原发性小肠淋巴管扩张症50例临床表现分析  

Clinical features of 50 patients with primary intestinal lymphangiectasia

作　　者：钱幼蕾 孙宇光 苏万春 信建峰 常鲲 夏松 沈文彬 Qian Youlei;Sun Yuguang;Su Wanchun;Xin Jianfeng;Chang Kun;Xia Song;Shen Wenbin(Department of Lymphatic Surgery,Beijing Shijitan Hospital,Capital Medical University,Beijing100038,China)

机构地区：[1]首都医科大学附属北京世纪坛医院淋巴外科,北京100038

出　　处：《中华外科杂志》2024年第12期1150-1156,共7页Chinese Journal of Surgery

基　　金：北京市医管中心培育项目(2022-A01)。

摘　　要：目的探讨原发性小肠淋巴管扩张症(PIL)的临床表现。方法本研究为回顾性病例系列研究。连续收集首都医科大学附属北京世纪坛医院淋巴外科2019年3月至2021年3月收治的50例原发性小肠淋巴管扩张症患者的临床资料。男性20例,女性30例,发病年龄为[M(IQR)]14(40)岁(范围:0 d至67岁),均无家族史。其中儿童26例,男9例,女17例,年龄0.7(5.8)岁(范围:0 d至14岁);成年人24例,男性11例,女性13例,年龄40(26)岁(范围:20~67岁)。收集患者的临床和病理学资料,包括临床表现、实验室检查结果、消化内镜检查结果、^(99)Tc标记的人血白蛋白(^(99)Tc^(m)-HSA)核素显像、^(99)Tc标记的右旋糖酐(^(99)Tc^(m)-DX)淋巴核素显像、直接淋巴管造影(DLG)及组织病理学检查结果、是否低脂饮食、手术方式、出院时临床症状缓解情况等。组间比较采用独立样本t检验,Wilcoxon秩和检验或χ^(2)检验。结果50例PIL患者的主要表现为水肿(86.0%)、腹泻(76.0%)和腹水(48.0%),淋巴水肿(36.0%)与乳糜性腹水(18.0%)亦较常见。95.9%(47/49)患者的^(99)Tc^(m)-HAS核素显像提示肠蛋白丢失;91.7%(44/48)患者的^(99)Tc^(m)-DX淋巴核素显像有阳性发现。DLG示97.8%(45/46)的患者存在胸导管梗阻,82.6%(38/46)的患者存在腹膜后淋巴管增生扩张,23.9%(11/46)的患者可见造影剂反流至肠管。成年人组与儿童组在性别、病程、临床表现、白蛋白、球蛋白、淋巴细胞绝对值、便潜血阳性率等方面均无差异(P值均>0.05)。结论PIL在儿童和成年人中均可发病,且临床表现无明显差异。PIL的诊断需结合临床表现、实验室检查、^(99)Tc^(m)-HSA核素显像、^(99)Tc^(m)-DX淋巴核素显像、DLG、消化内镜及病理学检查结果综合判断。Objective To investigate the clinical features of primary intestinal lymphangiectasia(PIL).Methods This study was a retrospective case series study.Fifty consecutive patients diagnosed with PIL in Department of Lymphatic Surgery,Beijing Shijitan Hospital,Capital Medical University from March 2019 to March 2021 were included and their clinical data was retrospectively reviewed.There were 20 males and 30 females included,with an age of(M(IQR))14(40)years(range:0 to 67 years).No patient had the family history.There were 26 children,including 9 males and 17 females,aged 0(7)years(range:0 to 14 years).There were 24 adults,including 11 males and 13 females,aged 40(26)years(range:20 to 67 years).The clinical manifestations and the results of laboratory examinations,gastrointestinal endoscopy,^(99)Tc^(m)-labeled human albumin(^(99)Tc^(m)-HSA)scintigraphy,^(99)Tc^(m)-DX scintigraphy,direct lymphangiography(DLG),histopathology,diet treatment,surgical intervention,and clinical symptom remission at discharge were collected.Comparisons between groups were performed using independent samples t-test,Mann-Whitney U test,orχ^(2) test.Results Among the 50 cases of PIL,the main manifestations were edema(86.0%),diarrhea(76.0%),and abdominal effusion(48.0%).Lymphedema(36.0%)and chylous ascites(18.0%)were not rare in PIL patients.In ^(99)Tc^(m)-HAS scintigraphy,95.9%(47/49)cases showed signs of intestinal protein loss,and 91.7%(44/48)ceses showed positive findings in ^(99)Tc^(m)-DX scintigraphy.In DLG,97.8%(45/46)cases showed signs of thoracic duct obstruction,82.6%(38/46)cases showed retroperitoneal lymphatic hyperplasia,and 23.9%(11/46)cases showed backflow of contrast agent into intestine.No significant difference was seen in gender,course of disease,clinical manifestation,serum level of albumin or globulin,lymphocyte count,positive rate of fecal occult blood and prevalence of lymphedema between adults and children(all P>0.05).Conclusion sThe clinical presentations of PIL between children and adults had no significant difference.T

关 键 词：淋巴管扩张 肠 淋巴水肿 原发性小肠淋巴管扩张症 直接淋巴管造影 淋巴核素显像 

分 类 号：R574.5[医药卫生—消化系统]