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严重凝血障碍作为一名先前被诊断为乳糜泻患者乳糜泻危象的罕见特征。

Severe Coagulopathy as a Rare Feature of Celiac Crisis in a Patient Previously Diagnosed with Celiac Disease.

作者信息

Gonzalez Juan J, Elgamal Mohamed, Mishra Shikha, Adekolujo Orimisan S

机构信息

Department of Internal Medicine-Hospital Medicine, University of Michigan, Ann Arbor, MI, USA.

Internal Medicine Residency Program, McLaren Flint/Michigan State University, Flint, MI, USA.

出版信息

Am J Case Rep. 2019 Mar 5;20:290-293. doi: 10.12659/AJCR.913731.

DOI:10.12659/AJCR.913731
PMID:30833539
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6410609/
Abstract

BACKGROUND Celiac crisis is an uncommon but critical complication of celiac disease (CD) manifesting with copious diarrhea, dehydration, and severe metabolic imbalances. Celiac crisis occurring in individuals who have been formerly diagnosed with CD and displaying severe coagulopathy is tremendously rare. CASE REPORT We report a case of a 76-year-old male, previously diagnosed with CD and non-compliant with gluten free diet, who presented with severe coagulopathy manifesting as gastrointestinal bleeding in addition to other features of celiac crisis, including severe diarrhea, dehydration, metabolic acidosis, electrolyte disturbances, and renal dysfunction. Esophagogastroduodenoscopy revealed flattened mucosa and mucosal nodularity in the duodenum. Duodenal biopsies exhibited active chronic inflammation with intraepithelial lymphocytosis and subtotal villous blunting. The patient was diagnosed with celiac crisis and treatment with vitamin K, parenteral nutrition, and steroids was commenced. After initial clinical improvement, a gluten-free diet was implemented with complete resolution of symptoms. CONCLUSIONS Though celiac crisis typically presents in patients with undiagnosed CD, it should be considered in patients who have been previously diagnosed CD but who are non-compliant with gluten free diet. Severe coagulopathy, though extremely rare, can be a feature of celiac crisis and should be consider when encountered in a patient with history of steatorrhea and gastrointestinal bleeding.

摘要

背景

乳糜泻危象是乳糜泻(CD)一种罕见但严重的并发症,表现为大量腹泻、脱水及严重代谢失衡。在先前已诊断为CD且出现严重凝血病的个体中发生乳糜泻危象极为罕见。病例报告:我们报告一例76岁男性,既往诊断为CD且未遵循无麸质饮食,除了乳糜泻危象的其他特征(包括严重腹泻、脱水、代谢性酸中毒、电解质紊乱及肾功能不全)外,还出现表现为胃肠道出血的严重凝血病。食管胃十二指肠镜检查显示十二指肠黏膜扁平及黏膜结节。十二指肠活检显示有活动性慢性炎症伴上皮内淋巴细胞增多及绒毛部分变钝。该患者被诊断为乳糜泻危象,并开始用维生素K、肠外营养及类固醇进行治疗。在最初临床改善后,实施无麸质饮食,症状完全缓解。结论:虽然乳糜泻危象通常出现在未诊断出CD的患者中,但对于先前已诊断为CD但未遵循无麸质饮食的患者也应予以考虑。严重凝血病虽然极为罕见,但可能是乳糜泻危象的一个特征,在有脂肪泻和胃肠道出血病史的患者中遇到时应予以考虑。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/547c/6410609/6b32dac4fbc5/amjcaserep-20-290-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/547c/6410609/f631cda385dc/amjcaserep-20-290-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/547c/6410609/6b32dac4fbc5/amjcaserep-20-290-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/547c/6410609/f631cda385dc/amjcaserep-20-290-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/547c/6410609/6b32dac4fbc5/amjcaserep-20-290-g002.jpg

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本文引用的文献

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Celiac Disease Presenting as Profound Diarrhea and Weight Loss - A Celiac Crisis.以严重腹泻和体重减轻为表现的乳糜泻——乳糜泻危象
Am J Case Rep. 2016 Aug 5;17:559-61. doi: 10.12659/ajcr.898004.
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Clin Gastroenterol Hepatol. 2010 Jul;8(7):587-90. doi: 10.1016/j.cgh.2010.04.009. Epub 2010 Apr 24.
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Celiac disease.乳糜泻
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