deamino-arginine-vasopressin and Pituitary-Diseases

Alcohol-induced hypercortisolism (AIH) is underrecognized and may masquerade as neoplastic hypercortisolism [Cushing syndrome (CS)] obscuring its diagnosis.. In order to characterize AIH, we performed a chart review of eight patients (4 males and 4 females; 2014-2022) referred for evaluation and treatment of neoplastic hypercortisolism - six for inferior petrosal sinus sampling, one due to persistent CS after unilateral adrenalectomy, and one for pituitary surgery for Cushing disease (CD). Five underwent dDAVP stimulation testing.. All eight patients had clinical features of hypercortisolism and plasma ACTH levels within or above the reference interval confirming hypothalamic-pituitary mediation. All had abnormal low-dose dexamethasone suppression test and increased late-night salivary cortisol. Only one had increased urine cortisol excretion. In contrast to CD, the 5 patients tested had blunted or absent ACTH and cortisol responses to desmopressin. Two had adrenal nodules and one had abnormal pituitary imaging. Most patients underreported their alcohol consumption and one denied alcohol use. Elevated blood phosphatidyl ethanol (PEth) was required in one patient to confirm excessive alcohol use. All patients had elevations of liver function tests (LFTs) with AST>ALT.. AIH is an under-appreciated, reversible cause of non-neoplastic hypercortisolism that is indistinguishable from neoplastic CS. Incidental pituitary and adrenal imaging abnormalities as well as under-reporting of alcohol consumption further confound the diagnosis. Measurement of PEth helps to confirm an alcohol use disorder. Elevations of LFTs (AST>ALT) and subnormal ACTH and cortisol responses to dDAVP help to distinguish AIH from neoplastic hypercortisolism.

Topics: Adrenocorticotropic Hormone; Cushing Syndrome; Deamino Arginine Vasopressin; Ethanol; Female; Humans; Hydrocortisone; Male; Pituitary ACTH Hypersecretion; Pituitary Diseases

GPA with pituitary involvement is a rare condition which is prone to be misdiagnosed. The aim of this study was to summarize clinical features of pituitary involvement in GPA and facilitate early diagnosis. Twelve GPA patients were retrospectively analyzed at a single hospital between 2000 and 2017. A literature review was conducted to compare previous findings with our clinical results. The incidence rate of pituitary involvement in GPA was 3.9% (12/304) without sexual predilection. Other impairments included ear, nose and throat (n = 12), oculi (n = 10), lung (n = 6), meninges (n = 4), kidney (n = 3), and skin (n = 2). Antineutrophil cytoplasmic antibodies (ANCA) were positive in all patients with lung or kidney involvement (n = 6/6), while ANCA were negative in almost all patients without lung or kidney involvement (n = 5/6). Endocrine abnormalities included central diabetes insipidus (CDI, n = 11/12) hypogonadotropic hypogonadism (n = 6/11), adrenocorticotropic hormone deficiency (n = 4/7), thyroid-stimulating hormone deficiency (n = 5/11), and growth hormone deficiency (n = 3/9). Enlarged pituitary gland (n = 6), absence of posterior hyperintense signal on T1-weighed images (n = 11) and hypertrophic cranial pachymeningitis (n = 4) were common radiological manifestations. After treatment, nine patients experienced remission but one died. Pituitary images of 3/4 patients showed size of pituitary lesions decreased. CDI was not alleviated and hypopituitarism remained in two patients. Pituitary involvement in GPA can occur at any time throughout the course of disease, including at the initial presentation. GPA could not be excluded based on negative-ANCA in patients with pituitary abnormality alone. CDI and hypogonadotropic hypogonadism are dominant endocrine abnormalities. Systemic diseases may alleviate and pituitary images may improve after treatment, though the recovery of pituitary function is rare.

Topics: Adult; Antibodies, Antineutrophil Cytoplasmic; Biomarkers; Biopsy; Deamino Arginine Vasopressin; Female; Granulomatosis with Polyangiitis; Hormone Replacement Therapy; Hormones; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Middle Aged; Pituitary Diseases; Remission Induction; Retrospective Studies; Thyroxine; Tomography, X-Ray Computed; Treatment Outcome; Young Adult

A 20-year-old woman with a history of pituitary resection complicated by diabetes insipidus was able to fully breastfeed for three months despite apparent hyposecretion of pituitary hormones. This case report adds to the growing body of evidence that control of milk production shifts from endocrine to autocrine control shortly after delivery. Autocrine control allows efficient regulation of milk supply to match the needs of the infant. A recently discovered factor in human milk that inhibits lactose and casein synthesis in vitro is believed to be responsible for local, short term control of milk production. This study suggests that practices which result in infrequent or incomplete removal of milk from the breast lead to decreased milk production and should be abandoned.

Topics: Abscess; Adult; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Humans; Infant, Newborn; Lactation; Oxytocin; Patient Education as Topic; Pituitary Diseases; Postoperative Complications; Pregnancy; Prolactin

Neonatal diabetes insipidus (DI) is extremely rare. An efficacious substitutive treatment can be particularly difficult to ensure. We have treated two children with central DI, revealed during the neonatal period, successively with an intranasal preparation of desmopressin at one month of age and with an oral preparation at one and 3 years respectively. We conclude that oral treatment with desmopressin is possible, secure and effective in DI presenting in the first months of life. This new form of administration seems even better for younger children when the difficulties of intranasal administration can be responsible for severe complications. As with the intranasal form, the doses given orally are very variable and individual.

Topics: Administration, Intranasal; Administration, Oral; Animals; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Growth Hormone; Humans; Infant; Infant, Newborn; Infant, Premature; Male; Pituitary Diseases; Pregnancy

Topics: Benzothiadiazines; Chlorpropamide; Deamino Arginine Vasopressin; Diabetes Insipidus; Diuretics; Humans; Hypothalamo-Hypophyseal System; Pituitary Diseases; Postoperative Complications; Prognosis; Prostaglandin Antagonists; Sodium Chloride Symporter Inhibitors; Vasopressins

Cushing's syndrome (CS), when fully expressed, is easily diagnosed. Mild cases, however, may require careful distinction from pseudo-Cushing's states as may occur in depression, alcoholism, polycystic ovary disease and visceral obesity. The aim of the present study is a reappraisal of the diagnostic accuracy of the two tests most commonly used to differentiate CS from pseudo-Cushing's: corticotropin-releasing hormone (CRH) stimulation after low dose dexamethasone administration and desmopressin stimulation.. The study population comprised 32 patients with CS and 23 with pseudo-Cushing's evaluated retrospectively.. Urinary free cortisol (UFC), serum cortisol at midnight and after low dose dexamethasone (1 mg overnight and 2 mg over two days) were measured. Further, patients were tested with dexamethasone + CRH and desmopressin and the diagnostic performances of the two tests were compared in the entire series as well as in patients with mild hypercortisolism only (i.e. UFC < 690 nmol/24 h).. As expected, measurement of UFC, assessment of cortisol rhythmicity and inhibition after 1 mg/2 mg dexamethasone failed to clearly classify patients with pseudo-Cushing's. Administration of CRH following 2-mg dexamethasone achieved 100% sensitivity but 62.5% specificity. Conversely, desmopressin testing correctly classified all but two patients with pseudo-Cushing's (90% specificity) with 81.5% sensitivity. Diagnostic accuracy was comparable in the subgroup with mild hypercortisolism (21 CS, all 23 pseudo-Cushing's patients). Desmopressin offered an incremental diagnostic effectiveness of 35.8/million inhabitants compared with dexamethasone + CRH as a second-line test.. Neither of the two tests guarantees absolute diagnostic accuracy. The specificity of dexamethasone + CRH is less brilliant than previously reported and appears to be inferior to desmopressin stimulation. The greatest diagnostic effectiveness results from the low-dose dexamethasone test combined with the desmopressin test. Skilful use of dynamic testing and balanced clinical judgement are necessary to distinguish between Cushing's syndrome and pseudo-Cushing's.

Topics: Adolescent; Adrenocorticotropic Hormone; Adult; Chi-Square Distribution; Child; Corticotropin-Releasing Hormone; Cushing Syndrome; Deamino Arginine Vasopressin; Dexamethasone; Female; Glucocorticoids; Humans; Hydrocortisone; Male; Middle Aged; Pituitary Diseases; Pituitary Function Tests; Sensitivity and Specificity

The prophylactic efficacy of desmopressin acetate (DDAVP) on diabetes insipidus (DI) after hypophysectomy was investigated in the dog. In the control group, hypernatremia with a plasma level of 155 mEq/l or higher persisted for 12 hr from the 4th to the 16th hour after hypophysectomy, and symptoms of DI developed within five days after surgery. In the DDAVP treatment group, these changes were not observed, showing that administration of DDAVP (4 microg, installation, twice daily) effectively prevented hypernatremia that develops immediately after surgery and DI-like symptoms that persists for about one week after surgery.

Topics: Animals; Deamino Arginine Vasopressin; Diabetes Insipidus; Dog Diseases; Dogs; Female; Hypophysectomy; Male; Pituitary Diseases; Pituitary Gland; Renal Agents; Time Factors

Hypothalamic-pituitary axis dysfunction is a common complication in post-operative craniopharyngioma(CP) patients, and it greatly impacts the long-term quality of life of such patients. To better understand the effects of postoperative hypothalamic-pituitary dysfunction and long-term hormone replacement therapy in patients with childhood CP, we assessed approximately 200 patients with childhood-onset CP postoperatively.. Clinical details of patients with childhood-onset CP who underwent sellar tumor resection in Beijing Children's Hospital and Beijing Tiantan Hospital from 2018 to 2019 were retrieved retrospectively. The participants were followed up to assess the effects of post-operative long-term hormone replacement therapy and assess the tumor recurrence rate.. The median age of admission was 8.1 (1.8, 14.3) years. Headache (45.5%), visual impairment (39.5%), and nausea (33.0%) were the most common clinical manifestations. ACP accounted for 95% of all CP cases. The incidence of central adrenal insufficiency and central hypothyroidism within the first week after surgery was 56.2% and 70.3%, respectively. At the same time 85.5% of the patients required at least one dose of desmopressin to control urine output. Total survival and tumor recurrence rates were 98.6% and 26.1%, respectively, with a median follow-up time of 29.7 (19.0, 40.3) months. During the follow-up period, 28.1% patients met the diagnostic criteria for short stature, while 54.4% fit the criteria for obesity. In addition, 94.4% of the patients were taking at least one kind of hormone substitution, and 74.7% were taking three or more. The prevalence of levothyroxine, glucocorticoid, desmopressin, and growth hormone replacement therapy was 87.3%, 77.5%, 78.9% and 31.0%, respectively. The proportion of patients treated with the substitutive combination of levothyroxine, hydrocortisone, and desmopressin was 54.9%.. This study is a large-sample systematic postoperative endocrine function evaluation of patients with childhood-onset CP. Due to the high prevalence of post-operative hypothalamic-pituitary dysfunction, patients with CP usually require long-term multiple hormone substitution therapy. Individualized management and accurate hormone replacement dosage for postoperative childhood-onset CP patients are of great importance.

Topics: Child; Craniopharyngioma; Deamino Arginine Vasopressin; Hormone Replacement Therapy; Humans; Hydrocortisone; Hypothalamic Diseases; Neoplasm Recurrence, Local; Pituitary Diseases; Pituitary Neoplasms; Quality of Life; Retrospective Studies; Thyroxine

Endocrinopathy can occur as a postoperative sequel in children treated for supratentorial tumors (STTs). We assessed prediction of a residual hypothalamic/pituitary insufficiency (HPI) in these patients and factors associated with prolonged length of hospital stay (LOS).. This is a retrospective cohort study of children who had surgery for STTs in two tertiary centers in Saudi Arabia (2009-2019). We assessed PICU postoperative management and risk of HPI. Data were analyzed using SPSS V24.0 and a logistic regression model for a prediction of a prolonged LOS.. Data included 55 children (1-18 years, mean 9.5 ± 4.9 years) who required STT surgeries, 32 (54%) females. Craniopharyngioma (27.3%) was the commonest STTs and 20% of patients had initial symptoms of HPI. PICU management included the use of different types of intravenous fluids (IVFs) and medications such as steroids and desmopressin (DDAVP). An early postoperative DI was reported in 21.8% (n=12/55). Residual HPI included 24 (43.6%) presumed cortisol deficient and 18 (32.7%) central DI patients. Risk factors for postoperative HPI were female gender, age <6 years, headache and preoperative pituitary symptoms. LOS (Median=25.5 ± 12.2 days) was significantly prolonged in patients who required two or more doses of DDAVP [B=13; 95% CI= (1.7-24.3) days] and reduced in patients who had suspected preoperative HPI [B=-19.6; 95% CI= (-31.1, -8.2) days].. Prediction of postoperative HPI in pediatric STTs enhances an early initiation of treatment in PICU and reduces LOS. A meticulous use of IVF and medications supervised by a multidisciplinary team is essential for a favorable outcome.

Topics: Child, Preschool; Critical Care; Deamino Arginine Vasopressin; Female; Humans; Length of Stay; Male; Pituitary Diseases; Pituitary Neoplasms; Retrospective Studies; Supratentorial Neoplasms

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

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

A 29-year-old man was referred to a multidisciplinary pituitary clinic with a 3.5-year history of central diabetes insipidus, initially presumed to be idiopathic based on a normal MRI scan of the pituitary gland. Subsequent scanning revealed a suprasellar mass, which demonstrated progressive enlargement on serial imaging. He also developed hypogonadotropic hypogonadism.. Measurement of levels of serum morning fasting cortisol, adrenocorticotropic hormone, total testosterone, luteinizing hormone, follicle-stimulating hormone, prolactin, insulin-like growth factor 1, TSH and free T(4), MRI of the pituitary gland and a transsphenoidal biopsy of a pituitary mass were performed.. Lymphocytic hypophysitis presenting with diabetes insipidus, with development of hypogonadotropic hypogonadism and a suprasellar mass.. The patient was treated with intranasal desmopressin and transdermal testosterone. The underlying lymphocytic hypophysitis was initially managed conservatively with serial MRI and visual field testing. No immunosuppressant medication was given and, aside from the diagnostic transsphenoidal biopsy, no surgical intervention was required. He subsequently developed secondary hypothyroidism, secondary adrenal insufficiency and growth hormone deficiency. These disorders were managed with levothyroxine and prednisone.

Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus; Humans; Hypogonadism; Male; Pituitary Diseases; Testosterone

Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus; Disseminated Intravascular Coagulation; Female; Humans; Hypogonadism; Pituitary Diseases; Pituitary Gland; Pregnancy

Lymphocytic infundibuloneurohypophysitis is a rare disorder in which neurohypophyseal function is impaired by an autoimmune process. Although several etiologies for this rare entity have been suggested, its occurrence following transsphenoidal adenomectomy has not been reported. A 20-year-old man presented with diabetes insipidus - seven years after successful transsphenoidal microadenomectomy for Cushing's disease, first diagnosed at the age of 13. Seven years later, he developed fairly rapid onset of polydipsia and polyuria. Magnetic resonance imaging demonstrated swelling of the posterior pituitary gland with thickening of the pituitary stalk. Endocrinological evaluation revealed neurohypophyseal dysfunction without the adenohypophysis being affected. On the basis of these findings, a diagnosis of lymphocytic infundibuloneurohypophysitis was made. The mass lesion of the posterior pituitary resolved after the administration of corticosteroids for two months and no operation was required. Lymphocytic infundibuloneurohypophysitis should be considered in the differential diagnosis of pituitary mass lesions following transsphenoidal surgery, especially when the mass is confined to the posterior pituitary gland with neurohypophyseal function being compromised.

Topics: Adenoma; Adult; Anti-Inflammatory Agents; Cushing Syndrome; Deamino Arginine Vasopressin; Humans; Magnetic Resonance Imaging; Male; Neurosurgical Procedures; Pituitary Diseases; Pituitary Gland, Posterior; Pituitary Hormones, Posterior; Pituitary Neoplasms; Postoperative Complications; Prednisolone

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

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

Topics: Antigens, CD1; Biopsy, Needle; Child, Preschool; Deamino Arginine Vasopressin; Diabetes Insipidus; Follow-Up Studies; Histiocytosis, Langerhans-Cell; Humans; Immunohistochemistry; Male; Pituitary Diseases; Risk Assessment; Sensitivity and Specificity; Treatment Outcome

We report the case of a 53 year old patient who was admitted with polyuria, polydipsia associated with fatigue, depression and sexual dysfunction. Central diabetes insipidus with hypogonadotrophic hypogonadism was diagnosed by a water restriction test and different static and dynamic hormonal dosages. Nodular thickening of the pituitary stalk was noted on the MRI and the biopsy permitted a histological diagnosis of infundibulitis.

Topics: Biopsy; Deamino Arginine Vasopressin; Diabetes Insipidus; Drinking; Humans; Hypogonadism; Male; Middle Aged; Pituitary Diseases; Pituitary Function Tests; Pituitary Gland, Posterior; Polyuria; Renal Agents

Four cases of hypothalamic-pituitary Langerhans cell histiocytosis (LCH) are reported, highlighting the expanding spectrum of clinical and magnetic resonance imaging (MRI) features in adults. The diagnostic challenge of hypothalamic-pituitary LCH is emphasized in cases revealed as supra-sellar tumors with panhypopituitarism or as isolated central diabetes insipidus. Diagnosis is confirmed by histological examination showing infiltration with CD1a positive histiocytes. General guidelines for diagnosis procedure are drawn out, including the neurosurgical biopsy in particular cases.

Topics: Adult; Aged; Blindness; Deamino Arginine Vasopressin; Fatal Outcome; Female; Histiocytosis, Langerhans-Cell; Humans; Lung Diseases; Magnetic Resonance Imaging; Male; Pituitary Diseases; Pituitary Neoplasms; Renal Agents

The desmopressin test has recently been introduced in clinical practice as an adjunctive tool in the differential diagnosis of ACTH-dependent Cushing's syndrome (CS). It has been reported that the majority of patients with pituitary-dependent CS (Cushing's disease, CD) respond to desmopressin, while no such response is usually observed in other forms of this syndrome. In the present study, the responsiveness of the HPA axis to desmopressin was studied in a group of obese subjects. In addition, the ability of desmopressin administration to differentiate between patients with obesity and the various forms of Cushing's syndrome was investigated.. Cortisol and ACTH responses to the administration of desmopressin (10 microg bolus i.v.) were examined in 20 consecutive patients with obesity (14 women and six men; BMI range: 34.5-66.7 kg/m2). Obese subjects had no clinical stigmata of CS. In all obese patients, either an overnight (dex 1 mg at 2300 h) (n = 8) or a formal low-dose (dex 0.5 mg 6-hourly for 2 days) (n = 12) dexamethasone suppression test was performed for the exclusion of Cushing's syndrome. Three of eight subjects showed failure of cortisol suppression (i.e. F > 28 nmol/l) to the overnight dexamethasone suppression test, but they had undetectable cortisol levels (< 28 nmol/l) on further testing with the formal 2-day test. All but two of the remaining subjects had undetectable cortisol levels (< 28 nmol/l) following the formal 2-day, low-dose, dexamethasone suppression test. For comparison, desmopressin responses were also tested in 33 patients with CS of varied aetiologies (25 patients with pituitary-dependent CS, three patients with occult ectopic ACTH secretion and five patients with primary adrenal CS). A positive response was considered to be an increment greater than 20% and 50% from baseline levels of cortisol and ACTH, respectively.. Mean cortisol (F) and ACTH levels did not differ from the baseline at any time point following desmopressin administration in the obese group (basal F: 417 +/- 41, peak F: 389 +/- 32 nmol/l, P > 0.05; basal ACTH: 33.5 +/- 4.3, peak ACTH: 50.6 +/- 16.6 ng/l, P > 0.05), or in patients with occult ectopic or primary adrenal CS. In contrast, in the group of patients with CD, there was a significant rise in the mean ACTH and F levels from baseline (basal F: 725 +/- 50, peak F: 1010 +/- 64 nmol/l, P < 0.01; basal ACTH: 88.6 +/- 11.8, peak ACTH: 351 +/- 64 ng/l, P < 0.01). Cortisol responses greater than 20% from baseline were observed in 21/25 (84%) patients with CD, but in only 3/20 (15%) of the obese patients. With regard to ACTH, increments greater than 50% over baseline were observed in 23/25 (92%) of patients with CD, and in only 3/20 (15%) of the obese patients. As previously reported, none of the patients with occult ectopic ACTH secretion or primary adrenal CS had a positive response.. The prevalence of subjects who met the criteria adopted to define positive cortisol and ACTH responses to the desmopressin test was significantly higher in the group of patients with Cushing's disease than in the group of patients with obesity. It is therefore suggested that this test may be occasionally useful in the differentiation between simple obesity and the pituitary-dependent form (but not other forms) of Cushing's syndrome.

Topics: Adrenocorticotropic Hormone; Adult; Cushing Syndrome; Deamino Arginine Vasopressin; Diagnosis, Differential; Female; Humans; Hydrocortisone; Male; Middle Aged; Obesity; Pituitary Diseases; Predictive Value of Tests; Renal Agents

We present the eldest case ever reported of central diabetes insipidus (DI) associated with infundibulo-neurohypophysitis. A 77-year old woman, who complained of recent development of excessive thirst, polyuria and polydipsia, was referred to our hospital. The daily urine volume was markedly increased to 6 L. DDAVP administration effectively reduced urine volume and increased urine osmolality. The loading test using high-osmolar sodium chloride showed impaired excretion of vasopressin discordant with plasma osmolar changes. The anterior pituitary function was normal. Pituitary magnetic resonance imaging (MRI) showed thickening of the pituitary stalk and a lack of high-intensity signal of the neurohypophysis on T1-weighted images, suggestive of lymphocytic infundibulo-neurohypophysitis. The thickness of pituitary stalk on MRI improved 6 months later. DI was controlled with DDAVP for 40 days. This was followed by stabilization of the daily urine volume to less than 2.5 L without DDAVP. Our case is the eldest case of central DI associated with infundibulo-neurohypophysitis. The rapid remission of pituitary changes on MRI provides an insight that spontaneously partial remission of central DI may occur, resulting in transient polyuria and polydipsia.

Topics: Aged; Blood; Chlorides; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Headache; Humans; Lymphocytes; Magnetic Resonance Imaging; Osmolar Concentration; Pituitary Diseases; Pituitary Gland, Posterior; Potassium; Sodium; Thirst; Urine; Vasopressins

We reported 4 cases of lymphocytic infundibuloneurohypophysitis. All four patients had diabetes insipidus as initial symptoms without anterior pituitary dysfunction. All patients showed pituitary stalk swelling and two patients showed enlargement of the pituitary gland. No patients were operated on for a histological diagnosis. No patients received corticosteroid treatment for this pathology. The mean follow-up period was 36 months. The diabetes insipidus continued in all cases, but radiological findings showed improvement in all cases. In one case, adrenal insufficiency occurred after 10 months, but had disappeared 6 months later. We think lymphocytic infundibuloneurohypophysitis can be diagnosed without histological examinations and can be treated conservatively without corticosteroid treatment. It seems to be a self-limiting disease. This disease can be distinguished from lymphocytic adenohypophysitis, but in some cases, both the anterior and posterior pituitary glands are invaded, and in this situation lymphocytic hypophysitis may be an appropriate name. Even if the initial symptom is diabetes insipidus, careful follow-up is needed for the duration of the disease.

Topics: Aged; Chronic Disease; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Humans; Inflammation; Lymphocytes; Magnetic Resonance Imaging; Male; Middle Aged; Pituitary Diseases; Pituitary Gland

A young white man with new-onset central diabetes insipidus was discovered to have a posterior pituitary mass on magnetic resonance imaging. No other radiological abnormalities were noted in the anterior pituitary, infundibulum or hypothalamus. No other endocrinopathies were present: laboratory investigations showed normal basal concentrations of anterior pituitary hormones, including prolactin. The patient was suspected to have sarcoidosis affecting the posterior pituitary, because of the discovery of pulmonary sarcoidosis during his diagnostic evaluation. His symptoms of polydipsia and polyuria responded promptly to intranasal administration of 1-desamino-8-D-arginine vasopressin (DDAVP). The patient demonstrated complete regression of the posterior pituitary mass after a course of corticosteroid therapy. However, his diabetes insipidus persisted and he continues to need DDAVP treatment, currently at 12 months of follow-up. The resolution of the neurohypophysial mass was compatible with the diagnosis of pituitary sarcoidosis and this precluded the need for a transsphenoidal biopsy or surgery.

Topics: Adrenal Cortex Hormones; Adult; Deamino Arginine Vasopressin; Diabetes Insipidus; Humans; Lung Diseases; Magnetic Resonance Imaging; Male; Pituitary Diseases; Pituitary Gland, Posterior; Radiography, Thoracic; Sarcoidosis

We describe five patients with chronic inflammation of the hypophysis including three pituitary granulomas of unknown aetiology. In contrast to the previously reported cases, the involvement of neurohypophysis or hypothalamus was a distinct clinical feature in these patients. Impairment of anterior pituitary function was less prominent, while polyuria and polydipsia occurred in all cases. Enlargement of the sella turcica was absent in three and slight in two cases. CT scan and MR images demonstrated a contrast-enhanced sellar mass in all patients; abnormally thickened pituitary stalk and infundibulum with contrast-enhancement was observed in four. The fibrous tissues were removed by the transsphenoidal approach in four patients, and by the subfrontal approach in one case. In all patients, the endocrinological dysfunction was prolonged. No increase in the size of the remaining pituitary mass was demonstrated on repeated MR images in any of the patients. On histological examination, granulomatous formation was present in three samples, and multinucleated Langhans' giant cells were seen in one. The epithelioid cells and multinucleated giant cells constituting the granulomas were positive for anti-macrophage antibody. No firm laboratory or histological evidence was obtained supporting the presence of systemic disease leading to granulomas. In the other two cases, the pituitary lesions were composed of chronic inflammation tissue, and serum antipituitary antibodies were present in a patient with concurrent Hashimoto's thyroiditis. Our experiences with chronic inflammation of the hypophysis indicate that these patients are best managed by histological confirmation of the lesion followed by adequate hormonal replacement.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Corticosterone; Deamino Arginine Vasopressin; Female; Fibrosis; Granuloma; Granuloma, Giant Cell; Humans; Inflammation; Magnetic Resonance Imaging; Male; Middle Aged; Pituitary Diseases; Pituitary Function Tests; Pituitary Gland; Postoperative Care; Thyroid Hormones; Tomography, X-Ray Computed

A rare case of a postmenopausal (60-year-old) female with lymphocytic adenohypophysitis manifesting as a sudden onset of diabetes insipidus is reported. Magnetic resonance imaging with gadolinium-diethylene-triaminepentaacetic acid enhancement showed a spherical lesion, approximately 1 cm in diameter, in the sella turcica and a thickened, deviated pituitary stalk. The abnormal tissue was totally removed. Histological examination showed marked infiltration of lymphocytes and plasma cells. Postoperatively, the pituitary stalk became normal. Preoperative differentiation of lymphocytic adenohypophysitis from pituitary adenoma is extremely difficult, and biopsy is essential.

Topics: Adenoma; Autoimmune Diseases; Biopsy; Deamino Arginine Vasopressin; Diabetes Insipidus; Diagnosis, Differential; Female; Humans; Lymphocytes; Magnetic Resonance Imaging; Menopause; Middle Aged; Pituitary Diseases; Pituitary Gland, Anterior; Pituitary Neoplasms; Plasma Cells

Topics: Deamino Arginine Vasopressin; Pituitary Diseases; Pituitary Gland