Summary
Two new clinical subvariants of virilizing adrenal hyperplasia are described, together with a current classification of the recognized clinical patterns. The two new subvariants are virilizing adrenal hyperplasia with (1) “late” sodium loss and (2) periodic fever.
1.In the first instance a girl with virilizing adrenal hyperplasia who had shown no overt signs of sodium loss in the first year of life is described. At the age of 14 months she went into a severe sodium-losing crisis, without having a previous history of such episodes. She has required therapy with desoxycorticosterone since that time.
2.The second patient is a boy with virilizing adrenal hyperplasia who, since the early years of life, had episodes of periodic fever, headaches, and/or abdominal pain, chilly sensation and/or shaking chills, flushing of the face, head, and ears, and prostration. He occasionally had nausea and vomiting and in two instances diastolic hypotension. In most of these attacks leukocytosis was present. Plasma 17-ketosteroid analyses suggest that the episodes of periodic fever and other symptoms and signs may be related to the presence of etiocholanolone in plasma. A classification of the different subvariants of virilizing adrenal hyperplasia is presented.
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*** This study was supported in part by Research Grant A-2504 (C1) and Training Grant 2G-346, National Institutes of Health, United States Public Health Service.Presented in part at the IX International Congress of Paediatrics, Montreal, Canada, July 19 to 25, 1959.
PII: S0022-3476(60)80254-5
© 1960 Published by Elsevier Inc.
