| Acute adrenal crisis complicating hypertensive congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. | |
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MedLine Citation:
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PMID: 8448909 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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In its classical form, congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency is characterized by hypertension and abnormal sexual development. Suppression of ACTH secretion by means of administering glucocorticoids fulfills the therapeutic goal of reducing blood pressure and decreasing androgen production. The present report describes the case of a patient suffering from congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency who developed an acute adrenal crisis, unprovoked by stress, following interruption of glucocorticoid replacement therapy. It is suggested that patients on a suppressive dose of glucocorticoids for adrenal hyperplasia are at increased risk for acute adrenal insufficiency if therapy is interrupted, and that deoxycorticosterone (DOC) in the absence of a glucocorticoid is insufficient to prevent manifestations of adrenal crisis. |
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Authors:
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Y Liel |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Clinical genetics Volume: 43 ISSN: 0009-9163 ISO Abbreviation: Clin. Genet. Publication Date: 1993 Feb |
Date Detail:
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Created Date: 1993-04-09 Completed Date: 1993-04-09 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0253664 Medline TA: Clin Genet Country: DENMARK |
Other Details:
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Languages: eng Pagination: 92-3 Citation Subset: IM |
Affiliation:
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Endocrine Unit, Soroka University Hospital, Kupat-Holim, Israel. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Acute Disease Adrenal Hyperplasia, Congenital / complications*, drug therapy Adrenal Insufficiency / drug therapy, etiology* Adult Dexamethasone / therapeutic use Epinephrine / therapeutic use Female Humans Hydrocortisone / therapeutic use Male Patient Compliance |
| Chemical | |
Reg. No./Substance:
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50-02-2/Dexamethasone; 50-23-7/Hydrocortisone; 51-43-4/Epinephrine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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