Lee P J, Patel A, Hindmarsh P C, Mowat A P, Leonard J V
London Centre for Paediatric Endocrinology and Metabolism, Institute of Child Health, UK.
Clin Endocrinol (Oxf). 1995 Jun;42(6):601-6. doi: 10.1111/j.1365-2265.1995.tb02686.x.
There has been much debate concerning the relative contribution of insulin resistance to the development of polycystic ovaries (PCO). We therefore aimed to assess ovarian morphology and insulin/androgen status in females with the hepatic glycogen storage diseases types Ia (GSD-Ia) and III (GSD-III), disorders associated with abnormalities of insulin secretion.
A cross-sectional study of ovarian ultrasonography, oral glucose tolerance tests (oGTTs) and single measurements of gonadotrophins and androgens were performed.
Twenty-seven patients were evaluated: 13 with GSD-Ia, median age 11.2 years (range, 3.3-26.7) and 14 with GSD-III, aged 13.2 years (4.2-31.3). None had clinical signs of hyperandrogenism and only two of the 13 adults (15%) had menstrual irregularities. They were compared to 9 normal adult female controls, aged 21-28 years.
Ovarian morphology and volume were measured. Blood glucose and plasma insulin concentrations were measured at the beginning and end of a 2-hour oGTT. Single measures of LH, FSH, testosterone, dehydroepiandrosterone sulphate, androstenedione, IGF-I and SHBG were made on samples taken at the beginning of the oGTT.
In both GSD-Ia and III, all those older than 4.8 years of age had a polycystic ovarian appearance. Pre-pubertal GSD-Ia patients had lower basal and 2-hour blood glucose and plasma insulin concentrations than pre-pubertal GSD-III patients. In adults with GSD-Ia and GSD-III, although basal and 2-hour blood glucose concentrations did not differ, both basal and 2-hour plasma insulin concentrations were significantly higher than controls. Serum gonadotrophins, androgens, IGF-I and SHBG were mostly normal.
A polycystic ovarian appearance is a common finding in patients with glycogen storage disease even before puberty. In GSD-III and adults with GSD-Ia, this ovarian appearance was associated with hyperinsulinism, suggesting an aetiological link, but this was not the case in pre-pubertal children with GDS-Ia. Inborn errors of carbohydrate metabolism may act as useful models for examining control mechanisms of ovarian physiology and development.
关于胰岛素抵抗在多囊卵巢（PCO）发生发展中的相对作用，一直存在诸多争议。因此，我们旨在评估患有Ia型（GSD-Ia）和III型（GSD-III）肝糖原贮积病的女性的卵巢形态以及胰岛素/雄激素状态，这两种疾病与胰岛素分泌异常有关。
进行了一项关于卵巢超声检查、口服葡萄糖耐量试验（oGTT）以及促性腺激素和雄激素单次测量的横断面研究。
对27例患者进行了评估：13例GSD-Ia患者，中位年龄11.2岁（范围3.3 - 26.7岁）；14例GSD-III患者，年龄13.2岁（4.2 - 31.3岁）。所有患者均无高雄激素血症的临床体征，13例成年患者中只有2例（15%）有月经不规律。将他们与9名年龄在21 - 28岁的正常成年女性对照进行比较。
测量卵巢形态和体积。在2小时oGTT开始和结束时测量血糖和血浆胰岛素浓度。在oGTT开始时采集的样本上，对促黄体生成素（LH）、促卵泡生成素（FSH）、睾酮、硫酸脱氢表雄酮、雄烯二酮、胰岛素样生长因子-I（IGF-I）和性激素结合球蛋白（SHBG）进行单次测量。
在GSD-Ia和GSD-III患者中，所有年龄大于4.8岁的患者均有多囊卵巢表现。青春期前的GSD-Ia患者的基础血糖和2小时血糖以及血浆胰岛素浓度低于青春期前的GSD-III患者。在患有GSD-Ia和GSD-III的成年患者中，尽管基础血糖和2小时血糖浓度无差异，但基础和2小时血浆胰岛素浓度均显著高于对照组。血清促性腺激素、雄激素、IGF-I和SHBG大多正常。
即使在青春期前，多囊卵巢表现也是糖原贮积病患者的常见发现。在GSD-III患者以及成年GSD-Ia患者中，这种卵巢表现与高胰岛素血症相关，提示存在病因学联系，但青春期前的GDS-Ia患儿并非如此。碳水化合物代谢的先天性缺陷可能是研究卵巢生理和发育控制机制的有用模型。
