Nephrogenic diabetes insipidus and intracerebral calcification

Article Abstract:

Nephrogenic diabetes insipidus and intracerebral calcification are two rare conditions. Nephrogenic diabetes insipidus is characterized by unresponsiveness of the distal tubule and collecting duct of the kidney to normal or increased concentrations of the antidiuretic hormone vasopressin. The primary form of this condition is believed to be inherited and linked to the X chromosome, while the secondary form is related to certain neoplastic diseases, medullary cystic disease, and hypercalcemia (a condition of excess calcium in the blood). Skull X-rays from 34,941 patients were examined. Pathological calcification was seen in 293 of them. These calcifications were associated with prenatal infections with Toxoplasma gondii, inflammatory brain diseases, central nervous system tumors, intracranial hemorrhage, and parathyroid disorders. Three cases are described; two boys and one girl with the combination of nephrogenic diabetes insipidus and massive intracerebral calcification. No underlying pathology other than the nephrogenic diabetes insipidus was found to explain the intracerebral calcification. The association of these two disorders is believed to have been previously unreported. The association of these two rare conditions suggests that there may be a common pathogenesis. It would be useful to investigate a large group of patients with nephrogenic diabetes insipidus during infancy to determine the incidence of intracerebral calcification. (Consumer Summary produced by Reliance Medical Information, Inc.)

Brain, Calcification

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Continuous vasopressin replacement in diabetes insipidus

Article Abstract:

Diabetes insipidus, a condition of excessive urination caused by inadequate secretion of vasopressin, is a well recognized complication of severe brain injury. Damage to the hypothalamoneurohypophyseal axis, a region of the brain, results in the impairment of vasopressin secretion. If left untreated, the child can develop dehydration, low blood pressure, and other fluid and electrolyte disturbances. Intravenous vasopressin and appropriate fluids were administered to five children who developed diabetes insipidus following brain injury. All children were in the intensive care unit, and levels of fluids and electrolytes, particularly sodium, and urine output were carefully monitored. Injection of an aqueous solution of the antidiuretic hormone vasopressin was valuable in the acute stages of diabetes insipidus for treating these patients. The treatment regimen employed resulted in a steady decrease in the concentration of sodium in the blood, and dose regulation corresponded to changing hourly urine production. Vasopressin administration was effective, safe, and convenient when given to these patients with head injuries. This agent may also be useful in the treatment of diabetes insipidus following neurosurgery; however, further study is needed. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Care and treatment, Drug therapy, Juvenile diabetes, Diabetes in children, Vasopressin, Vasopressins

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Nephrogenic diabetes insipidus: a personal perspective

Article Abstract:

Cases of nephrogenic diabetes insipidus among members of a multigenerational family illustrate the interplay between genes, life experiences, development, and the biological appearance of the disease. Nephrogenic diabetes insipidus is a metabolic condition characterized by excessive thirst and urination. The family matriarch carried the gene for the disease and had mild symptoms. One of her daughters who was also a carrier bore 10 children to four husbands. Five sons and two daughters were affected to varying degrees by the disease. Males exhibited the most severe form of the disease that is characterized by dehydration, growth failure, mental retardation, and water-electrolyte imbalances. The frequent need to urinate and the unquenchable thrist experienced by all affected family members interrupted their daily activities, social adaptation, and family life.

Familial diseases

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