Liang C D, Ko S F, Huang C B, Niu C K
Department of Pediatrics, Chang Gung Children's Hospital, Kaohsiung, Taiwan, ROC.
J Formos Med Assoc. 1997 Nov;96(11):917-20.
We report a very rare case of systemic sclerosis in a 6-year-old girl. She presented with diffuse scleroderma, Raynaud's phenomenon, pulmonary interstitial fibrosis, pulmonary hypertension, and right ventricular failure. The diagnosis was confirmed by skin manifestations, high resolution computed tomography, cardiac catheterization, and anti-nuclear antibodies. Nifedipine, prednisolone, digoxin, and furosemide were given. There was remission of the right ventricular failure and dyspnea, and the skin showed partial improvement. The patient remained asymptomatic for a year. The symptoms of respiratory and right heart failure developed again after an episode of lower respiratory tract infection and she eventually died. We discuss the clinical manifestations, treatment, and outcome.
我们报告了一例非常罕见的6岁女童系统性硬化症病例。她表现为弥漫性硬皮病、雷诺现象、肺间质纤维化、肺动脉高压和右心衰竭。通过皮肤表现、高分辨率计算机断层扫描、心导管检查和抗核抗体确诊。给予硝苯地平、泼尼松龙、地高辛和呋塞米治疗。右心衰竭和呼吸困难有所缓解，皮肤也有部分改善。患者一年内无症状。一次下呼吸道感染后，呼吸和右心衰竭症状再次出现，最终死亡。我们讨论了临床表现、治疗及预后情况。
