Finkelstein S M, Wielinski C L, Elliott G R, Warwick W J, Barbosa J, Wu S C, Klein D J
Department of Laboratory Medicine, University of Minnesota, Minneapolis 55455.
J Pediatr. 1988 Mar;112(3):373-7. doi: 10.1016/s0022-3476(88)80315-9.
The prevalence of overt diabetes mellitus and carbohydrate intolerance was studied in 448 patients with cystic fibrosis (CF). Insulin-dependent diabetes (IDDM) developed in 7.6% of patients (13 male and 21 female). Survival was significantly lower (P less than 0.01) in the IDDM-CF group, with fewer than 25% surviving to age 30 years, whereas nearly 60% of the nondiabetic CF population reached this age. A significant deterioration in CF clinical status, based on NIH score, became apparent 2 years before onset of overt IDDM (P less than 0.05 at 2 years prior, P less than 0.01 at IDDM diagnosis). Total glycosylated hemoglobin (HbA1) was significantly (P less than 0.001) higher for the total CF population (7.3% +/- 1.2%) than for the general non-CF population (6.5% +/- 0.7%), and in the IDDM-CF group (P less than 0.05) compared with normoglycemic CF control patients. Female patients had a higher mean HbA1 after 12 years of age than their male counterparts did (P less than 0.02). HBA1 did not predict the development of IDDM, but there was a weak inverse relationship between HbA1 and both NIH clinical score (r = -0.41, P less than 0.02) and standard pulmonary function tests (forced vital capacity, r = -0.25, P less than 0.01) in the general CF population. Therefore, impaired carbohydrate tolerance in CF is associated with progressive clinical deterioration.
对448例囊性纤维化（CF）患者的显性糖尿病和碳水化合物不耐受患病率进行了研究。7.6%的患者（13名男性和21名女性）发生了胰岛素依赖型糖尿病（IDDM）。IDDM-CF组的生存率显著降低（P<0.01），30岁前存活者不到25%，而近60%的非糖尿病CF人群达到了这个年龄。基于美国国立卫生研究院（NIH）评分，CF临床状态在显性IDDM发病前2年出现显著恶化（发病前2年P<0.05，IDDM诊断时P<0.01）。CF总体人群的总糖化血红蛋白（HbA1）显著高于一般非CF人群（7.3%±1.2%对6.5%±0.7%，P<0.001），且IDDM-CF组与血糖正常的CF对照患者相比也显著升高（P<0.05）。12岁后女性患者的平均HbA1高于男性患者（P<0.02）。HbA1不能预测IDDM的发生，但在总体CF人群中，HbA1与NIH临床评分（r=-0.41，P<0.02）和标准肺功能测试（用力肺活量，r=-0.25，P<0.01）之间存在弱的负相关。因此，CF患者的碳水化合物耐受性受损与临床进行性恶化有关。
