â-thalassemia and gonadal axis: a cross-sectional, clinical study in a Greek population

ABSTRACT â-thalassemia (â-thal) is characterized by disturbances of the reproductive system. The aim of the present study was: 1) to assess the hypothalamic pituitary -gonadal axis in patients with â-thal in relation to their phenotype and 2) to determine prognostic features of current gonadal status. We studied 135 patients (67 males and 68 females) with â-thal through history, physical examination, spermiograms and GnRH test. These patients were divided into â-thal major (51 males and 62 females) and â-thal intermedia phenotypes (16 males and 6 females). Male patients with â-thal major were subdivided into three groups a) eugonadal (35%, Tanners stage V, normal testicular volume, normal spermiograms, normal basal and stimulated hormone values), b) patients with hypogonadotrophic hypogonadism (HH) of late onset (24%, Tanners stage II-V, low-normal testicular volume, abnormal spermiograms, normal basal gonadotrophin values and abnormal response to GnRH test) and c) patients with HH of early onset (41%, Tanners stage I, small testicular volume, abnormal spermiograms, abnormal basal and stimulated hormone values). Female patients with â-thal major were subdivided into: a) eugonadal (32%, Tanners stage V, regular menstruation, normal basal and stimulated hormone values), b) patients with hypogonadotrophic hypogonadism (HH) of late onset (34%, Tanners stage II-V, secondary amenorrhea, subnormal basal and stimulated gonadotrophin values) and c) patients with HH of early onset (34%, Tanners stage I, primary amenorrhea, subnormal basal and stimulated hormone values). Patients with â-thal intermedia were subdivided into eugonadal (75% of males -33% of females) and hypogonadal (25% of males -67% of females). Current gonadal status could not be predicted by means of transfusion or chelation parameters. In conclusion, â-thal patients could be eugonadal or develop early or late onset HH. â-thal intermedia patients have a more favorable profile than â-thal major individuals. Current gonadal status of â-thal patients cannot be predicted by means of history, clinical or laboratory parameters

Decreased active, total and altered active to total ghrelin ratio in normal weight women with the more severe form of polycystic ovary syndrome

Objective: To assess total, active and active to total serum ghrelin ratio in normal weight women with polycystic ovary syndrome (PCOS) and in healthy ovulatory control women.Study design: The study included 50 normal weight women with PCOS with a mean age of 23.70 +/- 4.99 years and 10 control women with a mean age of 30 +/- 5.80 years. The diagnosis of PCOS was based on the presence of biochemical hyperandrogenemia, chronic anovulation and polycystic ovarian morphology according to the Rotterdam ESHRE/ASRM-sponsored PCOS Consensus Workshop Group. Serum total and active ghrelin were measured by RIA, using commercially available kits. Results: A significantly lower serum active/total ghrelin ratio was noted in the more severe form of PCOS with hyperandrogenemia, chronic anovulation and polycystic ovarian morphology. Both total and active serum ghrelin levels were negatively correlated to hirsutism score, to plasma glucose levels and to QUICKI and HOMA-IR indices of Insulin Resistance. A statistically significant difference was detected between the more severe and the milder forms of PCOS, concerning serum levels of total ghrelin (p = 0.017), active ghrelin (p = 0.007) and the active/total ghrelin ratio (p = 0.026). Conclusions: The results of the present study demonstrate an altered active to total ghrelin ratio, as well as a tendency towards lower both total and active fasting serum ghrelin levels in normal weight PCOS, more pronounced in the more severe forms of the syndrome. (C) 2009 Elsevier Ireland Ltd. All rights reserved