Linked Life Data

161161

Source:http://linkedlifedata.com/resource/pubmed/id/161161

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
7
pubmed:dateCreated
1980-4-23
pubmed:abstractText
Five cases of congenital 3 beta-hydroxysteroid deshydrogenase deficiency in children are reported: four boys with perineal posterior hypospadias and one girl with clitoromegaly. The salt losing syndrome was clinically overt in only three patients. The main biological character was the very high level of plasma dehydroepiandrosterone (DHA) with an elevated DHA/delta 4 androstenedione ratio. The 17 alpha-OH progesterone, though in normal biosynthesis of glucocorticoids being produced beyond the enzymatic block, was raised, but this apparently paradoxical observation may assist making the diagnosis. Deficient production of testosterone was demonstrated in the prepubertal boys by absence of postnatal rise in plasma testosterone or a decreased reponse of plasma testosterone to chorionic gonadotrophin. It is concluded that deficiency of 3 beta-hydroxysteroid deshydrogenase, now easily recognizable with the use of plasma steroids radioimmunoassay, is probably less rare than was apparent with the use of urinary steroid estimations.
pubmed:language
fre
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
0003-9764
pubmed:author
pubmed:issnType
Print
pubmed:volume
36
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
647-55
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:articleTitle
[Congenital adrenal hyperplasia due to blockade of 3-beta-hydroxysteroid dehydrogenase].
pubmed:publicationType
Journal Article, English Abstract, Case Reports