Objective To investigate the diagnosis and treatment of pulmonary arterial hypertension ( PAH) due to rare causes. Methods The clinical presentation, laboratory testing, diagnosis and treatment of 4 patients with PAH associated with rare causes in Beijing Anzhen Hospital from January 2001 to March 2008 were analysed retrospectively. Results Primary biliary cirrhosis, hyperthyroidism, antiphospholipid syndrome and pulmonary artery sarcoma may cause PAH, which were improved after corresponding diagnosis and management. Conclusion PAH can result from rare causes. The enhancement of its recognition will help earlier diagnosis and treatment and improve the prognosis.
目的:分析原发性胆汁性肝硬化(PBC)患者的临床表现,试验室检查及治疗情况。方法:回顾性分析48例PBC患者临床资料。结果:93.8%是中年女性患者,平均年龄53.2±8.73。主要的临床表现包括肝功能异常(ALT、GGT、AKP升高)95.8%,乏力纳差83.3%,黄疸79.2%,瘙痒66.6%,肝肿大62.5%,脾大58.3%等。常合并干燥综合征(25%),类风湿关节炎(16.7%)等自身免疫性疾病及结缔组织疾病。所以患者AMA及AMAM-2均为阳性。全部病例使用熊去氧胆酸治疗,但仅31.3%患者病情有不同程度好转。结论:加强对PBC的认识,重视对AMA 或AMA-M2的检测,尤其对长期不明原因肝功能异常的女性患者。