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Transient gestational diabetes insipidus: report of two cases and review of pathophysiology and treatment.

Gestational diabetes insipidus is a rare disorder characterized by polyuria and polydipsia due to the inability of the kidneys to concentrate urine. We report two cases of transient gestational diabetes insipidus in which patients responded to intranasal DDAVP (1-deamino-8-D-arginine vasopressin) with greater than 50% increase in urine osmolality and marked reduction in urine output. Intranasal DDAVP was discontinued after their discharge and both patients maintained normal urine output and appropriate urine osmolality. In determining whether diabetes insipidus is present in a patient who is polyuric and hypernatremic, a urine osmolality below that of the plasma suggests the presence of diabetes insipidus. Understanding of the pathophysiology may soon lead to improved methods of prevention, diagnosis and treatment.

Adult↗

Diabetes insipidus.

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Diabetes Insipidus↗

Diabetes insipidus and histiocytosis X: a case of isolated pituitary involvement.

Diabetes insipidus is associated with histiocytosis X in approximately 50% of cases with multiple lesions that involve both intracranial and extracranial structures. Although approximately 8% of cases of diabetes insipidus in children are caused by involvement of the hypothalamus or pituitary infundibulum (or both) by histiocytosis X, diabetes insipidus can be the initial manifestation in many disorders most commonly, intracranial neoplasms. Herein we report a case of isolated histiocytosis X of the pituitary gland in a child for whom medical assistance was sought because of symptoms of diabetes insipidus.

Journal Article↗

[Familial occurrence of central diabetes insipidus].

Two cases are presented of familial diabetes insipidus with analysis of pedigree involving four generations. The analysis of diabetes insipidus occurrence in this family indicates the autosomal dominant type of inheritance.

Adult↗

Molecular biology of hereditary diabetes insipidus.

The identification, characterization, and mutational analysis of three different genes-the arginine vasopressin gene (AVP), the arginine vasopressin receptor 2 gene (AVPR2), and the vasopressin-sensitive water channel gene (aquaporin 2 [AQP2])-provide the basis for understanding of three different hereditary forms of "pure" diabetes insipidus: Neurohypophyseal diabetes insipidus, X-linked nephrogenic diabetes insipidus (NDI), and non-X-linked NDI, respectively. It is clinically useful to distinguish two types of hereditary NDI: A "pure" type characterized by loss of water only and a complex type characterized by loss of water and ions. Patients who have congenital NDI and bear mutations in the AVPR2 or AQP2 genes have a "pure" NDI phenotype with loss of water but normal conservation of sodium, potassium, chloride, and calcium. Patients who bear inactivating mutations in genes (SLC12A1, KCNJ1, CLCNKB, CLCNKA and CLCNKB in combination, or BSND) that encode the membrane proteins of the thick ascending limb of the loop of Henle have a complex polyuro-polydipsic syndrome with loss of water, sodium, chloride, calcium, magnesium, and potassium. These advances provide diagnostic and clinical tools for physicians who care for these patients.

Animals↗

Juvenile diabetes mellitus, optic atrophy, sensory nerve deafness, and diabetes insipidus--a syndrome.

Four patients with diabetes mellitus, optic atrophy, and high-frequency neurosensory hearing loss, two of whom also had diabetes insipidus, are described. The frequency of this syndrome among patients with juvenile diabetes appears to be between 1/148 and 1/175. Because of the progressive nature of the disabilities and the autosomal recessive mode of inheritance, careful monitoring of all juvenile diabetic patients for other signs of the syndrome is warranted.

Adolescent↗

Diabetes insipidus associated with leukaemia.

Three cases of leukaemia complicated by pituitary diabetes insipidus are presented. In one patient diabetes insipidus developed five months before acute myelomonocytic leukaemia became apparent. The two other patients suffered from acute myelomonocytic leukaemia and from a blastic transformation of chronic myeloid leukaemia. In one case a transient cure of diabetes insipidus was obtained after intrathecal chemotherapy and irradiation of the skull.

Adult↗

Co-existent diabetes mellitus and diabetes insipidus, a familial disease.

Three male siblings with diabetes mellitus are described, two of whom also had coexistent diabetes insipidus. The co-existence of diabetes mellitus and insipidus appears to represent a single genetic abnormality and may or may not be accompanied by primary optic atrophy. Chlorpropamide was effective in controlling the symptoms of diabetes mellitus and diabetes insipidus.

Adult↗

[Anterio-hypophyseal function in central diabetes insipidus].

In our recent experience, the posttraumatic diabetes insipidus (PT.DI) and idiopathic (I.DI) are the most common forms of central diabetes insipidus (C.DI) in adult patients. The hypothalamo-pituitary function in these patients may be quite heterogeneous. We evaluated this aspect in 32 patients with different forms of C.DI (19 males and 13 females; aged 16-55 yrs): 12 with previous severe cranial or general trauma; 8 with CNS lesion due to Tuberculosis, Sarcoidosis, Histiocytosis X or to other pathogenic noxa (Secondary DI); 12 with idiopathic form. In all we measured ACTH, TSH, FSH, LH, PRL and target hormones (pl. cortisol, T3 T4, Testosterone) in baseline conditions with and without substitutive DDAVP therapy. In all cases the hormonal pattern was within the normal range. In several patients stimulation test with specific releasing factors (TRH, LHRH, oCRH) were carried out. Although basal anterior pituitary function is usually normal in patients with central DI (post-traumatic, idiopathic or secondary), an isolated subclinical secondary or tertiary hypothyroidism can be observed in some cases. Thus, a more accurate, periodical, and complete hormonal evaluation is indicated in some patients. The maintained response of ACTH to CRH (even increased after acute withdrawal therapy) indicated that AVP is not necessary to ensure normal function to the CRH-ACTH axis.

Adolescent↗