同胞兄弟特发性腹膜后纤维化2例报告及文献复习

目的:提高对腹膜后纤维化疾病的认识。方法:回顾性分析2例同胞兄弟特发性腹膜后纤维化的临床资料,并结合文献复习对其病因、诊断和治疗等进行归纳。结果:弟弟首先发病,就诊时病理提示为晚期腹膜后纤维化伴有严重的肾功能损害,药物和手术治疗效果欠佳,通过肾造瘘维持肾功能,预后不良;哥哥随后发病,出现不典型的临床症状,由于有弟弟的相关病史而获得早期诊断和手术治疗,得到治愈。结论:腹膜后纤维化早期缺乏特异性临床表现,影像学的特征和病理表现有助于明确诊断和选择治疗方案。早期诊断疗效好,晚期患者应保护肾功能,提高生活质量,并做好长期随访。

Idiopathic retroperitoneal fibrosis in 2 brothers(Report of 2 cases)

Objective:To improve the diagnosis and treatment of Idiopathic retroperitoneal fibrosis(IRF).Methods:The clinical data of adult siblings with idiopathic retroperitoneal fibrosis were reviewed.Results:The young brother first presented with serious renal failure.Biopsy of surgery showed advanced retroperitoneal fibrosis.Ureterolysis and intraperitoneal transposition wrapping with omental plus steroids showed ineffective.Percutaneous nephrostomy bilaterally kept his renal function to death.Then because of his young brother's history the elder were diagnosed IRF with moderate renal impairment.Surgery alone and Follow-up of 6 years showed well.Conclusions:In the majority of IRF patient,the early symptoms are of no-specific.Analysis of imaging and pathology conduce to refine the diagnostic process and treatment strategies.Early diagnosis and treatment promise favourable outcomes.Sustaining renal function is key point to advanced RF patient.Long-term follow-up is mandatory in all patients.