1/10. Clearance of acanthosis nigricans associated with the hair-AN syndrome after partial pancreatectomy: an 11-year follow-up.We describe a woman with the syndrome characterised by hyperandrogenism, insulin resistance and acanthosis nigricans (the hair-AN syndrome), and an associated insulinoma (islet B-cell tumour), whose signs and symptoms cleared after partial pancreatectomy.- - - - - - - - - - ranking = 1keywords = acanthosis nigricans, nigricans, acanthosis (Clic here for more details about this article) |
2/10. Seckel-like syndrome: a patient with precocious puberty associated with nonclassical congenital adrenal hyperplasia.Seckel syndrome is a rare, recessively inherited disorder of severe growth retardation with low birth weight and distinct craniofacial, orodental and skeletal anomalies. In addition to these characteristics, some cardiovascular, hematopoietic, endocrine and central nervous system abnormalities have also been described. We report a patient with Seckel-like syndrome who has precocious puberty associated with non-classical congenital adrenal hyperplasia (NCCAH). She was admitted to our clinic three times. She was diagnosed as having Seckel-like syndrome and premature thelarche at the age of 8.9 years. At 10.9 years old she was admitted to our clinic with pubic hair and cliteromegaly. Hormonal findings revealed NCCAH and hydrocortisone therapy was offered but the patient was non-compliant. At 13.6 years she had acanthosis nigricans as an additional clinical finding and her pubertal stage was 4. She had irregular menses. On hormonal evaluation she had euglycemic hyperinsulinism accompanying mild hypertriglyceridemia and functional ovarian hyperandrogenism. Premature pubarche, hyper-insulinism, dyslipidemia, and hyperandrogenism, and some combinations of these, can be associated with reduced fetal growth. This is the first report of hyperinsulinism, and probably NCCAH, in association with Seckel syndrome.- - - - - - - - - - ranking = 0.2keywords = acanthosis nigricans, nigricans, acanthosis (Clic here for more details about this article) |
3/10. Clinical course of genetic diseases of the insulin receptor (type A and Rabson-Mendenhall syndromes): a 30-year prospective.The interaction of insulin with its cell surface receptor is the first step in insulin action and the first identified target of insulin resistance. The insulin resistance in several syndromic forms of extreme insulin resistance has been shown to be caused by mutations in the receptor gene. We studied 8 female patients with the type A form of extreme insulin resistance and 3 patients (2 male and 1 female) with the Rabson-Mendenhall syndrome and followed the natural history of these patients for up to 30 years. The 11 patients ranged in age from 7 to 32 years at presentation. All 11 patients had extreme insulin resistance, acanthosis nigricans, and hyperandrogenism in the female patients, and all but 1 were of normal body weight. This phenotype strongly predicts mutations in the insulin receptor: of the 8 patients studied, 7 were found to have mutations. Similar results from the literature are found in other patients with type A and Rabson-Mendenhall syndromes and leprechaunism. The hyperandrogenic state resulting from hyperinsulinemia and insulin resistance in these patients was extreme: 6 of 8 patients had ovarian surgery to correct the polycystic ovarian syndrome and elevation of serum testosterone. By contrast, a larger group of insulin-resistant patients who were obese with hyperandrogenism, insulin resistance, and acanthosis nigricans (hair-AN syndrome) did not have a high probability of mutations in the insulin receptor. The morbidity and mortality of these patients were high: 3 of 11 died, 9 of 11 were diabetic and 1 had impaired glucose tolerance, and 7 of 9 patients had 1 or more severe complication of diabetes. Our literature review revealed that the mortality of leprechaunism is so high that the term leprechaunism should be restricted to infants or young children under 2 years of age. Analogous to patients with the common forms of type 2 diabetes, these patients had a heterogeneous course. In 2 patients who were able to maintain extremely high endogenous insulin production, the fasting blood glucose remained normal even though post-glucose-challenge levels were elevated. Most patients, however, required large doses of exogenous insulin to ameliorate the severe hyperglycemia. Preliminary results of a recent study suggest that recombinant leptin administration may benefit these patients with severe insulin resistance.- - - - - - - - - - ranking = 0.4keywords = acanthosis nigricans, nigricans, acanthosis (Clic here for more details about this article) |
4/10. biliopancreatic diversion as a novel approach to the hair-AN syndrome.BACKGROUND: The hair-AN syndrome is a rare multisystem disorder in women, that consists of hyperandrogenism (HA), insulin resistance (IR) and acanthosis nigricans (AN). The IR is likely due to a primary defect of the insulin receptor. methods: We report the case of a 42-year-old Caucasian woman with hair-AN syndrome, impaired glucose tolerance (IGT), mild hyperlipemia, and hypertension, who underwent biliopancreatic diversion (BPD). RESULTS: Within 24 months follow-up after BPD, impaired glucose tolerance, mild hyperlipemia, and hypertension completely reversed. Although insulin sensitivity, estimated by the euglycemic hyperinsulinemic clamp, did not improve, signs and symptoms of hyperandrogenism and acanthosis nigricans resolved fully. CONCLUSION: In hair-AN syndrome, malabsorptive bariatric surgery is effective in improving hyperandrogenism and acanthosis nigricans, with noteworthy esthetic consequences. BPD was followed by disappearance of co-morbidities of the syndrome, such as IGT, hypertension and hyperlipemia.- - - - - - - - - - ranking = 0.6keywords = acanthosis nigricans, nigricans, acanthosis (Clic here for more details about this article) |
5/10. Type A insulin resistance syndrome revealing a novel lamin A mutation.Particular forms of polycystic ovary syndrome with severe hyperandrogenism, acanthosis nigricans, and marked insulin resistance, defining the type A insulin resistance syndrome, are due to insulin receptor gene mutations. However, the majority of affected individuals do not have such mutation, arguing for the genetic heterogeneity of this syndrome. The familial partial lipodystrophy of the Dunnigan type, one of the diseases due to mutations in the lamin A/C (LMNA) gene, is characterized by a lipodystrophic phenotype and shares some clinical and metabolic features with the type A syndrome. We describe here the case of a nonobese 24-year-old woman affected with type A syndrome without clinical lipodystrophy. We linked this phenotype to a novel heterozygous missense mutation in the LMNA, predicting a G602S amino acid substitution in lamin A. This mutation cosegregated with impaired glucose tolerance, insulin resistance, and acanthosis nigricans in the absence of clinical lipodystrophy in the family. The skin fibroblasts from the proband exhibited nuclear alterations similar to those described in other laminopathies, and showed several defects in the insulin transduction pathway. This study further extends the vast range of diseases linked to LMNA mutations and identifies another genetic cause for the type A insulin resistance syndrome.- - - - - - - - - - ranking = 0.25179122017051keywords = acanthosis nigricans, nigricans, acanthosis (Clic here for more details about this article) |
6/10. insulin resistance in a patient with ovarian stromal hyperthecosis and the hyperandrogenism, insulin resistance and acanthosis nigricans syndrome. Report of a case with a possible endogenous ovarian factor.insulin resistance is a common feature of ovarian stromal hyperthecosis and is usually accompanied by hyperandrogenemia. A patient had ovarian stromal hyperthecosis, and her hyperandrogenemia resolved, possibly due to the development of a type of ovarian fibrosis similar to so-called ovarian fibromatosis, without a concomitant improvement in her insulin resistance. The insulin resistance improved markedly, however, after bilateral oophorectomy.- - - - - - - - - - ranking = 0.8keywords = acanthosis nigricans, nigricans, acanthosis (Clic here for more details about this article) |
7/10. acromegaly with polycystic ovaries, hyperandrogenism, hirsutism, insulin resistance and acanthosis nigricans: a case report.We describe a woman with acromegaly who had acanthosis nigricans and hirsutism. serum growth hormone (GH) and testosterone levels were markedly elevated. Standard oral glucose tolerance test (OGTT) showed a diabetic curve and no suppressed GH levels. fasting insulin levels were very high while plasma glucose levels were not hypoglycemic. Insulin tolerance test revealed blunted hypoglycemic response. acanthosis nigricans was present in the right axilla and face. Ultrasonogram demonstrated bilateral polycystic changes in the ovaries. From the above findings this patient's condition is characteristic of a very rare syndrome consisting of acromegaly, polycystic ovaries (PCO), hyperandrogenism, hirsutism, insulin resistance and acanthosis nigricans.- - - - - - - - - - ranking = 1.2300124403976keywords = acanthosis nigricans, nigricans, acanthosis (Clic here for more details about this article) |
8/10. The hyperandrogenic-insulin-resistant acanthosis nigricans syndrome: therapeutic response.The hyperandrogenic-insulin-resistant acanthosis nigricans syndrome affects between 2% and 5% of hirsute women and is characterized by INS resistance, elevated INS levels, acanthosis nigricans, and androgen excess. These patients' response to therapy is unclear, although long-acting GnRH-a suppression has been proposed. The objective of this study was to determine the success of OC in suppressing the hyperandrogenemia of five patients with the hyperandrogenic-insulin-resistant acanthosis nigricans syndrome and the subsequent response to GnRH-a suppression of those women failing initial therapy. After 6 months of OC and SPA therapy, four patients experienced adequate suppression of free T, an increase in SHBG levels, and a subjective improvement in hair growth rate. Two also reported an improvement in hair texture. The total and free T levels in the fifth patient did not suppress after 8 months of OC therapy. Nevertheless, in this patient the administration of a GnRH-a along with hormonal replacement and SPA adequately suppressed free and total T, increased SHBG, and dramatically decreased the shaving interval. In conclusion, although GnRH-a suppression and hormonal replacement can be helpful in patients not responding to standard therapy, the majority of patients with the hyperandrogenic-insulin-resistant acanthosis nigricans syndrome will respond favorably to OC treatment. It is also clear that although hyperinsulinemia augments LH-stimulated androgen biosynthesis in vivo, it does not appear to be able to initiate or maintain androgen production in the absence of adequate gonadotropin stimulation.- - - - - - - - - - ranking = 1.6keywords = acanthosis nigricans, nigricans, acanthosis (Clic here for more details about this article) |
9/10. hyperandrogenism, insulin resistance, and acanthosis nigricans (hair-AN) syndrome: spontaneous remission in a 15-year-old girl.acanthosis nigricans is associated with many diseases, including internal malignancies, genetic disorders, and endocrine abnormalities. insulin resistance frequently accompanies endocrine-associated acanthosis nigricans. We describe a patient with acanthosis nigricans associated with hyperandrogenism and extreme insulin resistance that spontaneously resolved after a marked decrease in insulin receptor antibodies.- - - - - - - - - - ranking = 1.2300124403976keywords = acanthosis nigricans, nigricans, acanthosis (Clic here for more details about this article) |
10/10. A case of severe hyperandrogenism, acanthosis nigricans and over diabetes: the use of non-invasive methods for diagnosis, pathogenesis and management.hyperandrogenism is characterized clinically by hirsutism, acne, androgens-dependent alopecia and elevated serum concentrations of androgens (testosterone and androstenedione). polycystic ovary syndrome is the most frequent cause of hyperandrogenism. Nevertheless, the differential diagnosis includes androgen-secreting tumors of the ovary or adrenal gland. Although rare, it is important to consider this diagnosis in patients with serum testosterone concentrations greater than 7 nmol/l. A 35-year-old woman who presented with hirsutism, amenorrhea and acanthosis nigricans is described. The endocrine abnormalities included a serum testosterone concentration of 9 nmol/l and overt type II diabetes mellitus. Imaging studies, including magnetic resonance imaging and Doppler ultrasonography, did not disclose a secreting tumor. After cyproterone acetate was prescribed the serum testosterone concentration returned to normal. The recent application of modern, high-resolution diagnostic ultrasonography and magnetic resonance imaging enabled a morphologically based diagnosis in a case of severe hyperandrogenism, with no need for invasive procedures. The therapeutic response to antiandrogens is reassuring.- - - - - - - - - - ranking = 1keywords = acanthosis nigricans, nigricans, acanthosis (Clic here for more details about this article) |
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