Nguyen Chuong Dinh, Buchman Alan, Bui Hoang Huu, Thi-Lan Pham Anh, Thi-Ngoc Vo Diem, Dang Luan Minh, Vo Van Huy
Department of Gastroenterology, University Medical Center Ho Chi Minh City, Vietnam.
Intestinal Rehabilitation and Transplant Center, Department of Surgery/UI Health, University of Illinois at Chicago, Chicago, IL.
ACG Case Rep J. 2022 Dec 26;9(12):e00939. doi: 10.14309/crj.0000000000000939. eCollection 2022 Dec.
Primary intestinal lymphangiectasia is a rare disorder that may result in protein-losing enteropathy. We report a 21-year-old man with malabsorption syndrome, an unintentional weight loss of 30 kg over 10 months, lymphocytopenia, and hepatic aminotransferase elevation. His diagnosis was established by a combination of enteroscopy, histopathology, and secondary etiology exclusion. Institution of parenteral nutrition, followed by a low-fat diet, medium-chain triglycerides, and octreotide, resulted in the resolution of his symptoms and laboratory abnormalities and led to weight gain. Aminotransferase abnormalities are an atypical finding in primary intestinal lymphangiectasia and were most likely due to nonalcohol steatohepatitis after rapid weight loss. Primary intestinal lymphangiectasia should be considered in patients with protein-losing enteropathy and lymphocytopenia.
原发性肠淋巴管扩张症是一种罕见的疾病，可能导致蛋白丢失性肠病。我们报告一名21岁男性，患有吸收不良综合征，在10个月内无意体重减轻30kg，淋巴细胞减少，肝转氨酶升高。通过小肠镜检查、组织病理学检查和继发性病因排除相结合的方法确诊。给予肠外营养，随后采用低脂饮食、中链甘油三酯和奥曲肽治疗，使他的症状和实验室异常情况得到缓解，并实现了体重增加。转氨酶异常在原发性肠淋巴管扩张症中是不典型表现，很可能是快速体重减轻后发生的非酒精性脂肪性肝炎所致。对于患有蛋白丢失性肠病和淋巴细胞减少的患者，应考虑原发性肠淋巴管扩张症。
