Late-onset adrenal hyperplasia in hirsutism.

@article{Kuttenn1985LateonsetAH,
  title={Late-onset adrenal hyperplasia in hirsutism.},
  author={F. Kuttenn and P. Couillin and F. Girard and L. Billaud and M. Vincens and C. Boucekkine and J. Thalabard and T. Maudelonde and P. Spritzer and I. Mowszowicz},
  journal={The New England journal of medicine},
  year={1985},
  volume={313 4},
  pages={
          224-31
        }
}
We studied the incidence of late-onset adrenal hyperplasia as a cause of hirsutism, its association with the major histocompatibility complex, and its clinical expression. Twenty-four of 400 women seen because of hirsutism were found to have late-onset adrenal hyperplasia, diagnosed on the basis of a high plasma level of 17-hydroxyprogesterone, and its marked increase after ACTH stimulation. The degree of hirsutism varied widely. Plasma antigen levels were high, especially the level of… Expand
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It is apparent that the determination of basal plasma 17-OHP may be sufficient to discover late-onset 21-hydroxylase deficiency, and therefore ACTH testing is not considered an advisable step in the routine screening for hirsutism. Expand
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It is concluded that the adrenocorticotropic hormone stimulation test should be more widely utilized in patients presenting with hirsutism or menstrual dysfunction. Expand
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Some recent studies have shown that the ovary contributes to the hyperandrogenism of patients and some alternative therapies have been proposed, which forms the matter of the present review. Expand
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The incidence of NC21OHD in hirsute adolescent girls in this population was investigated, and it was found in only one of 32 patients, and human leukocyte antigen (HLA) analysis supported these diagnoses. Expand
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The aims of the treatment are to suppress androgen overproduction, if present; to block androgen action on hair follicles; to identify and treat patients at risk for metabolic disturbances or reproductive neoplasias. Expand
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TLDR
Late‐onset adrenal hyperplasia due to partial deficiency of this enzyme should always be considered as a possible diagnosis in women who present with symptoms of hyperandrogenism, and synacthen stimulation is an important diagnostic tool in elucidating partial enzyme deficiency as baseline 17‐hydroxyprogesterone may be normal in such patients. Expand
21-Hydroxylase deficiency in female hyperandrogenism: screening and diagnosis.
TLDR
In this population of hyperandrogenic women the 3 measures of 21-hydroxylase studied clearly differentiated the LOAH women from all others, although a single 17-HP level 30 min post-ACTH was the simplest and most cost effective. Expand
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Late-onset congenital adrenalhyperplasia appears to be an allelic variant of congenital virilizing adrenal hyperplasia with a milder enzymatic defect. Expand
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Severe hirsutism, virilization, early onset of symptoms, short stature, familial occurrence, and regular menses were identified as the clinical characteristics associated with late-onset CAH. Expand
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Serum 17-hydroxyprogesterone (17OHP) was measured in normal women and 25 hirsute oligomenorrheic patients, five of whom were shown to have CAH, and human lymphocyte antigen (HLA) typing on family members indicated that the inheritance of the disorder may be linked to B antigens. Expand
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The study of families A and B shows that the so-called cryptic and attenuated forms of CAH have the same pathophysiological basis, and suggests that most nonclassical subjects are allelic compounds for variable degrees of severity in the mutation at the 21-hydroxylase locus. Expand
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