The frequency and natural history of diabetes insipidus in children with Langerhans-cell histiocytosis

Article Abstract:

Histiocytosis X is a rare disease of childhood in which there is an increased number of cells of a particular type, histiocytes. The disease has many forms, ranging from a disease of a single bone (often the skull) to a very dangerous disease of infants that resembles leukemia (white blood cell cancer). Diabetes insipidus, not to be confused with the more common diabetes mellitus, is a frequent complication of histiocytosis, but it is not well understood. The current study documents the natural history of Langerhans-Cell histiocytosis which frequently produces pathologies of the posterior (neural) portion of the pituitary gland, causing the pituitary to fail to manufacture or release adequate supplies of a hormone, vasopressin or antidiuretic hormone (ADH). ADH causes the kidneys to resorb water back into the circulatory system that had accompanied the active excretion of electrolytes . When ADH levels are low, a severe loss of fluid occurs from excessive urination, and resulting in diabetes insipidus, where the loss of even 20 quarts of fluid in a single day is not unusual. A group of 52 children with histiocytosis were studied, 2 of which had symptoms of diabetes insipidus when first seen. A total of 12 of these children ultimately developed diabetes insipidus (42 percent). The development of the disease was measured by depriving the children of water and measuring the urinary levels and blood levels of vasopressin. When the 21 children who had not developed diabetes insipidus were tested, 5 had subnormal levels of ADH. Two of these five later developed diabetes insipidus. The study demonstrates that the use of a simple water-deprivation test can examine defects of the posterior pituitary in children with histiocytosis X and may lead to improved therapy.

Measurement, Complications and side effects, Development and progression, Reticuloendotheliosis, Endocrine gland diseases, Endocrine diseases

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Molecular insights into diabetes mellitus

Article Abstract:

Recent studies on nephrogenic diabetes insipidus offer insights to the cause of the disease but little hope for a cure. Nephrogenic diabetes insipidus is a rare, inherited disease in which the kidneys do not respond to vasopressin. Vasopressin is a hormone that regulates the excretion of water in the urine. Vasopressin binds to two receptors: V1 and V2. Patients with nephrogenic diabetes insipidus have an impaired V2 receptor. Using DNA analysis, two recent family studies of the disorder identified a mutation in the gene for the vasopressin V2 receptor that appears to be the cause of nephrogenic diabetes insipidus. This knowledge, however, will do little to improve therapy because technology is not capable of altering or replacing malfunctioning receptors.

Editorial

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Hypoglycemia due to an insulin-secreting small-cell carcinoma of the cervix

Article Abstract:

Non-islet-cell tumors may be capable of producing insulin and therefore causing low blood sugar (hypoglycemia). Most tumors that produce insulin occur in the islet cells of the pancreas, where insulin is normally produced. Researchers describe the case of a 29-year-old woman with cervical cancer who had hypoglycemia. Her blood insulin level was found to be 200 times higher than normal. Her symptoms of hypoglycemia were relieved by intravenous glucose. An autopsy following her death revealed that the cervical tumor contained insulin and also produced other pancreatic hormones. However, her pancreas was healthy and contained no cancer.

Health aspects, Cervical cancer, Insulin, Hypoglycemia

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