Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To evaluate whether frank or subtle disorders of adrenal steroidogenesis exist in human immunodeficiency virus (HIV)-infected children, the adrenal steroid response to an iv bolus of ACTH-(1-24) (0.25 mg Cortrosyn) was determined. Ten children (six males and four females, aged 7 months to 7.5 yr) were studied. Five underwent repeat testing 3-5 months after initial assessment. Nine patients were classified as P2 or symptomatic according to the Center for Disease Control criteria for HIV infection in children. Eight had failure to thrive, six had opportunistic infections and neurological deficits, and two were receiving ketoconazole at the time of ACTH testing. Only one patient had a neonatally acquired transfusion-related HIV infection. Three of the children died 2-5 months after ACTH testing. All patients had normal or slightly elevated baseline and stimulated cortisol levels compared to the control population. The mean post-ACTH cortisol level was significantly higher than the mean post-ACTH level in the control population. No patient demonstrated an impaired aldosterone response to ACTH. The basal and ACTH-stimulated dehydroepiandrosterone levels were normal. Although individual deoxycorticosterone and corticosterone levels were variable, the mean stimulated deoxycorticosterone and corticosterone levels in the patients were suggestive of a selective defect of the 17-desoxy pathway in the adrenal fasciculata. No changes were noted in the patients' cortisol, dehydroepiandrosterone, and aldosterone responses on repeat ACTH testing. In HIV-infected children we have demonstrated that cortisol and aldosterone synthesis is intact. Thus, the chronic debilitation observed cannot be explained on the basis of adrenal insufficiency. However, a selective deficiency of 17-desoxysteroid hormone production from the adrenal fasciculata may be present.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Steroid response to adrenocorticotropin stimulation in children with human immunodeficiency virus infection. 230 20