摘要
为提高对婴儿隐匿性原发性小肠淋巴管扩张症的临床、内镜及病理特点的认识,减少漏诊及误诊,我们对厦门市儿童医院收治的1例原发性小肠淋巴管扩张婴儿的临床表现和诊疗相关资料进行了回顾性分析。该例患儿以支气管肺炎收住入院,轻度腹泻和水肿,未见外周血淋巴细胞数减少,呈现低蛋白血症。输注白蛋白未能缓解低蛋白血症,随后胃镜检查显示十二指肠段可见大小不等密集的白色粟粒样颗粒状隆起,黏膜呈广泛白斑样病变,病理检查显示明显的小肠淋巴管扩张,进而确诊小肠淋巴管扩张症。确诊后每两日输注白蛋白,连续输注两个疗程并予portagen中链脂肪酸奶粉喂养。经处理后血浆中总蛋白和白蛋白基本升至正常水平,腹部及胸部彩超显示双胸腔及双肺积液均已被吸收。对该病例的回顾性分析结果表明对于有血浆白蛋白及球蛋白下降而导致浮肿的婴儿,排除肝肾疾患及肿瘤、结核等慢性疾病所致蛋白消耗过多的可能性,均应考虑小肠淋巴扩张症,需尽可能尽早行胃镜检查,进一步完善病理活检,实现早日确诊、尽早进行对症治疗及饮食干预治疗,避免处理不及时导致的严重后果。
Objective In order to improve the understanding of the clinical,endoscopic and pathological characteristics of occult primary intestinal lymphangiectasia in infants,and to reduce misdiagnosis and missed diagnosis,we analyzed the clinical manifestations and diagnosis and treatment related data of a case of primary intestinal lymphangitis in Xiamen Children′s Hospital.The patient was admitted to the hospital with bronchopneumonia,mild diarrhea and edema,without decreasing in the number of peripheral blood lymphocytes,showing hypoproteinemia.Albumin infusion failed to alleviate hypoalbuminemia,and then gastroscopy showed that the duodenal segment had white millet like granular protrusion with different sizes and dense density,and the mucosa had extensive leukoplakia like lesions.Pathological examination showed that the small intestinal lymphadenectasia was obvious,and then the small intestinal lymphadenectasia was confirmed.After diagnosis,albumin was infused every two days for two consecutive courses and portagen medium chain fatty acid milk powder was fed.After treatment,the total protein and albumin in the plasma basically rose to the normal level,and the color Doppler ultrasonography of abdomen and chest showed that both pleural effusion and bilateral pulmonary effusion had been absorbed.The retrospective analysis of this case shows that for infants with dropsy caused by the decrease of plasma albumin and globulin,the possibility of excessive protein consumption caused by liver and kidney disease,tumor,tuberculosis and other chronic diseases should be excluded,and small intestinal lymphadenopathy should be considered,gastroscopy should be performed as soon as possible,pathological biopsy should be further improved,early diagnosis and treatment should be carried out as soon as possible And diet intervention treatment to avoid serious consequences caused by not timely handling.
作者
修阳艳
刘秀华
李明珍
陈競芳
Xiu Yangyan;Liu Xiuhua;Li Mingzhen;Chen Jingfang(Department of Respiratory and Digestive,Children′s Hospital of Fudan University Xiamen Branch&Xiamen Children′s Hospital of Fujian Province 361006)
出处
《中国综合临床》
2020年第3期278-281,共4页
Clinical Medicine of China
关键词
小肠淋巴管扩张症
婴儿
电子胃镜
低蛋白血症
Intestinal lymphangiectasia
Infant
Electronic gastroscope
Hypoproteinemia
