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乳糜泻危象:麸质敏感性肠病的一种不寻常表现。

Celiac Crisis: an unusual presentation of gluten-sensitive enteropathy.

作者信息

do Vale Rômulo Ribeiro, Conci Nathalia da Silva, Santana Alexandre Pinheiro, Pereira Mauricio Baptista, Menezes Natália Yume Hissayasu, Takayasu Vilma, Laborda Lorena Silva, da Silva Aloísio Souza Felipe

机构信息

Universidade de São Paulo (USP), Hospital das Clínicas, Department of Internal Medicine. São Paulo, SP, Brazil.

Instituto de Infectologia Emilio Ribas. São Paulo, SP, Brazil.

出版信息

Autops Case Rep. 2018 Jul 30;8(3):e2018027. doi: 10.4322/acr.2018.027. eCollection 2018 Jul-Sep.

Abstract

Celiac disease (CD)-also known as gluten-sensitive enteropathy-is a chronic, genetically predisposing and autoimmune entity with a wide range of clinical manifestations triggered by gluten ingestion, which affects 1% of the general population. Currently, up to 60% of the diagnosis of CD is in adults due to the atypical course of the disease. The severe acute onset of CD-also called celiac crisis-is very uncommon and is still not well documented in adults. We report the case of a 58-year-old man who presented a 45-day history of subtle-onset diarrhea followed by malabsorption syndrome with progressive weight loss, anasarca, and electrolyte disturbances. The diagnostic work-up included an upper digestive endoscopy, which showed scalloping of the duodenal mucosa with pathological features confirmed on biopsies. Specific antibodies were positive, and a satisfactory clinical response was obtained once a gluten-free diet was started. Celiac crisis is a rare initial presentation of CD characterized by severe diarrhea, dehydration, weight loss, hypoproteinemia, and metabolic and electrolyte disturbances. Although rare, it should be considered in patients with apparently unexplained chronic diarrhea.

摘要

乳糜泻(CD)——也称为麸质敏感性肠病——是一种慢性、具有遗传易感性的自身免疫性疾病,由摄入麸质引发广泛的临床表现,影响着1%的普通人群。目前,由于该病病程不典型,高达60%的乳糜泻诊断是在成人中做出的。乳糜泻的严重急性发作——也称为乳糜泻危象——非常罕见,在成人中仍缺乏充分的文献记载。我们报告一例58岁男性病例,其有45天的隐匿性腹泻病史,随后出现吸收不良综合征,伴有体重逐渐减轻、全身性水肿和电解质紊乱。诊断性检查包括上消化道内镜检查,显示十二指肠黏膜呈扇贝样改变,活检证实有病理特征。特异性抗体呈阳性,开始无麸质饮食后获得了满意的临床反应。乳糜泻危象是乳糜泻一种罕见的初始表现,其特征为严重腹泻、脱水、体重减轻、低蛋白血症以及代谢和电解质紊乱。尽管罕见,但对于明显原因不明的慢性腹泻患者应考虑此病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/372e/6066267/32f7a3161d7d/autopsy-08-03e2018027-g01.jpg

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