Clinical review 56: Nonclassic adrenal hyperplasia: current concepts.

Azziz, Ricardo; Dewailly, Didier; Owerbach, David

doi:10.1210/jcem.78.4.8157702

Cited by 158 publications

(132 citation statements)

References 11 publications

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“…Congenital adrenal hyperplasia was excluded with an intravenous adrenocorticotropic hormone (ACTH) stimulation test. To identify those patients who might be heterozygous for 21-hydroxylase defect, serum 11-desoxycorticosterone levels below 13 ng/mL and serum 17-hydroxyprogesterone levels below 10 ng/mL were considered normal 2122. Patients who had first- or second-degree relatives with established PCOS were also excluded.…”

Section: Methodsmentioning

confidence: 99%

Effect of thyroid hormone replacement therapy on ovarian volume and androgen hormones in patients with untreated primary hypothyroidism

Müderris

Boztosun

Öner

et al. 2011

Annals of Saudi Medicine

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BACKGROUND AND OBJECTIVES:Primary hypothyroidism may be associated with ovarian enlargement and/ or cyst formation. We evaluated the effect of thyroid hormone replacement therapy on hormonal changes, ovarian volume and sonographic appearance.DESIGN AND SETTING:Open, prospective study of women admitted to university gynecology clinic.PATIENTS AND METHODS:The study included 26 patients with untreated hypothyroidism who had polycystic (n=10) or normal-appearing (n=16) ovaries and 20 euthyroidic controls. Basal serum total testosterone, free testosterone, androstenedione, dehydroepiandosterone-sulfate, prolactin, estradiol, luteinizing hormone, follicle-stimulating hormone, free T3, free T4 and thyroid-stimulating horone, together with ovarian volumes, were determined and repeated after euthyroidism was achieved.RESULTS:Ovarian volumes of patients with hypothyroidism were significantly greater compared with controls, and their magnitudes diminished significantly during thyroid hormone replacement therapy. Hypothyroidic patients with polycystic ovaries had significantly higher serum free testosterone and dehydroepiandosterone-sulfate, but lower androstenodione levels compared with those who had normal-appearing ovaries. Serum total testosterone concentrations were significantly higher in hypothyroidic patients without polycystic ovaries, and thyroid hormone replacement therapy achieved a significant reduction in total as well as free testosterone.CONCLUSION:Severe longstanding hypothyroidism leads to increased ovarian volume and/or cyst formation. A decrease in ovarian volume, resolution of ovarian cysts and reversal of the polycystic ovary syndrome-like appearance, together with improvement in serum hormone levels, occurred after euthyroidism was achieved.

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Section: Methodsmentioning

confidence: 99%

Effect of thyroid hormone replacement therapy on ovarian volume and androgen hormones in patients with untreated primary hypothyroidism

Müderris

Boztosun

Öner

et al. 2011

Annals of Saudi Medicine

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“…All had normal serum prolactin and thyroid function tests. Late-onset congenital adrenal hyperplasia was excluded by a serum 17α-hydroxyprogesterone level <10 nmol/l (3.0 μg/l) [26]. Pelvic ultrasound was not performed, and so this criterion of the 2003 Rotterdam consensus conference was not considered [1].…”

Section: Methodsmentioning

confidence: 99%

Brothers of women with polycystic ovary syndrome are characterised by impaired glucose tolerance, reduced insulin sensitivity and related metabolic defects

Baillargeon

Carpentier

2007

Diabetologia

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Aims/hypothesis Since it has been shown that polycystic ovary syndrome (PCOS) is highly inherited and characterised by insulin resistance, we hypothesised that male siblings of PCOS women would also be insulin resistant. Thus, our aim was to assess insulin sensitivity and metabolic parameters in brothers of women with PCOS and male control individuals. Methods Seventeen brothers of PCOS women and 28 male control volunteers were assessed with 75 g OGTTs and euglycaemic-hyperinsulinaemic clamps. PCOS index women were identified using criteria developed at the 1990 National Institutes of Health conference. Results Brothers and control individuals were similar in terms of BMI, waist circumference, percentage body fat and BP. However, brothers had increased triacylglycerol (p=0.02), plasminogen activator inhibitor-1 (PAI-1; p=0.02), factor VIII (p=0.02), 2 h glucose (p<0.001), AUC glucose (p<0.001) and AUC insulin (p<0.001). Insulin sensitivity was reduced by 38% in brothers (p<0.001), and this was primarily due to a 65% decrease in insulin-stimulated non-oxidative carbohydrate metabolism (p<0.001). These differences remained significant after adjustment for age and BMI, except for triacylglycerol, PAI-1 and fasting glucose. The main findings also persisted after excluding individuals with impaired glucose tolerance or diabetic siblings. Significant interactions with BMI status were found for sex hormone-binding globulin, androstenedione, PAI-1 and AUC insulin , which were significantly altered only in obese brothers (vs control individuals). Conclusions/interpretation Brothers of PCOS women are characterised by decreased insulin sensitivity and glucose tolerance, as well as hypercoagulability, independently of obesity. Therefore, brothers of PCOS women may have inherited the insulin resistance and metabolic syndrome typical of PCOS.

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“…A clear diagnosis of nonclassic congenital adrenal hyperplasia requires a steroid intermediate peak that is more than five standard deviations above average (eg, 17-hydroxyprogesterone over 1000 to 1500 ng/dL for 21-hydroxylase deficiency or 17-hydroxypregnenolone over 5000 ng/dL for 3β-hydroxysteroid dehydrogenase deficiency) [80,107,108]. Mildly elevated or normal responses to cosyntropin are consistent with FAH or idiopathic hyperandrogenism, respectively.…”

Section: Determining the Source Of Androgen Excessmentioning

confidence: 99%

“…The most common of these is nonclassic congenital adrenal hyperplasia caused by 21-hydroxylase deficiency [80]. This accounts for less than 5% of cases [13].…”

Section: Differential Diagnosismentioning

confidence: 99%

“…This accounts for less than 5% of cases [13]. Polycystic ovaries occur in about one-third of these, however [80], and the adrenal rests of the ovaries that can occur in this condition may be difficult to distinguish from PCOS [55]. Androgen-secreting tumors of the ovaries or adrenal glands occur in only about 0.2% of cases [81], but many are malignant.…”

Section: Differential Diagnosismentioning

confidence: 99%

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Polycystic Ovary Syndrome in Adolescence

Driscoll

2003

Semin Reprod Med

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Polycystic ovary syndrome (PCOS) is a syndrome of variable combinations of menstrual irregularity, hirsutism or acne, and obesity. It can be diagnosed in adolescence and has early childhood antecedents. PCOS is the single most common endocrine cause of anovulatory infertility and a major risk factor for the metabolic syndrome and, in turn, development of type 2 diabetes mellitus (T2DM) in women. Thus, it appears that PCOS increases a woman's risk of developing cardiovascular disease. Therefore, identifying girls at risk for PCOS and implementing treatment early in the development of PCOS may be an effective means of preventing some of the long-term complications associated with this syndrome. This article reviews the definition, clinical features, diagnosis, and treatment of PCOS. Definition Classic polycystic ovary syndromeThe classic syndrome originally was described in 1935 by Stein and Leventhal as the association of amenorrhea with polycystic ovaries in women, of whom about two thirds were hirsute, and one half were obese [1]. The term PCOS was introduced upon recognition of a broader spectrum of clinical symptoms and ovarian histology, including stromal hyperplasia with multiple subcapsular follicles. Approximately two-thirds of patients with classic PCOS have hirsutism (or hirsutism equivalents, acne vulgaris or pattern alopecia), two-thirds have anovulatory symptoms (manifested as amenorrhea, oligomenorrhea, dysfunctional uterine bleeding, or unexplained infertility), and one-half are obese. Thus, only about one-third of classic cases have the full-blown clinical picture (Fig. 1). The laboratory diagnostic criteria for classic PCOS require biochemical evidence of hyperandrogenism with either a polycystic ovary by ultrasound or an increased serum level of luteinizing hormone (LH) or LH to follicle-stimulating hormone (FSH) ratio. These criteria have proven to not necessarily coincide (Fig. 2). Nonclassic and atypical polycystic ovary syndromeIn 1990, the National Institutes of Health (NIH) Conference on PCOS considered the implications of recent research findings for the diagnosis [2]. Fifty percent to 60% of those present concurred that the criteria for PCOS should consist of chronic anovulation with clinical or biochemical signs of hyperandrogenism that was not explained by other etiologies. This recognized the spectrum of the syndrome to include androgen excess in the absence of ultrasonographic and gonadotropic abnormalities (here termed nonclassic PCOS). NIH-PA Author ManuscriptNIH-PA Author Manuscript NIH-PA Author ManuscriptThe androgen excess of PCOS usually results from characteristic types of functional ovarian hyperandrogenism (FOH) or functional adrenal hyper-androgenism (FAH) [3,4]. FOH is gonadotropin-dependent excessive ovarian androgen production, and it occurs in most women with classic PCOS. It is also found in an equal number of hyperandrogenic women who do not meet the criteria for classic PCOS. It is characterized by 17-hydroxyprogesterone hyper-responsiveness to gonadotropin...

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Clinical review 56: Nonclassic adrenal hyperplasia: current concepts.

Cited by 158 publications

References 11 publications

Effect of thyroid hormone replacement therapy on ovarian volume and androgen hormones in patients with untreated primary hypothyroidism

Effect of thyroid hormone replacement therapy on ovarian volume and androgen hormones in patients with untreated primary hypothyroidism

Brothers of women with polycystic ovary syndrome are characterised by impaired glucose tolerance, reduced insulin sensitivity and related metabolic defects

Polycystic Ovary Syndrome in Adolescence

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