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一名接受Fontan手术后的患者发生与嗜酸性肉芽肿性多血管炎相关的灾难性颅内出血。

Catastrophic intracranial hemorrhage associated with eosinophilic granulomatous polyangiitis in a patient after Fontan operation.

作者信息

Ezaki Hiroki, Muneuchi Jun, Imamoto Naoyuki

机构信息

Department of Pediatrics Japan Community Healthcare Organization Kyushu Hospital Kitakyushu Japan.

Department of Neurosurgery Japan Community Healthcare Organization Kyushu Hospital Kitakyushu Japan.

出版信息

Clin Case Rep. 2020 Dec 11;9(1):547-550. doi: 10.1002/ccr3.3579. eCollection 2021 Jan.

DOI:10.1002/ccr3.3579
PMID:33489213
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7813104/
Abstract

A 29-year-old man after the Fontan operation had a catastrophic intracranial hemorrhage associated with eosinophilic granulomatous polyangiitis. Despite combination therapy with cyclophosphamide and methylprednisolone, he was dead at 6 months after the onset. The clinical course was worse owing to underlying coagulopathy and endothelial dysfunction associated with congenital heart disease. The majority of patients with complex congenital heart disease survive into adulthood. Potential endothelial dysfunction and coagulopathy can attribute to unexpected clinical manifestations of other extracardiac disease among them.

摘要

一名29岁男性在接受Fontan手术后发生了与嗜酸性肉芽肿性多血管炎相关的灾难性颅内出血。尽管接受了环磷酰胺和甲泼尼龙联合治疗，但发病后6个月死亡。由于潜在的凝血功能障碍和与先天性心脏病相关的内皮功能障碍，临床病程更糟。大多数复杂先天性心脏病患者存活至成年期。潜在的内皮功能障碍和凝血功能障碍可能是其中其他心脏外疾病出现意外临床表现的原因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ae/7813104/83967940bb9d/CCR3-9-547-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ae/7813104/c82b48be5392/CCR3-9-547-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ae/7813104/903f5f4023a9/CCR3-9-547-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ae/7813104/83967940bb9d/CCR3-9-547-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ae/7813104/c82b48be5392/CCR3-9-547-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ae/7813104/903f5f4023a9/CCR3-9-547-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ae/7813104/83967940bb9d/CCR3-9-547-g003.jpg

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J Thromb Thrombolysis. 2017 Jul;44(1):38-47. doi: 10.1007/s11239-017-1499-8.
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Thromboembolism and anticoagulation after Fontan surgery.
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Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):236-40. doi: 10.4103/0974-2069.189109.
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