Complications of diazoxide treatment in persistent neonatal hyperinsulinism

Article Abstract:

Hyperinsulinemia is a condition involving excess blood levels of insulin that is caused by the overproduction of insulin by the pancreas, which in turn results in hypoglycemia (very low levels of blood sugar). The initial treatment of hypoglycemia usually combines drug therapy with an adequate intake of glucose. One drug commonly used to normalize blood glucose levels is diazoxide. The treatment of seven infants with diazoxide is described. Out of 11 different instances of drug treatment, signs of cardiorespiratory failure (indicative of diazoxide toxicity) developed in eight. In each case, the ratio of insulin concentration to glucose concentration was greater than 66 and extended to 636; in the three times when signs of cardiorespiratory failure did not appear, the ratio of insulin to glucose was less than 33. A drug toxicity index was defined to relate the drug dose with the severity of the disease (insulin/glucose ratio multiplied by diazoxide dose) and to predict the chance of developing drug toxicity. The three times no symptoms of toxicity occurred, the toxicity index was below 676. However, the toxicity index was greater than 1532 when toxicity symptoms developed. Diazoxide is effective in controlling blood glucose levels during hyperinsulinemia, but serious complications have been reported. The toxicity index as described may be a useful way to identify those patients at high risk for complications during diazoxide treatment. (Consumer Summary produced by Reliance Medical Information, Inc.)

Drug therapy, Hyperglycemia, Diazoxide

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Short stature, mental retardation, and hypoparathyroidism: a new syndrome

Article Abstract:

This article describes a newly recognized pattern of congenital anomalies in eight children born in seven families of Middle Eastern origin. In each case, the parents were first cousins, and it is presumed the syndrome is the result of expression of one or more recessive genes, genes that are not expressed in the presence of their dominant partner gene. Half the children were female, and there were five siblings among the families who died under one year of age. All children were of low birth weight and had deformed, but distinctively similar, facial features. The children had low blood calcium levels, related to low or abnormal levels of parathyroid hormone, which, with vitamin D, regulates calcium metabolism. The children were delayed in movement and speech development and grew extremely poorly in terms of weight, height, and head size. Abnormalities in long bone structure and other bones were also present. Half of the children had abnormal levels of white blood cells, and overwhelming infections were common in the children. The grouping of these features has not been previously described. Intermarriage among close relatives is highly prevalent in some Middle Eastern populations, which increases the probability of recessive conditions. This description may allow earlier recognition and treatment of affected children. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Genetic aspects, Developmental disabilities, Hypoparathyroidism, Cross-cousin marriage, Cross cousin marriage

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