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一例特发性嗜酸性粒细胞增多综合征致二尖瓣乳头肌破裂病例

A Case of Idiopathic Hypereosinophilic Syndrome Causing Mitral Valve Papillary Muscle Rupture.

作者信息

Tamse Tiffany, Rampersad Avind, Jordan-Villegas Alejandro, Ireland Jill

机构信息

Florida Hospital for Children, Orlando, FL 32803, USA ; University of Central Florida, Orlando, FL 32827, USA.

Florida Hospital for Children, Orlando, FL 32803, USA ; Orlando Health, Orlando, FL 32806, USA.

出版信息

Case Rep Pediatr. 2015;2015:538762. doi: 10.1155/2015/538762. Epub 2015 Nov 12.

DOI:10.1155/2015/538762
PMID:26640733
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4660015/
Abstract

Idiopathic Hypereosinophilic Syndrome (IHES) is a rare disease that can be difficult to diagnose as the differential is broad. This disease can cause significant morbidity and mortality if left untreated. Our patient is a 17-year-old adolescent female who presented with nonspecific symptoms of abdominal pain and malaise. She was incidentally found to have hypereosinophilia of 16,000 on complete blood count and nonspecific colitis and pulmonary edema on computed tomography. She went into cardiogenic shock due to papillary rupture of her mitral valve requiring extreme life support measures including intubation and extracorporal membrane oxygenation (ECMO) as well as mitral valve replacement. Pathology of the valve showed eosinophilic infiltration as the underlying etiology. The patient was diagnosed with IHES after the exclusion of infectious, rheumatologic, and oncologic causes. She was treated with steroids with improvement of her symptoms and scheduled for close follow-up. In general patients with IHES that have cardiac involvement have poorer prognoses.

摘要

特发性嗜酸性粒细胞增多综合征(IHES)是一种罕见疾病,由于鉴别诊断范围广泛,可能难以诊断。如果不进行治疗,这种疾病会导致严重的发病率和死亡率。我们的患者是一名17岁的青少年女性,出现腹痛和不适等非特异性症状。她在全血细胞计数时偶然发现嗜酸性粒细胞增多至16,000,计算机断层扫描显示非特异性结肠炎和肺水肿。她因二尖瓣乳头肌破裂导致心源性休克,需要采取包括插管和体外膜肺氧合(ECMO)以及二尖瓣置换在内的极端生命支持措施。瓣膜病理学检查显示嗜酸性粒细胞浸润是潜在病因。在排除感染、风湿和肿瘤性病因后,该患者被诊断为IHES。她接受了类固醇治疗,症状有所改善,并安排了密切随访。一般来说,患有心脏受累的IHES患者预后较差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0509/4660015/daf99f71cf09/CRIPE2015-538762.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0509/4660015/a6c5695cfa3f/CRIPE2015-538762.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0509/4660015/59f4042a2a74/CRIPE2015-538762.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0509/4660015/daf99f71cf09/CRIPE2015-538762.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0509/4660015/a6c5695cfa3f/CRIPE2015-538762.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0509/4660015/59f4042a2a74/CRIPE2015-538762.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0509/4660015/daf99f71cf09/CRIPE2015-538762.003.jpg

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1
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Unusual cause of acute mitral regurgitation: idiopathic hypereosinophilic syndrome.急性二尖瓣反流的不常见病因:特发性嗜酸性粒细胞增多综合征。
Ann Thorac Surg. 2012 Mar;93(3):974-7. doi: 10.1016/j.athoracsur.2011.08.062.
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The idiopathic hypereosinophilic syndrome.特发性嗜酸性粒细胞增多综合征
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9
Eosinophilic colitis as a complication of the hypereosinophilic syndrome.嗜酸性粒细胞性结肠炎作为高嗜酸性粒细胞综合征的一种并发症。
Postgrad Med J. 1987 Jun;63(740):485-7. doi: 10.1136/pgmj.63.740.485.
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Systemic vasculitis associated with eosinophilia and marked degranulation of tissue eosinophils.与嗜酸性粒细胞增多及组织嗜酸性粒细胞显著脱颗粒相关的系统性血管炎。
Pediatrics. 1988 Jul;82(1):69-75.