Bismuth Elise, Laborde Kathleen, Taupin Pierre, Velho Gilberto, Ribault Virginie, Jennane Farida, Grasset Etienne, Sermet Isabelle, de Blic Jacques, Lenoir Gérard, Robert Jean-Jacques
Department of Pediatric Diabetology, Bichat Hospital, Paris, France.
J Pediatr. 2008 Apr;152(4):540-5, 545.e1. doi: 10.1016/j.jpeds.2007.09.025. Epub 2007 Nov 26.
To describe the history, mechanisms, and consequences of cystic fibrosis (CF)-related diabetes, from childhood to early adulthood.
Pancreatic beta-cell function was estimated from the plasma insulin/glucose ratios during oral glucose tolerance test (total area under the curve and deltaI(30-0min)/G(30min), homeostasis model assessment [HOMA]%B), insulin sensitivity with the HOMA%S index, in 237 children with CF (109 boys, 128 girls). Progression of glucose metabolism abnormalities was evaluated by analysis for interval censored data; rates of pulmonary transplantation and death by Kaplan-Meier analysis.
Impaired glucose tolerance was found in 20% of patients at 10 years, 50% at 15 years, 75% at 20 years, 82% at 30 years; for diabetes, >20% at 15 year, 45% at 20 years, 70% at 30 years; for insulin treatment, 30% at 20 years, 40% at 30 years. Early impairment was associated with lower survival rates and higher rates of lung transplantation. The area under the curve(glucose) correlated with decreased body mass index and height. Decrease in early insulin secretion (deltaI(30-0min)/G(30min)) was associated with impaired glucose tolerance, in all estimates of insulin secretion with diabetes. HOMA%S did not differ between the groups. Increased inflammation correlated with insulin resistance and impaired glucose tolerance.
CF-related diabetes, mainly because of beta-cell deficiency, is frequent early in life and associated with impaired nutritional state and growth, increased rates of terminal respiratory failure, and death.
描述从儿童期到成年早期与囊性纤维化（CF）相关的糖尿病的病史、机制及后果。
对237名CF患儿（109名男孩，128名女孩）进行口服葡萄糖耐量试验，根据血浆胰岛素/葡萄糖比值评估胰腺β细胞功能（曲线下总面积及ΔI(30 - 0min)/G(30min)，稳态模型评估[HOMA]%B），用HOMA%S指数评估胰岛素敏感性。通过区间删失数据分析评估糖代谢异常的进展；用Kaplan - Meier分析评估肺移植率和死亡率。
10岁时20%的患者存在糖耐量受损，15岁时为50%，20岁时为75%，30岁时为82%；糖尿病患者，15岁时>20%，20岁时为45%，30岁时为70%；接受胰岛素治疗的患者，20岁时为30%，30岁时为40%。早期功能受损与较低的生存率和较高的肺移植率相关。曲线下面积（葡萄糖）与体重指数和身高降低相关。在所有糖尿病胰岛素分泌评估中，早期胰岛素分泌减少（ΔI(30 - 0min)/G(30min)）与糖耐量受损相关。各组间HOMA%S无差异。炎症增加与胰岛素抵抗和糖耐量受损相关。
与CF相关的糖尿病主要因β细胞缺陷，在生命早期常见，且与营养状态和生长受损、终末期呼吸衰竭率增加及死亡相关。
