Polak M, Le Luyer B, Rybojab M, Czernichow P
Department of Pediatric Endocrinology and Diabetology, Hôpital Robert Debré, Paris, France.
Diabetes Metab. 1996 Jun;22(3):192-6.
A 14-year-old boy presented with Type I diabetes mellitus and subsequently developed pancreatic exocrine insufficiency and systemic sclerosis (SSc). His diabetes had been diagnosed when he was about 5 years old, after the onset of ketoacidosis. Insulin treatment was provided from then until the time he was treated in our department. Exocrine pancreatic insufficiency, which occurred at age 9, was treated with pancreatic extracts. Cystic fibrosis was excluded after a chloride sweat test. The diagnosis of SSc was confirmed at age 14 on the basis of skin sclerosis, sclerodactyly and oesophageal and pulmonary involvement and then at age 18 by the occurrence of Raynaud's disease. Thus, this patient demonstrated a rare and previously unreported association of Type I diabetes mellitus and systemic scleroderma. Limited joint mobility and skin abnormalities are frequent in childhood diabetes mellitus but should not be misdiagnosed as systemic scleroderma.
一名14岁男孩患有I型糖尿病,随后出现胰腺外分泌功能不全和系统性硬化症(SSc)。他的糖尿病在大约5岁时酮症酸中毒发作后被诊断出来。从那时起直到在我们科室接受治疗,他一直接受胰岛素治疗。9岁时出现的外分泌性胰腺功能不全用胰腺提取物进行治疗。氯化物汗液试验排除了囊性纤维化。14岁时根据皮肤硬化、指端硬化以及食管和肺部受累确诊为SSc,18岁时因出现雷诺病再次确诊。因此,该患者表现出I型糖尿病与系统性硬皮病一种罕见且此前未报道过的关联。儿童糖尿病中关节活动受限和皮肤异常很常见,但不应误诊为系统性硬皮病。