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Query: UMLS:C0028754 (obesity)
 124,988 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The incidence of ultrasonically diagnosed polycystic ovaries (PCO) was studied in 389 Arab patients with different types of menstrual dysfunction and 100 normal women with regular menstruation. Two-hundred-and-forty-six patients (63.2%) were found to have PCO but only 206 (53.0%) were confirmed as cases of polycystic ovarian disease (PCOD) on endocrine grounds. Polycystic ovaries were diagnosed in 50% of patients with hyperprolactinaemia, 36.4% with hypothyroidism, 23.7% with hypothalamic dysfunction, 100% with adrenal 21-hydroxylase deficiency and in 16.0% of normal women. More women with PCOD presented with oligomenorrhoea or dysfunctional uterine bleeding (77.7%) and hirsutism (72.3%) but obesity had no discriminating value between the groups with different diagnoses. Ultrasonic diagnosis of PCO should be supplemented with an endocrine biochemical assessment to prevent overdiagnosis of PCOD and to exclude other endocrine dysfunctions.
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PMID:Implications of ultrasonically diagnosed polycystic ovaries. I. Correlations with basal hormonal profiles. 152 85

We have compared three treatments of congenital adrenal hyperplasia (CAH) for their effect on physical development. Thirteen girls and two boys with CAH due to 21-hydroxylase deficiency were treated with three different treatments, hydrocortisone (HC), dexamethasone (DXM) and cyproterone acetate (CA). The results showed that height growth was better with HC and CA than DXM, and bone excessive maturation was more suppressed with DXM and CA than HC. A dose-dependent relationship was revealed between body weight and dose of HC. Iatrogenic obesity was found in 42.9% and 38.1% of the patients treated with DXM and HC, but none of the patients treated with CA did became obese. Physical growth was better with CA treatment than HC or DXM treatment, but CA may have a suppressive effect on the pituitary-adrenal axis observed carefully, especially on prepubertal and pubertal cases.
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PMID:[A clinical study of congenital adrenal hyperplasia]. 255 16

A 29-year-old woman presented with hirsutism, obesity, oligomenorrhea, and infertility caused by oligoovulation and tubal occlusion. Partial 21-hydroxylase deficiency of the adrenal was suggested by an abnormal adrenocorticotropic hormone (ACTH) stimulation test. The patient subsequently developed bilateral tuboovarian abscesses and underwent abdominal hysterectomy and bilateral salpingo-oophorectomy. Thus, an opportunity was presented to study the adrenal endocrine disorder in the absence of ovaries and to investigate the effect of human chorionic gonadotropin (hCG) on the adrenals. There was no change in the adrenal response to ACTH stimulation after oophorectomy. hCG stimulation resulted in an increase in dehydroepiandrosterone sulfate and 17 beta-estradiol levels, suggesting that hCG had a stimulatory effect on the adrenal. The ovarian-adrenal relationship and effects of adrenal stimulation in the absence of ovaries are discussed.
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PMID:Effect of adrenocorticotropic hormone and human chorionic gonadotropin before and after bilateral oophorectomy in a patient with acquired adult-onset adrenal hyperplasia: a case report. 629 2

The importance of late-onset congenital adrenal hyperplasia as a cause of hirsutism is controversial. Two of 35 women with a chief complaint of hirsutism met the criteria of 21-hydroxylase deficiency. In one, who presented with hirsutism, oligomenorrhea, obesity, infertility, and enlarged cystic ovaries, the initial diagnosis was polycystic ovarian syndrome. Family data showed that her disorder was autosomal recessive and linked to the histocompatibility leukocyte antigens (HLA), as in the classic form of congenital adrenal hyperplasia. Carriers were thus detectable by HLA typing. Thus late-onset congenital adrenal hyperplasia appears to be an allelic variant of congenital virilizing adrenal hyperplasia with a milder enzymatic defect. The diagnosis cannot be made clinically because the disease has the same presentation as idiopathic hirsutism or polycystic ovarian disease. Basal plasma 17-hydroxyprogesterone levels, unlike in classic congenital adrenal hyperplasia, can be normal, and an ACTH stimulation test or sequential measurements of plasma 17-hydroxyprogesterone throughout the day may be needed to show the abnormality. The incidence among hirsute women is estimated to be 6% to 12%, and the calculated gene frequency for the allele coding for attenuated expression of 21-hydroxylase deficiency is 0.015 to 0.057.
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PMID:Late-onset 21-hydroxylase deficiency mimicking idiopathic hirsutism or polycystic ovarian disease. 697 82

Medical management of congenital adrenal hyperplasia (CAH) patients has led to suboptimal results in most cases. High glucocorticoid doses, often needed to suppress adrenal androgen production, may lead to signs of Cushing syndrome. Incompletely suppressed androgen levels commonly lead to premature closure of growth centers, acne, virilization, precocious puberty, irregular or absent menses, and decreased fertility in female CAH patients. A newly proposed therapy for CAH patients is bilateral adrenalectomy. Three Caucasian female patients with 21-hydroxylase deficiency were treated with bilateral adrenalectomy. Two of the three procedures were accomplished laparoscopically. In each patient, medical management alone was unsuccessful. Two patients had salt-losing 21-hydroxylase deficiency. The third patient had uncontrolled hyperandrogenism complicated by obesity and glucose intolerance. All patients had low height percentiles with respect to their normalized percentiles for weight. Bone age was advanced in one patient. Androgen and renin levels were well controlled in two patients, whereas the third patient had persistent hyperandrogenism. Bilateral adrenalectomy was performed at the ages of 14, 19, and 30 years with follow-up, to date, of 25 months, 10 months, and 26 months, respectively. Postoperatively, all patients were free from hyperandrogenism. One patient experienced one episode of urosepsis precipitating an addisonian crisis. Bilateral adrenalectomy may successfully address the problems of increasing steroid requirements and hyperandrogenism in patients with severe CAH. The ability to perform this operation laparoscopically coupled with the overall metabolic benefits make bilateral adrenalectomy a reasonable alternative to lifelong androgen suppression in select patients.
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PMID:Study of three patients with congenital adrenal hyperplasia treated by bilateral adrenalectomy. 1103 5

Clinical characteristics of PCOS Syndrome Two fundamental characteristics: hyperandrogenism and anovulation which lead to hirsutism and oligo-or amenorrhea. Other features include obesity, acanthosis nigricans, and metabolic disruption (insulin resistance with hyperinsulinemia, glucose intolerance, or type II diabetes mellitus). Complementary tests Serum testosterone and DHEA-S levels: to exclude androgen-producing tumors. Serum 17-hydroxyprogesterone level: to exclude congenital adrenal hyperplasia, 21-hydroxylase deficiency. Ultrasound: increased size of the ovaries and central stroma with presence of peripheral follicular cysts (8-10) measuring about 8 mm in diameter. Pathophysiology Therapeutic approaches Therapeutic approaches
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PMID:[Polycystic ovaries in 2001: physiology and treatment]. 1198 85

Patients with congenital adrenal hyperplasia attributable to 21-hydroxylase deficiency are treated with glucocorticoids. Glucocorticoid administration, even in substitution doses, may cause decreased bone mineral density (BMD) and obesity. The purpose of this study was to determine BMD, lean mass, and fat mass in young adult male (M, n = 15) and female (F, n = 15) patients with 21-hydroxylase deficiency, who had been treated with currently recommended low doses of glucocorticoids. Measurements were performed with dual-x-ray absorptiometry. In addition, calcaneal ultrasound measurements were performed (broadband ultrasound attenuation and speed of sound). Results were compared with those in age- and sex-matched controls; to adjust for height, lean and fat mass were divided by (height)(2). M and F patients [M, 21.7 +/- 2.4; F, 20.6 +/- 2.9 yr old (mean +/- SD)] were shorter than the controls (M, P < 0.001; F, P < 0.003) and their body mass indices were higher [M patients (25.0 +/- 3.6) vs. controls (22.3 +/- 1.9 kg/m(2)) (P < 0.02); F patients (25.5 +/- 4.5) vs. controls (21.9 +/- 2.3 kg/m(2)) (P < 0.02)]. BMD values (lumbar spine L1-L4, femoral neck, and total body) were not different from controls. Calcaneal ultrasound measurements showed that M patients had higher speed of sound values [M patients (1564 +/- 38) vs. controls (1529 +/- 29 m/sec) (P < 0.01)]. Lean mass in M and F patients was not different from controls when adjusted for height. Fat mass was higher in M and F patients when adjusted for height [M patients 5.6 +/- 2.9 vs. controls 2.7 median (1.7-7.0 min-max) kg/m(2) (P < 0.04); F patients 8.7 +/- 2.8 vs. controls 5.8 (4.3-10.7) kg/m(2) (P < 0.02)]. Relative fat mass (fat mass as a percentage of the total body mass) was higher in patients, compared with controls [M patients 22.0 +/- 9.1 vs. controls 12.8 (8.5-27.0)% (P < 0.04); F patients 34.1 +/- 5.0 vs. controls 29.0 +/- 5.1% (P < 0.02)]; this resulted from increased fat mass, not from decreased lean mass. Fat distribution over the body was not different in patients and controls. No significant correlations were found between cumulative glucocorticoid doses in the last 0.5, 2, or 5 yr or mean salivary morning levels of 17-hydroxyprogesterone and androstenedione in the last 5 yr on one hand and bone parameters, lean mass, or fat mass on the other hand. We conclude that, at prevailing low-dose glucocorticoid regimens, young adult patients with 21-hydroxylase deficiency have normal BMD. Their lean mass is in accordance with height, but fat mass is increased, with a normal distribution over the body. This results in a higher fat percentage of the total body and a higher body mass index than in healthy peers. Because overweight and increased fat mass are associated with the metabolic syndrome and increased cardiovascular risk, weight management should have appropriate attention in the follow-up of congenital adrenal hyperplasia patients, to prevent overweight-associated morbidity.
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PMID:Normal bone mineral density and lean body mass, but increased fat mass, in young adult patients with congenital adrenal hyperplasia. 1262 82

Congenital adrenal hyperplasia is a general term applied to several disorders caused by inherited recessive defects of cortisol synthesis. The most common form is 21-hydroxylase deficiency, accounting for 95% of cases. The classical forms have an incidence of one in 15,000 and the non-classical forms about one in 1,000. The classical or severe phenotype presents in the newborn period or early infancy with virilization and adrenal insufficiency, with or without salt-losing; the non-classical or mild phenotype presents in late childhood or early adulthood with signs of hyperandrogenism. This wide range of clinical expression is explained by genetic variation. Although there is a certain amount of genotype-phenotype correlation, discrepancies have been described. During the last 30 years there has been a substantial improvement in diagnosis and treatment of this disease, and patients with CAH now reach adulthood. Treatment of this condition is intended to reduce excessive corticotropin secretion and replace glucocorticoids and mineralocorticoids as physiologically as possible. Clinical management is often complicated by periods of inadequately treated hyperandrogenism, iatrogenic hypercortisolism, or both. Long-term consequences in adult life may include short stature, obesity, diminished bone mass, gonadal dysfunction with low fertility rates and psychosexual dysfunction in females. New treatment approaches are under investigation, such as the use of anti-androgens, inhibitors of estrogen production and adrenalectomy for severely resistant cases.
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PMID:Childhood-onset congenital adrenal hyperplasia: long-term outcome and optimization of therapy. 1513 1

The diagnosis of polycystic ovary syndrome (PCOS) is primarily achieved through clinical history and physical findings. The principle features are hirsutism or biochemical evidence of excess androgen production and irregular menstrual bleeding caused by the chronic anovulation. Associated findings include insulin resistance with compensatory hyperinsulinemia and obesity. Ultrasound imaging of the ovary has facilitated the diagnosis. It is important to exclude conditions that may mimic PCOS, such as hyperthecosis, congenital adrenal hyperplasia, 21-hydroxylase deficiency, Cushing's syndrome, and androgen-producing neoplasms. These disorders are usually revealed by appropriate laboratory assessment. Screening tests include measurement of serum total testosterone, DHEA sulfate, and 17-hydroxyprogesterone. In addition, in the obese individual, determinations of glucose and insulin levels, as well as a lipid profile, are highly recommended.
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PMID:A practical approach to the diagnosis of polycystic ovary syndrome. 1546 30

The development of Cushing's disease among patients with deficiency of 21-hydroxylase has not been observed to date. The clinical manifestations and the hormonal profile of this exceptional association are herein described through the study of two cases. The first one was a 39-yr-old woman who had undergone non-curative transsphenoidal surgery for a pituitary-dependent Cushing's syndrome 12 yr before. She showed hypertension, central obesity, severe hirsutism, alopecia and hyperpigmentation. Urinary excretion of cortisol was normal, but ACTH levels were very high and hormonal dynamic studies (cortisol circadian rhythm, insulin-induced hypoglycemia and dexamethasone suppression tests) revealed the qualitative disturbances that characterize Cushing's disease. Serum concentrations of androstenedione, free testosterone and 17-hydroxyprogesterone were clearly increased. Reexamination of the tissue samples from previous surgery confirmed the presence of an ACTH-producing pituitary adenoma. CYP21 gene analysis found the splicing 655G mutation at intron 2 and the V281L mutation at exon 7. The second case was a 21-yr-old woman who was diagnosed with pituitary ACTH-dependent Cushing's syndrome according to unequivocal clinical and laboratory findings. However, hirsutism was particularly severe and both serum androgens and 17-hydroxyprogesterone were elevated. The patient was heterozygote for a large conversion of CYP21 gene. In these cases, the clinical and biochemical expression of Cushing's syndrome was determined by the different severity of 21-hydroxylase deficiency and the subsequent residual ability of adrenal cortex to synthesize cortisol.
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PMID:Clinical manifestations and hormonal profile of two women with Cushing's disease and mild deficiency of 21-hydroxylase. 1571 59


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