Flick J A, Boyle J T, Tuchman D N, Athreya B H, Doughty R A
Division of Gastroenterology, Children's Hospital of Philadelphia.
Pediatrics. 1988 Jul;82(1):107-11.
To determine the frequency and nature of esophageal motor abnormalities in children and adolescents with scleroderma syndromes and mixed connective tissue disease, esophageal manometry was performed on seven patients with progressive systemic sclerosis, four patients with mixed connective tissue disease, and two patients with linear scleroderma. A total of 73% of patients with progressive systemic sclerosis and mixed connective tissue disease had symptoms of esophageal dysfunction. A significant association between the presence of Raynaud phenomenon and esophageal symptoms was noted. Esophageal motor abnormalities were detected in 73% of patients with progressive systemic sclerosis and mixed connective tissue disease; these abnormalities were characterized by decreased lower esophageal sphincter pressure and abnormal peristalsis in the distal two thirds of the esophageal body. They resemble those described among adults with progressive systemic sclerosis and mixed connective tissue disease but were not related to disease duration or to the presence of Raynaud phenomenon. Patients with linear scleroderma did not have esophageal symptoms and demonstrated only nonspecific motor abnormalities that did not worsen during several years of follow-up.
为了确定硬皮病综合征和混合性结缔组织病患儿及青少年食管运动异常的频率和性质，对7例进行性系统性硬化症患者、4例混合性结缔组织病患者和2例线状硬皮病患者进行了食管测压。进行性系统性硬化症和混合性结缔组织病患者中共有73%有食管功能障碍症状。观察到雷诺现象的存在与食管症状之间存在显著关联。在进行性系统性硬化症和混合性结缔组织病患者中，73%检测到食管运动异常；这些异常的特征是食管下括约肌压力降低以及食管体远端三分之二蠕动异常。它们与在患有进行性系统性硬化症和混合性结缔组织病的成年人中所描述的异常相似，但与病程或雷诺现象的存在无关。线状硬皮病患者没有食管症状，仅表现出非特异性运动异常，在数年的随访中未加重。
