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一名儿童患伴有血小板减少和凝血病的乳糜泻危象。

Celiac crisis with thrombocytopenia and coagulopathy in a child.

作者信息

Mazman Duygu İskender, Dereci Selim, Hızlı Şamil, Doğan Hayriye Tatlı, Ateş Burcu Berberoğlu, Hızal Gülin, Demir Arzu Meltem, Aksu Aysel Ünlüsoy

机构信息

Department of Pediatric Gastroenterology, University of Health Sciences, Ankara City Hospital, Ankara.

Departments of Pediatric Gastroenterology, Ankara Yıldırım Beyazıt University Faculty of Medicine, Ankara, Türkiye.

出版信息

Turk J Pediatr. 2022;64(6):1156-1160. doi: 10.24953/turkjped.2022.216.

Abstract

BACKGROUND

Celiac disease rarely presents with edema, hypoalbuminemia, acute metabolic deterioration, and electrolyte imbalances. This life-threatening condition is defined as a celiac crisis and may mimic disorders with metabolic derangement and sepsis. The crisis may present at onset or develop in celiac disease patients with poor compliance to a gluten-free diet. The fluid resuscitation and replacement of electrolyte deficits are life-saving modalities.

CASE

A 14-month-old girl was admitted with fever, lethargy, severe dehydration, edema, hypotension, and commenced sepsis therapy. However, the patient had a growth delay and loss of weight with diarrhea and delayed motor skills. On admission, laboratory evaluation showed anemia, coagulopathy, hypoalbuminemia, electrolyte disturbances, and metabolic acidosis and developed thrombocytopenia during follow-up. The celiac serological tests and upper gastrointestinal endoscopic duodenal mucosa appearance, and duodenum histopathology findings suggested celiac disease.

CONCLUSIONS

This case highlights that a celiac patient may present with a severe illness like sepsis and may be associated with cytopenia and coagulopathy in the celiac crisis.

摘要

背景

乳糜泻很少表现为水肿、低蛋白血症、急性代谢恶化和电解质失衡。这种危及生命的情况被定义为乳糜泻危象,可能类似于伴有代谢紊乱和脓毒症的疾病。危象可能在起病时出现,或在对无麸质饮食依从性差的乳糜泻患者中发生。液体复苏和纠正电解质缺乏是挽救生命的措施。

病例

一名14个月大的女孩因发热、嗜睡、严重脱水、水肿、低血压入院,并开始接受脓毒症治疗。然而,该患者有生长发育迟缓、体重减轻、腹泻以及运动技能发育迟缓。入院时,实验室检查显示贫血、凝血功能障碍、低蛋白血症、电解质紊乱和代谢性酸中毒,随访期间出现血小板减少症。乳糜泻血清学检查、上消化道内镜十二指肠黏膜外观以及十二指肠组织病理学检查结果提示为乳糜泻。

结论

本病例强调乳糜泻患者可能表现为类似脓毒症的严重疾病,且在乳糜泻危象中可能伴有血细胞减少和凝血功能障碍。

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