Cytomegalic Adrenocortical Hypoplasia and Increased Plasma 20α-Hydroxypregn-4-en-3-one in a Man Exhibiting the Features of Selective Mineralocorticoid Deficiency1
| Autor: | Francis H. Straus, Edward N. Ehrlich, Robert L. Hunter, Walter G. Wiest |
|---|---|
| Rok vydání: | 1969 |
| Předmět: |
endocrine system
Pregnanetriol medicine.medical_specialty medicine.drug_class Endocrinology Diabetes and Metabolism Urinary system medicine.medical_treatment Biochemistry (medical) Clinical Biochemistry Autopsy medicine.disease Biochemistry Steroid Excretion chemistry.chemical_compound Endocrinology chemistry Mineralocorticoid Internal medicine Pregnanediol medicine Hypoaldosteronism |
| Zdroj: | The Journal of Clinical Endocrinology & Metabolism. 29:523-538 |
| ISSN: | 1945-7197 0021-972X |
| DOI: | 10.1210/jcem-29-4-523 |
| Popis: | This report describes a man who developed adrenocortical dysfunction late in adult life, characterized mainly by manifestations of aldosterone deficiency; 17-hydroxycorticoids were normal but unresponsive to exogenous ACTH; urinary 17-ketosteroids were high normal; urinary pregnanetriol was slightly elevated and pregnanediol was increased substantially. These findings at first suggested that this case might represent a peculiar variant of the salt-losing type of CAH.3 However, in view of the atypical preponderance of urinary pregnanediol rather than pregnanetriol, plasma steroid analyses were performed to determine whether the increased urinary pregnanediol was derived from progesterone, which would be expected if it reflected a 21-hydroxylation defect. Surprisingly, the plasma progesterone level was very low, but the plasma contained a high concentration of 20α-OH-P. These findings suggested that the increased excretion of urinary pregnanediol was derived from 20α-OH-P rather than progesterone. ... |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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