deamino-arginine-vasopressin and Craniopharyngioma

Hypothermia has been demonstrated to induce pancytopenia in animals, but whether this association exists in humans is unknown. The authors report the case of an 8-year-old girl in whom hypothermia (temperature 33 degrees C-35 degrees C) is the cause of pancytopenia. The patient developed thermoregulatory dysfunction subsequent to surgical resection of a craniopharyngioma. Her recurrent cytopenias could not be explained by any etiology except chronic hypothermia. The pancytopenia improved upon rewarming the patient to a temperature of 36 degrees C. This association between hypothermia and pancytopenia has rarely been reported in humans and may be underdiagnosed especially in cases of transient or milder presentations. The authors recommend careful hematologic monitoring of patients with thermoregulatory dysfunction.

Topics: Adrenal Insufficiency; Blood Cell Count; Cerebral Infarction; Child; Chronic Disease; Consciousness Disorders; Craniopharyngioma; Deamino Arginine Vasopressin; Dehydration; Diabetes Insipidus; Female; Frontal Lobe; Humans; Hyponatremia; Hypophysectomy; Hypopituitarism; Hypothalamus; Hypothermia; Hypothyroidism; Pancreatitis; Pancytopenia; Pituitary Neoplasms; Postoperative Complications; Seizures; Sleep Stages

We describe a case of spontaneous muscle necrosis of the anterior tibial compartment occurring in a patient who had a hypophysectomy for a craniopharyngioma five years previously. We know of no other reported cases of spontaneous muscle necrosis in association with diabetes insipidus and feel that there should be increased awareness of the association between the two.

Topics: Adult; Biopsy; Compartment Syndromes; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Humans; Hypophysectomy; Leg; Male; Pituitary Neoplasms; Postoperative Complications

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

Presented case demonstrates a rare diencephalic pathology - adipsic diabetes insipidus (ADI) with severe hypernatremia in a 58-year-old woman after ttranssphenoidal removal of stalk intraventricular craniopharyngioma. ADI was diagnosed because of hypernatremia (150-155 mmol/L), polyuria (up to 4 liters per day) and absence of thirst. Normalization of water-electrolyte balance occurred on the background of desmopressin therapy and sufficient hydration in postoperative period. After release from the hospital, the patient independently stopped desmopressin therapy and did not consume an adequate amount of fluid of the background of polyuria. This led to severe hypernatremia (155-160 mmol/L) and rough mental disorders.Patients with ADI need closely monitoring of medical condition and water-electrolyte parameters, appointment of fixed doses of desmopressin and adequate hydration.

Topics: Central Nervous System Cysts; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Mellitus; Female; Humans; Hypernatremia; Middle Aged; Neurosurgical Procedures; Polyuria; Postoperative Complications

Topics: Adult; Craniopharyngioma; Deamino Arginine Vasopressin; Drug Administration Schedule; Female; Humans; Hypernatremia; Hypopituitarism; Neurologic Examination; Pituitary Neoplasms; Postoperative Complications; Sodium; Substance Withdrawal Syndrome

Adipsic diabetes insipidus is an infrequent disease which may be associated with craniopharyngioma. It may be secondary to the tumour's extension, as well as to resection of the mass. We present the case of a 24-year-old woman with a history of delayed puberty and hypothyroidism, but no prior study reports. She consulted due to a headache with warning signs associated with altered visual acuity. Brain MRI was performed which showed signs of a non-adenomatous lesion with suprasellar and hypothalamic extension. Following transcranial surgery, she developed diabetes insipidus criteria, with absence of thirst documented during the hospitalisation. The histopathological findings confirmed the diagnosis of craniopharyngioma. The patient was treated with desmopressin and received recommendations regarding rehydration according to the quantification of losses, with electrolyte stabilisation.

Topics: Antidiuretic Agents; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Humans; Magnetic Resonance Imaging; Pituitary Neoplasms; Prednisolone; Sodium; Thirst; Thyroxine; Treatment Outcome; Young Adult

Pituitary stalk sectioning is only essential in cases of craniopharyngioma originating from the stalk or metastatic tumor to the stalk. Some patients can discontinue postoperative antidiuretic hormone (ADH) supplementation with special conditions.. Sixty-three patients with craniopharyngiomas who were treated by surgery with pituitary stalk sectioning were included in this study. Great care was taken to preserve the fine arteries running along the lateral walls of the third ventricle. Removal rates, change of endocrinologic status, and magnetic resonance imaging (MRI) findings were investigated.. Total removal was achieved in 52 of 54 patients in initial surgery (96.3%), and in 5 of 9 patients in retreatment (55.6%). ADH supplementation was required in all patients from the day of surgery, but was discontinued in 29 of 54 patients among the initial surgery group (53.7%) and in 2 of 9 patients among the retreatment group (22.2%). Preservation of thyroid hormone secretion was observed in 24 of 31 patients who could discontinue ADH (77.4%), but only in 12 of 32 patients who could not discontinue ADH (37.5%). Recovery from diabetes insipidus (DI) was significantly associated with preservation of thyroid function (P < 0.01). Postoperative MRI showed that part of the hypothalamus was enhanced in patients with recovery from DI.. Total removal was achieved in 91% of all cases. Half of the patients could discontinue ADH supplementation, which was associated with preservation of thyroid function. The findings of hypothalamic enhancement on postoperative MRI may be associated with recovery from DI.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antidiuretic Agents; Child; Child, Preschool; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Glucocorticoids; Hormone Replacement Therapy; Humans; Hypopituitarism; Hypothalamo-Hypophyseal System; Hypothyroidism; Magnetic Resonance Imaging; Male; Middle Aged; Neurosurgical Procedures; Pituitary Gland; Pituitary Neoplasms; Postoperative Complications; Prognosis; Recovery of Function; Thyroid Hormones; Young Adult

Topics: Adolescent; Child, Preschool; Craniopharyngioma; Deamino Arginine Vasopressin; Estrogens; Female; Hemostatics; Hormone Replacement Therapy; Humans; Hypopituitarism; Monitoring, Physiologic; Postoperative Complications; Progesterone; Risk Factors; Thrombophilia; Venous Thrombosis; von Willebrand Factor

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

To report the first case of pregnancy and spontaneous delivery after total hypophysectomy.. A case report.. University hospital.. A 34-year-old woman with panhypopituitarism after hypophysectomy in childhood for craniopharyngioma.. Successful fertility treatment followed by sufficient hormone substitution therapy during pregnancy.. Clinical variables.. The patient had an uncomplicated spontaneous delivery without any substitution of oxytocin before, during, or after delivery.. Normal pregnancy and delivery are possible in women with lack of pituitary function. Maternal pituitary oxytocin release seems to play a limited role in the onset and progression of labor.

Topics: Adult; Craniopharyngioma; Deamino Arginine Vasopressin; Estrogen Replacement Therapy; Female; Hemostatics; Humans; Hydrocortisone; Hypophysectomy; Male; Ovulation Induction; Pregnancy; Pregnancy Outcome; Thyroxine

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

In a retrospective study, the intra- and early postoperative data of 39 children with 46 operations for craniopharyngioma were analyzed. Diabetes insipidus (DI) occurred in 30 out of 32 cases without preoperative evidence of DI. We observed that all children who did not have a pituitary stalk preserved and 5 out of 7 patients with preserved pituitary stalk developed DI within 18 h of surgery. Short-term inappropriate secretion of antidiuretic hormone (SIADH) occurred in 2 children, but was quickly followed by DI. The time of onset of DI and SIADH did not correlate with sex, age, body weight, location of tumor, or duration or extent of surgery. Parenteral desmopressin was an effective treatment for intra- and postoperative DI. The duration of the clinical effect of desmopressin administration varied in different patients between 4 and 23 h. An approach to the immediate intra- and postoperative management of children with craniopharyngioma is presented.

Topics: Administration, Intranasal; Adolescent; Child; Child, Preschool; Combined Modality Therapy; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Fluid Therapy; Humans; Inappropriate ADH Syndrome; Infant; Infant, Newborn; Male; Perioperative Care; Pituitary Neoplasms; Postoperative Complications; Retrospective Studies; Water-Electrolyte Imbalance

A 46-year-old man who had a history of hypogonadism, bilateral hydronephrosis and huge residual urine volume during the past ten years was admitted complaining of fever and flank pain. Polyuria which was more than 4 liters per day and inability of urine concentration suggested diabetes insipidus. Magnetic resonance imaging (MRI) demonstrated a tumor which was compatible with craniopharyngioma. Tumor resection and administration of desmopressin improved polyuria and urinary tract dilatation with marked reduction of residual urine volume from 400 ml to 20 ml.

Topics: Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Dilatation, Pathologic; Humans; Hydronephrosis; Male; Middle Aged; Pituitary Neoplasms; Renal Agents; Urinary Tract

The postoperative course of children undergoing surgery for craniopharyngioma was reviewed. Typically they were below height at presentation. All had an attempt at radical surgical resection of the tumor. Most developed diabetes insipidus in the postoperative period, which was permanent in all but 1 child. 94% required thyroid replacement therapy, and sex steroids were administered in 100% when they reached the age of puberty. 91% required maintenance corticosteroids. 54% required growth hormone replacement, but some children showed continued growth despite apparent growth hormone deficiency. Postoperative obesity develops in one half of patients, and may be improved with administration of growth hormone; a controlled trial is underway.

Topics: Adolescent; Body Height; Body Weight; Child; Child, Preschool; Combined Modality Therapy; Craniopharyngioma; Deamino Arginine Vasopressin; Female; Follow-Up Studies; Growth Hormone; Humans; Hypophysectomy; Hypopituitarism; Infant; Male; Pituitary Function Tests; Pituitary Hormones; Pituitary Neoplasms; Postoperative Complications; Thyroxine

1. We have studied the response of six patients with cranial diabetes insipidus and six age-matched control subjects to dietary sodium restriction during constant administration of the synthetic vasopressin analogue desamino-[8-D-arginine]vasopressin. 2. Urine flow increased on the first low salt day in the normal control subjects but not in the patients with cranial diabetes insipidus. Body weight fell 1.35 kg in the control subjects but was constant in the patients with cranial diabetes insipidus. 3. Urinary sodium excretion fell at the same rate in both groups. Diurnal variation of urinary sodium excretion and creatinine clearance was present in the control subjects but not in the patients with cranial diabetes insipidus. 4. Changes in plasma sodium concentration and osmolality were similar. Plasma protein concentration increased more in the control subjects (from 69.1 +/- 1.5 to 73 +/- 1.2 versus from 71.7 +/- 1 to 73.2 +/- 1.1 milligrams). The responses of plasma atrial natriuretic peptide, plasma renin activity and salivary aldosterone concentration were similar between the two groups. Salivary aldosterone concentration levels were consistently higher in the patients with cranial diabetes insipidus. 5. We confirm that the low salt diuresis is triggered by release from the antidiuretic activity of arginine vasopressin. In the patients with cranial diabetes insipidus extracellular fluid osmoregulation appeared to be achieved by the movement of water out of and sodium into the extracellular fluid. 6. Absent posterior pituitary function and hypothalamic disturbances did not alter renal sodium conservation.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adult; Blood Pressure; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Heart Rate; Humans; Male; Middle Aged; Pituitary Neoplasms; Sodium; Sodium, Dietary

A case is presented of a pregnancy complicated by a suprasellar mass diagnosed at 27 weeks' gestation. This patient developed diabetes insipidus, which was successfully treated with 1-desamino-8-D-arginine vasopressin. Thyrotropin-releasing hormone and ACTH stimulation tests were also abnormal, requiring the institution of thyroid and cortisol replacement therapy. The patient was delivered at 34 weeks' gestation secondary to worsening visual field testing. A craniotomy was performed in the postpartum period with removal of a craniopharyngioma. With successful medical treatment and careful observation, surgical intervention may be postponed until postpartum or until a gestational age with lower neonatal morbidity and mortality is reached.

Topics: Adult; Combined Modality Therapy; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Humans; Hydrocortisone; Pituitary Neoplasms; Pregnancy; Pregnancy Complications, Neoplastic; Thyroxine

Topics: Adolescent; Brain Neoplasms; Child; Child, Preschool; Craniopharyngioma; Craniotomy; Deamino Arginine Vasopressin; Diabetes Insipidus; Diuresis; Female; Humans; Hypernatremia; Male; Osmolar Concentration

Topics: Adolescent; Arginine Vasopressin; Chlorpropamide; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Drug Therapy, Combination; Humans; Male; Pituitary Neoplasms; Thirst

Topics: Adenoma; Adolescent; Adult; Arginine Vasopressin; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Humans; Male; Middle Aged; Pituitary Neoplasms; Postoperative Period

Topics: Child; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Headache; Humans; Male; Pituitary Neoplasms; Postoperative Complications; Vision Disorders

A patient is described who developed diabetes insipidus during pregnancy. During a revised Carter test performed at 36 wk gestation using DDAVP (1-desamino-8-D-arginine-vasopressin), uterine activity was recorded with a maximum activity of 120 Montevideo Units. The induction of uterine activity by DDAVP in our patient might be related to the high endogenous oxytocin levels or to the far advanced state of amenorrhea. Post partum, the patient reported decreased vision, and the visual fields were found to be abnormal. A neurosurgical procedure followed, and the diagnosis of craniopharyngioma was made.

Topics: Adult; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Humans; Oxytocin; Pituitary Neoplasms; Pregnancy; Pregnancy Complications; Uterine Contraction

Topics: Adolescent; Adult; Child; Craniopharyngioma; Deamino Arginine Vasopressin; Female; Humans; Male; Neoplasm Recurrence, Local; Pituitary Gland; Pituitary Neoplasms; Tomography, X-Ray Computed