| Multicomponent gas chromatographic analysis of urinary steroids excreted by an infant with a defect in aldosterone biosynthesis. | |
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MedLine Citation:
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PMID: 946566 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The urinary steroids excreted by an infant with a salt-wasting syndrome due to a suspected defect in the 18-oxidation of corticosterone have been analysed by gas chromatography-mass spectrometry. The excretion of tetrahydroaldosterone was low (3.5 mug/24 h) whilst the excretion of 3alpha,11beta,21-trihydroxy-5alpha-pregnan-20-one (allo-tetrahydrocorticosterone) and other corticosterone metabolites was high (total about 2 mg/24 h). The excretion of cortisol metabolites was apparently normal for age (total about 2 mg/24 h) but 3alpha,11beta,17alpha,21-tetrahydroxy-5alpha-pregnan-20-one (allo-tetrahydrocortisol) rather than tetrahydrocortisone, was the major component of the group. The excretion of an 18-hydroxycorticosterone metabolite 3alpha,18,21-trihydroxy-5beta-pregnane-11,20-dione (18-hydroxytetrahydroCompound A) was higher than normal for infants of this age (between 50 and 200 mug/24 h), suggesting that the defect was in 18-hydroxysteroid dehydrogenase rather than 1,-hydroxylase. In addition, 18-hydroxytetrahydrocorticosterone, another metabolite of 18-hydroxycorticosterone was tentatively identified and it was found that the rate of excretion of this compound was of similar magnitude to 18-hydroxytetrahydroCompound A. The salt balance of the infant has been sucessfully controlled by salt administration (77 mEq./24 h) and treatment with Fludrocortisone (0.5 mg/day). |
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Authors:
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C H Shackleton; J W Honour; M Dillon; P Milla |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Acta endocrinologica Volume: 81 ISSN: 0001-5598 ISO Abbreviation: Acta Endocrinol. Publication Date: 1976 Apr |
Date Detail:
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Created Date: 1976-06-02 Completed Date: 1976-06-02 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0370312 Medline TA: Acta Endocrinol (Copenh) Country: DENMARK |
Other Details:
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Languages: eng Pagination: 762-73 Citation Subset: IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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18-Hydroxycorticosterone
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urine Aldosterone / analogs & derivatives, biosynthesis*, urine Chromatography, Gas / methods Corticosterone / urine* Humans Hydrocortisone / urine* Hydroxysteroid Dehydrogenases / deficiency Infant Male Metabolic Diseases / blood, drug therapy, urine* Potassium / blood Sodium / blood Sodium Chloride / metabolism, therapeutic use Tetrahydrocortisol / urine Tetrahydrocortisone / urine |
| Chemical | |
Reg. No./Substance:
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50-22-6/Corticosterone; 50-23-7/Hydrocortisone; 52-39-1/Aldosterone; 53-02-1/Tetrahydrocortisol; 53-05-4/Tetrahydrocortisone; 561-65-9/18-Hydroxycorticosterone; 7440-09-7/Potassium; 7440-23-5/Sodium; 7647-14-5/Sodium Chloride; EC 1.1.-/Hydroxysteroid Dehydrogenases |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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