Tubiana-Rufi N, Czernichow P
Service d'Endocrinologie et Diabétologie Pédiatrique, Hôpital Robert Debré, Paris.
Ann Pediatr (Paris). 1991 Apr;38(4):244-8.
As a result of the improvement in life-expectancy in cystic fibrosis patients, simultaneous presence of cystic fibrosis and diabetes mellitus is no longer exceptional. In teenagers and young adults with cystic fibrosis, the prevalence of insulin-dependent diabetes mellitus (IDDM) is 7 to 10%. Fifty percent of cystic fibrosis patients have impaired glucose tolerance. These prevalences increase with advancing age. Insulin deficiency is a consistent feature. An endocrine pancreatic deficiency thus exists in addition to the exocrine pancreatic deficiency, as demonstrated by the fall in glucagon and pancreatic polypeptide productions. Development of insulin dependency is associated with deterioration in clinical status and indicates an adverse prognosis. Although in cystic fibrosis patients diabetes mellitus seems to occur as a result of different pathophysiologic mechanisms than those involved in autoimmune IDDM, the risk of degenerative complications is similar in both conditions. It follows that early detection of diabetes mellitus and appropriate insulin treatment are warranted in cystic fibrosis patients.
由于囊性纤维化患者预期寿命的提高,囊性纤维化与糖尿病同时存在已不再罕见。在青少年和年轻的囊性纤维化患者中,胰岛素依赖型糖尿病(IDDM)的患病率为7%至10%。50%的囊性纤维化患者存在糖耐量受损。这些患病率随着年龄的增长而增加。胰岛素缺乏是一个持续的特征。正如胰高血糖素和胰多肽分泌量下降所表明的那样,除了外分泌性胰腺功能不全外,还存在内分泌性胰腺功能不全。胰岛素依赖的发展与临床状况的恶化相关,预示着不良预后。尽管在囊性纤维化患者中,糖尿病的发生似乎是由与自身免疫性IDDM不同的病理生理机制引起的,但在这两种情况下,退行性并发症的风险相似。因此,有必要对囊性纤维化患者进行糖尿病的早期检测和适当的胰岛素治疗。