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腺瘤性甲状腺肿切除术后治愈的蛋白丢失性肠病:一例报告

Protein-losing enteropathy cured by resection of adenomatous goiter: report of a case.

作者信息

Takada Jun, Araki Hiroshi, Kubota Masaya, Ibuka Takashi, Shiraki Makoto, Shimizu Masahito, Moriwaki Hisataka

机构信息

Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan,

出版信息

Clin J Gastroenterol. 2015 Jun;8(3):120-5. doi: 10.1007/s12328-015-0565-z. Epub 2015 Apr 7.

DOI:10.1007/s12328-015-0565-z
PMID:25845937
Abstract

A 51-year-old Japanese woman presented to our hospital with systemic edema and general fatigue. Her serum albumin level was very low (1.5 g/dL). Technetium-99 m-human serum albumin ((99m)Tc-HSA) scintigraphy showed albumin leakage from the upper small bowel. Magnetic resonance lymphangiography showed dilated lymphatic vessels in the chest, whereas double-balloon enteroscopy (DBE) showed white villi and chyle leakage in the deeper part of the duodenal mucosa. A duodenal mucosa biopsy specimen revealed lymphangiectasia. She was diagnosed with protein-losing enteropathy (PLE). Treatment with a fat-restricted diet and tranexamic acid--previously reported to be effective against PLE--was attempted, but was ineffective. A thyroid tumor was simultaneously detected in her left neck, and was found to extend to the mediastinum on computed tomography. The tumor (size, >5 cm) was resected, and a pathological diagnosis of adenomatous goiter was made. The patient's serum albumin level increased to normal levels within 1 month postoperatively. After 6 months, (99m)Tc-HSA scintigraphy showed no albumin leakage from the gastrointestinal tract, and disappearance of white villi and chyle leakage on DBE. No lymphangiectasia was noted in the biopsy specimen. Adenomatous goiter was thus considered the cause of the PLE, possibly through lymph flow obstruction in the mediastinum.

摘要

一名51岁的日本女性因全身水肿和全身乏力前来我院就诊。她的血清白蛋白水平极低(1.5 g/dL)。锝-99m-人血清白蛋白((99m)Tc-HSA)闪烁扫描显示上小肠有白蛋白渗漏。磁共振淋巴造影显示胸部淋巴管扩张,而双气囊小肠镜检查(DBE)显示十二指肠黏膜深层有白色绒毛和乳糜渗漏。十二指肠黏膜活检标本显示淋巴管扩张。她被诊断为蛋白丢失性肠病(PLE)。尝试采用低脂饮食和氨甲环酸治疗——此前报道该治疗方法对PLE有效——但无效。同时在她的左颈部发现一个甲状腺肿瘤,计算机断层扫描显示该肿瘤已延伸至纵隔。切除了该肿瘤(大小>5 cm),病理诊断为腺瘤性甲状腺肿。术后1个月内患者的血清白蛋白水平升至正常水平。6个月后,(99m)Tc-HSA闪烁扫描显示胃肠道无白蛋白渗漏,DBE显示白色绒毛和乳糜渗漏消失。活检标本中未发现淋巴管扩张。因此,腺瘤性甲状腺肿被认为是PLE的病因,可能是通过纵隔内的淋巴流阻塞所致。

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本文引用的文献

1
Primary intestinal lymphangiectasia: Minireview.原发性肠淋巴管扩张症:综述
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Primary intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by medium-chain triglycerides: a case report.经双气囊小肠镜诊断并采用中链甘油三酯治疗的原发性小肠淋巴管扩张症:一例报告
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J Clin Gastroenterol. 2012 Aug;46(7):575-80. doi: 10.1097/MCG.0b013e31823832ac.
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Eur J Gastroenterol Hepatol. 2005 Dec;17(12):1417-9. doi: 10.1097/00042737-200512000-00024.
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Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy.原发性肠及胸导管扩张症:抗纤溶酶治疗的反应
Pediatrics. 2002 Jun;109(6):1177-80. doi: 10.1542/peds.109.6.1177.
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Characteristic endoscopic features of intestinal lymphangiectasia: correlation with histological findings.肠道淋巴管扩张症的特征性内镜表现:与组织学结果的相关性
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Resolution of longstanding protein-losing enteropathy in a patient with intestinal lymphangiectasia after treatment for malignant lymphoma.一名患有肠道淋巴管扩张症的患者在接受恶性淋巴瘤治疗后,其长期存在的蛋白丢失性肠病得到缓解。
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