| 摘要: |
| 小肠淋巴管扩张症是一种罕见病,常表现为腹泻、水肿、胸腹腔积液,乳糜性胸腹腔积液等,可出现生长发育迟缓、免疫
功能低下等临床表现。该病临床表现差异大,极易被误诊。原发性免疫缺陷病是先天性免疫功能异常疾病的统称,随着国内医
生对该病认知水平的提升,越来越多的患者被临床早期识别。本文报道1 例以反复腹泻、淋巴细胞数量明显下降为主要临床特
点,初诊考虑为原发性免疫缺陷病的小肠淋巴管扩张症患儿,同时文献复习小肠淋巴管扩张症的临床特点以及和原发性免疫缺
陷病的主要临床鉴别点,帮助临床医生更早期对两种疾病做出鉴别,及时做出更有利的临床判断。 |
| 关键词: 小肠淋巴管扩张症 淋巴细胞 原发性免疫缺陷病 |
| DOI：10.13407/j.cnki.jpp.1672-108X.2025.06.009 |
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| 基金项目:重庆市自然科学基金面上项目,编号CSTB2023NSCQ-MSX0486;重庆市教委重点项目,编号KJZD-K202400407。 |
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| Intestinal Lymphangiectasia: a Case Report and Literature Review |
| Shi Jing1, Yu Zhongsu2, Wang Li2, Tang Xuemei2, Zhao Xiaodong2, Yang Xi2 |
| (1. Chenzhou No. 1 People’s Hospital,Hunan Chenzhou 423000, China; 2. Children’s Hospital of Chongqing Medical University, Chongqing 400014, China) |
| Abstract: |
| Intestinal lymphangiectasia is a kind of rare disease, presenting with diarrhea, edema, thoracoabdominal effusion and
chylous thoracoabdominal effusion, and may manifest as growth retardation and immunodeficiency. The clinical presentation of the
disease varies widely and is highly susceptible to misdiagnosis. Primary immunodeficiency diseases are a collective term for disorders of
congenital immune function, with the increased awareness of the disease among clinicians in China, more and more patients have been
identified at an early clinical stage. This article reports a case of intestinal lymphangiectasis with the main clinical characteristics of
repeated diarrhea and significant decrease in the number of lymphocytes, which was initially considered as a primary immunodeficiency
disease. Meanwhile, the literature reviewed the clinical characteristics of intestinal lymphangiectasis and main clinical differentiation
points from primary immunodeficiency diseases, with the aim of helping a large number of clinicians to identify the two diseases at an
earlier stage, make a more favorable clinical judgment in a timely manner. |
| Key words: intestinal lymphangiectasia lymphocyte primary immunodeficiency disease |
