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Simultaneous occurrence of diabetes mellitus, diabetes insipidus, and optic atrophy in a brother and sister.

We report the cases of two siblings who died at age 21 and 15 years respectively. Both had optic atrophy, pitressinsensitive diabetes insipidus, and insulin-dependent diabetes mellitus, with onset occurring in early childhood. Although there are now 21 patients from 15 families with this syndrome, this is the first time that necropsy findings have become available. They include the expected atrophy of hypothalamic nuclei and degeneration of the optic nerves, chiasm and tract, as well as a totally unexpected degeneration of the pons and cerebellum.

Adolescent↗

[Case of severe motor and intellectual disabilities presenting with partial central diabetes insipidus triggered by infection].

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.

Adult↗

Lymphocytic hypophysitis with central diabetes insipidus and subsequent hypopituitarism masking a suprasellar germinoma in a 13-year-old girl.

CASE REPORT: We report a case of central diabetes insipidus, hypothyroidism, and subsequent hypopituitarism due to lymphocytic hypophysitis masking a germinoma in a 13-year-old pubertal girl. Magnetic resonance revealed an enlarged pituitary gland and a mass lesion in the pituitary stalk and inferior hypothalamus. Open cranial surgery of the anterior pituitary showed active hypophysitis with lymphocytic infiltrates but without necrosis. Despite prednisolone therapy, 1 year later an enlarged, irregular cystic mass lesion had developed; in the pituitary stalk and inferior hypothalamus, a endoscopic biopsy revealed germinoma. CONCLUSION: Lymphocytic hypophysitis in children may be the first sign of a host reaction to an occult germinoma. The diagnosis of central diabetes insipidus with a thickened pituitary stalk requires long-term follow-up to establish the underlying cause.

Adolescent↗

Atrial natriuretic peptides in brain nuclei of rats with inherited diabetes insipidus (Brattleboro rats).

The concentration of atrial natriuretic peptides (ANF) was measured radioimmunologically in 18 selected microdissected brain areas of rats with inherited diabetes insipidus (Brattleboro rats) and their control rats (Long-Evans rats). In 7 brain areas known to be involved in the regulation of fluid homeostasis or blood pressure (subfornical organ, organum vasculosum lamina terminalis, periventricular preoptic nucleus, perifornical nucleus, nucleus of the solitary tract, tegmentum pontis, arcuate nucleus) ANF concentration was significantly changed. After restoration of antidiuresis with the V2 receptor agonist 1-desamino-8-D-arginine vasopressin (DDAVP) ANF levels of Brattleboro rats adapted at least partly to those of the control rats in all brain areas except the subfornical organ. Furthermore, ANF was then significantly changed in the supraoptic and paraventricular nuclei of DDAVP-treated diabetes insipidus rats. These data show that the central ANF system is sensitive for changes in electrolyte and fluid homeostasis.

Animals↗

Generalized status epilepticus associated with massive pulmonary aspiration and transient central diabetes insipidus: case report.

Status epilepticus causes significant morbidity and mortality. A case of generalized status epilepticus followed by massive pulmonary aspiration, acute respiratory failure and transient central diabetes insipidus is presented. Seizures were promptly controlled, but the patient required mechanical ventilation and correction of polyuria with desmopressin acetate. During hospitalization mental status improved, diabetes insipidus spontaneously remitted and he was discharged without neurologic sequelae. The clinical and pathophysiological features of this case are discussed.

Acute Disease↗

Nephrogenic diabetes insipidus and tubulointerstitial nephritis during continuous therapy with rifampin.

While receiving continuous daily rifampin therapy, a 57-year-old man developed acute renal failure and nephrogenic diabetes insipidus due to acute tubulointerstitial nephritis which was reversible by discontinuing the rifampin. Tubulointerstitial nephritis rarely develops during continuous rifampin therapy, and associated nephrogenic diabetes insipidus has not previously been reported. The majority of cases of tubulointerstitial nephritis due to rifampin have occurred following reintroduction of rifampin after an interruption in therapy. The clinical differences between patients developing tubulointerstitial nephritis during interrupted and continuous therapy are discussed.

Acute Kidney Injury↗

Hypopituitarism associated with transient diabetes insipidus followed by an episode of painless thyroiditis in a young man.

A 16-year-old male complained of a headache and a high fever followed by polyuria. The endocrinological studies showed he had hypopituitarism and central diabetes insipidus, and magnetic resonance imaging (MRI) revealed a pituitary mass. Diabetes insipidus gradually improved and hydrocortisone treatment was begun at three months after onset, but a month later painless thyroiditis developed. An MRI demonstrated a spontaneous shrinkage of the pituitary mass nine months after onset. Lymphocytic hypophysitis followed by painless thyroiditis was the most probable diagnosis, although it is very uncommon especially among men.

Adolescent↗

Acute myelogenous leukemia (AML) and diabetes insipidus (DI): further association with monosomy 7.

Diabetes insipidus (DI) is a rare complication of acute myelogenous leukemia (AML). Five of the six such cases described in the literature who had banded chromosomal studies performed had monosomy 7 and the sixth patient had del(7)(q22). A further case of AML complicated by DI in whom banded chromosomal studies revealed a complex karyotypic abnormality, including monosomy 7, is reported. The association between monosomy 7 and DI in AML appears specific but the reason for this association remains unclear.

Chromosomes, Human, Pair 7↗