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川崎病患儿的蛋白丢失性肠病:一种罕见的临床表现。

Protein-losing enteropathy in a paediatric patient with Kawasaki disease: a rare clinical manifestation.

作者信息

Yuwattana Rattakarn, Saengsin Kwannapas, Sittiwangkul Rekwan

机构信息

Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand

出版信息

BMJ Case Rep. 2025 Sep 9;18(9):e265781. doi: 10.1136/bcr-2025-265781.

Abstract

Kawasaki disease (KD) is an acute vasculitis of childhood, which can lead to complications affecting multiple organ systems. Protein-losing enteropathy (PLE) is an extremely rare complication of KD, characterised by excessive protein loss through the gastrointestinal tract, leading to hypoalbuminaemia, oedema and immune dysfunction. We report a case of an early childhood boy with intravenous immune globulin (IVIG)-resistant incomplete KD who developed PLE. The patient presented with prolonged fever, conjunctivitis, mucosal changes and cervical lymphadenopathy. He subsequently developed generalised oedema, hypoalbuminaemia and pleural effusion. Diagnosis of PLE was confirmed by elevated stool alpha-1 antitrypsin levels. The patient improved with a second dose of IVIG and supportive therapy. This case highlights the importance of recognising PLE as a potential complication of KD and the importance of early diagnosis and proper treatment to reduce the risk of adverse sequelae.

摘要

川崎病(KD)是一种儿童急性血管炎,可导致影响多个器官系统的并发症。蛋白丢失性肠病(PLE)是KD极其罕见的并发症,其特征是通过胃肠道过度丢失蛋白质,导致低白蛋白血症、水肿和免疫功能障碍。我们报告一例患有静脉注射免疫球蛋白(IVIG)抵抗性不完全KD的幼儿发生PLE的病例。该患者表现为持续发热、结膜炎、黏膜改变和颈部淋巴结病。随后他出现全身水肿、低白蛋白血症和胸腔积液。粪便α-1抗胰蛋白酶水平升高证实了PLE的诊断。该患者在接受第二剂IVIG和支持治疗后病情好转。该病例强调了认识到PLE作为KD潜在并发症的重要性,以及早期诊断和适当治疗以降低不良后遗症风险的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df58/12421297/ac0ca98c6663/bcr-18-9-g001.jpg

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