小肠淋巴管扩张症2例

目的 提高对儿童小肠淋巴管扩张症(IL)的认识.方法　对2例IL患儿的临床表现、诊断、治疗及预后等临床资料进行分析.2例患儿除行血液、尿液、腹部B超等检查外,均接受电子小肠镜检查,并取小肠黏膜行病理活检.结果 例1,9岁发病,以腹泻、水肿起病.WBC 5.80×109L-1,L 0.15,清蛋白17 g·L-1,IgG 2.83 g·L-1,IgA 0.61 g·L-1.小肠镜检查发现自回盲瓣至回肠黏膜弥散性白色结节样隆起,绒毛欠清晰,病理提示黏膜淋巴管扩张症,考虑原发性IL.饮食对症治疗有效,随访情况良好.例2,12岁发病,以双下肢、颜面及眼睑水肿起病.WBC 5.60×109L-1,L 0.216,清蛋白24 g·L-1,IgG 1.83 g·L-1,IgA 0.58 g·L-1.小肠镜检查发现空回肠交界以下小肠黏膜散在结节,不平,颜色偏白,病理结果提示部分绒毛顶端可见扩张的淋巴管,考虑先天性心脏病和手术引起的继发性IL.饮食对症治疗效果不理想.结论　对于慢性腹泻或反复水肿伴低蛋白血症的患儿要警惕IL的可能,尽早行内镜及病理活检检查以明确诊断,并尽量寻找病因,给予有效治疗,以改善预后.     

儿童小肠淋巴管扩张症18例临床分析

目的 探讨儿童小肠淋巴管扩张症(intestinallymphangiectasia,IL)的临床表现、诊断、治疗和预后,提高对儿童IL的认识和诊治水平.方法 回顾性分析2010年1 月至2019年12 月在广州市妇女儿童医疗中心消化科诊断IL 18例患儿临床资料、实验室检查、胃肠镜、组织病理学等结果,比较1 岁内起病...     

儿童小肠淋巴管扩张症的诊断及营养干预对预后的影响:附5例报告

目的 探讨小肠淋巴管扩张症(intestinal lymphangiectagia,IL)的诊断、营养干预及预后.方法 对确诊为IL的5例患儿(原发性4例,继发性1例)进行回顾性分析,总结并比较其临床表现、诊断方法、营养支持治疗及预后情况.结果 4例原发性lL患儿经过3～4周高蛋白、限制长链三酰甘油及补充中链三酰甘油的饮食干预,并辅以静脉营养支持治疗后,临床症状及实验室检查均有明显改善.例5经手术去除原发病后,症状和体征消失.结论 IL的治疗取决于不同的病因和病情严重程度,饮食干预及肠外营养支持是非常有效的治疗方法,可以很好改善患儿的临床症状和体征.     

原发性小肠淋巴管扩张症2例并文献复习

目的 探讨小肠淋巴管扩张症的临床表现，诊断、治疗及预后等。方法 2例患儿除一般的血清学、尿液、超声心动图、腹部B超和腹部CT等检查外，均接受胃镜和（或）小肠镜检查并取黏膜行病理活检。结果 例1，5岁发病，以反复水肿起病，小肠镜检查发现空肠、回肠弥漫性白色粟米样改变，病理提示肠黏膜固有膜内及黏膜肌层内可见到较多扩张淋巴管，淋巴管内皮细胞增生不明显。例2，5月龄发病，以水肿、腹泻起病，胃镜检查发现十二指肠黏膜弥漫性白色粟米样改变，正常绒毛结构消失，病理提示个别绒毛黏膜内可见轻度扩张淋巴管。2例患儿均除外引起小肠淋巴管扩张的继发性因素，结合起病年龄小，考虑原发性小肠淋巴管扩张症，其中例1诊断为小肠弥漫性淋巴管扩张。这2例患儿通过中链三酰甘油乳剂治疗后，随访症状明显缓解。结论 小肠淋巴管扩张症在儿童中可能并不少见。对于反复低蛋白血症、反复水肿或慢性腹泻的患儿要警惕原发性小肠淋巴管扩张症，应尽早行内镜及病理活检，并通过特殊的饮食控制疾病。     

以腹部膨隆为突出表现的原发性小肠淋巴管扩张症1例

原发性小肠淋巴管扩张症(primary intestinal lymphangiectasia,PIL)是一种少见的蛋白丢失性肠病,其特征是小肠黏膜淋巴管结构缺陷导致淋巴管扩张和功能性阻塞,使乳糜微粒和淋巴回流障碍,淋巴液漏出至小肠管腔,最终导致吸收不良和蛋白丢失[1]。临床主要表现为水肿、低蛋     

原发性小肠淋巴管扩张症1例报告并文献复习

目的探讨原发性小肠淋巴管扩张症(PIL)的临床表现及预后。方法回顾性分析1例PIL患儿的临床资料,并复习相关文献。结果患儿,女,5岁,生后逐渐出现进行性加重的腹胀、腹泻、全身水肿。实验室检查显示低白蛋白血症、淋巴细胞减少症、低丙种球蛋白血症。胃镜检查见十二指肠空肠弥漫的白色斑点,活检证实黏膜间质淋巴管扩张。确诊后予静脉输注白蛋白、利尿和调整饮食等治疗,病情很快缓解出院。结论原发性小肠淋巴管扩张症是一种罕见的蛋白丢失性肠病,诊断主要依靠小肠病理活检。早期诊断、及时饮食干预及肠外营养支持等可以很好改善患儿的症状和体征。     

婴儿原发小肠淋巴管扩张症8例

目的 提高婴儿原发小肠淋巴管扩张症(PIL)的诊治水平.方法 回顾性分析2007年1月至2012年2月北京儿童医院收治的8例PIL婴儿的临床表现、实验室检查、胃镜和影像、淋巴核素显像、病理及治疗经验,结合文献对婴儿PIL诊治进行探讨.结果 年龄4～8个月;水肿(8/8例)、腹泻(8/8例)、感染(8/8例)、腹水(7/8例)为常见症状,其他症状有呕吐(5/8例)、低体质量(2/8例)、惊厥(2/8例);感染较为突出,累及呼吸道、血液、消化道等,病原有细菌、病毒、真菌、原虫等;实验室检查血淋巴细胞绝对值、清蛋白、球蛋白降低(8/8例),免疫学检查CD4及IgG降低(7/7例),B淋巴细胞和NK细胞基本正常;胃镜可见十二指肠黏膜细小颗粒样白色结节(8/8例);病理为黏膜及黏膜下层淋巴管扩张(5/8例);淋巴核素显像检查多有阳性发现(6/8例),3例病理阴性者淋巴核素显像有阳性发现.治疗包括低脂或富含中链三酰甘油饮食、补充清蛋白及球蛋白、控制感染等;内科治疗后感染及水肿效果较好,但腹泻治疗不理想,仅有2例清蛋白及淋巴细胞恢复正常,外科手术治疗1例,至今未再复发.结论 婴儿PIL临床表现与一般小肠淋巴管扩张症基本相同,但感染是婴儿PIL较为突出的表现.推荐淋巴核素显像检查作为诊断婴儿PIL的方法之一.婴儿PIL内科治疗效果仍不理想、病变局限者建议手术治疗.     

小肠淋巴管扩张症1例报告并文献复习

目的探讨小肠淋巴管扩张症的诊断方法及治疗。方法分析1例小肠淋巴管扩张症患儿的病史、临床和病理检查结果,以及随访观察半年后复查的各项相关指标,同时复习相关文献。结果小肠淋巴管扩张症确诊依据病理检查,内镜下典型表现也可以诊断,胶囊胃镜、双气囊小肠镜、胃镜及结肠镜、腹部CT、小肠造影、粪便α1抗胰蛋白酶清除率测定等均有诊断意义。治疗上主张控制饮食,给予低脂、高蛋白、补充中链脂肪酸的饮食。该患儿经控制饮食治疗6个月、1年后临床症状、体征好转,部分实验室检查指标亦好转,但随访6个月时镜下小肠改变不明显。结论双气囊小肠镜并病理检查是诊断小肠淋巴管扩张症的最佳方法,胃镜结合结肠镜或小肠造影在一定程度上对诊断有帮助。可用低脂、高蛋白、补充中链脂肪酸的饮食疗法,其治疗是一个长期连续的过程,可能与小肠黏膜修复慢有关。     

原发性肠淋巴管扩张症

原发性肠淋巴管扩张症是一种罕见的疾病,它以小肠淋巴管回流受阻、乳糜管扩张及绒毛结构扭曲为特征,淋巴管阻塞以及小肠淋巴管压力升高使淋巴液漏出至小肠管腔,最终导致吸收不良和蛋白丢失.其病因和发病机制尚不清楚,免疫机制异常越来越得到人们的重视.随着内镜技术的不断改进,该病的诊断率明显提高.饮食调节是该病有效的治疗方法,尤其对...     

儿童原发性肠淋巴管扩张症临床特点

<正>肠淋巴管扩张症(intestinal lymphangiectasia,IL)是一种罕见的疾病,它以小肠淋巴管回流受阻、乳糜管扩张及绒毛结构扭曲为特征,淋巴管的阻塞以及小肠淋巴管压力的升高导致淋巴液漏出至小肠管腔,最终导致吸收不良和蛋白丢失。1961年,Waldmann等[1]首次通过51Cr标记白蛋白的方法研究蛋白质渗出部位,进而提出该病。根据病因可分     

原发性小肠淋巴管扩张症四例

例1男,13岁,因反复水肿腹胀3年,加重1个月入院.3年前无诱因出现颜面水肿,腹胀,在当地医院发现血白蛋白及IgG降低,予输白蛋白等对症治疗,病情反复,1个月前又出现全身水肿,腹胀明显.     

Failure of blind small bowel biopsy in the diagnosis of intestinal lymphangiectasia

A case of primary intestinal lymphangiectasia is presented in which multiple blind peroral jejunal biopsies were unable to document any abnormality, despite strongly suggestive clinical history and radiographic findings. Endoscopically directed biopsy was necessary to document the characteristic pathologic lesion. This report documents the importance of endoscopy in the diagnosis of intestinal lymphangiectasia when clinical history is suggestive of intestinal lymphangiectasia but standard small bowel biopsy fails to show any abnormality.     

Congenital intestinal lymphangiectasia: a rare differential diagnosis in hypoproteinemia in infants

BACKGROUND: Congenital intestinal lymphangiectasia is a rare disease in childhood, which may already cause protein-losing enteropathy in newborns. PATIENT, METHODS AND RESULTS: This is a case report of an infant with generalized edema and protein-losing enteropathy, in whom intestinal lymphangiectasia was diagnosed at the age of two months. Following repetitive intravenous albumin und gamma globulin infusions, the elimination of long-chain fats from the diet and the substitution with medium-chain triglycerides (MCT) led to an improvement of the protein-losing enteropathy. CONCLUSION: In newborns with low level of serum protein and edema protein-losing enteropathy caused by congenital lymphangiectasia might be considered as a differential diagnosis.     

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??Abstract:Objective To improve the diagnosis and treatment level of primary intestinal lymphangiectasia ??PIL) in children. Methods Retrospectively analyze the clinical manifestations?? laboratory examinations?? gastroscopy and imaging features?? lymph radionuclide imaging?? pathological results?? therapy experience in 13 PIL children who were admitted to our hospital from January ??2007 to February?? 2012?? related literatures were reviewed. Results The youngest case was a 4-month-old infant ?? the oldest case was a 11-year-old child?? and 8 cases were within 1 year. Edema??13??100%?????infection??12??92.3%?????diarrhea??11??84.6%?????fever??10??76.9%?????and ascites??9??69.2%????were common manifestations?? hematochezia??3??23.1%????? pleural effusion??3??23.1%?????convulsions?? 3??23.1%????and abdominal mass?? 1??7.7%????were relatively rare. Infection was predominant manifestation?? including respiratory tract??blood??lower limbs fascia and esophagus?? pathogens include bacterium??virus??fungus and ectosarc. The laboratory findings in these patients were lymphocytopenia??13??100%????? hypoalbuminemia??13??100%????? hypogammaglobulinemia??13??100%????? the CD4 cell counts??9??90.0%???? and serum IgG levels ??10??100%?????which were significantly decreased?? while B cell and NK cell counts were reduced in one of ten cases. The gastroscopy revealed nodular lesions in duodenal that appeared white opaque spots??13??100%????. Pathological examinations indicated dilated lymphatic channel in mucosal and submucosal ??9??69.2%????. Lymph radionuclide imaging discovered abnormalities consistent with PIL in ten out of twelve patients?? including four cases that were negative for pathological examinations. The main ways of treatment for PIL were introduction of a low-fat and full-MCT diet?? supplement of albumin and globulin??and control of the infection. The temperature returned to normal and edema were alleviated in most patients after internal therapy. However??therapeutic efficacy of diarrhea was not satisfactory??Only three children recovered from hypoalbuminemia and six children recovered from lymphocytopenia. Two cases had a complete remission after surgical therapy without relapse until 2012-12-31. Conclusion Compared to adult IL??PIL in children has similar manifestations??but infection is the predominant clinical manifestation in these patients??lymph radionuclide imaging is suggested to be used as one of the valuable methods in diagnosing PIL in children??internal therapy is not satisfactory in child PIL??surgical therapy can be used if pathological focus is localized.