蛋白丢失性肠病的临床特点分析

Analysis of clinical characteristics with protein losing enteropathy

目的概括蛋白丢失性肠病(protein losing enteropathy,PLE)的临床特点及诊治情况。方法以"蛋白丢失性肠病"或"小肠淋巴管扩张"为主题词,在万方、维普、CNKI检索我国2000～2010年公开发表的PLE病例报道。结果纳入的77份病例均除外摄入不足、肝脏合成减少和肾脏丢失,并且证实蛋白从肠道丢失或有影像学和内镜下的诊断依据。其中男30例,女47例,性别比为1∶1.57,成人起病平均年龄(41.06±5.88)岁,儿童起病平均年龄(5.57±2.33)岁。男女起病年龄与病程差异均无统计学意义。本文PLE的病因儿童组以小肠淋巴管扩张症(55.6%)为主,成人组以系统性红斑狼疮(43.9%)为主。PLE的首发症状为浮肿(84.4%),其次分别为腹泻(48.1%)、腹胀(31.2%)、腹痛(28.6%)、消瘦乏力(18.2%)等。结论国内PLE以中年为主,女性多见,起病年龄与病程无性别差异。由于缺乏特异性的临床表现,容易误诊为其他疾病。病程迁延,但预后相对较好。

Objective To summarise the clinical features,diagnosis and treatment of protein losing enteropathy（PLE） in China.Methods Protein losing enteropathy or intestinal lymphangiectasia as Key words,PLE cases were retrieved in Wanfang,CNKI,Weipu in China from 2000 to 2010.Results 77 cases were included,except for inadequate intake,reduced the synthesis and lost by liver and kidney diseases,confirmed by radiological and endoscopic diagnosis.There were 30 male and 47 female（1∶ 1.57）,adult age was（41.06±5.88） years old,childhood onset age was（5.57±2.33） years old.Age of onset and duration of men and women had no statistical significance.The main etiology of children PLE was intestinal lymphangiectasia（55.6%）,adult PLE was systemic lupus erythematosus（43.9%）.The initial symptom of PLE was edema（48.1%）,abdominal distension（31.2%）,abdominal pain（28.6%）,weight loss and weakness（18.2%）.Conclusion In China,the protein losing enteropathy is predominant in middle-aged females.Most of the patients have a good prognosis process.