| Haojie Li,Daoyu Hu,Lili Liang,Yao Hu,Zhen Li. Intestinal lymphangiectasia associated with chylothorax and multiple lower extremity arteriovenous malformation: A case report and literature review. Oncol Transl Med, 2015, 1: 177-180. |
| 小肠淋巴管扩张症伴发乳糜胸及双下肢动静脉血管畸形：病例报道 与文献复习 |
| Intestinal lymphangiectasia associated with chylothorax and multiple lower extremity arteriovenous malformation: A case report and literature review |
| Received:June 18, 2015 Revised:July 27, 2015 |
| DOI：10.1007/s10330-015-0107-7 |
| 中文关键词: 小肠淋巴管扩张症; 病例报道; CT; MRI |
| 英文关键词: Intestinal lymphangiectasia (IL); case report; CT; MRI |
| 基金项目: |
| Author Name | Affiliation | E-mail | | Haojie Li | Departments of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology | 897891279@qq.com | | Daoyu Hu | Departments of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology | cjr.hudaoyu@vip.163.com | | Lili Liang | Departments of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology | 409114750@qq.com | | Yao Hu | Departments of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology | 2320577158@qq.com | | Zhen Li* | Departments of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology | doclizhen@qq.com |
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| 中文摘要: |
| 小肠淋巴管扩张症是一种罕见的蛋白丢失性肠病，以小肠粘膜或浆膜淋巴管扩张和淋巴液丢失为特征。目前，无论是在儿童或者成人，小肠淋巴管扩张症都是一种非常罕见的疾病，典型的临床症状包括低蛋白血症，淋巴细胞绝对减少，腹腔积液，浮肿等。我们报告一例成人小肠淋巴管扩张症患者，同时伴有乳糜胸和臀部及双下肢动静脉畸形。CT和MRI可显示弥漫性水肿和小肠增厚，并伴有脾肿大及胸腔积液。肠镜检查显示回肠下段及回盲部广泛的结节状隆起。最后，小肠黏膜固有层淋巴管扩张病理检查证实。为了提高对该病的认识，我们比较并复习之前病例报告及相关文献，并讨论小肠淋巴管扩张症的诊断和治疗。 |
| 英文摘要: |
| Intestinal lymphangiectasia (IL) is an uncommon protein losing enteropathy, characterized by small intestinal mucosa or serosa lymphangiectasia and intestine lymph loss. Currently, IL is a very rare disease in children or adults, with typical clinical symptoms including hypoalbuminemia, absolute lymphocyte reduction, ascites, edema, etc. We report a case of an adult with intestinal lymphatic ectasia accompanied by chylothorax and multiply arteriovenous malformations of the hip and lower extremity. CT and MRI revealed diffuse edema and thickening of the small intestine, accompanied by splenomegaly and pleural effusion. Extensive nodularity of lower ileum and the ileocecal region could be seen during intestinal endoscopy. Finally, small intestinal lamina propria lymphangiectasis was confirmed by pathological examination. To raise awareness of the disease, here we compare our case and those previously reported, and discuss the
diagnosis and management of IL. |
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