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[Lymphocytic infundibulo-hypophysitis with diabetes insipidus as a new clinical entity: a case report and review of the literature].

In 1992, we reported a lymphocytic adenohypophysitis (LIH) (Neurol Med Chir). We considered this case unusual in that the case was that of a menopausal female and that it was accompanied with diabetes insipidus as classical lymphocytic adenohypohysitis (LAH). Subsequently, Ahmed reported two cases which presented a similar pathological manifestation, except for necrosis, as did our case and named them "necrotizing infundibulo-hypophysitis." Recently we encountered another similar case, which is reported hereunder. A female, 34 years of age, had suffered from headache, polyuria, and amenorrhea. CT scan showed a pituitary mass, and pituitary tumor was surgically removed transcranially at a local hospital. The pathological examination revealed the findings of chronic inflammation and necrosis. One month after the operation, however, she was an in-patient again under the suspicion of meningitis for fever and, when antibiotic therapy at the local hospital resulted in no improvement, she was referred to our hospital. Endocrinological studies showed low FSH, LH, ACTH and plasma cortisol level. Antibodies of serum to RNP, Sm, mitochondria, nucleus, AChR, and DNA were all negative. Because of an intrasellar mass with suprasellar extension on MRI, transsphenoidal operation was conducted four months after the initial operation. The pathological examination revealed the infiltration of lymphocytes, plasma cells, and foamy macrophages, and necrosis. After this operation, the headache was cured and the patient was discharged. Two months subsequent to the second operation, headache recurred and temporal upper quadrantic anopsia was noted. An enlarged tumor was found, but prednisolone worked to cure the pain and the visual field defect was found to have been remedied. The patient's diabetes insipidus is presently persisting, and she still relies on the use of desmopressin acetate and is still in need of cortisol replacement therapy. Including our cases, ten cases of lymphocytic hypophysitis, not related to pregnancy or delivery but with diabetes insipidus, have been reported. Several clinical and anatomical features distinguish these 10 cases from classical LAH. The classical LAH was predominantly related to pregnancy or delivery. However 6 of 10 cases were male in LIH. LAH related to pregnancy or delivery does not accompany diabetes insipidus, but all reported cases of LIH had a diabetes insipidus. Visual field and/or ocular movement disturbance are LAH's chief complaints (15 out of 25 cases) but visual field disturbance seldom occurs in LIH (1 out of 10 cases). Hypopituitarism is more serious in LAH, and 4 cases became fatal from an adrenal crisis. Anatomically, inflammatory change of LIH is located anterior and posterior to the pituitary gland and extends to the pituitary stalk and, at times, hypothalamus. On the other hand, LAH relates to pregnancy or delivery, the inflammatory change localizes to the adenohypophysis. Ahmed emphasized necrosis, while necrosis was not a prominent histological finding in LIH. Necrosis was noted only in 3 of 10 cases. To be stressed, rather, are the inflammatory changes seen on the neurohypophysis and the pituitary stalk, together with the characteristic diabetes insipidus. We believe, in view of the above, that what Ahmed named necrotizing infundibulo-hypophysitis should be named "LIH with diabetes insipidus." Whereas differential diagnosis is necessary between this said new disorder and the conventional LAH, we advocate that the latter, which is related to pregnancy or parturition but is free from neurohypophysitis be identified as "LAH related to pregnancy or delivery." With respect to treatment, steroid therapy is essential. If the symptoms do not improve, a transsphenoidal operation for diagnosis (LIH and LAH) and decompression (the case of LAH with visual or external ocular movement disturbance) is advisable. However, extensive surgery is not recommended, because per

Adult↗

Diabetes insipidus in Langerhans' cell histiocytosis: report of a case.

The incidence of diabetes insipidus secondary to Langerhans' cell histiocytosis (LCH) varies among different reports, ranging from 9.5 to 50%, but it has never been reported in literature in Taiwan. Therefore, we presented a case suffering from polyuria, polydipsia, body weight loss for more than one year and seborreic dermatitis-like skin lesions over the scalp and trunk for more than two years. Her body weight and body length were both less than 3 percentile. Fluid restriction and vasopressin test were performed to differentiate nephrogenic from neurogenic diabetes insipidus. Skin biopsy revealed picture of LCH and LCH with complete central diabetes insipidus was diagnosed. Brain MRI and other laboratory examinations were all within normal limits. She received nasal DDAVP treatment and chemotherapy with TPOG-H 94 protocol. After 3 months treatment, her skin lesions disappeared and daily urine amount returned to normal range.

Child, Preschool↗

[Treatment of diabetes insipidus].

Although water alone taken in sufficient amount should correct any metabolic abnormality, a proper therapy is available to reduce the symptoms of the diabetes insipidus. In patients with reduced circulating levels of vasopressin, chlorpropamide, clofibrate, idroclorotiazide etc., enhance the effect of vasopressin on the renal tubule or induce a release of vasopressin, thus reducing the diuresis. On the contrary patients with complete diabetes insipidus require replacement therapy. Different forms of extractive vasopressin unfortunately are available: "aqueous vasopressin", "tannate vasopressin" in a suspension of peanut oil etc., are provided of many side-effects because of the impurities in their preparation. DDAVP is a synthetic analogous of natural vasopressin more specific for antidiuresis and with a long-activity. This compound available in a buffered aqueous solution is administrated by blowing into the nose. In our experience, 23 patients with different forms of central diabetes insipidus have successfully treated with 2-3 daily administrations of this drug without any side effect. The recent demonstration that DDAVP given orally exerts an antidiuretic activity in patients with diabetes insipidus is of therapeutic interest. It is conceivable that DDAVP in a properly formulated tablet will become a therapeutic option for the future treatment of patients with central diabetes insipidus.

Deamino Arginine Vasopressin↗

Effect of a short-term treatment with alendronate on bone density and bone markers in patients with central diabetes insipidus.

The aim of this open prospective randomized study was to evaluate the effect of a 6-month treatment with alendronate on the bone mineral density (BMD) at lumbar spine in patients with central diabetes insipidus. Eighteen patients with central diabetes insipidus and 18 sex- and age-matched healthy subjects entered this study. At study entry, all subjects underwent BMD assessment at the lumbar spine and measurement of serum osteocalcin (OC) and cross-linked N-telopeptides of type I collagen (Ntx). Thereafter, 9 of the 18 patients were randomized to receive treatment with alendronate at a dose of 10 mg, orally, once daily for 6 months (group 1), whereas the remaining 9 patients did not receive any treatment affecting bone status during this period (group 2). After 6 months, bone metabolism and bone density study were repeated in all patients. At baseline, lumbar BMD values (0.86+/-0.03 vs. 1.01+/-0.02 g/cm2; P<0.001) and serum OC levels (4.7+/-0.3 vs. 7.9+/-0.2 microg/L; P<0.001) were significantly lower, whereas urinary Ntx levels were significantly higher [72.0+/-1.9 vs. 64.6+/-1.7 nmol bone collagen equivalents (BCE)/nmol creatinine (Cr); P<0.01] in patients than in controls. After randomization, no difference in lumbar BMD, serum OC, or urinary Ntx was found between patients of group 1 and group 2. At the 6 month follow-up, no difference in serum OC levels was found compared to baseline evaluation in patients of both group 1 and group 2. By contrast, a significant decrease in urinary Ntx levels was found in patients of group 1 (70.3+/-3.0 vs. 75.4+/-2.1 nmol BCE/nmol Cr; P<0.05), but not in patients of group 2 (68.8+/-3.3 vs. 68.5+/-3.0 nmol BCE/nmol Cr; P = NS). A significant increase in lumbar BMD values was found in patients of group 1 (0.88+/-0.04 vs. 0.83+/-0.04 g/cm2; P<0.05), whereas a significant decrease in lumbar BMD values was found in patients of group 2 (0.86+/-0.05 vs. 0.89+/-0.05 g/cm2; P<0.05). Lumbar BMD increased 7.0+/-1.5% in patients of group 1 and decreased 4.2+/-1.8% in patients of group 2 (P<0.001). In conclusion, this study demonstrated that a 6-month treatment with alendronate in patients with central diabetes insipidus was effective in significantly improving BMD at the lumbar spine, which was significantly worsened in untreated patients. Therefore, alendronate treatment could be used in patients with central diabetes insipidus with documented osteopenia or osteoporosis.

Adolescent↗

Transient nephrogenic diabetes insipidus during toxemia in pregnancy.

A pregnant woman is presented who developed transient nephrogenic diabetes insipidus. The patient had chronic hypertension with evidence of superimposed toxemia. The association of nephrogenic diabetes insipidus and pregnancy is reviewed. To our knowledge this is the first description of a patient who developed nephrogenic diabetes insipidus during pregnancy.

Adult↗

[A case of pulmonary embolism with diabetes insipidus developed after removal of craniopharyngioma].

A case of pulmonary embolism associated with diabetes insipidus is reported in an 18-year-old male. The patient, who had been treated with DDAVP for diabetes insipidus and hydrocortisone for hypocorticism for two years after first operation for the removal of craniopharyngioma, was admitted with recurrence of that tumor. Diabetes insipidus immediately after second operation was controlled with intermittent drip infusion of a small amount of aqueous pitressin under monitorings of body weight hourly using a patient weighing system to keep the weight changes within +/- one kilogram. Serum and urine electrolytes levels, osmolarity, and free water clearance were also monitored every three hours to maintain water-electrolytes balances appropriately. Postoperative course had been uneventful except that CSF rhinorrhea occurred 7 days after operation. The patient was, then, kept in bed with horizontal plane to avoid further leakage of CSF. Two days later, he developed chest pain suddenly with tachypnea, tachycardia, and general cyanosis. The arterial-BGA showed PaO2 of 53.5mmHg and PaCO2 of 35.3mmHg in room air. The definite diagnosis of pulmonary embolism was made by technetium microaggregate lung perfusion scans and by pulmonary angiograms. The patient was treated with heparin, 15000IU/day, and urokinase, 720000IU/day. The symptoms due to pulmonary embolism had improved gradually within a couple of weeks. Recent articles have shown an unexpected high incidence of deep vein thrombosis and pulmonary embolism in neurosurgical patients associated with the elevation of blood coagulability. Brain tumors, especially suprasellar mass with hypothalamic dysfunction have been suggested to cause thromboembolic disorders frequently. The clinical course was described and factors causing pulmonary embolism on this patient was discussed.

Adolescent↗

[Vasopressin-sensitive diabetes insipidus. Case histories].

Twelve patients, aged 16-66 years, affected by cranial diabetes insipidus either idiopathic or secondary (head injuries, histiocytosis X, metastatic tumors, pituitary or hypothalamic surgery) were studied. Diagnosis was made on the basis of both dehydration test and sensitivity to exogenous vasopressin. The relationship between plasma and urine osmolality at the end of the dehydration test was evaluated (and its usefulness for diagnostic purpose confirmed). According to this test two different conditions, namely partial and complete, of cranial diabetes insipidus were defined. All the patients underwent the following diagnostic procedures: skull x-ray, evaluation of visual fields, encephalic CT-scan. Idiopathic central diabetes insipidus incidence was found to be 33.3% of all cases, with a sharp prevalence for male sex; the median age of onset was 12.5 years. In our experience the frequency of idiopathic condition was similar to that reported in most recent literature confirming a decreasing incidence, most likely due to the use of more sophisticated diagnostic procedures.

Adolescent↗

[Inhibition of prostaglandin synthesis during the therapy of nephrogenic diabetes insipidus].

A report is presented of a male infant with nephrogenic diabetes insipidus type I. A low-solute-load diet and large fluid intake were not able to prevent hypertonic dehydration and to control the disease. It responded within 3 days to indomethacin (3 mg/kg body weight), a prostaglandin synthetase inhibitor. The boy is doing well on this regimen and no side-effects have been observed after 2 years of treatment. Hitherto only a few case reports have been published on this therapeutic regimen, but all confirm the high efficacy of prostaglandin synthesis inhibition as the treatment of choice in nephrogenic diabetes insipidus.

Diabetes Insipidus↗

Isolated Langerhans cell histiocytosis of the infundibulum presenting with fulminant diabetes insipidus.

INTRODUCTION: Langerhans cell histiocytosis (histiocytosis X) is a rare disorder that can manifest with solitary bony lesions or disseminated disease in various tissues. A common manifestation of the disease is diabetes insipidus even in the absence of neural involvement. Disseminated disease often involves the central nervous system, but only six isolated lesions in the infundibulum have been reported. CASE REPORT: An 18-year-old male had failed to develop secondary sexual characteristics. He also had diabetes insipidus for more than 1 year. Magnetic resonance imaging (MRI) of the brain showed a 1-cm homogeneously enhancing mass in the region of the infundibulum. No other lesions were found in other organ systems. The patient underwent an orbitozygomatic osteotomy and pterional craniotomy. The lesion, which was adherent to the infundibulum, was partially resected. Microscopic analysis showed multiple lymphocytes and eosinophils with Langerhans cells that stained positive for S-100. Langerhans cell histiocytosis was diagnosed, and the patient was discharged on postoperative day 6 with no neurological deficits. His diabetes insipidus persisted, and he underwent intensity-modulated radiation therapy to the sellar region. CONCLUSIONS: Isolated Langerhans cell histiocytosis of the infundibulum is rare. The usual presentation is hypothalamic-pituitary axis dysfunction and diabetes insipidus. Patients with diabetes insipidus of unknown origin should undergo MRI of the sellar region to rule out infundibular abnormalities.

Adolescent↗

Correlation between magnetic resonance imaging of posterior pituitary and neurohypophyseal function in children with diabetes insipidus.

The posterior pituitary lobe and stalk were studied by magnetic resonance imaging in 20 children with diabetes insipidus of different origins: primary familial autosomal dominant (n = 2) or idiopathic (n = 2), and secondary to craniopharyngioma (n = 6, resected in 5), to Langerhans cell histiocytosis (n = 5), to excessive water intake (dipsogenic; n = 3), to renal vasopressin insensitivity (n = 1), and to osmoreceptor dysfunction (n = 1). Of the four children with primary diabetes insipidus, the posterior bright signal was recognizable in two with the familial autosomal dominant form and one with the idiopathic form; in the latter, the pituitary stalk was thin, while it was normal in the first two patients; no posterior hyperintense signal with enlarged and gadolinium-enhanced pituitary stalk was observed in the fourth. The posterior hyperintense signal was absent without evidence of ectopic posterior pituitary tissue regeneration in five children with surgically removed craniopharyngioma and was doubtful in the child with unresected craniopharyngioma; the stalk was unrecognizable in all patients. In the five children with Langherans cell histiocytosis, the posterior bright signal was absent, while the stalk was normal in two and unexpectedly enlarged in three (uniformly in two and mainly at the level of median eminence and hypothalamus in one). All five patients with dipsogenic or nephrogenic diabetes insipidus or osmoreceptor dysfunction had normal images of posterior pituitary lobe and stalk. Normal posterior pituitary bright signal and stalk were found in all 25 healthy control children. Plasma vasopressin was undetectable in all patients except in nephrogenic one, in the child with osmoreceptor dysfunction, and in two of three dipsogenic children, the third mimicking partial neurogenic diabetes insipidus.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

The renal pathology in a case of lithium-induced diabetes insipidus.

A case of lithium-induced diabetes insipidus is reported. At necropsy microscopy shoed unique and extensive damage to cells lining the distal nephron. It is suggested that these changes represent a specific toxic effect of lithium, reported here for the first time in man.

Adult↗

[Gestational diabetes insipidus].

We report a case of transient diabetes insipidus in late pregnancy. This rare condition is characterised by excessive thirst and polyuria and is associated with pre-eclampsia. The aetiology is most likely due to increased vasopressinase activity. Suspected cases should be referred to an obstetric centre, since termination of pregnancy is recommended. The condition normally resolves within a few days of delivery.

Adult↗

Hypernatremia in a non insulin dependent (type 2) diabetic patient with central diabetes insipidus.

We describe a patient with central diabetes insipidus who presented with hyperosmolar, non-ketotic hyperglycaemia. The role in this case of reduced thirst sensation with decreased water intake and abnormal AVP production illustrates the importance of these protective mechanisms in normal physiology regarding maintenance of normal plasma osmolality. Despite the complex pathophysiology in this patient, fluid resuscitation aimed at normalisation of the water deficit resulted in full recovery.

Brain Neoplasms↗

Anitdiuretic effect of carbamazepine in diabetes insipidus.

A study was made of the effect of the antiepileptic carbamazepine on the water metabolism in 13 vasopressin-sensitive and 4 ADH-resistant diabetes insipidus patients. It was found that in 12 ADH-sensitive diabetes insipidus cases the drug decreased the urinary output and the free water clearance, and increased the urinary osmolarity. The diuresis did not change in ADH-resistant diabetes insipidus patients. The investigations suggest that carbamazepine exerts an ADH-like effect, and can be applied with good results in the treatment of ADH-sensitive diabetes insipidus.

Adolescent↗

Diabetes insipidus.

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Diabetes Insipidus↗

Diabetes insipidus.

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Diabetes Insipidus↗