Management of Turner’s syndrome in adult life: case-series and systematic review

Objective: To assess the symptoms and outcomes of clinical management in adult patients with Turner’s Syndrome. Design: Retrospective case-series and systematic review of the literature. Setting: Gynaecological Endocrinology Unit in a teaching hospital. Patients: Patients followed in the Gynaecological Endocrinology Unit. Interventions: Review of medical records and a computer search via several databases to identify journals relevant to the subject were performed. Main outcome measures: Final height, weight, previous treatments with growth hormone, cardiac or renal malformations, metabolic profile, and additional treatment for osteoporosis. Results: Thirty-one patients were analysed. Differences in final height were found between groups with monosomy and other karyotypes. Four patients bore congenital cardiac malformations, and six, renal congenital malformations. Nine patients had a previous diagnosis of hypercholesterolemia. The most abnormal hepatic parameter was GGT, with fifteen patients having values over the normality limit. Ten patients were receiving treatment for osteopenia or osteoporosis. Conclusions: This case-series provides recommendations for the management of adult patients with Turner’s syndrome and insight into the different medical complaints of this syndrome. A link between karyotypes and clinical features suggests a novel hypothesis to explain the different phenotypes and clinical abnormalities of these patients.     

Study on mechanism of Bushen Yugong Granule in treating uterine dysgenesiain treating uterine dysgenesia

Objective: To explore the therapeutic mechanism of Bushen Yugong Granule (BSYGG, a Chinese medicinal preparation) in treating infertility due to uterine dysgenesia.Methods: (1) Clinical study: Serum level of sex hormone and endometrial contents of estradial receptor (ER) and progesterone receptor (PR) in follicle period of 60 patients (the tested group) were measured and compared before and after treatment, and also compared with those of 70 normal women (the control group). (2) Experimental study: Rats were divided into the control group and 3 tested groups fed with different doses of BSYGG. Pathologic examination of pituitary, ovary and uterus of rats after BSYGG treatment were performed, and serum levels of ER, PR measured. Also, an estrogen activity experiment was conducted in some young oophorectomized rats.Results: (1) Clinical study showed the difference of serum sex hormone levels between the tested and control group was insignificant. The percentage of patients in the tested groups with endometrial ER content over ++ was lower than that in the control group (46.9 % vs 72.4 %) significantly (P < 0.05), after treatment, it rose to 65.4 %, and the change was significant, P<0.05. (2) Experimental study showed that the number of follicle, weight of uterus and serum E₂ level increased significantly after BSYGG treatment. The endometrial content of ER in the control group was 29.25±17.03%, while in the tested groups, it was 67.75 ± 24.56% to 83.00 ± 9.87% after treatment, the difference between the two groups was significant (P<0.005).Conclusion: BSYGG has an estrogen-like activity, it could increase the endometrial content of ER, enhance the effect of estrogen on uterus and promote the development of uterus, so as to elevate fertilization rate.     

Inhibin bodies: a new marker for immature Sertoli cells

Nistal M, Pastrián L G, González‐Peramato P & De Miguel M P
(2011) Histopathology 58, 1019–1027
Inhibin bodies: a new marker for immature Sertoli cells Aims: To provide a marker for immature and dysgenetic Sertoli cells which allows easy identification in patients in which Sertoli cell maturation does not take place properly, such as those consulting for cryptorchidism, testicular tumours and infertility. Methods and results: We performed immunohistochemistry against inhibin‐α subunit and the endoplasmic reticulum marker Grp78 in normal human testes from fetal life to adulthood, and in several testicular lesions where Sertoli cell maturation is abnormal. We describe a pattern of inhibin immunostain (inhibin bodies of 2–9 μm in diameter at the Sertoli cells cytoplasm apical pole) in immature and dysgenetic Sertoli cells that facilitates their identification. Inhibin bodies were found in tubules with either no germ cells or only spermatogonia or carcinoma in situ (CIS) and seminoma cells, but not in tubules containing more advanced germ cells. Conclusions: Our data provide a new marker of immature and dysgenetic Sertoli cells. In addition, our data suggest that inhibin bodies represent a slower transit of inhibin through the endoplasmic reticulum, as inhibin bodies were associated with Grp78.     

四联法治疗输卵管阻塞性不孕症疗效分析

目的：观察中医药内、外配合治疗输卵管阻塞性不孕症的疗效。方法：应用自拟疏通汤方一方三用即内服、保留灌肠、药渣热敷下腹部并配合复方鱼腥草注射液宫腔灌注治疗本证60例。结果：治愈率73.3%,总有效率总有效率91.6%。结论：采用疏通汤方一方三用及宫腔灌注四种方法联合治疗本症,安全且疗效肯定。     

抗心磷脂抗体与不孕不育关系的研究进展

从病理生理的基础研究，药物治疗的实验研究，临床治疗的观察研究等方面研究分析，探讨抗心磷脂抗体与不孕不育之间的关系，认为抗心磷脂抗体主要通过影响血小板等形成血栓影响胎盘血液循环，使胎盘发育不良，妊娠早期可引起流产，妊娠中晚期发生胚胎死亡，抗心磷脂抗体的存在可导致不孕。     

抗心磷脂抗体与不孕不育关系的研究进展

从病理生理的基础研究,药物治疗的实验研究,临床治疗的观察研究等方面研究分析,探讨抗心磷脂抗体与不孕不育之间的关系,认为抗心磷脂抗体主要通过影响血小板等形成血栓影响胎盘血液循环,使胎盘发育不良,妊娠早期可引起流产,妊娠中晚期发生胚胎死亡,抗心磷脂抗体的存在可导致不孕。     

补肾育宫冲剂治疗子宫发育不良的机理研究

目的：探讨补肾育宫冲剂治疗子宫发育不良性不孕症的作用机理。方法：（１）以子宫发育不良和发育正常的育龄妇女为观察组和对照组，于卵泡期测血清性激素和子宫内膜雌、孕激素受体（ＥＲ、ＰＲ）含量，并作观察组治疗前后比较。（２）用大鼠作动物实验，设对照组及补肾育宫冲剂３个剂量组，于服药前后测血清雌二醇（Ｅ２），对服药后大鼠的垂体、卵巢、子宫作病理检验并测ＥＲ、ＰＲ含量。对切除卵巢的幼龄大鼠作本冲剂的雌激素活怀     

CONGENITAL IMMUNODEFICIENCY AND AGRANULOCYTOSIS (RETICULAR DYSGENESIA)

Abstract. A patient is presented who manifested the typical clinical and pathological features of congenital immunodeficiency and agranulocytosis (reticular dysgenesia). Treatment under gnotobiotic conditions enabled the measurement of immunological parameters up to the 17th week of life with the following results: negative skin tests, low response to phytohaemagglutinin, weak response in the mixed leukocyte culture and very few E rosettes. Peripheral lymphocytes and lymphocytes in the lymphatic tissues were markedly decreased. Humoral immunoglobulins and plasma cells in the organs were decreased. The in vitro culture of hemopoietic cells showed a diminished content of myelopoietic progenitor cells ("committed stem cells"). It is concluded that the disease may be primarily a defect of stem cells with regard to differentiation in myelopoiesis or lymphopoiesis.