deamino-arginine-vasopressin and Diabetes-Insipidus--Neurogenic

The authors report permanent central diabetes insipidus (CDI) in a patient after severe traumatic brain injury (TBI) in traffic accident. A 16-year-old boy entered to a medical facility in coma (GCS score 6) with the following diagnosis: acute TBI, severe cerebral contusion, subarachnoid hemorrhage, depressed comminuted cranial vault fracture, basilar skull fracture, visceral contusion. CDI was diagnosed in 3 days after injury considering polyuria and hypernatremia (155 mmol/l). Desmopressin therapy was initiated through a feeding tube. Thirst appeared when a patient came out of the coma after 21 days despite ongoing desmopressin therapy. Considering persistent thirst and polyuria, we continued desmopressin therapy in a spray form. Under this therapy, polyuria reduced to 3-3.5 liters per a day. Symptoms of CDI persisted in long-term period (2 years after TBI) while function of adenohypophysis was intact. This case demonstrates a rare development of permanent diabetes insipidus after TBI. CDI manifested only as polyuria and hypernatremia in coma. Thirst joined after recovery of consciousness. Probable causes of CDI were damage to neurohypophysis and partially injury of pituitary stalk because of extended basilar skull fracture and/or irreversible secondary lesion of hypothalamus following diffuse axonal damage after TBI.. В статье представлен клинический случай развития постоянной формы центрального несахарного диабета (ЦНД) у пациента после тяжелой черепно-мозговой травмы (ТЧМТ) в результате дорожно-транспортного происшествия. Подросток 16 лет поступил в лечебное учреждение в состоянии комы (6 баллов по шкале комы Глазго) с диагнозом: сочетанная травма; острая ТЧМТ; ушиб головного мозга тяжелой степени; субарахноидальное кровоизлияние; вдавленный многооскольчатый перелом свода черепа справа; протяженный перелом основания черепа; ушиб внутренних органов. На 3-и сутки развились полиурия и гипернатриемия (155 ммоль/л); диагностирован ЦНД и начата терапия десмопрессином в таблетированной форме через зонд. При выходе из комы (21-е сутки) отмечено появление жажды на фоне продолжения терапии. В связи с сохраняющейся жаждой и полиурией произведен перевод на терапию десмопрессином в виде спрея, на этом фоне отмечено уменьшение выделения мочи до 3—3,5 л в сутки. Симптоматика ЦНД наблюдалась и через 2 года после ТЧМТ, при этом функция аденогипофиза оставалась сохранной. Представленный случай является примером развития постоянного несахарного диабета у подростка с ТЧМТ, находившегося под длительным наблюдением. Клиническая картина ЦНД в состоянии комы проявлялась только полиурией и гипернатриемией, а по мере повышения уровня сознания присоединилась жажда. Вероятными причинами развития ЦНД явились повреждение нейрогипофиза и частичное повреждение стебля гипофиза в результате протяженного перелома основания черепа и/или необратимого вторичного повреждения гипоталамуса вследствие диффузного аксонального повреждения головного мозга после ТЧМТ.

Topics: Adolescent; Brain Injuries, Traumatic; Coma; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Humans; Hypernatremia; Male; Polyuria

Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis of small vessels that affect the pituitary gland in less than 1% of cases being exceptionally rare. To describe the clinical, biochemical, radiological findings, treatment, and outcomes of 4 patients with GPA-related hypophysitis. A systematic review of published cases with the same diagnosis is presented as well. A cross-sectional case series of patients with hypophysitis due to GPA from 1981 to 2018 at a third level specialty center. Literature review was performed searching in seven different digital databases for terms "granulomatosis with polyangiitis" and "pituitary gland" or "hypophysitis," including in the analysis all published cases between 1950 and 2019 with a minimum follow-up of 6 months. We found 197 patients with GPA in our institution of whom 4 patients (2.0%) had pituitary involvement. Clinical characteristics and outcomes are described. We also reviewed 7 case series, and 36 case reports describing pituitary dysfunction related to GPA from 1953 to 2019, including the clinical picture of an additional 74 patients. Pituitary dysfunction due to GPA is rare. Treatment is targeted to control systemic manifestations; nevertheless, the outcome of the pituitary function is poor. Central diabetes insipidus, particularly in younger women with other systemic features, should raise suspicion of GPA.Key Points• Involvement of the pituitary gland is an uncommon manifestation in GPA patients. The presence of central diabetes insipidus in the setting of systemic symptoms should prompt its suspicion.• In patients with pituitary involvement due to GPA, affection of other endocrine glands is rare, neither concomitant nor in different times during the disease course. This may arise the hypothesis of a local or regional pathogenesis affection of the gland.• There is no consensus on the best therapy strategy for GPA hypophysitis. Although the use of glucocorticoids with CYC is the most common drug combination, no differences in the outcome of the pituitary function and GPA disease course are seen with other immunosuppressants.• Poor prognosis regarding pituitary function is expected due to possible permanent pituitary tissue damage that results in the need of permanent hormonal replacement.

Topics: Antidiuretic Agents; Autoimmune Hypophysitis; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Glucocorticoids; Granulomatosis with Polyangiitis; Humans; Hyperprolactinemia; Hypopituitarism; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Middle Aged

The treatment of central diabetes insipidus has not changed significantly in recent decades, and dDAVP and replacement of free water deficit remain the cornerstones of treatment. Oral dDAVP has replaced nasal dDAVP as a more reliable mode of treatment for chronic central diabetes insipidus. Hyponatraemia is a common side effect, occurring in one in four patients, and should be avoided by allowing a regular break from dDAVP to allow a resultant aquaresis. Hypernatraemia is less common, and typically occurs during hospitalization, when access to water is restricted, and in cases of adipsic DI. Management of adipsic DI can be challenging, and requires initial inpatient assessment to establish dose of dDAVP, daily fluid prescription, and eunatraemic weight which can guide day-to-day fluid targets in the long-term.

Topics: Body Weight; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Humans; Hypernatremia; Hyponatremia; Neurophysins; Protein Precursors; Vasopressins

Some diseases contribute to hypopituitarism without clinical manifestations and the glucocorticoid therapy may unveil central diabetes insipidus. The condition is rare and usually causes problems for clinical physicians.. A 59-year-old woman presented to our hospital due to facial numbness and persistent eyelid heaviness.. Physical examination and cerebrospinal fluid examination supported a diagnosis of Guillain-Barre[Combining Acute Accent] syndrome. Magnetic resonance imaging showed an empty sella. Hormone test indicated hypopituitarism.. The patient received intravenous immunoglobulin and glucocorticoid. Central diabetes insipidus appeared after 20 days. Subsequently, the patient was prescribed 1-desamino-8-D-arginine vasopressin and prednisone.. During 6 months' follow-up, the patient's urine output was gradually reduced to normal level.. This case indicated that hypopituitarism may be caused by an empty sella and be masked by adrenal insufficiency. Central diabetes insipidus may present after glucocorticoid therapy.

Topics: Adolescent; Adrenal Insufficiency; Adult; Aged; Aged, 80 and over; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Drug Therapy, Combination; Empty Sella Syndrome; Female; Glucocorticoids; Guillain-Barre Syndrome; Humans; Hypopituitarism; Immunoglobulins, Intravenous; Magnetic Resonance Imaging; Male; Middle Aged; Prednisone; Treatment Outcome

Diabetes insipidus (DI) is a rare complication of pregnancy. It is usually transient, being due to increased placental production of vasopressinase that inactivates circulating vasopressin. Gestational, transient DI occurs late in pregnancy and disappears few days after delivery. Acquired central DI can also occur during pregnancy, for example in a patient with hypophysitis or neuroinfundibulitis during late pregnancy or postpartum. Finally, pre-existing central or nephrogenic DI may occasionally be unmasked by pregnancy. Treatment with dDAVP (desmopressin, Minirin(®)) is very effective on transient DI of pregnancy and also on pre-existing or acquired central DI. Contrary to vasopressin, dDAVP is not degraded by vasopressinase. Nephrogenic DI is insensitive to dDAVP and is therefore more difficult to treat during pregnancy if fluid intake needs to be restricted.

Topics: Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Female; Humans; Pregnancy; Pregnancy Complications; Vasopressins

Central diabetes insipidus (CDI) is a complex and heterogeneous clinical syndrome affecting the hypothalamic-neurohypophyseal network and water balance. A recent national surveillance in Denmark showed a prevalence rate of twenty-three CDI patients per 100,000 inhabitants in five years. The differential diagnosis between several presenting conditions with polyuria and polydipsia is puzzling, and the etiological diagnosis of CDI remains a challenge before the identification of an underlying cause. For clinical practice, a timely diagnosis for initiating specific treatment in order to avoid central nervous system damage, additional pituitary defects and the risk of dissemination of germ cell tumor is advisable. Proper etiological diagnosis can be achieved via a series of steps that start with careful clinical observation of several signs and endocrine symptoms and then progress to more sophisticated imaging tools. This review summarizes the best practice and approach for the diagnosis and treatment of patients with CDI.

Topics: Antidiuretic Agents; Brain Injuries; Brain Neoplasms; Child; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Disease Management; Histiocytosis, Langerhans-Cell; Humans; Magnetic Resonance Imaging; Neoplasms, Germ Cell and Embryonal

Enterovirus is a known cause of central nervous system infection in the neonatal population and typically has a benign course; however, neurologic complications have been reported. We describe what we believe to be the first documented case of enteroviral meningoencephalitis complicated by central diabetes insipidus in a neonate.

Topics: Ampicillin; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Enterovirus B, Human; Enterovirus Infections; Gentamicins; Humans; Infant; Infant, Newborn; Male; Meningoencephalitis

Diabetes insipidus (DI) is a common complication following pituitary surgery and can be transient or permanent. Neurogenic DI occurs following injury to the magnocellular neurons in the hypothalamus that produce and transport arginine vasopressin (AVP) and form the hypothalamo-hypophyseal tract. DI is defined by a constellation of signs and symptoms resulting in dilute high-volume urine output and increasing serum osmolality. The body's inability to concentrate urine leaves the patient dehydrated and leads to metabolic abnormalities that can be life threatening if not recognized and treated in a timely manner with an exogenous AVP analog. The reported incidence of postsurgical central DI varies from 1 to 67%. This wide range likely reflects inconsistencies in the working definition of DI across the literature. Factors affecting the rate of DI include pituitary tumor size, adherence to surrounding structures, surgical approach, and histopathology of pituitary lesion. The likelihood of postoperative DI can be reduced by careful preservation of the neurovascular structures of the hypothalamus, infundibulum, and neurohypophysis. Vigilance and meticulous surgical technique are essential to minimize injury to these critical regions that can lead to postsurgical DI.

Topics: Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Endoscopy; Humans; Microsurgery; Neurosurgical Procedures; Pituitary Gland; Pituitary Neoplasms; Postoperative Complications; Vasopressins

Central diabetes insipidus (CDI) is the end result of a number of conditions that affect the hypothalamic-neurohypophyseal system. The known causes include germinoma/craniopharyngioma, Langerhans cell histiocytosis (LCH), local inflammatory, autoimmune or vascular diseases, trauma resulting from surgery or an accident, sarcoidosis, metastases and midline cerebral and cranial malformations. In rare cases, the underlying cause can be genetic defects in vasopressin synthesis that are inherited as autosomal dominant, autosomal recessive or X-linked recessive traits. The diagnosis of the underlying condition is challenging and raises several concerns for patients and parents as it requires long-term follow-up. Proper etiological diagnosis can be achieved via a series of steps that start with clinical observations and then progress to more sophisticated tools. Specifically, MRI identification of pituitary hyperintensity in the posterior part of the sella, now considered a clear marker of neurohypophyseal functional integrity, together with the careful analysis of pituitary stalk shape and size, have provided the most striking findings contributing to the diagnosis and understanding of some forms of 'idiopathic' CDI. MRI STIR (short-inversion-time inversion recovery sequencing) is a promising technology for the early identification of LCH-dependent CDI.

Topics: Adult; Age Factors; Animals; Antidiuretic Agents; Child; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Nephrogenic; Diabetes Insipidus, Neurogenic; Drug Monitoring; Fluid Therapy; Humans; Infant

Over the past two decades, the genetic and molecular basis of familial forms of diabetes insipidus has been elucidated. Diabetes insipidus is a clinical syndrome characterized by the excretion of abnormally large volumes of diluted urine (polyuria) and increased fluid intake (polydipsia). The most common type of diabetes insipidus is caused by lack of the antidiuretic hormone arginine vasopressin (vasopressin), which is produced in the hypothalamus and secreted by the neurohypophysis. This type of diabetes insipidus is referred to here as neurohypophyseal diabetes insipidus. The syndrome can also result from resistance to the antidiuretic effects of vasopressin on the kidney, either at the level of the vasopressin 2 receptor or the aquaporin 2 water channel (which mediates the re-absorption of water from urine), and is referred to as renal or nephrogenic diabetes insipidus. Differentiation between these two types of diabetes insipidus and primary polydipsia can be difficult owing to the existence of partial as well as complete forms of vasopressin deficiency or resistance. Seven different familial forms of diabetes insipidus are known to exist. The clinical presentation, genetic basis and cellular mechanisms responsible for them vary considerably. This information has led to improved methods of differential diagnosis and could provide the basis of new forms of therapy.

Topics: Animals; Aquaporin 2; Arginine Vasopressin; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Nephrogenic; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Female; Humans; Male; Mutation; Phenotype; Polydipsia; Polyuria; Receptors, Vasopressin; Vasopressins

Central or neurogenic diabetes insipidus results from a deficiency in antidiuretic hormone (ADH) or arginine-vasopressin (AVP). Treatment is based on replacement therapy with the hormone analog desmopressin (d-DAVP). d-DAVP can be administered subcutaneously to infants or patients with postoperative or posttraumatic brain injury being monitored for transient diabetes insipidus. Intranasal and oral forms are also available. The recently introduced lyophilisate, which melts under the tongue, has replaced the tablet form (recently withdrawn from the market in France) and provides better bioavailability. Irrespective of the mode of administration, it is usually the patient who finds the effective minimal dose necessary for a normal life, i.e. without excessive polyuria, particularly at night. Patient education is necessary to avoid the risk of water intoxication and hyponatremia.

Topics: Antidiuretic Agents; Brain Injuries; Chemistry, Pharmaceutical; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Dose-Response Relationship, Drug; Drug Administration Routes; Humans; Infant

Wegener's granulomatosis (WG) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis of small and medium-sized vessels. Pituitary involvement in WG is rare with only 22 previous case reports in the English medical literature between 1966 and 2006. Herein we report another patient with WG-related diabetes insipidus (DI) and partial disruption of the anterior pituitary axes. We also review the clinical features, imaging findings, treatment and outcome of WG-related pituitary involvement. Isolated pituitary involvement in the absence of lung or renal complications in WG is rare and described in only one previous patient. Pituitary involvement in WG is usually associated with other organ involvement (96% of cases)-commonly upper respiratory tract (93%), lungs (73%) and kidneys (67%). Abnormalities are often seen in the hypothalamo-pituitary region on magnetic resonance imaging (MRI) or computed tomography (CT) of the head (90% of cases). In 65% of reported cases, cyclophosphamide-based induction therapy was used with a subsequent relapse rate of 27%, occurring at a median of 10.5 months (range: 7-36 months) after initiation of treatment. In comparison, induction treatment without cyclophosphamide was associated with relapse in 50% at a median of 4.5 months (range: 4-18 months after starting treatment) suggesting more frequent and earlier relapse. Therefore, we recommend treatment with cyclophosphamide-based regimen. Despite treatment of WG, only 17% (4 patients) had full recovery in their pituitary function. The long-term prognosis of patients with WG and pituitary involvement is not known.

Topics: Adult; Antidiuretic Agents; Cyclophosphamide; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Drug Therapy, Combination; Glucocorticoids; Granulomatosis with Polyangiitis; Humans; Hypopituitarism; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Methylprednisolone; Pituitary Function Tests; Pituitary Gland; Recurrence; Treatment Outcome

Infants consume most of their calories as formula. Because of this large fluid intake, infants normally produce dilute urine, not far off from that seen in individuals with diabetes insipidus (DI). Infants with DI are therefore prone to water intoxication if fixed antidiuresis is achieved using the long-acting vasopressin analog desmopressin (DDAVP), which induces a state of high urine concentration. DI treatment approaches applied to older children and adults, who consume the their calories as solids, are difficult to apply to infants with DI. When used in infants, oral and intranasal DDAVP can be associated with wide swings in serum sodium concentration (SNA). In comparison, precisely administered subcutaneous doses of DDAVP can be successfully used in infants with DI, and appear to be superior to oral or intranasal DDAVP therapy. Alternatively, consistent eunatremia can be simply achieved in infantile DI using low renal solute load (RSL) formula and thiazide diuretics. Low RSL formula reduces obligatory urinary water losses, and thiazide diuretics concentrate the urine to levels seen in normal formula-fed infants. This report addresses treatment options of DI in infancy and the delicate management issues involved.

Topics: Adult; Antidiuretic Agents; Arginine Vasopressin; Child; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Infant; Infant Formula; Models, Biological; Osmolar Concentration; Sodium Chloride Symporter Inhibitors; Water-Electrolyte Balance

Topics: Administration, Intranasal; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Humans; Prognosis; Vasopressins

Topics: Antidiuretic Agents; Biomarkers; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Humans; Treatment Outcome

The management of central diabetes insipidus has been greatly simplified by the introduction of desmopressin (DDAVP). Its ease of administration, safety and tolerability make DDAVP the first line agent for outpatient treatment of central diabetes insipidus. The major complication of DDAVP therapy is water intoxication and hyponatremia. The risk of hyponatremia can be reduced by careful dose titration when initiating therapy and by close monitoring of serum osmolality when DDAVP is used with other medications affecting water balance. Herein we review the adverse effects of DDAVP and its predecessor, vasopressin, as well as discuss important clinical considerations when using these agents to treat central diabetes insipidus.

Topics: Antidiuretic Agents; Child; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Vasopressins

Topics: Arginine Vasopressin; Chlorothiazide; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Infant; Meningitis, Pneumococcal; Osmolar Concentration; Renal Agents; Water-Electrolyte Balance

Central diabetes insipidus (CDI) is associated with arginine vasopressin (AVP) deficiency with resultant polyuria and polydipsia. Intranasal desmopressin provides physiological replacement but oral formulations are preferred for their ease of administration. This study aimed to demonstrate the efficacy and safety of desmopressin orally disintegrating tablet (ODT) in the treatment of Japanese patients with CDI, and confirm that antidiuresis is maintained on switching from intranasal desmopressin to desmopressin ODT. A total of 20 patients aged 6-75 years with CDI were included in this 4-week multicenter, open-label study. Following observation, patients switched from intranasal desmopressin to desmopressin ODT with titration to optimal dose over ≤5 days at the study site. Following three consecutive doses with stable patient fluid balance, patients were discharged with visits at Weeks 2 and 4. Following titration from intranasal desmopressin to ODT, the mean 24-hour urine volume was unchanged, indicating similar antidiuresis with both formulations. The proportion of patients with endpoint measurements (urine osmolality, 24-hour urine volume, hourly diuresis rate and urine-specific gravity) within normal range at Days 1-2 (intranasal desmopressin) and Week 4 (desmopressin ODT) was similar. The mean daily dose ratio of intranasal desmopressin to desmopressin ODT (Week 4) was 1:24 but a wide range was observed across individuals to maintain adequate antidiuretic effect. Hyponatraemia was generally mild and managed by dose titration. Desmopressin ODT achieved sufficient antidiuretic control compared to intranasal therapy and was well tolerated over long-term treatment. The wide range of intranasal:ODT dose ratios underline the importance of individual titration.

Topics: Administration, Oral; Adolescent; Adult; Aged; Antidiuretic Agents; Chemical Phenomena; Child; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diuresis; Dose-Response Relationship, Drug; Drug Monitoring; Female; Follow-Up Studies; Humans; Hyponatremia; Japan; Male; Mechanical Phenomena; Middle Aged; Tablets; Young Adult

The key question answered by this study is whether it is possible to deliver a pharmacokinetic and pharmacodynamic duration of antidiuretic action long enough to ensure adequate antidiuresis with two daily administrations of desmopressin in patients with central diabetes insipidus (CDI). We studied the efficacy and safety of desmopressin i.v. in 13 CDI patients using two 3-way crossover designs, in the doses 30, 60, 125 ng, and 125, 250 and 500 ng. Duration of action, minimum output rate, max osmolality and average osmolality during action (AUC osmolality) were measured every 30 min for the first 2 h during the infusion, and then every hour or every second hour until the urine output rate was greater than 2 ml/kg/30 min. The duration of antidiuretic action was 4, 8 and 11 h, respectively, for 125, 250, and 500 ng, increasing from 250 to 500 ng but for the remaining secondary dynamic efficacy parameters no difference could be detected based on descriptive statistics between the doses 250 and 500 ng, indicating that the upper plateau region of the dose-response curve had been reached. All treatment emergent adverse events were classified as unrelated or unlikely related to trial medication. No serious adverse events occurred. Data on duration of action indicates that it is possible to achieve antidiuretic control with 500 ng i.v. corresponding to 160 μg orodispersible tablets twice daily in CDI patients. Today, the Minirin Melt label recommends the majority of CDI patients a dose of 60 to 120 μg t.i.d.

Topics: Administration, Oral; Adult; Antidiuretic Agents; Cross-Over Studies; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Dose-Response Relationship, Drug; Female; Humans; Infusions, Intravenous; Kaplan-Meier Estimate; Male; Middle Aged; Osmolar Concentration; Sodium; Thirst; Time Factors; Treatment Outcome; Urine

IGF binding protein-1 (IGFBP-1) is essential for IGF-I bioavailability. High levels of IGFBP-1 are encountered in critically ill patients and are a good predictor marker in acute myocardial infarction. The mechanisms responsible for the elevated IGFBP-1 levels in these conditions are still unclear. Interestingly, high levels of vasopressin have been reported in the above-mentioned conditions.. To study the effect of vasopressin on IGFBP-1 in humans.. Placebo-controlled cross-over study in patients with central diabetes insipidus (CDI) in whom potential interference from endogenous vasopressin secretion is minimized. After a 3-day desmopressin washout period, each patient received i.v. saline on day 1 and desmopressin (3 mug) on day 2. Blood samples were taken after administration, every 2 h during the whole night, starting at 2000 h.. Fourteen inpatients with CDI in an endocrinology department of a university hospital.. Serum IGFBP-1 increased within 4 h after 1-desamino-8-d-arginine vasopressin (DDAVP) by 375+/-73%, compared with a spontaneous fasting increase by 252+/-46% following placebo administration (P<0.05). No changes were registered in the levels of either classically regulators of IGFBP-1 (insulin, glucagon, and cortisol) or of IGF-I and glucose. The decrease in plasma osmolarity induced by DDAVP did not precede the increase in IGFBP-1.. DDAVP increases serum levels of IGFBP-1. Further investigation is essential to unravel the clinical potential of this interaction in conditions associated with high IGFBP-1 levels.

Topics: Antidiuretic Agents; Cross-Over Studies; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Glucagon; Humans; Hydrocortisone; Insulin; Insulin-Like Growth Factor Binding Protein 1; Placebos

Melatonin secretion is modulated by the light-dark schedule, mainly through a sympathetic input to the pineal gland. Besides this, arginine vasopressin (AVP) has been found in the pineal glands of several animal species and there is experimental evidence that AVP modulates melatonin secretion in animals. However, the interaction between vasopressin and melatonin secretion in humans has not been systematically investigated. We proposed to study the nocturnal melatonin pattern in patients with central diabetes insipidus (CDI) who lack endogenous secretion of AVP, and the effect on their melatonin secretion of the agonist for V2 type receptors: desmopressin (1-Desamino [8-D Arginine] vasopressin). Plasma melatonin levels were measured in 14 patients with CDI, every 2 h starting from 22:00 h until 06:00 h, following iv injection of saline (day 1) and 3 microg desmopressin (day 2) at 20:00 h. The lights were turned off at 22:30 h and the samples were taken in a dim light. The plasma melatonin secretion pattern was normal in patients with CDI. Desmopressin at a dose 3 times higher than the antidiuretic one did not modify the melatonin levels or the time of the peak secretion. In conclusion melatonin secretion is not modulated by AVP in humans.

Topics: Circadian Rhythm; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Drug Administration Schedule; Female; Hormone Replacement Therapy; Humans; Male; Melatonin; Middle Aged; Vasopressins

We studied the efficacy and safety of oral 1-deamino-8-D-arginine-vasopressin (DDAVP) tablets in 9 patients, aged 17-36 years, with central diabetes insipidus (DI). The tablet contained 100 microg of desmopressin acetate. Maximum plasma concentration was obtained at 90 min after a single oral administration of 100 microg DDAVP with a mean plasma level of 14.7 +/- 5.4 (range: 5.3-50.9) pg/ml. The onset of action was observed 2 h after oral administration, while the maximum effect was obtained at 4 h. Mean urine volume in patients decreased significantly from 402 +/- 52 to 26 +/- 3 ml/hr and urine osmolality increased from 91 +/- 8 to 732 +/- 21 mosm/kg at 4 h after the intake of oral DDAVP. Plasma osmolality level and serum sodium concentration remained unchanged throughout the study. Long-term treatment for 5 years with oral DDAVP resulted in control of diuresis in 8 of the 9 patients. The average oral DDAVP dose required to obtain this control was 19 +/- 2 (range: 15-30) times more than that of prior intranasal treatment. No adverse effects were observed during this follow-up period. These results indicate that oral DDAVP is a safe therapeutic agent that may be a good alternative treatment of central DI, particularly in patients who have chronic rhinitis and visual disturbances.

Topics: Administration, Oral; Adolescent; Adult; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diuresis; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Humans; Male; Osmolar Concentration; Treatment Outcome

In this study, we analyzed plasma lipid and lipoprotein levels before and after treatment with 1-desamino-8-D-arginine vasopressin (DDAVP) in subjects with partial and complete central diabetes insipidus (DI) in order to determine how a shortage and supplement of this hormone affect plasma lipid metabolism. The subjects consisted of 6 patients with partial and 6 with complete central DI. After treatment with DDAVP through nasal cavity, plasma total cholesterol (TC) level did not decrease either in complete or partial form. Plasma triglyceride (TG) levels decreased from 306+/-175 mg/dl to 198+/-91 (35% decrease, p=0.027) in complete form, while TG did not change significantly in partial form. A detailed investigation of plasma lipoprotein metabolism during treatment with DDAVP was carried out in 3 of the 6 subjects with complete form of DI. Lipoprotein lipase activity and mass in post-heparin plasma from those three subjects tended to increase after treatment with DDAVP, along with the complete disappearance of an unusual lipoprotein between low density lipoprotein (LDL) and very low density lipoprotein (VLDL) as analyzed by polyacrylamide gel electrophoresis. These results suggest that the DDAVP treatment has a favorable effect on lipid and lipoprotein metabolism, especially triglyceride-rich lipoproteins, either directly or through modifying factors contributing to lipid metabolism.

Topics: Administration, Intranasal; Cholesterol; Cholesterol, HDL; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Hyperlipidemias; Lipase; Lipoprotein Lipase; Liver; Male; Middle Aged; Triglycerides

A 36-year-old female was pointed out to have liver enzyme elevation by routine health checkup. Subsequent contrast-enhanced CT scan identified gigantic uterine fibroids and retroperitoneal tumor. She was referred to the gynecologist at JA Toride Medical Center and planned to undergo a uterus enucleation and biopsy of the retroperitoneal tumor. The surgery was conducted without any troubles. After the surgery, the patient presented polyuria with urine volume 10-20 L a day and developed hypovolemic shock. Laboratory test revealed hypotonic urine and hypernatremia. Arginine vasopressin (AVP) loading test suggested shortage of endogenous vasopressin. Since the subcutaneous administration of AVP was not sufficient to control the urine volume, continuous intravenous infusion of AVP was initiated. After achieving hemodynamic stability, the treatment was switched to oral desmopressin. MRI finding indicated attenuation of high signal in posterior pituitary in T1 weighted image while neither enlargement of pituitary nor thickening of pituitary stalk was indicated by enhanced MRI. Hypertonic salt solution test indicated no responsive elevation of AVP, confirming the diagnosis of central diabetes insipidus (CDI). Her anterior pituitary function was preserved. Only anti-rabphilin-3A antibody was found positive in the serum of the patient, while other secondary causes for CDI were denied serologically and radiologically. Hence, lymphocytic infundibuloneurohypophysitis　(LINH) was suspected as the final diagnosis. Hormonal replacement therapy by nasal desmopressin was continued and the patient managed to control her urine volume. In cases of CDI considered idiopathic with conventional examinations, anti-rabphilin-3A antibody may be a clue for determining the cause as LINH.

Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Female; Humans; Retroperitoneal Neoplasms

Topics: Arginine; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Humans; Oxytocin

Experience with nasogastric administration of oral DDAVP [desamino-D-arginine-8-vasopressin] lyophilisate (ODL) for central diabetes insipidus (CDI) in disabled children with swallowing coordination difficulties is limited.. We aimed to assess the safety and efficacy of nasogastric use of ODL in disabled children with CDI. Time to serum sodium normalisation was compared with that of children with normal intellect and CDI treated with sublingual DDAVP.. Clinical, laboratory and neuroimaging characteristics were evaluated for 12 disabled children with CDI treated with ODL through nasogastric tube at Dr Behcet Uz Children's Hospital, Turkey, between 2012 and 2022.. Six boys and six girls with a mean (±SD) age of 43 (± 40) months were evaluated. These children (mean [±SD] weight standard deviation score [SDS] - 1.2 ± 1.7; mean [±SD] height SDS - 1.3 ± 1.4) presented with failure to thrive, irritability, prolonged fever, polyuria and hypernatraemia (mean serum sodium 162 [±3.6] mEq/L). At diagnosis, mean serum and urine osmolality were 321 (± 14) mOsm/kg and 105 (± 7.8) mOsm/kg, respectively. Arginine vasopressin (AVP) levels were undetectable (< 0.5 pmol/L) at diagnosis in all patients. Nasogastric tube administration of DDAVP lyophilisate (120 µg/tablet) dissolved in water (10 mL) was commenced at a dose of 1-5 µg/kg/day in two divided doses together with controlled water intake to avoid hyponatraemia. The frequency and dose of DDAVP were titrated based on urine output and serum sodium concentration. Serum sodium declined at a rate of 0.11 ± 0.03 mEq/L/h and reached normal range in a mean duration of 174 ± 46.5 h. Serum sodium declined faster in children with normal intellect and CDI treated with sublingual DDAVP (1.28 ± 0.39 mEq/L/h; p = 0.0003). Three disabled children needed rehospitalisation because of hypernatraemia due to unintentional DDAVP omission by caregivers. No episode of hyponatraemia was observed. Weight gain and growth were normal during the median (± interquartile range) follow-up duration of 32 ± 67 months.. Nasogastric administration of oral DDAVP lyophilised formulation was safe and effective in the treatment of CDI in disabled children in this small retrospective series.

Topics: Child; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Disabled Children; Female; Humans; Hypernatremia; Hyponatremia; Male; Retrospective Studies; Sodium

Novel coronavirus disease 2019 (COVID-19) mainly affects the lungs, but can involve several other organs. The diagnosis of acute and chronic sequelae is one of the challenges of COVID-19. The current literature proposes that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may involve the hypothalamic-pituitary axis. In this case report, we present a unique case of new-onset central diabetes insipidus secondary to the COVID-19 disease in a 54-year-old woman.. A 54-year-old woman presented with the history of excessive thirst, polyuria, and polydipsia, six weeks after being infected by COVID-19. Laboratory tests revealed low urine osmolarity and increased serum osmolarity, and the patient was diagnosed with central diabetes insipidus. After administration of nasal desmopressin, urinary osmolarity increased, and the patient's symptoms improved. However, to stabilize her condition, desmopressin treatment was required.. We reported a unique case of diabetes insipidus in a COVID-19 patient. Central diabetes insipidus may be included in clinical manifestations of the COVID-19, in case of new-onset polyuria and polydipsia following COVID-19 disease. Nevertheless, a causal relationship has not been established between the symptoms of the patient and the SARS-CoV-2 infection.

Topics: COVID-19; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Female; Humans; Middle Aged; Polydipsia; Polyuria; SARS-CoV-2

There is growing recognition of morbidity and mortality that can occur in patients with cranial diabetes insipidus (CDI) during hospitalisation, due to prescribing errors and dysnatraemia, often related to confusion between CDI and diabetes mellitus among non-specialists. We aimed to investigate this.. Data for each hospitalisation in patients with CDI attending Oxford University Hospital (OUH) were collected retrospectively. The same cohort were invited to complete a questionnaire by telephone.. One hundred and nine patients were included, median age was 42 (range: 6-80) years. Route of desmopressin was tablet, melt and nasal spray in 74%, 7% and 17% of patients, respectively, while two patients used a combination of tablet and nasal spray. There were 85 admissions to OUH by 38 patients between 2012 and 2021. Daily measurement of serum sodium was performed in 39% of admissions; hyponatraemia and hypernatraemia occurred in 44 and 15% of admissions, respectively. Endocrine consultation was sought in 63% of admissions post-2018. Forty-five of 78 patients (58%) self-reported ≥1 admission to any hospital since diagnosis. Of these, 53% felt their medical team did not have a good understanding of the management of CDI during hospital admission. Twenty-four per cent reported delay in administration of desmopressin, while 44% reported confusion between CDI and diabetes mellitus, often leading to unnecessary blood glucose monitoring.. Dysnatraemia is common in hospitalised patients with CDI. More than half of patients perceived their medical team's understanding of CDI to be poor when admitted with intercurrent illness. A coordinated approach, including early consultation of specialists, frequent serum sodium monitoring, and education of hospital specialists is needed to address this.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Blood Glucose; Blood Glucose Self-Monitoring; Child; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Humans; Middle Aged; Nasal Sprays; Perception; Retrospective Studies; Sodium; Tablets; Young Adult

A 66-year-old female patient with the initial diagnosis of acute myeloid leukemia is reported. Paraneoplastic syndrome manifested as hypernatremia due to central diabetes insipidus (CDI), which could be controlled with the administration of desmopressin. After initiation of the induction therapy, the required desmopressin administration could be reduced and terminated. In the further course, the early increasing polyuria and hypernatremia indicated the primary refractory acute myeloid leukemia.. Dargestellt wird der Krankheitsverlauf einer 66-jährigen Patientin mit der Erstdiagnose einer akuten myeloischen Leukämie. Als paraneoplastisches Syndrom zeigten sich eine Hypernatriämie sowie Polyurie infolge eines zentralen Diabetes insipidus (CDI), welcher mittels Desmopressingabe kontrolliert werden konnte. Nach Einleitung einer Induktionstherapie kam es im Verlauf nach Beendigung der Desmopressintherapie noch vor Anstieg des Blastenanteils zu einer erneuten Hypernatriämie und Polyurie als Hinweis auf eine primäre Refraktärität.

Topics: Aged; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Hypernatremia; Leukemia, Myeloid, Acute; Neoplasms, Second Primary; Polyuria

Central diabetes insipidus is a rare neuroendocrine condition. Data on treatment-associated side-effects, psychological comorbidities, and incorrect management are scarce. The aim of this study was to investigate patients' perspectives on their disease.. This study used a cross-sectional, web-based, anonymous survey, developed by endocrinologists and patient representatives, to collect the opinions of patients with central diabetes insipidus on management and complications of their disease, psychological comorbidities, degree of knowledge and awareness of the condition among health-care professionals, and renaming the disease to avoid confusion with diabetes mellitus (diabetes).. Between Aug 23, 2021, and Feb 7, 2022, 1034 patients with central diabetes insipidus participated in the survey. 91 (9%) participants were children and adolescents (37 [41%] girls and 54 [59%] boys; median age 10 years [IQR 6-15]) and 943 (91%) were adults (757 [80%] women and 186 [20%] men]; median age 44 years [34-54]). 488 (47%) participants had isolated posterior pituitary dysfunction and 546 (53%) had combined anterior and posterior pituitary dysfunction. Main aetiologies were idiopathic (315 [30%] of 1034 participants) and tumours and cysts (pre-surgical 217 [21%]; post-surgical 254 [25%]). 260 (26%; 95% CI [0·23-0·29]) of 994 patients on desmopressin therapy had hyponatraemia leading to hospitalisation. Patients who routinely omitted or delayed desmopressin to allow intermittent aquaresis had a significantly lower prevalence of hyponatraemia compared with those not aware of this approach (odds ratio 0·55 [95% CI 0·39-0·77]; p=0·0006). Of patients who had to be hospitalised for any medical reason, 71 (13%; 95% CI 0·10-0·16) of 535 patients did not receive desmopressin while in a fasting state (nil by mouth) without intravenous fluid replacement and reported symptoms of dehydration. 660 (64%; 0·61-0·67) participants reported lower quality of life, and 369 (36%; 0·33-0·39) had psychological changes subjectively associated with their central diabetes insipidus. 823 (80%; 0·77-0·82) participants encountered a situation where central diabetes insipidus was confused with diabetes mellitus (diabetes) by health-care professionals. 884 (85%; 0·83-0·88) participants supported renaming the disease; the most favoured alternative names were vasopressin deficiency and arginine vasopressin deficiency.. This is the largest survey of patients with central diabetes insipidus, reporting a high prevalence of treatment-associated side-effects, mismanagement during hospitalisation, psychological comorbidities, and a clear support for renaming the disease. Our data are the first to indicate the value of routinely omitting or delaying desmopressin.. Swiss National Science Foundation, Swiss Academy of Medical Sciences, and G&J Bangerter-Rhyner-Foundation.

Topics: Adolescent; Adult; Arginine; Child; Cross-Sectional Studies; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Female; Humans; Hyponatremia; Internet; Male; Middle Aged; Morbidity; Quality of Life

We present a 9-year-old boy with diabetes insipidus. The boy is treated with desmopressin (DDAVP) therapy. Under this therapy, the drinking quantity and the laboratory parameters were normal. No nocturia occurred any more. In the context of a clinically mild infection with SARS-CoV-2, the duration of action of DDAVP was significantly prolonged (approximately +50%). The original dosage was then reintroduced and was still sufficient until months later. A possible connection to the infection with SARS-CoV-2 can be suspected. Our case report should make physicians who care for patients with diabetes insipidus aware of such a possible prolongation of the effect of DDAVP. More frequent monitoring may be needed in such patients to assess the risk of symptomatic dilutional hyponatremia.

Topics: Child; COVID-19; COVID-19 Drug Treatment; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Half-Life; Humans; Male; SARS-CoV-2

Central diabetes insipidus (DI) is a complex disease that requires firm adherence to desmopressin therapy. There is little information on the onset of hypernatremia after withdrawal of desmopressin.. We present a case of an elderly woman with central DI whose serum sodium jumped from 141 to 171 mEq/L after 48-72 h of holding oral desmopressin. Her DI crisis resolved with intravenous desmopressin and free water administration.. Based on this precipitous onset of DI crisis, we recommend not withholding desmopressin for more than 24 h.

Topics: Administration, Intravenous; Aged; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Disasters; Female; Humans; Hypernatremia

There is inconsistency in the amount of oral desmopressin that children with central diabetes insipidus require. We investigated whether clinical characteristics influenced desmopressin dose requirements in 100 children with central diabetes insipidus. Extremely large doses were associated with acquired etiology (P = .04), greater body mass index z score, intact thirst, and additional pituitary hormone deficiencies (P < .001).

Topics: Administration, Oral; Adolescent; Antidiuretic Agents; Child; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Humans; Infant; Male; Retrospective Studies; Treatment Outcome

Central diabetes insipidus (CDI) may occur in the setting of intracranial abnormalities that affect the hypothalamus-pituitary system. It occurs rarely in neonates, especially in the premature population, and represents a challenging disease process to treat pharmacologically. Little is known regarding the treatment options in premature infants, including dose and route of administration of intravenous desmopressin (DDAVP). We present a case of a late premature male infant with gastroschisis and septo-optic dysplasia who developed transient CDI. He was treated with intravenous DDAVP but required frequent laboratory monitoring and a multidisciplinary approach, and ultimately his CDI resolved. Although there are minimal guidelines regarding the appropriate formulation and dosage of DDAVP for management of CDI in infants, we initiated the lowest dose available and titrated the medication based on close monitoring of urine output and serum sodium levels in order to successfully treat his transient CDI.

Topics: Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Infant, Premature; Infant, Premature, Diseases; Male; Septo-Optic Dysplasia

Central diabetes insipidus is a heterogeneous condition characterized by decreased release of antidiuretic hormone by the neurohypophysis resulting in a urine concentration deficit with variable degrees of polyuria. The most common causes include idiopathic diabetes insipidus, tumors or infiltrative diseases, neurosurgery and trauma. Temozolomide is an oral DNA-alkylating agent capable of crossing the blood-brain barrier and used as chemotherapy primarily to treat glioblastoma and other brain cancers.. Two men (aged 38 and 54 years) suddenly developed polyuria and polydispsia approximately four weeks after the initiation of temozolomide for a glioblastoma. Plasma and urine parameters demonstrated the presence of a urinary concentration defect.. The clinical and laboratory abnormalities completely resolved with intranasal desmopressin therapy, allowing the continuation of temozolomide. The disorder did not relapse after cessation of temozolomide and desmopressin and relapsed in one patient after rechallenge with temozolomide.. Our report highlights the importance of a quick recognition of this exceptional complication, in order to initiate promptly treatment with desmopressin and to maintain therapy with temozolomide.

Topics: Adult; Antineoplastic Agents, Alkylating; Brain Neoplasms; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Fatal Outcome; Glioblastoma; Humans; Male; Middle Aged; Temozolomide; Vasopressins

We herein report a rare case of advanced lung adenocarcinoma with central diabetes insipidus due to pituitary metastasis. Although treatment with gefitinib was dramatically effective, the symptoms of diabetes insipidus did not improve. Radiotherapy for pituitary metastasis was effective to control diabetes insipidus; however, we could not cease the administration of 1-deamino-8-D-arginine vasopressin (DDAVP). It is important for physicians to positively consider radiotherapy for pituitary metastases even if favorable tumor control is achieved with chemotherapy when diabetes insipidus becomes clinically overt. Furthermore, continuous DDAVP administration may be needed to treat central diabetes insipidus.

Topics: Adenocarcinoma of Lung; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Gefitinib; Humans; Lung Neoplasms

Polyuria in post-kidney transplant (KT) patients is a common condition generally attributed to delayed tubular function, fluid administration, and solute diuresis. Since excessive water intake post-KT physiologically suppresses arginine vasopressin (AVP) secretion, central diabetes insipidus (CDI) caused by deficient primary AVP release can be overlooked. Although DDAVP (desmopressin) - a selective AVP V2 receptor agonist - has been used to treat massive polyuria, CDI rarely progresses to kidney injury due to the preservation of fluid balance by thirst-dependent osmoregulation. Administration of DDAVP in post-KT recipients with mild polyuria and subclinical CDI is difficult to assess, and whether long-term use of DDAVP is beneficial for the transplanted kidney has not been established. We present the case of a 36-year-old Japanese female who was diagnosed with subclinical/partial CDI post KT. CDI was caused by a sequela of suprasellar germinoma. Graft function gradually declined without evidence of hypovolemia or hypernatremia, and a kidney biopsy revealed advanced ischemic kidney injury. Although daily oral DDAVP administration did not increase extracellular fluid volume, treatment resulted in a gradual improvement of graft function, and a follow-up transplanted kidney biopsy indicated substantial recovery.

Topics: Administration, Oral; Adult; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Ischemia; Kidney; Kidney Transplantation

Topics: Aldosterone; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Glycopeptides; Humans; Hypovolemia; Middle Aged; Polyuria; Postural Orthostatic Tachycardia Syndrome; Quality of Life; Renin; Treatment Outcome

Topics: Antidiuretic Agents; COVID-19; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Female; Humans; Hypophysitis; Magnetic Resonance Imaging; Middle Aged; Pituitary Gland; Post-Acute COVID-19 Syndrome; SARS-CoV-2; Treatment Outcome

Modulating PD-1 expression can constrain tumor growth. Hodgkin's lymphoma patients commonly express PD-L1 on tumor cells. We report the case of a 60-year-old male patient with relapsed classical Hodgkin's lymphoma who suffered from immediate-onset chill, hyperthermia and polyuria following initial treatment with sintilimab, an anti-PD-1 monoclonal antibody. The results revealed central diabetes insipidus (cDI). After 3 months of treatment with glucocorticoids and desmopressin acetate, his symptoms and the results were consistent with the resolution of cDI and the treatment course was discontinued. Diabetes insipidus is a rare complication of immunotherapeutic treatment, and this is the first case report to our knowledge to have described immediate-onset cDI caused by anti-PD-1 treatment.. Lay abstract For relapsed classical Hodgkin's lymphoma, anti-PD-1 monoclonal antibodies have been related to potent safety profiles and efficacy. Reported here is a case of a 60-year-old man diagnosed as having relapsed classical Hodgkin's lymphoma. He achieved a durable response over 4 months with four rounds of traditional chemotherapy, and then the disease relapsed. Sintilimab, an anti-PD-1 monoclonal antibody, was executed for salvage therapy. In spite of the patient-acquired durable response, central diabetes insipidus was detected after the first application of sintilimab. According to our knowledge this is the first case to report immediate-onset central diabetes insipidus caused by the treatment of anti-PD-1. In the present study, we discussed and analyzed the types and clinical causes of diabetes insipidus for this patient.

Topics: Antibodies, Monoclonal, Humanized; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Glucocorticoids; Hodgkin Disease; Humans; Immunotherapy; Male; Middle Aged; Programmed Cell Death 1 Receptor

Topics: Abnormalities, Multiple; Administration, Intravenous; Administration, Oral; Cleft Lip; Cleft Palate; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Drug Administration Routes; Drug Compounding; Female; Gastrostomy; Holoprosencephaly; Humans; Infant; Infusions, Parenteral

This case report describes the clinical presentation, and the diagnostic and therapeutic approaches of a 4-year-old gelding presented with severe polyuria and polydipsia. The horse was diagnosed with central diabetes insipidus. After diagnosis, different therapeutic regimens with intraocular desmopressin acetate (Minirin, Ferring GmbH, Kiel, Germany) (a synthetic arginine vasopressin analog) were tested, but without success. Only the subcutaneous injection of desmopressin acetate (Minirin, Ferring GmbH) led to an increase in urine specific gravity and a decrease in water intake and urine output. Daily subcutaneous treatment with desmopressin acetate (Minirin, Ferring GmbH) was initiated and maintained for at least 5 years. The horse did not develop adverse effects or re-occurrence of the initial complaints. This case report describes successful long-term treatment of central diabetes insipidus in a horse.

Topics: Animals; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Horse Diseases; Horses; Injections, Subcutaneous; Male

Topics: Achlorhydria; Adult; Cyclophosphamide; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Hormones; Humans; Hypogonadism; Hyponatremia; Hypophysitis; Lupus Erythematosus, Systemic; Methylprednisolone; Treatment Outcome; Young Adult

COVID-19 has changed the nature of medical consultations, emphasizing virtual patient counseling, with relevance for patients with diabetes insipidus (DI) or hyponatraemia. The main complication of desmopressin treatment in DI is dilutional hyponatraemia. Since plasma sodium monitoring is not always possible in times of COVID-19, we recommend to delay the desmopressin dose once a week until aquaresis occurs allowing excess retained water to be excreted. Patients should measure their body weight daily. Patients with DI admitted to the hospital with COVID-19 have a high risk for mortality due to volume depletion. Specialists must supervise fluid replacement and dosing of desmopressin. Patients after pituitary surgery should drink to thirst and measure their body weight daily to early recognize the development of the postoperative syndrome of inappropriate antidiuresis (SIAD). They should know hyponatraemia symptoms. The prevalence of hyponatraemia in patients with pneumonia due to COVID-19 is not yet known, but seems to be low. In contrast, hypernatraemia may develop in COVID-19 patients in ICU, from different multifactorial reasons, for example, due to insensible water losses from pyrexia, increased respiration rate and use of diuretics. Hypernatraemic dehydration may contribute to the high risk of acute kidney injury in COVID-19. IV fluid replacement should be administered with caution in severe cases of COVID-19 because of the risk of pulmonary oedema.

Topics: Antidiuretic Agents; Brain Injuries; Coronavirus Infections; COVID-19; Deamino Arginine Vasopressin; Dehydration; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Disease Management; Fluid Therapy; Humans; Hypernatremia; Hyponatremia; Hypotonic Solutions; Inappropriate ADH Syndrome; Neurosurgical Procedures; Pandemics; Pneumonia, Viral; Postoperative Complications; Practice Guidelines as Topic; Saline Solution; Shock

Autoimmune hypophysitis is a rare disease characterized by the infiltration of lymphocytic cells into the pituitary gland.. Here, we report a 40-year-old female diagnosed with central diabetes insipidus and multiple pituitary hormone deficiencies, and MRI revealed homogeneous signals in the pituitary gland as well as thickened in the pituitary stalk. FDG PET localized the pituitary and pituitary stalk lesions and displayed an SUVmax of 5.5. FDS, a sensitive radiotracer for bacterial infections but remains unproven under aseptic inflammation, also demonstrated elevated radioactivity, with an SUVmax of 1.1 at 30 min and 0.73 at 120 min. Transnasal biopsy suggested a diagnosis of autoimmune hypophysitis, and the patient displayed radiological and clinical improvement after treatment with glucocorticoids and hormone replacement.. Autoimmune hypophysitis can display elevated FDG uptake, which aids in the localization of the lesions. In addition to revealing bacterial infection specifically, FDS can also accumulate under autoimmune conditions, suggesting that it could serve as a potential radiotracer for both bacterial and aseptic inflammation.. The patient was enrolled in study NCT02450942 (clinicaltrials.gov, Registered May 21, 2015).

Topics: Adult; Antidiuretic Agents; Autoimmune Hypophysitis; Biopsy; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Fluorodeoxyglucose F18; Glucocorticoids; Hormone Replacement Therapy; Humans; Hypopituitarism; Magnetic Resonance Imaging; Positron-Emission Tomography; Radiopharmaceuticals; Sorbitol; Thyroxine

We report the case of a pituitary stalk germinoma initially misdiagnosed and treated as infundibuloneurohypophysitis (INH). A 27-year-old man presented with a 1-year history of polydipsia, polyuria, nycturia consistent with central diabetes insipidus and a hyperintense pituitary stalk lesion on MRI. A possible INH diagnosis was considered, after excluding other pathologies. Lesion biopsy was discarded at that time on the ground of a small target and the high risk of added morbidity. Oral desmopressin led to initial symptoms resolution but, in the following months, an anterior panhypopituitarism developed, in spite of appropriate treatment and, by that time, the brain MRI also revealed lesion growth, which prompted a biopsy recommendation. The pathology analysis revealed a germinoma. After chemotherapy and radiotherapy, there was complete disappearance of the pituitary lesion, but the panhypopituitarism persisted. In conclusion, this case highlights the importance and difficulty of precise diagnosis in the initial assessment of pituitary stalk lesions and the need for close monitoring of treatment response. Diagnostic reassessment and biopsy in atypical cases is the only path to achieve the correct diagnosis and treatment.

Topics: Adult; Autoimmune Hypophysitis; Biopsy; Chemoradiotherapy; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Drug Monitoring; Germinoma; Hormone Replacement Therapy; Hormones; Humans; Hydrocortisone; Hypopituitarism; Magnetic Resonance Imaging; Male; Pituitary Gland; Pituitary Neoplasms; Thyroxine; Treatment Outcome

The clinical features, course and outcome of hantavirus infection is highly variable. Symptoms of the central nervous system may occur, but often present atypically and diagnostically challenging. Even though the incidence of hantavirus infection is increasing worldwide, this case is the first to describe diabetes insipidus centralis as a complication of hantavirus infection in the Western world.. A 49-year old male presenting with severe headache, nausea and photophobia to our neurology department was diagnosed with acute haemorrhage in the pituitary gland by magnetic resonance imaging. In the following days, the patient developed severe oliguric acute kidney failure. Diagnostic workup revealed a hantavirus infection, so that the pituitary haemorrhage resulting in hypopituitarism was seen as a consequence of hantavirus-induced hypophysitis. Under hormone replacement and symptomatic therapy, the patient's condition and kidney function improved considerably, but significant polyuria persisted, which was initially attributed to recovery from kidney injury. However, water deprivation test revealed central diabetes insipidus, indicating involvement of the posterior pituitary gland. The amount of urine production normalized with desmopressin substitution.. Our case report highlights that neurological complications of hantavirus infection should be considered in patients with atypical clinical presentation.

Topics: Acute Kidney Injury; Antibodies, Viral; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Follow-Up Studies; Hantavirus Infections; Hormone Replacement Therapy; Humans; Hypophysitis; Hypopituitarism; Magnetic Resonance Imaging; Male; Middle Aged; Orthohantavirus; Phylogeny; Polymerase Chain Reaction; Polyuria; Treatment Outcome

Central or neurogenic diabetes insipidus (DI) is uncommon in the pediatric age group and rarely occurs in neonates. It should be suspected in any neonate presenting with excessive urine output and hypernatremia that persists despite increased fluid administration. Diabetes insipidus may be secondary to asphyxia, intraventricular hemorrhage, infection, and structural abnormalities or may be idiopathic or genetic. Diagnosis includes a careful history, laboratory testing, and magnetic resonance imaging. Management of neonatal DI involves a careful balance between fluid intake and pharmacologic treatment. In this article we report a case of an extremely low birth weight infant presenting with central DI possibly caused by abnormality of the pituitary gland. Persistent hypernatremia was the initial presentation. Increased fluids were given initially but were only partially helpful. Eventually subcutaneous desmopressin (DDAVP) was required. The infant was unresponsive to intranasal DDAVP and required subcutaneous DDAVP upon discharge.

Topics: Administration, Intranasal; Cerebral Hemorrhage; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diabetes Mellitus; Humans; Infant, Extremely Low Birth Weight; Infant, Newborn; Infant, Premature; Magnetic Resonance Imaging

Orbital apex syndrome (OAS) manifests as multiple cranial nerve palsies caused by an abnormal nerve response to inflammation or other processes. Central diabetes insipidus (CDI) is characterized by deficient synthesis or secretion of antidiuretic hormone. A 62-year-old woman underwent myringotomy for otitis media with effusion. Two months after the procedure, symptoms of hearing loss had not improved, and she underwent left tympanoplasty and mastoidectomy. After surgery, she presented with left ocular pain and visual loss. Neurologic examination revealed ptosis, total ophthalmoplegia, and a relative afferent pupillary defect on the left eye. Magnetic resonance imaging showed an asymmetric contrast-enhancing lesion in the left orbital apex and left cavernous sinus, with adjacent dural thickening and enhancement. OAS was diagnosed, and steroid treatment was started. During the regular follow-up period, she reported polyuria, and CDI was diagnosed. Treatment with intranasal desmopressin 10 μg twice daily was started, and symptoms greatly improved. The mechanism underlying the association of CDI with OAS is unclear, and further research is needed. The present case suggests that polyuria in OAS should alert neurologists and ophthalmologists to possible CDI.

Topics: Administration, Intranasal; Cavernous Sinus; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diffusion Magnetic Resonance Imaging; Female; Humans; Middle Aged; Ophthalmoplegia; Otitis Externa; Polyuria; Prefrontal Cortex; Pupil Disorders; Syndrome; Treatment Outcome

Langerhans cell histiocytosis (LCH) is a rare disease that mainly affects children, but this disease is significantly rarer in patients who are older than 15 years. In this disease, any organ can be involved. The skeleton, skin and lung are commonly affected, and isolated hypothalamic-pituitary (HP) involvement is relatively rare. Here we report a 17-year-old adolescent with isolated HP-LCH of enlarged pituitary stalk presented with central diabetes insipidus (CDI).. A 17-year-old male adolescent with polydipsia and polyuria accompanied with elevated serum sodium level and low urine osmolality for 3 weeks was referred to our hospital. After admission, hormonal evaluation showed that his growth hormone (GH) was slightly elevated, and serum osmolality and glucose were normal. The fluid deprivation-vasopressin test demonstrated CDI. Imaging examination showed an obvious thickening of the pituitary stalk. Lymphocytic hypophysitis, sarcoidosis and granulation tissue lesions were suspected. After oral 1-deamino-8-Darginine vasopressin (DDAVP) and prednisone were administered for 2 months, symptoms were relieved, and he discontinued taking the drugs by himself. On reexamination, imaging revealed changes in the size and shape of the pituitary stalk, with thickened nodules. Then, a diagnostic biopsy of the pituitary stalk lesion was performed. Immunohistochemistry confirmed the definitive diagnosis of LCH. The clinical symptoms subsided with oral hormone replacements.. CDI is a rare symptom in children and adolescents. Most of the causes are idiopathic, while others are caused by central nervous system (CNS) disorders. Meanwhile, lymphocytic hypophysitis, germinoma, LCH and other CNS disorders can all present as thickening of the pituitary stalk, diffuse enlargement of the pituitary gland, and weakening of high signal intensity in the neurohypophysis on magnetic resonance imaging (MRI). The differential diagnosis among these diseases depends on immunohistochemistry evidence.

Topics: Adolescent; Autoimmune Hypophysitis; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Histiocytosis, Langerhans-Cell; Humans; Hypothalamic Diseases; Male; Pituitary Diseases; Prednisone

Patients with central diabetes insipidus (CDI) are known to lose weight because their polydipsia interferes with their nutritional intake. We retrospectively examined weight changes in CDI patients when they switched from nasal to oral desmopressin.. Twenty-three patients with CDI were included. Weight change was defined as an increase or decrease of more than 3 kg or 3% body weight. As factors contributing to the weight change, we studied the patients' clinical characteristics and quality of life (QOL) scores as determined by our original questionnaire.. Five patients showed a weight loss of 5.9 kg (2.4-9.0 kg), and two patients showed weight gain, while 16 out of 23 patients were weight neutral. When the patients with weight gain and weight neutral were analyzed together, the mean weight change was +0.3 kg (-0.5 to +1.1 kg). All the patients who lost weight had a Body Mass Index ≥22 kg/m2 (38% vs. 0%, P=0.027) and higher frequencies of abnormally high serum levels of AST (40% vs. 0%, P=0.005). The sum of the QOL scores was similar between the two groups, but higher in patients who lost weight after switching to oral desmopressin (43.3±2.7) than in those who did not (38.2±5.0, P=0.01).. Switching the treatment from nasal to oral desmopressin may cause weight loss in patients with CDI who seemed to have polydipsia-associated weight gain.

Topics: Administration, Intranasal; Administration, Oral; Adult; Aged; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Hypoglycemic Agents; Male; Middle Aged; Polydipsia; Retrospective Studies; Weight Loss

A 710 g male infant was born at a referring hospital at a gestational age of 23 weeks and 2 days via vaginal delivery and was transferred to our facility at 14 days of age. His delivery was complicated by the breech presentation with difficult head extraction. The infant's initial course was significant for respiratory distress syndrome, grade III-IV intraventricular hemorrhage (IVH), acute kidney injury, and large PDA. On the day of life 29, a gradual increase in serum sodium level refractory to increasing total fluid volume was noted. The combination of persistent hypernatremia (150-160 mmol/l), polyuria (8.4 ml/kg/hr), high plasma osmolality (323 mosm/kg), hyposthenuria (75 mosm/kg) and an undetectable serum ADH (<0.8 pg/ml) confirmed the diagnosis of central diabetes insipidus (CDI). Serum sodium and urine output decreased and urine osmolality increased after subcutaneous DDAVP administration.CDI is an uncommon cause of hypernatremia in the neonatal period. The diagnosis can be difficult as excessive urine output and high serum sodium can often be attributed to high insensible water loss in the extremely premature newborn. CDI in our patient was thought to be due to grade III-IV IVH complicated by post-hemorrhagic hydrocephalus.In conclusion, the diagnosis of central DI should be considered as a complication of severe IVH in the extremely premature neonate who demonstrates persistent hypernatremia, polyuria, decreased urine osmolality, and increased plasma osmolality. Serum ADH levels can be helpful in confirming the central origin of DI and subcutaneous desmopressin can be an effective treatment in the preterm infant.

Topics: Cerebral Intraventricular Hemorrhage; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Gestational Age; Hemostatics; Humans; Infant, Extremely Premature; Infant, Newborn; Infant, Very Low Birth Weight; Male; Respiratory Distress Syndrome, Newborn; Treatment Outcome

Topics: Antineoplastic Combined Chemotherapy Protocols; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Disease Progression; Histiocytoma; Histiocytosis, Non-Langerhans-Cell; Humans; Infant; Male; Pituitary Gland; Skin; Skin Neoplasms; Treatment Outcome

A 7-year-8-month-old boy with cardiofaciocutaneous syndrome caused by the D638E mutation of the B-Raf proto-oncogene (BRAF) presented with new-onset seizures. He was incidentally found to have advanced Tanner staging on physical examination. Hormonal testing revealed pubertal levels of gonadotropins and sex steroid hormones. On brain imaging, a lack of visualisation of the posterior pituitary bright spot was observed, in addition to mild thinning of the corpus callosum and the lateral gyri of the cerebellar hemispheres. A diagnosis of idiopathic central precocious puberty was made and the patient was started on leuprolide depot treatment. Pituitary hormone testing revealed hyperprolactinemia for which the patient did not receive treatment as he was asymptomatic. During a subsequent hospital admission for seizures, the patient was diagnosed with transient central diabetes insipidus for which he required treatment with a desmopressin infusion.

Topics: Antineoplastic Agents, Hormonal; Child; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Ectodermal Dysplasia; Facies; Failure to Thrive; Heart Defects, Congenital; Hemostatics; Humans; Leuprolide; Male; Proto-Oncogene Mas; Proto-Oncogene Proteins B-raf; Puberty, Precocious; Seizures; Treatment Outcome

Central diabetes insipidus (DI) is a rare disease characterized by the excretion of excessive volumes of dilute urine due to reduced levels of the antidiuretic hormone arginine vasopressin (AVP), caused by an acquired or genetic defect in the neurohypophysis. The aim of this study was to identify any autonomic dysfunction (AD) in patients with DI as a possible cofactor responsible for their reportedly higher mortality.. The study involved 12 patients (6 females) with central idiopathic DI and a well-controlled electrolyte balance, and 12 controls matched for age, sex and cardiovascular risk factors, who were assessed using the tilt, lying-to-standing, hand grip, deep breath, Valsalva maneuver and Stroop tests.. The tilt test showed a significantly more pronounced decrease in both systolic (- 20.67 ± 18 vs. - 1.92 ± 6.99 mmHg, p = 0.0009) and diastolic blood pressure (- 10.5 ± 14.29 vs. - 1.5 ± 5 mmHg, p = 0.012) in patients than in controls. Three patients with DI had to suspend the test due to the onset of syncope. The lying-to-standing test also revealed a marked reduction in blood pressure in patients with DI (1.05 ± 0.13 vs. 1.53 ± 0.14, p = 0.0001). Similar results emerged for the Valsalva maneuver (Valsalva ratio, 1.24 ± 0.19 vs. 1.79 ± 0.11, p < 0.0001) and deep breath test (1.08 ± 0.11 vs. 1.33 ± 0.08, p < 0.0001).. All the principal autonomic tests performed in the study were concordant in indicating that patients with central DI have an impaired autonomic nervous system function despite a normal hydroelectrolytic balance under desmopressin therapy. This impairment may reflect damage to the autonomic system per se and/or the absence of any vasoactive effect of AVP on vascular smooth muscle. In our opinion, patients with central DI should be educated on how to prevent orthostatic hypotension, and pharmacological treatment should be considered for patients with a more marked impairment.

Topics: Adult; Antidiuretic Agents; Autonomic Nervous System; Cardiovascular System; Case-Control Studies; Cross-Sectional Studies; Deamino Arginine Vasopressin; Death, Sudden, Cardiac; Diabetes Insipidus, Neurogenic; Female; Hemodynamics; Humans; Hypotension, Orthostatic; Male; Middle Aged; Stroop Test; Tilt-Table Test; Valsalva Maneuver

Central diabetes insipidus is a rare disease in children caused by a deficiency of vasopressin. Its main clinical manifestations are polyuria and polydipsia. Brain malformations are one of the main causes. Desmopressin is the synthetic drug of choice for the treatment. One of the routes of administration is sublingual and its use in infants is very limited. We describe two infants with central diabetes insipidus and hydranencephaly who were successfully treated with sublingual desmopressin.. La diabetes insípida central es una patología infrecuente en pediatría ocasionada por un déficit de vasopresina. Sus manifestaciones clínicas principales son poliuria y polidipsia. Las malformaciones cerebrales son una de las principales causas. La desmopresina es la droga sintética de elección para el tratamiento. Una de las vías de administración es la sublingual y su uso en lactantes es muy limitado. Se describe a dos lactantes con hidranencefalia y diabetes insípida central que fueron tratados satisfactoriamente con desmopresina sublingual.

Topics: Administration, Sublingual; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Hydranencephaly; Infant; Male

A 58-year-old right-handed woman presented to our institution with a 1-month history of polydipsia and polyuria. She had a remote history of neurofibroma excision by dermatology and, on examination, was noted to meet the clinical diagnostic criteria for neurofibromatosis type 1. Laboratory investigations revealed hypernatraemia and elevated serum osmolality, accompanied by reduced urinary osmolality. A subsequent water deprivation test confirmed central diabetes insipidus, which responded to treatment with desmopressin. MRI of the brain showed pituitary enlargement, which raised the possibility of an underlying pituitary adenoma or, alternatively, lymphocytic hypophysitis. Both conditions have rarely been described in neurofibromatosis.

Topics: Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Neurofibromatosis 1; Neuroimaging; Polydipsia; Polyuria; Treatment Outcome; Water

Central diabetes insipidus (DI) was detected in a patient with hemorrhagic fever with renal syndrome (HFRS) who had been molecularly and serologically diagnosed with Hantaan virus infection. We recommend that clinicians differentiate central DI in HFRS patients with a persistent diuretic phase even when pituitary MRI findings are normal.

Topics: Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Hantaan virus; Hemorrhagic Fever with Renal Syndrome; Humans; Hypopituitarism; Magnetic Resonance Imaging; Male; Middle Aged; Phylogeny; Thyroxine

We report a case of subclinical central diabetes insipidus (DI), due to Rathke's cleft cysts, that was initially misdiagnosed as transient DI of pregnancy because it presented in the third trimester of pregnancy. A 37-year-old primigravida visited the Department of Obstetrics in the 30th week of gestation due to polyuria. She was admitted due to oligohydramnios; the amniotic fluid index was 3.24. A vasopressin challenge test was performed and her urine osmolality increased by >100% from baseline after the administration of desmopressin. Because central DI or transient DI of pregnancy was suspected, we prescribed her a desmopressin nasal spray. She gave birth to a relatively healthy baby at 37 weeks and 4 days of gestation. Several months after delivery, discontinuation of desmopressin resulted in recurrence of her polyuria. Magnetic resonance imaging of her brain revealed Rathke's cleft cysts, and finally central DI was diagnosed.

Topics: Administration, Intranasal; Brain; Central Nervous System Cysts; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnostic Errors; Female; Humans; Infant, Newborn; Magnetic Resonance Imaging; Nasal Sprays; Oligohydramnios; Polyuria; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Pregnancy Trimester, Third; Recurrence

Topics: Adult; Biopsy; Bone Marrow; Brain; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Erdheim-Chester Disease; Humans; Interferons; Magnetic Resonance Imaging; Male; Pituitary Gland

Central diabetes insipidus (CDI) is a rare disease during the neonatal period, making it diagnosis difficult and delaying medical treatment.. We report here a case of a premature infant born at 26 weeks gestation who, during his 1st month of life, presented persistent hypernatremia with polyuria despite increased fluid supply and low sodium intake. CDI diagnosis was suspected and then confirmed by the therapeutic test with vasopressin analog, in its oral form. Electrolyte disorders were normalized after treatment, which allowed normal weight and height growth with standard fluid supply. Biological and radiological tests were all normal; this CDI was considered idiopathic.. Persistent hypernatremia with excessive diuresis should alert to CDI diagnosis.

Topics: Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Hypernatremia; Infant, Extremely Premature; Infant, Newborn; Male

The triphasic response of pituitary stalk injury has previously been described in a minority of patients following intracranial surgery, however, this phenomenon can also occur after traumatic brain injury. We present the case of a 20-year-old male who experienced the triphasic response of pituitary stalk injury (central diabetes insipidus, syndrome of inappropriate antidiuretic hormone and central diabetes insipidus again) after striking his head on a concrete curb. His history and presentation highlight the importance of recognising the distinctive symptoms of each individual stage of pituitary stalk injury, and using the appropriate diagnostic tools and therapies to guide further management.

Topics: Antidiuretic Agents; Brain Injuries, Traumatic; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Headache Disorders; Humans; Inappropriate ADH Syndrome; Male; Pituitary Gland; Polyuria; Thirst; Young Adult

Topics: Antidiuretic Agents; Antineoplastic Agents; Biopsy; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Histiocytosis, Langerhans-Cell; Humans; Magnetic Resonance Imaging; Mercaptopurine; Pituitary Gland; Prednisolone; Skin; Treatment Outcome; Vinblastine

Langerhans cell histiocytosis (LCH) is a rare disorder, characterised by a monoclonal proliferation of aberrant histiocytes that accumulate in and infiltrate into different organs. When the hypothalamic-pituitary axis is involved, central diabetes insipidus (CDI) can be its first manifestation. Three cases of LCH with central diabetes insipidus were retrospectively analyzed: Case 1 is a 41-year old female presenting with polyuria and polydipsia. Diabetes insipidus was diagnosed and treated with desmopressin. MRI pituitary showed hypophysitis. Subsequently, she developed bone lesions and a biopsy demonstrated LCH. Case 2 is a 51-year old female presenting in 2009 with polyuria and polydipsia. Diabetes insipidus was diagnosed and treated with desmopressin. MRI pituitary revealed hypophysitis. LCH was suspected because of known pulmonary histiocytosis. Coexisting bone lesions were biopsied and confirmed LCH. Case 3 is a 44-year old female presenting with diabetes insipidus. She was treated with desmopressin as well. MRI of the pituitary gland showed impressive thickening of the infundibulum. A few months later, she developed skin lesions and a biopsy revealed LCH. Conclusively, LCH is a rare, elusive and probably underdiagnosed disease with a broad disease spectrum. Due to infiltration of the hypothalamic-pituitary axis, CDI can be the first manifestation, even before LCH is diagnosed. Therefore, LCH should be considered in the diagnostic workup of CDI.

Topics: Adult; Antidiuretic Agents; Biopsy; Bone Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Female; Histiocytosis, Langerhans-Cell; Humans; Magnetic Resonance Imaging; Middle Aged; Pituitary Diseases; Pituitary Gland; Skin Diseases

Neonatal central diabetes insipidus (CDI) with or without adipsia is a very rare complication of various complex hypothalamic disorders. It is associated with greater morbidity and a high risk of developing both hypernatremia and hyponatremia, due to the condition itself or secondary to treatment with vasopressin analogs or fluid administration. Its outcomes have yet to be evaluated.. To investigate the clinical outcomes of patients with neonatal-onset CDI or adipsic CDI with hypernatremia.. All patients diagnosed with neonatal CDI in a university hospital-based observational study and followed between 2005 and 2015 were included and analyzed retrospectively.. The various causes of CDI were grouped. Clinical outcome and comorbidities were analyzed.. Ten of the 12 patients had an underlying condition with brain malformations: optic nerve hypoplasia (n = 3), septo-optic dysplasia (n = 2), semilobar holoprosencephaly (n = 1), ectopic neurohypophysis (n = 3), and unilateral absence of the internal carotid artery (n = 1). The other two were idiopathic cases. During the median follow-up period of 7.8 (4.9-16.8) years, all but one patient displayed anterior pituitary deficiency. Transient CDI was found in three (25%) patients for whom a posterior pituitary hyperintense signal was observed with (n = 2) and without (n = 1) structural hypothalamic pituitary abnormalities, and with no other underlying cerebral malformations. Patients with permanent CDI with persistent adipsia (n = 4) and without adipsia (n = 5) required adequate fluid intake and various doses of desamino-D-arginine-8-vasopressin. Those with adipsia were more likely to develop hypernatremia (45 vs 33%), hyponatremia (16 vs 4%) (P < .0001), and severe neurodevelopmental delay (P < .05) than those without adipsia. Comorbidities were common. The underlying cause remains unknown at the age of 23 years for one patient with CDI and normal thirst.. Neonatal CDI may be transient or permanent. These vulnerable patients have high rates of comorbidity and require careful monitoring.

Topics: Adolescent; Age of Onset; Child; Child, Preschool; Cohort Studies; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Drinking; Female; Humans; Hypernatremia; Hypoglycemic Agents; Hypothalamic Diseases; Infant; Infant, Newborn; Male; Pituitary Function Tests; Retrospective Studies; Sodium; Treatment Outcome

The treatment of neurogenic diabetes insipidus (DI) in infancy is challenging and complicated by fluid overload and dehydration. Therapy with subcutaneous (SC), intranasal (IN), or oral tablet desmopressin acetate (1-desamino-8-D-arginine vasopressin [DDAVP]) remains difficult to titrate in infants.. Assess the efficacy and safety of buccally administered IN DDAVP for the management of infants with neurogenic DI.. Retrospective review of clinical and laboratory data of 15 infants (mean age, 4.5 mo) with neurogenic DI treated at a tertiary care center. Treatment was with diluted IN DDAVP formulation (10 mcg/mL) administered buccally via a tuberculin syringe to the buccal mucosa.. After initial DDAVP titration of 2-3 days, IN DDAVP doses ranged from 1 to 5 mcg twice daily given buccally. Mean serum sodium concentration at DI diagnosis was 159 ± 6.6 mmol/L (range, 151-178) and improved to 142 ± 3.5 mmol/L (range, 137-147) with the buccally administered IN DDAVP. Normal sodium concentrations were established without major fluctuations. Serum sodium was then maintained in the outpatient setting at a mean of 145.7 ± 4.8 mmol/L (mean duration of follow-up, 11 mo).. Buccally administered IN formulation of DDAVP provides a practical and safe treatment alternative for neurogenic DI in infancy. Our approach avoided severe hypo- and hypernatremia during DDAVP titration and ongoing outpatient management of DI. The possibility for smaller dosage increments and ease of administration make IN DDAVP administered buccally preferable over other DDAVP treatment options in infants.

Topics: Administration, Buccal; Administration, Intranasal; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Infant; Infant, Newborn; Male; Retrospective Studies; Treatment Outcome

Central diabetes insipidus (CDI) is a rare cause of hypernatremia during the neonatal period. The diagnosis is particularly difficult in very low birth weight (VLBW) newborns.. We report on a preterm newborn who presented CDI soon after birth. On the third day of life, signs of dehydration were present despite normal fluid supply. The diuresis rate was 4.4 ml/kg/h. Although the fluid supply was then increased, the dehydration continued, with hypernatremia, normal glycemia, diuresis of 7.4 ml/kg/h and urine density of 1005 mOsmol/l. Thus, a diagnostic hypothesis of diabetes insipidus was raised. A test with a nasal vasopressin analogue (dDAVP) was performed and CDI was confirmed. Reduction of the fluid supply became possible through appropriate treatment.. The diagnosis of CDI is rarely made during the neonatal period, especially in VLBW newborns, because of the difficulty in detecting elevated diuresis. Persistent hypernatremia, usually accompanied by hyperthermia despite abundant fluid supply, weight loss and low urine osmolality are important signs of alert.

Topics: Administration, Intranasal; Deamino Arginine Vasopressin; Dehydration; Diabetes Insipidus, Neurogenic; Diuresis; Early Diagnosis; Female; Hemostatics; Humans; Hypernatremia; Infant, Newborn; Infant, Very Low Birth Weight; Male; Osmolar Concentration; Premature Birth; Treatment Outcome

Central diabetes insipidus (CDI), which is characterized by polyuria and polydipsia, is caused by a deficiency of the antidiuretic hormone arginine vasopressin (AVP). While CDI is treated with desmopressin, an analogue of AVP, the intranasal formulation is inconvenient and CDI patients reportedly prefer the oral formulation to the intranasal one. In Japan, intranasal desmopressin had been the only formulation for the treatment of CDI until 2012, when the desmopressin orally disintegrating tablet (ODT) was approved for treatment. In this study we analyzed 26 patients with CDI in whom intranasal desmopressin was switched to desmopressin ODT. The mean daily dose of intranasal desmopressin was 10 ± 8 μg/day, and that of desmopressin ODT was 142 ± 59 μg/day. The mean serum sodium levels were 140 ± 5 mmol/L and 140 ± 3 mmol/L with intranasal desmopressin and desmopressin ODT, respectively, and there were no significant differences between these values. The frequency of hyponatremia (<135 mmol/L) with intranasal desmopressin was 11.7% and that with desmopressin ODT was 7.6%, while the frequency of hyponatremia (<130 mmol/L) with intranasal desmopressin was 4.2% and that with desmopressin ODT was 1.3%. Statistical analyses revealed that incidence of hyponatremia was significantly decreased after the switch to desmopressin ODT. Thus, it is suggested that water balance is better controlled with desmopressin ODT than with intranasal desmopressin in patients with CDI.

Topics: Administration, Intranasal; Administration, Oral; Adolescent; Adult; Aged; Antidiuretic Agents; Child; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Drug Approval; Drug Monitoring; Electronic Health Records; Female; Hospitals, University; Humans; Hyponatremia; Incidence; Japan; Male; Middle Aged; Risk; Young Adult

Recently, an increased incidence of central diabetes insipidus (CDI) in pregnancy, and less frequently in the post partum period, has been reported, most probably favoured by some conditions occurring in pregnancy. This study was aimed at investigating the influence of pregnancy on a pre-existing potential/subclinical hypothalamic autoimmunity. We studied the longitudinal behaviour of arginine-vasopressin cell antibodies (AVPcAbs) and post-pituitary function in two young women with a positive history of autoimmune disease and presence of AVPcAbs, but without clinical CDI, and who became pregnant 5 and 7 months after our first observation. The behaviour of post-pituitary function and AVPcAbs (by immunofluorescence) was evaluated at baseline, during pregnancy and for 2 years after delivery. AVPcAbs, present at low/middle titres at baseline in both patients, showed a titre increase during pregnancy in one patient and after delivery in the other patient, with development of clinically overt CDI. Therapy with 1-deamino-8-d-arginine vasopressin (DDAVP) caused a prompt clinical remission. After a first unsuccessful attempt of withdrawal, the therapy was definitively stopped at the 6th and the 7th month of post partum period respectively, when AVPcAbs disappeared, accompanied by post-pituitary function recovery, persisting until the end of the follow-up. The determination of AVPcAbs is advisable in patients with autoimmune diseases planning their pregnancy, because they could be considered good predictive markers of gestational or post partum autoimmune CDI. The monitoring of AVPcAb titres and post-pituitary function during pregnancy in these patients may allow for an early diagnosis and an early replacement therapy, which could induce the disappearance of these antibodies with consequent complete remission of CDI.

Topics: Adult; Arginine Vasopressin; Autoantibodies; Autoimmune Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Pregnancy

Central diabetes insipidus (CDI) is an infrequent complication of neurosarcoidosis (NS). Its presentation may be masked by adrenal insufficiency (AI) and uncovered by subsequent steroid replacement. A 45-year-old woman with a history of NS presented 2 weeks after abrupt cessation of prednisone with nausea, vomiting, decreased oral intake and confusion. She was diagnosed with secondary AI and intravenous hydrocortisone was promptly begun. Over the next few days, however, the patient developed severe thirst and polyuria exceeding 6 L of urine per day, accompanied by hypernatraemia and hypo-osmolar urine. She was presumed to have CDI due to NS, and intranasal desmopressin was administered. This eventually normalised her urine output and serum sodium. The patient was discharged improved on intranasal desmopressin and oral prednisone. AI may mask the manifestation of CDI because low serum cortisol impairs renal-free water clearance. Steroid replacement reverses this process and unmasks an underlying CDI.

Topics: Adrenal Insufficiency; Antidiuretic Agents; Central Nervous System Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Glucocorticoids; Humans; Hydrocortisone; Hypernatremia; Middle Aged; Polyuria; Prednisone; Sarcoidosis; Thirst

Topics: Deamino Arginine Vasopressin; Demyelinating Diseases; Diabetes Insipidus, Neurogenic; Female; Humans; Hyponatremia; Saline Solution, Hypertonic; Syndrome; Young Adult

There is ongoing discussion regarding the mechanisms underlying edema formation in nephrotic syndrome (NS). Many studies published in the last decade reported that primary renal sodium retention was a major factor in edema formation. However, many of the factors influencing edema formation in NS remain unclear, including the role of arginine vasopressin (AVP).. We report a 12-year-old boy with steroid-dependent NS complicated by idiopathic central diabetes insipidus (CDI). He did not develop edema during his first relapse of NS after developing CDI, despite having hypoalbuminemia. He had polydipsia, polyuria, low urine osmolality, and a low serum arginine AVP level. His fractional excretion of sodium was only slightly low. Endocrinological testing and magnetic resonance imaging revealed idiopathic CDI. After starting desmopressin therapy, he developed edema when his NS relapsed.. This is the first known reported case of NS in a patient with CDI. The findings suggest that appropriate AVP secretion in response to an increase in serum osmolality caused by renal sodium retention is necessary for excess extracellular fluid accumulation in NS. Further investigation is needed to more fully understand the role of AVP in edema formation in NS.

Topics: Anti-Inflammatory Agents; Arginine Vasopressin; Child; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Edema; Humans; Male; Nephrotic Syndrome; Prednisolone; Water Deprivation

Epidemiological data for central diabetes insipidus (CDI) are sparse.. The purpose of this study was to provide accurate epidemiological data on CDI on a national level.. This was a drug utilization and patient registry study during a 5-year period from 2007 to 2011.. We used the Danish National Prescription Registry data linked with the Danish National Patient Registry to study the epidemiology of CDI using waiting time distribution and other pharmacoepidemiological methods.. A total of 1285 patients with CDI were recorded in the observation period and given 9309 prescriptions for desmopressin in the nasal formulation, orodispersible tablet, or conventional tablet.. The period prevalence rate of CDI in Denmark over the 5-year period investigated was 23 CDI patients per 100 000 inhabitants, with a higher prevalence in children and older adults (>80 years of age). The 1-year period prevalence rate of CDI decreased in Denmark over the 5 years from approximately 10 to 7 CDI patients per 100 000 inhabitants. The yearly incidence rate of new cases of CDI was found to be 3 to 4 patients per 100 000. The incidence of (presumable) congenital CDI was found to be 2 infants per 100 000 infants. Half of the patients with CDI prescribed as oral treatment were provided dosing instructions to only administer the drug before bedtime, and one third of the CDI patients either had no specific instructions or were instructed to use the drug as needed. Hospital admissions due to severe hyponatremia occurred in 0.9% of patients over a 5-year period, predominantly in females with an incidence ratio of women to men of 1.8:1.. Half of the cases of CDI are acquired later in life. At least half of the patients with CDI are instructed to prevent nocturnal polyuria, but it is not clear whether their CDI remains uncontrolled during the daytime or, alternatively, whether they use desmopressin only as needed. Female patients with CDI had approximately twice the number of hospital admissions due to severe hyponatremia than male patients with CDI.

Topics: Administration, Intranasal; Adolescent; Adult; Aged; Aged, 80 and over; Antidiuretic Agents; Child; Child, Preschool; Deamino Arginine Vasopressin; Denmark; Diabetes Insipidus, Neurogenic; Drug Prescriptions; Drug Utilization; Female; Humans; Infant; Infant, Newborn; Male; Middle Aged; Population Surveillance; Registries; Young Adult

Central diabetes insipidus (CDI) is a rare disease characterized by polyuria and polydipsia. Patients with CDI have been successfully treated with desmopressin administered either by intranasal instillation or oral tablets. Recently, a desmopressin orally disintegrating tablet (ODT) was approved as the first oral desmopressin tablet for CDI treatment in Japan. We conducted a retrospective single-center study of 15 Japanese CDI patients treated with desmopressin ODT therapy, which aimed to evaluate the efficacy and safety of switching to desmopressin ODT and to analyze the clinical factors that affect the desmopressin ODT dose in Japanese patients. The daily mean dose of desmopressin ODT was 104 ± 46.30 μg and the mean ratio of oral to nasal desmopressin dose was 17.0 ± 7.6, both of which are considerably smaller than those of previous dose-titration study. Moreover, the nasal spray group needed significantly smaller ratios of nasal to oral desmopressin than the nasal drop group (11.7 ± 6.5 vs 21.0 ± 5.5, p = 0.02). The ratio of oral to nasal desmopressin dose had a significant inverse correlation with the required nasal desmopressin dose. Multiple regression analysis demonstrated the ratios of nasal to oral desmopressin dose depended on intranasal formulations. In conclusion, desmopressin ODT was safe and effective in the treatment of Japanese adult CDI patients. When switching to ODT, we should care about the possibility that patients require smaller ODT doses than what was initially expected based on previously published data and also nasal formulations in terms of their differences of expected switching ratio.

Topics: Administration, Intranasal; Administration, Oral; Adult; Aged; Aged, 80 and over; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Drug Substitution; Female; Humans; Japan; Male; Middle Aged; Retrospective Studies; Tablets

To assess the efficiency of oral desmopressin lyophilisate (ODL) in neonatal central diabetes insipidus (CDI).. The characteristics of four newborns with CDI treated with ODL were evaluated.. Four newborns with polyuria and hypernatremia were included [male, 2 (50%); mean postnatal age, 19±17 days]. At the time of hypernatremia, the mean serum and urine osmolality values were 310±16 and 179±48 mOsm/kg, respectively. Antidiuretic hormone levels were undetectable (<0.5 pmol/L) in all cases. Magnetic resonance imaging revealed anatomical malformations in all cases. ODL (60 μg/tablet) dissolved in water (3-5 mL) was initiated with a dose of 5 μg/kg/day in two equal doses, together with limitation of water intake to avoid hyponatremia. Serum sodium levels returned to normal in a mean duration of 58±9.9 h with a mean decline rate of 0.37±0.1 mEq/L/h after desmopressin administration. Rehospitalization was required for one of the infants because of hypernatremia due to non-compliance. No episode of hyponatremia was encountered. Weight gain and growth of the infants were normal during the mean follow-up duration of 8.5±1 months.. ODL appears to be practical and safe in the treatment of CDI during the first year of life.

Topics: Administration, Oral; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Dose-Response Relationship, Drug; Humans; Hypernatremia; Infant; Infant, Newborn; Male; Polyuria; Treatment Outcome

Topics: Adult; Antidiuretic Agents; Brain Edema; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Hyponatremia; Magnetic Resonance Imaging; Male; Myelinolysis, Central Pontine; Syndrome

A 14-year-old girl weighing 32 kg was diagnosed with suprasellar tumor causing hydrocephalus, hypothyroidism, adrenal dysfunction and central diabetes insipidus. She was treated with levothyroxine and hydrocortisone and urged to take fluid to replace urine. She was scheduled to undergo ventricular drainage to relieve hydrocephalus prior to tumor resection. For the first surgery, desmopressin was not started and urine output reached 4,000 to 6,000 ml x day(-1), urine osmolality 64 mOsm x l(-1) and urine specific gravity 1.002. Anesthesia was induced with sevoflurane and maintained with propofol and remifentanil. Maintenance fluid was with acetated Ringer's solution and urine loss was replaced with 5% dextrose. Bradycardia and hypotension occurred after intubation, which was treated with volume load. Infusion volume was 750 ml and urine output was 1100 ml during 133 min of anesthesia. Postoperative day 1 nasal desmopressin was started. Ten days later, partial tumor resection was performed. Anesthesia was induced with propofol and fentanyl and maintained with sevoflurane and remifentanil. Infusion volume was 610 ml, urine output 380 ml, and blood loss 151 ml during 344 min of anesthesia. Hemodynamic parameters were stable throughout the procedure. Pathology of the tumor was revealed to be germinoma. Bradycardia and hypotension experienced during the first surgery was suspected to be caused by preoperative hypovolemia brought by polyuria. Desmopressin was proved to be effective to treat excessive urine output and to maintain good perioperative water balance.

Topics: Adolescent; Anesthesia, General; Antidiuretic Agents; Brain Neoplasms; Cerebrospinal Fluid Shunts; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Germinoma; Humans; Perioperative Care

Topics: Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Male

In recent years, there have been several improvements in the treatment of neurohypophyseal diabetes insipidus (DI). They include new formulations of the vasopressin analog, desmopressin; a better understanding of the effect of fluid intake on dosing; and more information about treatments of infants, children, and pregnant women who present special challenges. This review aims to summarize past and current information relative to the safety and efficacy of treatments for the types of DI caused by a primary deficiency of vasopressin.. The review is based on publications identified primarily by a PubMed search of the international literature without limitations of date.. In acute settings where fluid intake is determined by factors other than thirst, desmopressin should be given iv in doses that have a short duration of action and can be adjusted quickly in accordance with changes in hydration as indicated by plasma sodium. In ambulatory patients, the oral formulations (tablet or melt) are preferred for their convenience. If fluid intake is regulated normally by the thirst mechanism, the tablets or melt can be taken safely 1 to 3 times a day in doses sufficient to completely eliminate the polyuria. However, if fluid intake consistently exceeds replacement needs as evidenced by the development of hyponatremia, the dose should be reduced to allow higher than normal rates of urine output or intermittent breakthrough diuresis. This regimen is often indicated in infants or children because their rate of fluid intake tends to be greater than in adults. In all cases, the appropriate dose should be determined by titration, owing to considerable interindividual differences in bioavailability and antidiuretic effect.. Desmopressin can provide effective and safe therapy for all patients with neurohypophyseal or gestational DI if given in doses and by a route that takes into account the determinants of fluid intake.

Topics: Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Polyuria; Treatment Outcome

Diabetes insipidus (DI) is common following surgery for a pituitary/sellar lesion. Postoperative DI persisting beyond a few weeks is usually regarded as permanent, requiring lifelong desmopressin therapy.. A 16-year-old girl underwent pituitary decompression following a pituitary tumour apoplexy. She developed DI with thirst and hypotonic polyuria in the immediate post-operative period, which was controlled with parenteral DDAVP. Her symptoms persisted and she was discharged on oral desmopressin. Subsequently, she commenced growth hormone replacement. A water deprivation test 6 weeks postoperatively confirmed persistent DI with failure to concentrate urine after 8 hours of dehydration. She was maintained on desmopressin and reported intense thirst and polyuria every time she delayed taking the medication up until 1 year postoperatively. After 1 year, she complained of bloating and stopped taking desmopressin but her symptoms did not recur. A repeat water deprivation test confirmed very late resolution of DI with normal urinary concentration. She has remained asymptomatic ever since.. Delayed recovery of normal posterior pituitary function is exceptionally rare. We hypothesise that the underlying mechanism may be the revascularisation and regeneration of the posterior pituitary tissues or of the axons terminating in the posterior pituitary.

Topics: Adolescent; Antidiuretic Agents; Deamino Arginine Vasopressin; Decompressive Craniectomy; Diabetes Insipidus, Neurogenic; Female; Humans; Pituitary Apoplexy; Pituitary Neoplasms; Postoperative Complications; Recovery of Function; Time Factors

Gunshot injuries (GSI) of the cranial area have an extremely high mortality rate. Herein, we present a girl who has been living with a bullet in the posterior sellar region. A 6-year-old girl was admitted with complaints of headache, polyuria and polydypsia, which started after a GSI. At the time of admission the patient's anthropometric, physical and neurological examinations were normal. Urine output was 5.5 L/m2/24h. A water deprivation test suggested central diabetes insipidus, which responded to treatment. Evaluation of other pituitary hormones revealed central hypothyroidism and growth hormone deficiency. Pituitary hormone deficiency must be kept in mind in patients injured by a gunshot to the sellar/parasellar region.

Topics: Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Human Growth Hormone; Humans; Hypopituitarism; Hypothyroidism; Pituitary Hormones; Thyroxine; Wounds, Gunshot

A 13-month-old female domestic shorthair cat presented with a 10-month history of polyuria and polydipsia that began after having been hit by a car. Neurological examination revealed visual deficits and an absent bilateral menace response. Hematological and serum biochemical analyses were within reference values, but hyposthenuria was identified. Failure to concentrate urine during the water deprivation test followed by an increase in urine specific gravity after administration of synthetic antidiuretic hormone (ADH) suggested a diagnosis of central diabetes insipidus. Subcutaneous or oral administration of synthetic ADH was effective in central diabetes insipidus treatment during the 19-month follow-up.

Topics: Administration, Oral; Animals; Cat Diseases; Cats; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Infusions, Subcutaneous

To determine the occurrence rate of central diabetes insipidus in pediatric patients with severe traumatic brain injury and to describe the clinical, injury, biochemical, imaging, and intervention variables associated with mortality.. Retrospective chart and imaging review.. Children's Hospital, level 1 trauma center.. Severely injured (Injury Severity Score ≥ 12) pediatric trauma patients (>1 month and <18 yr) with severe traumatic brain injury (presedation Glasgow Coma Scale ≤ 8 and head Maximum Abbreviated Injury Scale ≥ 4) that developed acute central diabetes insipidus between January 2000 and December 2011.. Of 818 severely injured trauma patients, 180 had severe traumatic brain injury with an overall mortality rate of 27.2%. Thirty-two of the severe traumatic brain injury patients developed acute central diabetes insipidus that responded to desamino-8-D-arginine vasopressin and/or vasopressin infusion, providing an occurrence rate of 18%. At the time of central diabetes insipidus diagnosis, median urine output and serum sodium were 6.8 ml/kg/hr (interquartile range = 5-11) and 154 mmol/L (interquartile range = 149-159), respectively. The mortality rate of central diabetes insipidus patients was 87.5%, with 71.4% declared brain dead after central diabetes insipidus diagnosis. Early central diabetes insipidus onset, within the first 2 days of severe traumatic brain injury, was strongly associated with mortality (p < 0.001), as were a lower presedation Glasgow Coma Scale (p = 0.03), a lower motor Glasgow Coma Scale (p = 0.01), an occurrence of fixed pupils (p = 0.04), and a prolonged partial thromboplastin time (p = 0.04). Cerebral edema on the initial computed tomography, obtained in the first 24 hrs after injury, was the only imaging finding associated with death (p = 0.002). Survivors of central diabetes insipidus were more likely to have intracranial pressure monitoring (p = 0.03), have thiopental administered to induce coma (p = 0.04) and have received a decompressive craniectomy for elevated intracranial pressure (p = 0.04).. The incidence of central diabetes insipidus in pediatric patients with severe traumatic brain injury is 18%. Mortality was associated with early central diabetes insipidus onset and cerebral edema on head computed tomography. Central diabetes insipidus nonsurvivors were less likely to have received intracranial pressure monitoring, thiopental coma and decompressive craniectomy.

Topics: Adolescent; Antidiuretic Agents; Brain Edema; Brain Injuries; Child; Child, Preschool; Coma; Deamino Arginine Vasopressin; Decompressive Craniectomy; Diabetes Insipidus, Neurogenic; Female; Glasgow Coma Scale; Humans; Hypnotics and Sedatives; Incidence; Intracranial Hypertension; Intracranial Pressure; Male; Monitoring, Physiologic; Partial Thromboplastin Time; Pupil Disorders; Radiography; Retrospective Studies; Thiopental; Time Factors

Polyuria is an uncommon clinical presentation in paediatric practice. When diabetes mellitus has been excluded by history taking and preliminary investigations and impaired renal concentrating ability is confirmed, water deprivation test (WDT) is necessary to differentiate among central diabetes insipidus (CDI), nephrogenic diabetes insipidus, or primary polydipsia. Traditionally, responsiveness to desmopressin injection is defined as urine osmolality >750 mOsm/kg. However, that level could not be reached in the review of our patients. We discuss how to arrive at the diagnosis of CDI in WDT. An approach to polyuria and WDT will also be discussed.

Topics: Adolescent; Child; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Female; Humans; Infant; Male; Polyuria; Retrospective Studies; Water Deprivation

Diabetes insipidus is a rare endocrine disorder in paediatric patients. Polyuria is a cardinal manifestation that is extremely difficult to recognize in diapered infants. Careful urine quantification is the key to diagnosis in appropriate clinical setting. We report a case of a 4 months old infant presenting with an acute life threatening event following an episode of vomiting and decreased oral intake. She had profound hypernatremia which persisted after stabilization. Polyuria unrecognized by the mother was revealed by 24-hour urine output measurement. A diagnosis of diabetes insipidus was made after appropriate laboratory investigations including serum and urine osmolality. The central nature of the disease was confirmed by neuroimaging which showed holoprosencephaly.

Topics: Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Female; Humans; Hypernatremia; Infant; Magnetic Resonance Imaging; Osmolar Concentration

Most cases of neonatal central diabetes insipidus are caused by an injury, which often results in other handicaps in the patient. The infant's prognosis will be determined by his or her own early age and disability as well as by the physician's skill. However, the rarity of this condition prevents the acquisition of personal experience dealing with it.. A neonatal hemorrhagic stroke, caused by an aortic coarctation, caused right lower limb paresis, swallowing disability, and central diabetes insipidus in a term infant. The scant oral intake, as a consequence of his disability, caused progressive undernutrition which closed a vicious circle, delaying his development and his ability to overcome the swallowing handicap. On the other hand, nasal desmopressin absorption was blocked by several common colds, resulting in brain bleeding because of severe dehydration. This even greater brain damage hampered the improvement of swallowing, closing a second harmful circle. Moreover, a devastating central myelinolysis with quadriplegia, caused by an uncontrolled intravenous infusion, consummated a pernicious sequence, possibly unreported.. The child's overall development advanced rapidly when his nutrition was improved by gastrostomy: This was a key effect of nutrition on his highly sensitive neurodevelopment. Besides, this case shows potential risks related to intranasal desmopressin treatment in young children.

Topics: Antidiuretic Agents; Aortic Coarctation; Deamino Arginine Vasopressin; Deglutition Disorders; Diabetes Insipidus, Neurogenic; Humans; Infant, Newborn; Male; Quadriplegia; Stroke

Topics: Antineoplastic Combined Chemotherapy Protocols; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Female; Humans; Leukemia, Myeloid, Acute; Middle Aged; Polyuria; Treatment Outcome

Diabetes insipidus (DI) is characterized by excessive urination and thirst. This disease results from inadequate output of antidiuretic hormone (ADH) from the pituitary gland or the absence of the normal response to ADH in the kidney. We present a case of transient central DI in a patient who underwent a cardiopulmonary bypass (CPB) for coronary artery bypass grafting (CABG). A 44-yr-old male underwent a CABG operation. An hour after the operation, the patient developed polyuria and was diagnosed with central DI. The patient responded to desmopressin and completely recovered five days after surgery. It is probable that transient cerebral ischemia resulted in the dysfunction of osmotic receptors in the hypothalamus or hypothalamus-pituitary axis during CPB. It is also possible that cardiac standstill altered the left atrial non-osmotic receptor function and suppressed ADH release. Therefore, we suggest that central DI is a possible cause of polyuria after CPB.

Topics: Adult; Antidiuretic Agents; Coronary Artery Bypass; Coronary Vessels; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Hypothalamus; Magnetic Resonance Imaging; Male; Pituitary Gland; Polyuria; Postoperative Complications; Radionuclide Imaging

We herein report the case of a patient who presented with an acute decrease of visual acuity, hypertension, focal seizures and transient mental dysfunction while undergoing desmopressin treatment. Neuroimaging revealed bilateral occipital-parietal lesions that presented with vasogenic edema. After controlling the hypertension and discontinuing the desmopressin treatment, the patient's condition improved. A follow-up imaging examination performed six months later showed complete resolution of the lesions. It is important to recognize posterior reversible encephalopathy syndrome (PRES) as a rare and serious complication of desmopressin administration. Both the blood pressure and water electrolyte balance should be carefully monitored in patients receiving desmopressin therapy.

Topics: Adult; Antidiuretic Agents; Blood Pressure; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Magnetic Resonance Imaging; Posterior Leukoencephalopathy Syndrome; Tomography, X-Ray Computed; Water-Electrolyte Balance

Central diabetes insipidus (CDI) is caused by deficient secretion of antidiuretic hormone (ADH) due to different conditions that can affect the hypothalamic neurons. It results in an inability to retain normal quantities of free water, which leads to polyuria, including at night, and polydipsia. In adults, it is mostly due to the "idiopathic" form or present after pituitary surgery or a traumatic brain injury. In rare cases, an underlying systemic disease is found. The diagnosis of CDI is based on the water deprivation test. Pituitary MRI and specific clinical and biological work-up are recommended to precise etiology. Treatment of choice is desmopressin, a synthetic analogue of the endogenous ADH hormone. A multidisciplinary team generally provides management and monitoring of CDI.

Topics: Adult; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Magnetic Resonance Imaging; Patient Care Team; Polydipsia; Polyuria; Vasopressins; Water Deprivation

Topics: Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Equipment Failure; Humans; Hypernatremia; Magnetic Resonance Imaging; Nebulizers and Vaporizers; Renal Agents

Familial neurohypophyseal (central) diabetes insipidus (DI) is caused by mutations in the arginine vasopressin-neurophysin II (AVP-NPII) gene. The majority of cases is inherited in an autosomal dominant way. In this study, we present the clinical features of a mother and her son with autosomal dominant neurohypophyseal DI caused by a novel mutation.. A thirty-four-year-old woman and her three-year-old son were evaluated because of polyuria and polydipsia since the age of 1.5 years onwards. Both patients were subjected to a water deprivation test confirming the diagnosis of central DI. Magnetic resonance imaging of the brain of the mother showed a hypothalamus without apparent abnormalities and a relatively small neurohypophysis without a hyperintense signal. Mutation analysis showed a c.322G>T (p.?/p.Glu108X) in Exon 2 of the AVP-NPII gene in both mother and son.. This study reports neurohypophyseal DI in a mother and her son due to a novel mutation in Exon 2 of the AVP-NPII gene. Clinical and pathophysiological aspects of this disease are shortly reviewed and discussed.

Topics: Adult; Arginine Vasopressin; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Male; Neurophysins

Topics: Anti-Bacterial Agents; Antidiuretic Agents; Cefotaxime; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Drug Therapy, Combination; Gentamicins; Humans; Infant, Newborn; Male; Meningitis, Bacterial; Streptococcal Infections; Streptococcus agalactiae; Treatment Outcome

In 2003, a 64-year-old woman was diagnosed with mixed connective tissue disease and treated with oral prednisolone (30 mg/day). The prednisolone dose was gradually decreased, and a dose of 5 mg/day had been maintained since 2004. In 2009, she gradually developed vision loss, malaise, anorexia, and throat pain due to hydrodipsia. She was noted to have iritis and vitreous opacity by an ophthalmologist, and was referred for further evaluation. Fine rales were audible throughout the entire lung field, and chest CT showed diffuse small nodules that were more prominent on the upper and middle lobes, and swelling of the mediastinal and hilar lymph nodes. Transbronchial lung biopsy showed many epithelioid granulomas with multinuclear giant cells, compatible with sarcoidosis. Polyuria was identified as a cause of hydrodipsia and a diagnosis of partial central diabetes insipidus was made. High-dose prednisolone (40 mg/day) together with intranasal administration of desmopressin resulted in improvement of all of her clinical symptoms. MCTD followed by sarcoidosis is rare. Furthermore, this is the first reported case of MCTD complicated by sarcoidosis and central diabetes insipidus.

Topics: Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Middle Aged; Mixed Connective Tissue Disease; Prednisolone; Sarcoidosis, Pulmonary; Treatment Outcome

Familial neurohypophysial diabetes insipidus (FNDI) is a rare disease that is inherited in an autosomal dominant manner. In a previous study, we made a mouse model for FNDI, which showed progressive polyuria accompanied by inclusion bodies in the arginine vasopressin (AVP) neurons formed by aggregates in the endoplasmic reticulum. The present study was conducted to determine whether the activities of AVP neurons are related to the phenotype progression in the FNDI model. In the first experiment, female heterozygous mice were administered either desmopressin (dDAVP) or a vehicle (control) subcutaneously with osmotic minipumps for 30 days. The dDAVP treatment significantly decreased the urine volume, AVP mRNA expression, and inclusion bodies in the AVP neurons. Urine volume in the dDAVP group remained significantly less than the control for 14 days even after the minipumps were removed. In the second experiment, the males were fed either a 0.2% Na or 2.0% Na diet for 6 mo. Urine AVP excretion was significantly increased in the 2.0% Na group compared with the 0.2% Na group for the first 2 mo but gradually decreased thereafter. Throughout the experiments, urine volume increased progressively in the 2.0% Na group but not in the 0.2% Na group. Immunohistochemical analyses revealed that inclusion bodies in the AVP cells had significantly increased in the 2.0% Na compared with the 0.2% Na group. These data demonstrated that activation of AVP neurons could accelerate the aggregate formation as well as the progression of the polyuria in the FNDI model mice.

Topics: Animals; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Disease Progression; Drinking; Hematocrit; Hypoglycemic Agents; Immunohistochemistry; In Situ Hybridization; Male; Mice; Mice, Inbred C57BL; Neurons; Osmolar Concentration; Phenotype; Pituitary Gland, Posterior; Sodium, Dietary; Urodynamics; Vasopressins

To report a case of infundibuloneurohypophysitis treated with steroid.. A 65-year-old woman who was well until 4 weeks before admission and was not taking any medication presented with acute development of polydipsia and polyuria. Urinary volume was increased to 4,500 ml/day. She showed elevated serum osmolality and low urine osmolality, together with shortage of antidiuretic hormone. Magnetic resonance imaging (MRI) of the pituitary revealed marked nodular thickening of the neurohypophysis. Endocrinologically, anterior pituitary function appeared normal. Based on these examinations, she was diagnosed as having central diabetes insipidus due to lymphocytic infundibuloneurohypophysitis.. Prednisolone (1 mg/kg/day, p.o.) and D-deaminovasopressin (5 microg/day, intranasal) were commenced. Ten days after the administration of the agents, MRI showed a dramatic improvement in the thickening of the neurohypophysis. Ten weeks later, abnormalities found in earlier MRI had disappeared. The drugs were withdrawn gradually, and diabetes insipidus ceased 25 weeks later. Recurrence was not seen in the subsequent MRI, and the function of the posterior pituitary gland was completely normalized even 7 years after discontinuation of treatments.. This case shows that noninvasive diagnosis and appropriate steroid administration can effectively cure lymphocytic infundibuloneurohypophysitis; it is recommended with long-term follow-up.

Topics: Aged; Antiemetics; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Magnetic Resonance Imaging; Pituitary Gland; Prednisolone; Steroids

Vasopressin influences salt and water transport in renal epithelia. This is coordinated by the combined action of V2 receptor-mediated effects along distinct nephron segments. Modulation of NaCl reabsorption by vasopressin has been established in the loop of Henle, but its role in the distal convoluted tubule (DCT), an effective site for fine regulation of urinary electrolyte composition and the target for thiazide diuretics, is largely unknown. The Na+-Cl- cotransporter (NCC) of DCT is activated by luminal trafficking and phosphorylation at conserved NH2-terminal residues. Here, we demonstrate the effects of short-term vasopressin administration (30 min) on NCC activation in Brattleboro rats with central diabetes insipidus (DI) using the V2 receptor agonist desmopressin (dDAVP). The fraction of NCC abundance in the luminal plasma membrane was significantly increased upon dDAVP as shown by confocal microscopy, immunogold cytochemistry, and Western blot, suggesting increased apical trafficking of the transporter. Changes were paralleled by augmented phosphorylation of NCC as detected by antibodies against phospho-threonine and phospho-serine residues (2.5-fold increase at Thr53 and 1.4-fold increase at Ser71). dDAVP-induced phosphorylation of NCC, studied in tubular suspensions in the absence of systemic effects, was enhanced as well (1.7-fold increase at Ser71), which points to the direct mode of action of vasopressin in DCT. Changes were more pronounced in early (DCT1) than in late DCT as distinguished by the distribution of 11beta-hydroxysteroid dehydrogenase 2 in DCT2. These results suggest that the vasopressin-V(2) receptor-NCC signaling cascade is a novel effector system to adjust transepithelial NaCl reabsorption in DCT.

Topics: 11-beta-Hydroxysteroid Dehydrogenase Type 2; Animals; Antidiuretic Agents; Blotting, Western; Cell Membrane; Chlorides; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Disease Models, Animal; Immunohistochemistry; Kidney Tubules, Distal; Male; Microscopy, Confocal; Natriuresis; Phosphorylation; Protein Transport; Rats; Rats, Brattleboro; Rats, Wistar; Receptors, Drug; Receptors, Vasopressin; Sodium; Sodium Chloride Symporter Inhibitors; Solute Carrier Family 12, Member 3; Symporters; Time Factors; Up-Regulation

Topics: Animals; Antidiuretic Agents; Cell Membrane; Chlorides; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Kidney Tubules, Distal; Natriuresis; Phosphorylation; Protein Transport; Rats; Receptors, Drug; Receptors, Vasopressin; Sodium; Sodium Chloride Symporter Inhibitors; Solute Carrier Family 12, Member 3; Symporters; Up-Regulation

One of the synthetic analogues of antidiuretic hormone-desmopressin is used in patients with central diabetes insipidus and in those with coagulation disorders. However, its effects on bile secretion are not fully defined. We investigated the effect of desmopressin on bile formation and determined the role of V1a vasopressin receptors in the action of desmopressin on choleresis. Rats were injected intraportally with a bolus of desmopressin; the changes of bile flow, the content of free and conjugated cholates were compared with control animal group. Selective antagonist of V1a receptors was injected 10 minutes before desmopressin treatment and the findings were compared with the results after desmopressin injection alone. Desmopressin increased bile flow, secretion of total cholates like amino acids conjugated, while diminished free bile acids content. Secreted bile volume and conjugated bile acids content were reduced in V1a receptors antagonist+desmopressin-treated rats. In contrast, free bile acids content was more than the results in desmopressin-treated rats. Desmopressin at concentrations nearly equal to physiological concentrations of natural hormone in blood shows its choleretic effect. Antagonist of V1a vasopressin receptors modulates desmopressin action. This certifies the role of these receptors in the action of desmopressin on different processes of bile formation.

Topics: Animals; Antidiuretic Hormone Receptor Antagonists; Bile; Bile Acids and Salts; Cholagogues and Choleretics; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Male; Rats; Rats, Wistar; Receptors, Vasopressin; Vasopressins

Diabetes insipidus (DI) is a well documented complication observed after traumatic head injuries. We report a case of hyperacute onset DI in a 19-year-old male who sustained a hypothalamic-pituitary injury when he was stabbed in the head with a 30-cm long thin-bladed knife. At CT, our patient showed significant hemorrhagic contusions of the lower hypothalamus. He developed polydipsia, polyuria, and mild hypernatremia in the Emergency Department. Diagnostic digital subtraction angiography showed a hypervascular congestive pituitary gland with prominent draining veins. On the third day his hypernatremia became severe (183mEq/L). He was managed with parenteral fluids and a regimen of intranasal DDAVP (1-desamino 8-d-arginine vasopressin), leading to improved plasmatic sodium levels, urine output, and urinary specific gravity. In patients presenting with hyperacute posttraumatic DI, emergency room physicians and neurosurgeons should rule out direct injury to the hypothalamus and/or the posterior lobe of the pituitary, and initiate early pharmacological treatment.

Topics: Acute Disease; Brain Injuries; Confusion; Craniocerebral Trauma; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Hypernatremia; Hypoglycemic Agents; Hypothalamo-Hypophyseal System; Hypothalamus; Magnetic Resonance Angiography; Magnetic Resonance Imaging; Male; Polyuria; Tomography, X-Ray Computed; Wounds, Stab; Young Adult

A 45-year-old man was hospitalized because of weight loss, finger tremor, thirst, polydipsia and increased urinary frequency. He was diagnosed with Graves' disease (GD) and central diabetes insipidus (CDI). Magnetic resonance imaging revealed the enlarged posterior pituitary with thickened stalk. Histological examination obtained from biopsy of the pituitary revealed lymphocytic infundibulo-neurohypophysitis. He received treatment with thiamazole (MMI) for GD and desmopressin acetate (DDAVP) for CDI. However, DDAVP administration could be discontinued as GD was gradually improved. This course indicates that not only the recovered renal response to arginine-vasopressin but also the immunomodulative effects of MMI might attribute to the improvement of polyuria.

Topics: Arginine Vasopressin; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Fibrosis; Graves Disease; Humans; Inflammation; Lymphocytes; Male; Methimazole; Middle Aged; Osmolar Concentration; Pituitary Gland, Posterior; Polyuria; Remission Induction; Saline Solution, Hypertonic; Thyroxine; Urine

Essential hypernatremia is very rare in clinical practice and the pathogenesis is unclear. We performed a set of clinical tests to a patient with chronic and sustained hypernatremia as well as absence of thirst in order to investigate the clinical characteristics and make the diagnosis, yet most importantly to analyze the possible pathogenesis and explore a possible therapy regime.. Water deprivation test and acute water intravenous loading test were performed to observe the changes of urinary osmolality, plasma osmolality and plasma sodium. Free water clearance (C(H₂O) was calculated. Osmolality was detected using the method of freezing point depression, and thirst grade using visual analogue scales. Desmopressin acetate (0.05-0.1 mg/d) was administered to the patient in order to observe the therapeutic effects to his disorder.. The patient had sustained hypernatremia over a long period of time, decreased thirst, normal renal function, as well as absence of clinical hypovoluemia. The plasma sodium was 160-190 mmol/L and plasma osmolality was 330-370 mOsm/L without any thirst perception which could not be corrected by water intake. An 18-hour period of water deprivation increased the urinary osmolality from 368 mOsm/L to 420 mOsm/L with plasma osmolality increasing from 362 mOsm/L to 369 mOsm/L and rising further to 857 mOsm/L after an injection of 5 u vasopresin. With the infusion of 1 250 ml 5%-glucose during 2 hours in an acute water loading test setting, plasma osmolality decreased from 350 mOsm/L to 334 mOsm/L associated with a plasma sodium decrease from 164.7 mmol/L to 155 mmol/L urinary osmolality dropped from a maximum of 632 mOsm/L to 135 mOsm/L urinary volume from 0.25 ml/min to 2.33 ml/min and C(H₂O) from -0.18 ml/min to 1.19 ml/min after acute water loading with 1 250 ml glucose dissolved in water. Our results reveal that treatment of the patient with Desmopressin acetate relieved the adypsia, hypernatremia and hyperosmolality effectively.. The patient was considered as suffering from essential hypernatremia which was associated with partial central diabetes insipidus and adypsia. Desmopressin acetate as a common therapeutic agent of central diabetes insipidus proved to be an effective treatment for essential hypernatremia.

Topics: Adolescent; Antidiuretic Agents; Arginine Vasopressin; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Drinking; Humans; Hypernatremia; Male; Osmolar Concentration; Severity of Illness Index; Sodium; Treatment Outcome; Water Deprivation

Topics: Animals; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Horse Diseases; Horses; Male; Ophthalmic Solutions; Treatment Outcome

We report the clinical course of 2 patients with central diabetes insipidus and evolving to panyhypopituitarism which prompted the diagnosis of an isolated pituitary stalk thickening (PST). In both patients, all etiological investigations were normal and the first biopsy revealed an isolated lymphocytic infiltrate with no sign of malignancy. Close clinical follow-up accompanied by serial brain MRIs was proposed to determine a precise diagnosis and for early detection and treatment of neoplastic disease. In our first case, the diagnosis of germinoma was made 9 months after the PST diagnosis owing to tumor progression. In the second case, the time course was even longer with the diagnosis of germinoma 6 years following initial presentation. In these cases, it is speculated that the lymphocytic infiltrates represent the first sign of a host reaction to an occult germinoma. To our knowledge, this is the third case reported of lymphocytic infiltrates preceding a germinoma in a prepubertal girl, and the only case reported in a prepubertal boy. These cases underline the difficulties in establishing the diagnosis of germinoma in a patient with isolated PST.

Topics: Child; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Female; Germinoma; Human Growth Hormone; Humans; Hypopituitarism; Lymphocytes; Magnetic Resonance Imaging; Male; Pituitary Gland; Pituitary Gland, Anterior; Thyroxine

A 31-year-old man was sent to hospital for urgent treatment. He was in the terminal state of chronic renal failure, and was placed under hemodialysis immediately. Proteinuria and hypertension had been notified since adolescence, had been left untreated, and there was no record of his conditions, was. Living kidney transplantation was conducted 8 months later. The donor was his father. After the operation, rejection was not recognized, but urine volume per day was not reduced and maintained the level around 10.000 ml. At the same time, the decrease of body weight and the rise in the serum creatinine concentration were noted. The results of magnetic resonance imaging and the hypertonic saline test (Hickey Hare Test) have formed diagnosis of incomplete diabetes insipidus. Immediately after the administration of desmopressin (rhinenchysis), the decrease of urine volume was recognized, and the body weight and serum creatinine concentration became stable.

Topics: Administration, Intranasal; Adult; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Kidney Transplantation; Living Donors; Magnetic Resonance Imaging; Male; Postoperative Complications; Saline Solution, Hypertonic; Treatment Outcome

A 1-year-old neutered male domestic shorthair cat presented with a 4-week history of polydipsia that began immediately after an 8 m fall. Trauma-induced central diabetes insipidus was suspected on the basis of the identification of hyposthenuria, normal haematology and serum biochemistry profile and unremarkable abdominal ultrasound examination. Failure to concentrate urine with water deprivation followed by production of hypersthenuric urine with administration of the synthetic antidiuretic hormone, Damino-8-D-arginine vasopressin (DDAVP), confirmed the diagnosis of central diabetes insipidus. Treatment via conjunctival administration of DDAVP failed to attenuate the polydipsia, however, resolution of polydipsia was achieved with subcutaneous administration of DDAVP and the cat remains eudipsic with twice daily subcutaneous DDAVP administration 17 months after diagnosis.

Topics: Accidental Falls; Animals; Antidiuretic Agents; Cat Diseases; Cats; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Injections, Subcutaneous; Male

Multiple Sclerosis (ME) is a chronic progressive disease characterized by relapses of demyelination that can occur anywhere in the brain stem, spinal cord and optic nerve. Since central diabetes insipidus (DI) is mainly caused by central nervous system damage (such as trauma, surgery, tumor, infection, sarcoidosis), ME is included among its possible etiologies. However, this association is not commonly described. The clinical suspicion must be made in the presence of polyuria and polydipsia or refractory hypernatremia (in patients without free access to water) during the evolution of ME. We will describe a clinical report in which this association occurred and, after the beginning of desmopressin therapy, the clinical findings were reverted.

Topics: Adult; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Female; Humans; Magnetic Resonance Spectroscopy; Multiple Sclerosis; Polyuria

In Taiwan, Solanum indicum L. has been used in folk medicine for the treatment of inflammation, toothache, ascites, edema, and wound infection. The plant is rich in solanine, an alkaloidal glycoside. We report a 43-year-old man who developed polyuria and polydipsia after taking seven doses of concentrated solution of Solanum indicum L. over two weeks. A water deprivation test and a low serum antidiuretic hormone level helped to confirm a diagnosis of central diabetes insipidus. We suggest that excessive doses of Solanum indicum L. may cause central diabetes insipidus.

Topics: Adult; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Drugs, Chinese Herbal; Humans; Male; Plant Extracts; Polyuria; Solanaceous Alkaloids; Solanine; Solanum; Taiwan; Thirst

It is known that arginine vasopressin (AVP) has a stimulatory effect on corticotropin (adrenocorticotropic hormone; ACTH) and cortisol secretion especially during stress. The present study was designed to investigate the effect of stress on ACTH and cortisol levels in patients with central diabetes insipidus (DI) with endogenous AVP deficiency receiving AVP therapy, and to determine whether these children need steroid replacement during stress.. Seven patients with a median age of 12 years (range 7-13 years) with idiopathic central DI on appropriate Desmopressin (DDAVP) therapy (group 1) and seven healthy controls with a median age of 15 years (range 13-20 years; group 2) were included in the study. Acute stress was produced in all children by treadmill exercise, assessed by maximal oxygen consumption and heart rate. ACTH and cortisol levels were determined before and after exercise.. In group 1, median ACTH level after exercise (28.3 pg/mL) was not different from the median value (24.0 pg/mL) before exercise. However, median cortisol level (10.5 microg/dL) was significantly increased (14.9 microg/dL; P < 0.05) with exercise. In group 2, cortisol (median 9.3 microg/dL) and ACTH levels (median 6.3 pg/mL) were significantly increased after exercise (15 mug/dL and 13.6 pg/mL, respectively; P < 0.05). There was no significant difference between the groups with respect to cortisol levels before and after exercise, but the stimulated ACTH levels after exercise were higher in patients with DI than in the controls (P < 0.05). A positive correlation was observed between total daily DDAVP dose and cortisol level after exercise (r(s)= 0.786, P < 0.05).. Cortisol response during acute stress is normal in children with DI and these patients do not need extra steroid treatment during stress. In contrast, the normal cortisol response obtained by increased ACTH levels in these patients indicates an increased sensitivity of corticotroph cells.

Topics: Adolescent; Adrenocorticotropic Hormone; Antidiuretic Agents; Child; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Exercise; Exercise Test; Female; Humans; Hydrocortisone; Male

Topics: Carcinoma, Mucoepidermoid; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Hypothalamic Neoplasms; Lung Neoplasms; Magnetic Resonance Imaging; Male; Middle Aged; Parotid Neoplasms; Polyuria; Thirst

Lymphocytic hypophysitis is an unusual inflammatory lesion that is caused by autoimmune destruction of the pituitary gland. We report a case of 42-year-old man who presented with a 6-month history of severe headache, blurred vision in the right eye, hearing loss, polyuria, polydipsia, and impotence. Medical history showed that he and his mother had osteopetrosis. The results of the physical examination and laboratory tests showed that secondary hypothyroidism, hypogonadism, and hypocortisolism had developed. Central diabetes insipidus was diagnosed by water deprivation test. MRI of the sella showed pituitary enlargement with symmetrical suprasellar expansion, compression of the chiasma, thickened infundibulum, and involvement of both bilateral cavernous sinuses and clivus. Hormonal substitution with hydrocortisone, levothyroxine, and DDAVP resulted in rapid improvement of all symptoms and signs. Transsphenoidal biopsy was diagnostic of lymphocytic hypophysitis. In spite of extensive literature reviewing, we have not been aware of any case of lymphocytic hypophysitis with clivus involvement. The present case represents a variant of lymphocytic hypophysitis which has progressed to involve bilateral cavernous sinuses and the clivus.

Topics: Adult; Autoimmune Diseases; Cavernous Sinus; Cranial Fossa, Posterior; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Hormone Replacement Therapy; Humans; Hydrocortisone; Hypopituitarism; Lymphocytosis; Male; Thyroxine

A 15-year-old girl was admitted to our hospital because of polydipsia, polyuria, bilateral hilar lymphadenopathy and uveitis. A diagnosis of sarcoidosis with central diabetes insipidus was made by radiological, serological, bronchoalveolar lavage examinations, fluid restriction test and vasopression test. Prednisolone therapy improved all of her clinical findings except diabetes insipidus. So she had to continue intranasal 1-desamino-8-arginine vasopressin (DDAVP) therapy. In addition, we reviewed the clinical features of 27 patients of sarcoidosis with diabetes insipidus reported in Japan. They included 12 patients in young men and 21 patients having uveitis. These patients showed low frequency of lung complications in comparison with sarcoidosis without diabetes insipidus. Steroid therapy improved the symptoms of diabetes insipidus in only 3 patients, and all these 3 patients started steroid therapy within 1 month after the onset. Therefore we think that early diagnosis and treatment are important. Though central neurosarcoidosis was generally considered to have poor prognosis, there were only 3 patients who had recurrence by steroid tapering.

Topics: Adolescent; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Lymphatic Diseases; Sarcoidosis

We here report a 77-year-old Japanese male who suffered general fatigue with progressive thirst and polyuria. Central diabetes insipidus was diagnosed by depletion of vasopressin secretion in response to increases in serum osmolality. Secretory responses of anterior pituitary hormones including adrenocorticotropin, thyrotropin, gonadotropins and growth hormone were severely impaired. Diffuse swelling of the infundibulum as well as lack of T1-hyperintense signal in the posterior lobe was noted by pituitary magnetic resonance imaging. The presence of bilateral hilar lymphadenopathy and increased CD4/CD8 ratio in bronchoalveolar lavage fluid was diagnostic for lung sarcoidosis. Physiological doses of corticosteroid and thyroid hormone were administered in addition to desmopressin supplementation. Complete regression of the neurohypophysial swelling was notable two years after corticosteroid replacement. Diffuse damage of anterior pituitary combined with hypothalamic involvement leading to central diabetes insipidus is a rare manifestation in such elderly patients with neurosarcoidosis.

Topics: Aged; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Hormone Replacement Therapy; Humans; Hydrocortisone; Hypopituitarism; Male; Sarcoidosis, Pulmonary; Thyroxine

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Breast Neoplasms; Carcinoma, Ductal, Breast; Combined Modality Therapy; Cortisone; Cyclophosphamide; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Fluorouracil; Humans; Hypopituitarism; Mastectomy, Modified Radical; Methotrexate; Pituitary Neoplasms; Radiotherapy, Conformal; Tamoxifen; Thyroxine

To report a case of neurosarcoidosis with rapid progression of visual field defects.. A 28-year-old woman presented with bilateral uveitis and was diagnosed as having sarcoidosis after skin and cervical lymph node biopsy. Since bilateral excavations of the optic nerve head and visual field defects were observed, endocranial lesion was suspected. However, a computed tomography (CT) scan of the head detected nothing abnormal. It was regarded as a case of sarcoidosisaccompanied by normal-tension glaucoma and treatment was initiated with latanoprost. Four months later, the patient's visual field deteriorated rapidly. A CT scan showed a pituitary mass. Neurologicalfindings and hypopituitarism were found which improved with systemic prednisolone therapy. Diabetes insipidus developed after the start of treatment, and was treated with intranasal desmopressin therapy. After 6 weeks, head magnetic resonance imaging (MRI) showed a remarkable reduction of the enhanced regions.. Although ocular sarcoidosis is often accompanied by secondary glaucoma or optic nerve atrophy, the progression of neurosarcoidosis can lead to visual field defects. Central nervous system (CNS) sarcoidosis is rare, but a precise examination with enhanced MRI should be considered when the visual field defect progresses rapidly.

Topics: Adult; Central Nervous System Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Disease Progression; Female; Humans; Magnetic Resonance Imaging; Prednisolone; Sarcoidosis; Tomography, X-Ray Computed; Treatment Outcome; Uveitis; Vision Disorders; Visual Fields

Central diabetes insipidus (DI) is extremely rare during the neonatal period. Most cases of central DI are secondary to a known aetiology. Substitutive treatment with desmopressin is effective with nasal or oral preparation, but doses are variable and must be tailored individually. We report on a case in a very low birth weight infant with an idiopathic central DI during the first month of life. He was successfully treated with oral desmopressin. The treatment was maintained after discharge with low doses desmospressin.

Topics: Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Infant, Newborn; Infant, Premature; Infant, Very Low Birth Weight; Male

Topics: Adult; Antidiuretic Agents; Blood Urea Nitrogen; Deamino Arginine Vasopressin; Dehydration; Diabetes Insipidus, Neurogenic; Humans; Kidney Function Tests; Male; Treatment Outcome; Urea

Tumor metastasis to the pituitary gland has been infrequently reported, and this is probably because only a small proportion of these patients are symptomatic. Most of the symptoms of this malady are related to diabetes insipidus. A 78-year-old man was diagnosed 2 years previously with stage IIIA adenocarcinoma of the lung and treated with sequential chemoradiation therapy and later with whole-brain radiation therapy because of newly developed brain metastasis; he was then admitted to our hospital with symptoms of polydipsia and polyuria. He was confirmed to have central diabetes insipidus that was caused by the pituitary metastasis from lung cancer. His symptoms resolved after treatment with desmopressin. Because of the rarity of this manifestation in lung cancer patients, we report on this case along with a brief review of the relevant literature.

Topics: Aged; Antidiuretic Agents; Brain Neoplasms; Carcinoma, Non-Small-Cell Lung; Combined Modality Therapy; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Lung Neoplasms; Male; Pituitary Neoplasms; Polyuria; Radiography; Thirst

Topics: Aged, 80 and over; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Hypopituitarism; Hypothalamus; Immunoenzyme Techniques; Magnetic Resonance Imaging; Male; Neurocysticercosis; Pituitary Gland; Polyuria; Thailand; Water Deprivation

Topics: Administration, Intranasal; Calcinosis; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Hemostatics; Humans; Hydrocephalus; Infant, Newborn; Toxoplasmosis, Congenital

Traditional methods for treating central diabetes insipidus during infancy, such as fluid therapy or the use of intranasal hormone replacement, have significant potential limitations. In a retrospective study of infants with diabetes insipidus, we examined outcome using subcutaneous (sc) DDAVP, and compared this to infants treated with intranasal lysine vasopressin or DDAVP. After in-patient dosage titration, outpatients' serum sodium concentrations were maintained in a narrower range in the sc group compared with the intranasal group, and the percentage of serum sodium concentrations within the normal range was greater in the sc group. There were no significant complications in either group. We conclude that DDAVP administered subcutaneously can be a safe and effective alternative to traditionally recommended treatments of central diabetes insipidus during infancy.

Topics: Administration, Intranasal; Antidiuretic Agents; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Follow-Up Studies; Humans; Infant; Injections, Subcutaneous; Lypressin; Male; Retrospective Studies

We describe a child who has congenital nasal pyriform aperture stenosis with single maxillary central incisor, holoprosencephaly and central diabetes insipidus without any apparent anterior pituitary dysfunction. Conservative management of the congenital nasal pyriform aperture stenosis is adopted and management of diabetes insipidus is described. A literature review is undertaken.

Topics: Abnormalities, Multiple; Bronchoscopy; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Enteral Nutrition; Female; Holoprosencephaly; Humans; Infant, Newborn; Intubation, Intratracheal; Magnetic Resonance Imaging; Nasal Cavity; Nasal Obstruction; Renal Agents; Tomography, X-Ray Computed; Vasopressins

Prostaglandins have an important role in renal salt and water reabsorption. PGE2 is the main kidney prostaglandin and is thought to be mainly produced in the kidney inner medulla (IM). There are indications that PGE2 synthesis in nephrogenic (NDI) and central (CDI) diabetes insipidus is altered. We hypothesize that the expression of the major PGE2 synthesis enzymes cyclooxygenases 1 and 2 (COX-1, COX-2) and membrane-associated PGE2 synthase (mPGES) is altered in the kidneys of rats with NDI and CDI. Wistar rats treated with lithium for 4 wk were used as the NDI model. One-half of the NDI model rats were additionally dehydrated for 48 h. Brattleboro (BB) rats that lack endogenous antidiuretic hormone were used as the CDI model. Expression and localization of COX-1, COX-2, and mPGES in IM, inner stripe of outer medulla (ISOM), and cortex were determined by immunoblotting and immunohistochemistry. In lithium-induced NDI, expression of COX-1, COX-2, and mPGES was markedly decreased in IM. In ISOM and cortex, COX-1 expression was marginally reduced and mPGES expression was unaltered. COX-2 expression was undetected in ISOM and marginally increased in cortex. Consistent with this, the density of COX-2-expressing cells in macula densa was significantly increased, indicating differential regulation of COX-2 in IM and cortex. Dehydration of NDI rats resulted in a marked increase in COX-2 immunolabeling in IM interstitial cells, and there was no significant change in COX-1 and mPGES expression in any kidney zone. Treatment of DDAVP in BB rats for 6 days resulted in a markedly increased expression of COX-1, COX-2, and mPGES in IM. In the cortex, there were no changes in the expression of COX-1 and mPGES, whereas COX-2 expression was decreased. These results identify markedly reduced expression of COX-1, COX-2, and mPGES in IM in lithium-induced NDI. Furthermore, there were major changes in the expression of COX-1, COX-2, and mPGES in rats with CDI.

Topics: Animals; Cyclooxygenase 1; Cyclooxygenase 2; Deamino Arginine Vasopressin; Dehydration; Diabetes Insipidus, Nephrogenic; Diabetes Insipidus, Neurogenic; Dinoprostone; Intramolecular Oxidoreductases; Kidney Concentrating Ability; Kidney Cortex; Kidney Medulla; Lithium; Male; Membrane Proteins; Polyuria; Prostaglandin-Endoperoxide Synthases; Rats; Rats, Brattleboro; Rats, Wistar; Renal Agents; Vasopressins; Water Deprivation

Five cases of central diabetes insipidus (CDI) in domestic shorthair cats are described. All cats were under 3 years of age at the onset of clinical signs, and outdoor or outdoor/indoor cats, in which a prior trauma was either present or possible. The history included polydipsia and polyuria, and physical examination abnormalities included urinary bladder distention and dehydration. All cats had hyposthenuria with a urine specific gravity between 1.003 and 1.006. The diagnosis was confirmed by an observed inability to concentrate urine during a water deprivation test or compatible serum osmolality, followed by an increase in urine concentration after desmopressin administration. All cats in this report were treated successfully with oral desmopressin. The dose (25-50 microg q8-12h) and the response to therapy were variable. Oral desmopressin administration may serve as an effective alternative route for cat owners who find the conjunctival or nasal application of the solution an inconvenient mode of therapy.

Topics: Administration, Oral; Animals; Cat Diseases; Cats; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Male; Renal Agents; Treatment Outcome

Two young female patients presented with polyuria and polydipsia. In one patient we additionally found idiopathic vitiligo, there were no relevant previous diseases. The gynaecological history was unremarkable.. In both cases a water deprivation test confirmed the diagnosis of central diabetes insipidus, the MRI investigation of the pituitary region showed a prominent and thickened pituitary stalk.. After exclusion of a systemic granulomatous inflammation we diagnosed an autoimmune hypophysitis based on the typical morphological lesions of the pituitary gland and stalk. TREATMENT AND FOLLOW-UP: High-dose glucocorticoid therapy was without any beneficial effect on the central diabetes insipidus. Desmopressin treatment was initiated and led to a normalization of the pre-existing polyuria and polydipsia.. Autoimmune hypophysitis is a very rare disease and the diagnosis is mostly achieved by excluding other causes. Systematic evaluations on large patient cohorts are lacking in the literature with respect to diagnostic procedures, therapy and outcome, the existing knowledge and experience is largely based on case reports. For this reason it appears desirable to create a central register to collect and to evaluate the course of disease in patients with autoimmune hypophysitis.

Topics: Adult; Antidiuretic Agents; Autoimmune Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnosis, Differential; Female; Humans; Inflammation; Magnetic Resonance Imaging; Pituitary Diseases; Pituitary Gland; Polyuria; Thirst

A 1-year-old neutered male domestic shorthair cat presented with a 4-week history of polydipsia that began immediately after an 8 metre fall. Trauma-induced central diabetes insipidus was suspected on the basis of the identification of hyposthenuria, normal haematology and serum biochemistry profile and unremarkable abdominal ultrasound examination. Failure to concentrate urine with water deprivation followed by production of hypersthenuric urine with administration of the synthetic antidiuretic hormone, Deamino-8-D-arginine vasopressin (DDAVP), confirmed the diagnosis of central diabetes insipidus. Treatment via conjunctival administration of DDAVP failed to attenuate the polydipsia, however, resolution of polydipsia was achieved with subcutaneous administration of DDAVP and the cat remains eudipsic with twice daily subcutaneous DDAVP administration 17 months after diagnosis.

Topics: Accidental Falls; Animals; Antidiuretic Agents; Cat Diseases; Cats; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Injections, Subcutaneous; Male

A 71-year-old man was admitted with high fever, thirst, polyposia and polyuria. After examination, lung cancer (adenocarcinoma T1NOM1, Stage IV) and central diabitus insipidus caused by pituitary metastasis of lung cancer, were diagnosed. We gave him desmopressin acetate, gamma knife surgery for pituitary metastasis and chemotherapy with paclitaxel and carboplatin, and his symptoms improved. However, his lung cancer progressed. Diabitus insipidus caused by lung cancer is rare.

Topics: Adenocarcinoma; Aged; Antidiuretic Agents; Antineoplastic Combined Chemotherapy Protocols; Carboplatin; Combined Modality Therapy; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Docetaxel; Humans; Lung Neoplasms; Male; Paclitaxel; Pituitary Neoplasms; Radiosurgery; Taxoids

Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Hydronephrosis; Male; Polyuria; Renal Agents; Treatment Outcome

We report a 42-year-old woman with severe motor and intellectual disabilities (SMID) who showed partial central diabetes insipidus during severe pneumonia. Serum sodium levels were previously within the upper normal range from 140 to 147 mEq/L. During pneumonia, however, serum sodium rose rapidly to reach 185 mEq/L. The daily urinary volume exceeded the daily intake of water. Nasal administration of 1-desamino-8-D-arginine vasopressin (DDAVP) reduced the daily urinary volume and the serum sodium level to normal ranges. Consequently, we diagnosed her as having central diabetes insipidus (DI). She required a smaller dose of DDAVP (2.5 microg/day) than usual DI (5-15 microg/day) to maintain normal urinary volume and the serum sodium level for seven months. After the nasal administration of DDAVP was discontinued, the serum sodium levels again returned to within the upper normal range. A water deprivation study demonstrated poor elevation of both plasma antidiuretic hormone (ADH) level (range, 0.5-2.0 pg/ml) and urine osmolarity (peak level, 552 mOsm/kgH2O) despite the elevation of plasma osomolarity, suggesting latent partial central DI. Water balance and serum electrolyte levels should be closely monitored in cases of SMID.

Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Hypernatremia; Hypothalamic Diseases; Persons with Mental Disabilities; Pituitary Diseases; Pneumonia; Severity of Illness Index; Water-Electrolyte Balance

Most mutations of the arginine vasopressin-neurophysin II (AVP-NPII) gene cause autosomal dominant familial neurohypophyseal diabetes insipidus (adFNDI). Such mutations are predicted to alter the three-dimensional structure of the prohormone, which accumulates in the cell body, ultimately leading to neuronal degeneration and hormonal deficit. In this study we describe the case of a 26-year-old female reporting a long-lasting history of polyuria/polydipsia. The father of the patient was affected by diabetes insipidus and was under desmopressin treatment until the time of his death. Nevertheless, the patient had never been subjected to endocrine evaluation.. Clinical and genetic studies were performed. An 8-h fluid deprivation test plus desmopressin challenge and a 5% saline solution test were performed, in order to confirm the diagnosis. DNA was extracted from peripheral blood lymphocytes and subjected to direct sequencing of the entire coding region of the AVP-NPII gene.. Clinical assessment of the patient confirmed the diagnosis of neurohypophyseal diabetes insipidus. Desmopressin treatment was started, which effectively reversed the polyuria/ polydipsia syndrome. Genetic analysis revealed a novel mutation (1665T>A) in exon 2 of the AVP-NPII gene, disrupting one of the disulfide bonds present in the NPII moiety which play a fundamental role in determining the proper folding of the molecule. In summary, in the present study we have described a novel mutation of the AVP-NPII gene, which is consistent with the malfolding/toxicity hypothesis underlying the pathogenesis of adFNDI.

Topics: Adult; Arginine Vasopressin; Base Sequence; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Drinking Behavior; Female; Humans; Molecular Structure; Mutation; Neurophysins; Oxytocin; Pedigree; Polyuria; Protein Precursors; Renal Agents

We describe a novel missense mutant of arginine vasopressin (AVP)-dependent neurohypophyseal diabetes insipidus in an autosomal dominant family. A 54-year-old woman was admitted to our hospital because of thyroidectomy for thyroid cancer. After thyroidectomy she was found to have hypernatremia and polyuria and polydipsia both of which had been present from childhood. She had no obstructive hydronephrosis. Her father, father's younger sister and her third son also had polyuria and polydipsia. Basal plasma AVP concentration at normal plasma osmolality was normal but did not respond to increased plasma osmolality despite hyperosmolality during infusion of hypertonic saline infusion, indicating that plasma AVP secretion was impaired. Sodium concentration in urine and urine osmolality were low and increased after nasal administration of DDAVP. There was a diminished but bright signal of pituitary posterior gland on magnetic resonance T1 weighted image. Molecular genetic analysis demonstrated that the patient and her son had a single heterozygous missense mutation (G-->A) at nucleotide 1829 in 1 AVP allele, yielding an abnormal AVP precursor with lacking Glu-47 in its neurophysin II moiety. The abnormal AVP precursor may be related to the impaired AVP secretion.

Topics: Adult; Base Sequence; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Hyponatremia; Male; Middle Aged; Molecular Sequence Data; Mutation, Missense; Neurophysins; Pedigree; Polyuria; Protein Precursors; Sequence Analysis, DNA; Vasopressins

Topics: Administration, Intranasal; Adrenal Insufficiency; Aged; Aged, 80 and over; Deamino Arginine Vasopressin; Dermatitis, Exfoliative; Diabetes Insipidus, Neurogenic; Humans; Hydrocortisone; Hypopituitarism; Male; Prednisolone; Treatment Outcome

Vasculitis with central diabetes insipidus is a rare condition which must be recognized for an appropriate management. We report a case of Behçet disease with central diabetes insipidus. A forty seven year old men presented recurring oral and genital ulcers, skin lesions, polyarthralgia. Two years later, he showed right uveitis and central diabetes insipidus without dysfunction of the pituitary gland. Computed tomographic scan showed normal neurohypophysis and pituitary gland. Later on, he presented hemiplegia. The cerebral computed tomographic scan showed hypodense lesions. Central diabetes insipidus should not be systematically searched because it is a rare feature of the Behçet disease.

Topics: Behcet Syndrome; Brain Ischemia; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Hemiplegia; Humans; Magnetic Resonance Imaging; Male; Middle Aged

Neuroendocrine dysfunctions are among the various complications that occur after traumatic brain injury. We report a case of onset of diabetes insipidus during acute rehabilitation of a 20-yr-old patient with a traumatic brain injury caused by a gunshot wound. Our case is the latest onset of diabetes insipidus after traumatic brain injury that has been reported in the literature.

Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Head Injuries, Penetrating; Humans; Hypopituitarism; Magnetic Resonance Imaging; Male; Osmolar Concentration; Pituitary-Adrenal Function Tests; Sodium; Specific Gravity; Time Factors; Water Deprivation; Wounds, Gunshot

A 10-year-old male referred to our clinic with the chief complaint of nocturnal enuresis also complained of daytime polyuria, frequency, and polydipsia. The clinical diagnosis was central diabetes insipidus. Since the patient's father had complained of similar symptoms, the arginine vasopressin-neurophysin II gene was examined. This revealed a single base substitution in one of two alleles in the patient, his father, and his grandfather (a C to T transition at nucleotide position 280 at codon 19 in the first exon). In conclusion, a history of polyuria or polydipsia should be carefully noted and the urinary volume and urine gravity or osmolarity examined in cases of nocturnal enuresis.

Topics: Arginine Vasopressin; Brain; Child; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; DNA; Enuresis; Humans; Magnetic Resonance Imaging; Male; Neurophysins; Pedigree; Reverse Transcriptase Polymerase Chain Reaction; Saline Solution, Hypertonic

A 41-year-old woman who had undergone transfrontal craniotomy for a pituitary tumor 4 months before presentation was admitted with confusion and orientation only to self. She had a fever of 40 degrees C. Serum sodium and chloride levels on admission were 180 and 139 mEq/L, respectively. Measured serum osmolality was 380 mOsmol/L with a urine osmolality of 360 mOsmol/L. Magnetic resonance imaging revealed a 1.5-cm mass in the sella turcica, which was nonfunctioning on endocrine evaluation. The "bright spot" of a normal posterior pituitary was absent. Central diabetes insipidus was confirmed by a 300% increase in urine osmolality with desmopressin. The patient survived her severe hypernatremia, which has 70% mortality with a serum sodium level of 160 mEq/L or above. However, she developed permanent (6 months) disorientation to time and place even when hypernatremia was corrected, which has not been described previously.

Topics: Adult; Confusion; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Hypernatremia; Magnetic Resonance Imaging; Pituitary Gland; Pituitary Neoplasms; Renal Agents; Sella Turcica

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Brain; Chlorambucil; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Humans; Hypothalamus; Male; Mitoxantrone; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Prednisolone; Remission Induction; Renal Agents; Tomography, X-Ray Computed

Central diabetes insipidus is clinically masked in dialysis patients. We report a 12-year-old girl receiving a living-related donor graft for renal failure from Alport syndrome, in whom a craniopharyngioma had been resected 6 months before transplantation. Pretransplant evaluation had documented central hypothyroidism, growth hormone deficiency, and presumptive hypogonadotropic hypogonadism. The corticotropin-releasing factor test had been normal. Four hours after transplantation, urine output exceeded 1,000 ml/h without diuretic therapy. Serum sodium concentration was 155 mmol/l, serum osmolality 333 mmol/kg, and plasma antidiuretic hormone 4.9 ng/l, while urine osmolality was 233 mmol/kg. Desmopressin acetate was started by continuous intravenous infusion at 1 microgram/day. Serum electrolytes rapidly normalized, urine output stabilized at 2 l/day. The patient was discharged 4 weeks after transplantation with good allograft function, receiving intranasal desmopressin acetate 10 micrograms twice daily. Pre-existing central diabetes insipidus is unmasked after successful kidney transplantation, leading to rapid dehydration and hypernatremia, which can be prevented by prompt institution of desmopressin therapy.

Topics: Child; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Kidney Failure, Chronic; Kidney Transplantation; Living Donors; Magnetic Resonance Imaging; Pituitary Neoplasms; Renal Agents

Case report.. To present a case of central diabetes insipidus (CDI) that developed after a gunshot injury to the thorax and thoracic spinal cord and to discuss the disease process in light of the relevant literature.. Antidiuretic hormone (ADH) abnormalities may develop after spinal trauma and/or surgery. Although there are published reports of inappropriate ADH syndrome arising in this clinical picture, CDI is rare.. A 33-year-old woman with hemopneumothorax and a gunshot wound to her thoracic spine was treated with chest tube drainage. No surgery was performed for the spinal injury. The patient was paraplegic on admission and rapidly developed excessive urine output. Testing revealed that her serum ADH level was low, consistent with CDI. Desmopressin acetate nasal spray was the prescribed treatment.. The patient responded well to the desmopressin acetate spray.. CDI is a complicated hormonal disorder characterized by excessive urine output. It is typically linked to an abnormality in the hypothalamohypophyseal axis that markedly reduces ADH production. The most common inciting causes are craniocerebral trauma, brain tumor and/or surgery, and central nervous system infection. Although uncommon, CDI should be considered when a spinal trauma patient develops excessive urine output.

Topics: Administration, Intranasal; Adult; Atrophy; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Hemostatics; Humans; Magnetic Resonance Imaging; Spinal Cord; Spinal Cord Injuries; Thoracic Injuries; Thoracic Vertebrae; Thorax; Treatment Outcome; Wounds, Gunshot

Topics: Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Drinking Behavior; Humans; Magnetic Resonance Imaging; Osmolar Concentration; Prognosis; Renal Agents

We report the cases of two children with cranial diabetes insipidus who were treated with lamotrigine for seizures and who had accompanying changes in desmopressin requirements. Lamotrigine is a new anticonvulsant chemically unrelated to other existing antiepileptic drugs. Studies suggest it acts at voltage-sensitive sodium channels and also decreases calcium conductance. Both of these mechanisms of action are shared by carbamazepine, which can cause hyponatraemia secondary to inappropriate secretion of antidiuretic hormone. It is possible that the effect of lamotrigine on fluid balance in the cases described is also centrally mediated.

Topics: Adolescent; Anticonvulsants; Child; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Dose-Response Relationship, Drug; Female; Humans; Hyponatremia; Hypopituitarism; Lamotrigine; Seizures; Triazines

Topics: Antineoplastic Combined Chemotherapy Protocols; Carboplatin; Child; Craniotomy; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Etoposide; Female; Germinoma; Humans; Hypopituitarism; Pituitary Gland, Anterior; Pituitary Neoplasms; Renal Agents