Cicognani A, Mazzanti L, Tassinari D, Pellacani A, Forabosco A, Landi L, Pifferi C, Cacciari E
Second Paediatric Clinic, University of Bologna, Italy.
Eur J Pediatr. 1988 Oct;148(1):64-8. doi: 10.1007/BF00441818.
Carbohydrate homeostasis was evaluated in 47 girls with Turner syndrome and in 25 "short normal" girls by means of an oral glucose tolerance test. Of the Turner patients 34% showed an impaired glucose tolerance vs 8% of the controls (chi 2 = 5.9, P less than 0.05). Mean glucose levels were significantly higher and mean insulin levels and insulinogenic index significantly lower in young Turner patients aged 5-12 years but not in adolescents aged 12-16 years. In both groups of patients, insulin levels and the insulinogenic index were significantly lower than those of the controls. In Turner patients between 12 and 16 years, carbohydrate tolerance improved and this may be explained by the lack of oestrogen release in these patients. Glucose tolerance was normal in patients with mosaicism. We conclude that (1) carbohydrate tolerance is defective in young children with Turner syndrome but improves in puberty due to the almost complete absence of oestrogen-progestogen secretion; (2) a difference in carbohydrate tolerance is evident depending on karyotype.
通过口服葡萄糖耐量试验,对47名特纳综合征女孩和25名“身材矮小但正常”的女孩的碳水化合物稳态进行了评估。特纳综合征患者中34%表现出葡萄糖耐量受损,而对照组为8%(χ² = 5.9,P < 0.05)。5至12岁的年轻特纳综合征患者的平均血糖水平显著更高,平均胰岛素水平和胰岛素生成指数显著更低,但12至16岁的青少年患者则不然。两组患者的胰岛素水平和胰岛素生成指数均显著低于对照组。在12至16岁的特纳综合征患者中,碳水化合物耐量有所改善,这可能是由于这些患者缺乏雌激素释放所致。嵌合型患者的葡萄糖耐量正常。我们得出结论:(1)特纳综合征幼儿的碳水化合物耐量存在缺陷,但由于几乎完全缺乏雌激素 - 孕激素分泌,在青春期会有所改善;(2)根据核型不同,碳水化合物耐量存在明显差异。