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

A rare case of the Cushing syndrome, due to bilateral adrenal hyperplasia is described. Because of generalized obesity, normal height, normal bone age and family history of obesity, a boy 10 years of age had at first been misdiagnosed as simple obesity. A reduction in height velocity, advancement of bone age and development of precocious pseudopuberty led to a more detailed biochemical investigation and to the correct diagnosis. The clinical picture can be misleading in the differential diagnosis of Cushing's disease versus simple obesity. From a comparison of reports on pediatric Cushing's syndrome, it is obvious that in most pediatric cases fat distribution differs from that of adult Cushing's syndromes. Stunted growth is reported to be more frequent, but normal and excessive height in Cushing's syndrome is not uncommon. In adrenal hyperplasia a retardation of bone age is usually expected. Interpretation of endocrine functions must take into account that simple obesity leads to secondary endocrinopathies, which are similar to the findings in Cushing's syndromes. Plasma cortisol at midnight and 12 hourly excretion of free cortisol in urine as well as overnight Dexamethasone suppression of morning plasma cortisol are judged to be good screening parameters. Plasma ACTH assays can help in the initial diagnosis and are mandatory during the follow up for an early detection of Nelson's syndrome.
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PMID:Cushing's syndrome or obesity. Bilateral adrenal hyperplasia in a boy 10 years of age. 17 22

In 17 normal subjects and 126 patients with various endocrine disorders, including 13 patients with Cushing's syndrome, plasma levels of fluorogenic corticosteroid were measured at 9 AM and midnight, and again at 9 AM the following morning, after the patient had received 1 mg of dexamethasone by mouth immediately after the midnight sampling. Basal morning levels of plasma corticosteroids were of little diagnostic value in differentiating between Cushing's syndrome and non-Cushing's states because of the wide overlap of values observed. At midnight the overlap almost completely disappeared. After overnight suppression, only one patient with a mild form of Cushing's disease had normal (false-negative) results in two of four instances. There were virtually no false-positive results, except for two patients with anorexia nervosa showing minor abnormalities of the test. The results were in general agreement with those of the classic Liddle test. However, one patient with Cushing's disease had repeatedly abnormal responses to overnight suppression and normal responses to the Liddle test. When the inhibitory tests gave equivocal results in the differential diagnosis between exogenous obesity and Cushing's disease due to adrenal hyperplasia, the response of plasma corticosteroids to hypoglycemia, normal in obesity and absent in Cushing's disease, proved to be an excellent ancillary test in differentiating between the two conditions.
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PMID:Clinical experience with a simple screening test for Cushing's syndrome combining the determination of plasma cortisol circadian rhythm with the overnight dexamethasone suppression test. 66 10

Subnormal plasma 11-deoxycortisol (compound S) responses to metyrapone were found in patients with adrenal insufficiency or with Cushing syndrome caused by adrenal tumors and in those receiving long-term glucocorticoid or diphenylhydantoin sodium therapy. High normal or exaggerated responses were seen in women receiving oral contraceptives, patients with Cushing syndrome caused by adrenal hyperplasia, and those with untreated hypothyroidism. Diabetes mellitus, hypoglycemia, congestive failure, and obesity also were associated with exaggerated responses. Subnormal plasma S responses were observed in 15 patients who responded normally to a repeat test or to the standard metyrapone test. The abnormal response resulted from insufficient metyrapone, administration at the wrong time, or delay in obtaining the blood sample. The single-dose metyrapone test may be the procedure of choice in screening for adrenal insufficiency.
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PMID:Single-dose metyrapone test: review of a four-year experience. 105 66

The incidence of polycystic ovarian disease (PCOD) varies from 0.6 to 92%, depending on the parameters analysed, PCOD has been reported to appear in association with Cushing's Syndrome, adrenal hyperplasia, hypothyroidism, adrenal and ovarian tumours and some genetic abnormalities. The controversy regarding the pathophysiological mechanism underlying the disease still persists. Critical evaluation of old data, assessment of new findings concerning the possible role of insulin, growth factors and their binding proteins, and extrapolation of neuroendocrinological experiments enabled the construction of a concise hypothesis of the pathophysiology of PCOD. According to this hypothesis, PCOD is a multifactorial disease. The sequence of events finally leading to clinical manifestation of the disease (hyperandrogenism, abnormal luteinizing hormone pulsatility pattern and ovulation disturbances) may originate in different organs or be triggered by different mechanisms. It may stem from the adrenals, the hypothalamus or higher central nervous system centres, or from the ovary itself; it may originate from excess of fat tissue usually combined with hyperinsulinism; or may be the result of a net increase in active growth factors. Each of the above disturbances probably appears early in life, much before the clinical signs of the disease are evident. Predisposing factors such as gestational diabetes of the mother, childhood obesity, borderline adrenal hyperplasia and late menarche have to be looked for as early as possible in order to prevent the late consequences of the disease, such as increased risk of infertility, endometrial and breast cancer and cardiovascular disease.
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PMID:Pathophysiology of polycystic ovarian disease: new insights. 180 58

The long-term results of surgical and specific drug therapy were compared in a group of 57 patients with primary aldosteronism (PA) (46 with aldosterone-producing adenoma (APA), 11 with idiopathic hyperaldosteronism (IHA) and bilateral adrenal hyperplasia). Unilateral adrenalectomy completely normalized blood pressure (BP) in 77.1% of surgically treated APA, evidently improving hypertension in remaining 22.9%. No recurrence of the adenoma in the remaining adrenal was seen in any of the surgical APA cases. In 19 of the non-surgical patients (11 with APA, 8 with IHA) monotherapy with spironolactone reduced blood pressure in 73%, though total BP normalization was an exception. The treatment normalized hypokalemia, low total exchangeable potassium, tendency to hypernatremia, and high total exchangeable sodium. Surgical as well as conservative therapy increased to normal or above-normal levels plasma renin activity suppressed prior to treatment. Pre-operatively high urine and plasma aldosterone levels normalized in all adrenalectomized patients, but remained above the normal range during spironolactone therapy in spite of a small decline in its absolute values. The disturbances of maximum renal concentrating capacity due to impaired nephron responsiveness to sufficiently high endogenous vasopressin concentrations were completely eliminated after kaliopenic nephropathy had been repaired. The other renal functions remained within normal values. Echocardiographically diagnosed left ventricular hypertrophy was seen less often than in the other types of arterial hypertension, tending to regress after APA management. Our longitudinal study (2-16 years) showed primary aldosteronism as a well curable, albeit rare, cause of hypertension. As regards BP and laboratory tests normalization, better results were achieved in surgical APA cases than in patients treated with spironolactone. Older age, longer history of hypertension and more frequent incidence of obesity, nephrosclerosis and pyelonephritis may be responsible for hypertension persisting after surgical treatment.
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PMID:Long-term results of surgical and conservative treatment of patients with primary aldosteronism. 345 May 33

Comparative diagnostic assessment by ultrasonography (US) and computed tomography (CT) was performed in each of 17 patients with adrenal disorders. US is better than CT for demonstrating the intratumorous characteristics, the relationship between the tumor and the surrounding organs, and the organ from which large tumors arise, such as pheochromocytoma. On the other hand, CT is better able to detect small adrenal tumors in primary aldosteronism, although US with a sector or linear scanner can also detect small tumors in some cases. Adrenal hyperplasia in both Cushing's disease and congenital adrenogenital syndrome was more easily demonstrated by CT than by US. Of the two US instruments, a sector scanner was more useful in the delineation of the enlarged right nontumorous adrenal by scanning from the right intercostal region, but both scanners were of no use in the delineation of the enlarged nontumorous left adrenal in patients with obesity.
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PMID:Comparative assessment of ultrasonography and computed tomography in adrenal disorders. 351 14

Cushing's syndrome, a characteristic pattern of obesity with hypertension due to the hyperfunction of the adrenal cortex, is relatively rare in infancy. Thirty-six infants have been reported in world literature, most of whom have had adrenal tumours. There are only eight reported cases of infants under the age of 1 year with adrenal hyperplasia responsible for Cushing's syndrome. This is a report of an 8 month old child with bilateral nodular adrenal hyperplasia.
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PMID:Cushingoid adrenal hyperplasia in infancy. 372 27

Fifteen patients with idiopathic hirsutism, who had no attenuated adrenal hyperplasia, obesity, enlarged ovaries, or amenorrhea, were studied. Excessive androgen secretion by adrenal tissue was suggested by the finding of increased levels of dehydroepiandrosterone sulfate, which decreased after dexamethasone administration but did not change after human chorionic gonadotropin (hCG) injection. Excessive androgen secretion by ovarian tissue was suggested by the finding that testosterone and androstenedione levels were elevated, correlated significantly with the levels of luteinizing hormone, decreased with administration of estrogen-progestagen, and increased after hCG injection. Notably, free testosterone levels, which were significantly increased, were only partially suppressed during dexamethasone or estrogen-progestagen administration. These results provide further evidence that both the adrenals and the ovaries secrete androgens excessively in patients with idiopathic hirsutism.
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PMID:Evidence of excessive androgen secretion by both the ovary and the adrenal in patients with idiopathic hirsutism. 680 96

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

Two hundred fifty women with hirsutism were studied, with a mean age of 25.5 years (ranging from 13 to 38 years). The evolution of hirsutism varied from 3 months to 13 years, being minimal in 82 patients (33%), mild to 101 (40%), moderate in 56 (23%) and severe in the remaining 11 women (4%). Polycystic ovary syndrome (PCOS) was diagnosed in 134 patients (53%), overweight or obesity in 45 (18%), late-onset adrenal hyperplasia in five (2%), ovarian tumor in two (0.8%), drug-induced hirsutism and Cushing's syndrome in one patient each (0.4%), and idiopathic hirsutism in 62 cases (25%). Among 67 patients with moderate or severe hirsutism, testosterone was elevated in 21 (31%). In 117 out of 206 (57%) cases polycystic ovaries were observed by ultrasound. Fifty-four patients were treated with a combination of 2 mg cyproterone acetate and 0.035 mg ethinyl estradiol, observing improvement of hirsutism in 32 patients (59%). It is concluded that PCOS is the most frequent cause of hirsutism, but an important proportion of cases without evident etiology remain classified as idiopathic hirsutism.
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PMID:Etiological review of hirsutism in 250 patients. 780 80


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