儿童系统性红斑狼疮并发假性肠梗阻8例临床研究  

Intestinal pseudo-obstruction in children with systemic lupus erythematosus:A clinical study of 8 cases

作　　者：唐晓艳[1] 李冀[1] 苟丽娟[1] 张玉[1] 李正红[1] 宋红梅[1] TANG Xiao-yan;LI Ji;GOU Li-juan(Department of Pediatrics,Peking Union Medical College Hospital,Chinese Academy of Medical Science,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院儿科,北京100730

出　　处：《中国实用儿科杂志》2021年第1期38-41,共4页Chinese Journal of Practical Pediatrics

摘　　要：目的探讨儿童系统性红斑狼疮(SLE)并假性肠梗阻(IPO)的临床特点及诊治方法。方法回顾性分析中国医学科学院北京协和医院2010年1月至2018年12月收治的16岁以下8例SLE并IPO患儿的临床资料,总结其发病特点、诊治方案和预后。结果共8例纳入研究,其中男2例,女6例,发病年龄(13.6±1.7)岁(11~15岁)。8例患儿均表现为腹胀、恶心、呕吐、腹痛,其中1例并腹泻。所有患儿立位腹部平片可见气液平面,肠管积气,其中6例提示小肠积气,2例提示结肠积气。所有患儿抗核抗体滴度增高,抗RNP抗体阳性4例,3例抗SSA/Ro52抗体阳性;所有IPO均发生于SLE活动期,4例为SLE首发症状,其中1例因考虑急腹症于当地医院行剖腹探查术。4例IPO同时伴肾盂积水、输尿管扩张。所有IPO治疗采用糖皮质激素联合环磷酰胺,均反应良好,无死亡病例。结论IPO是SLE的一个少见但严重的并发症,及早诊断,联合糖皮质激素和环磷酰胺治疗对于改善预后非常重要。早期识别,正确诊断可减少不必要的手术。Objective To investigate the clinical characteristics and treatments of systemic lupus erythematosus(SLE)with intestinal pseudo-obstruction(IPO)in children.Methods We retrospectively analyzed the clinical data,summarizing the characteristics of the disease,the diagnosis,the treatments and prognosis of 8 cases which were diagnosed as SLE with IPO under16 years old at Peking Union Medical College Hospital from January 2010 to December 2018.Results All of 8 cases,2 males and 6 females,were included in the study.The mean age of onset was(13.6±1.7)years old(11-15 years).All of 8 children had abdominal distension,nausea,vomiting,and abdominal pain,and 1 case had diarrhea.Gas-liquid plane can be found in conventional plain abdominal X-ray in 8 children.Six patients had dilated small bowel loops,while 2 with dilated large bowel.All children had elevated anti-nuclear antibody titers.Anti-RNP antibodies were positive in 4 cases,3 positive in anti-SSA/Ro52.All IPO cases were in the active phase of SLE.In 4 cases,IPO was the initial manifestation of SLE,and one patient received abdominal surgery for being suspected as acute abdominal at the local hospital.Four children had hydronephresis and ureterectasis at the same time.All cases received glucocorticoid and cyclophosphamide with good response,and no death was reported.Conclusion IPO is a rare but severe complication of SLE.It is very important to make timely diagnosis,and the combination treatment with prednisone and cyclophosphamide is very important to better prognosis.Early diagnosis can reduce unnecessary surgery.

关 键 词：系统性红斑狼疮 儿童 假性肠梗阻 输尿管扩张 糖皮质激素 环磷酰胺 

分 类 号：R72[医药卫生—儿科]