特发性腹膜后纤维化致急性肾损害1例诊治及文献复习

Analysis of the Diagnosis and Treatment of 1 Cases and Literature Damage in Acute Kidney Caused by Idiopathic Retroperitoneal Fibrosis

目的加深对特发性腹膜后纤维化疾病的认识,提高早期诊断和治疗水平。方法分析我科收治1例特发性腹膜后纤维化导致急性肾损害患者的诊治过程,并复习近年国内外有关腹膜后纤维化的文献资料。结果本例患者以双下肢浮肿就诊。腹部彩超:双肾积水。CT腹主动脉周围及后方可见不规则软组织密度影,左侧髂动脉前移,双侧肾盂扩张积水改变,双侧输尿管中上端扩张,腹主动脉下段及髂动脉扩张。化验:肾功能:血肌酐186μmol/L,尿素氮10.89mmol/L,血沉74mm/h,γ球蛋白25.3%,α2球蛋白8%。结合影像学检查及化验确诊为:特发性腹膜后纤维化并急性肾损害。经过强的松治疗近期临床效果满意。结论特发性腹膜后纤维化发病率低,临床表现缺乏特异性,早期误诊率高,影像学检查是明确诊断的重要举措。糖皮质激素对特发性腹膜后纤维化治疗效果较好。

Objective To investigate the early diagnosis and treatment for retroperitoneal fibrosis（RPF）. Methods The processes of the diagnosis and therapy were analyzed for one patient with idiopathic retropcritoneal fibrosis（1RF）.Admitted to our department and the domestic and foreign literature materials about RPF in recent years were reviewed. Results Patients with a pair of lower limbs dropsy to clinic. Abdominal ultrasound results;bilateral hydronephrosis.A soft--tissue mass surrounding the abdominal aorta in retroperitoneal,left arterial iliac was antedisplacement,Bilateral renal pelvis Expansion The upper of Bi（ateral urethral expansion,Expansion of Abdominal aorta under paragraph and Iliac artery was showed by CT.SCR：186μmol/ L,BUN： 10.89mmol/L,ESR：7mm/ h, γ-Globulin25.3%, α 2Globulin8%. According to the imaging examination and laboratory diagnosis;The idiopathic retroperitoneal fibrosis and Acute renal damage.After Prednisone therapy has Satisfactory clinical effect.Cone lus ion RPF is a rare disease and often has a misdiagnosis and symptoms was nospecific in the early phase.Analysis of imaging conduce to refine the diagnostic process. Corticosteroid has a good effectiveness on the treatment of most cases with RPF.