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重组凝血因子VIIa成功治疗一名患有抑制物的血友病儿童的自发性血胸。

Successful treatment of a spontaneous haemothorax with recombinant factor VIIa in a haemophilic child with inhibitors.

作者信息

Gidaris D, Economou M, Valeri R, Gombakis N, Athanasiou-Metaxa M

机构信息

1 Paediatric Clinic, Hippokratio General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Creece.

出版信息

Hippokratia. 2010 Oct;14(4):289-90.

Abstract

Haemophilia A is an X-linked disorder caused by a deficiency of factor VIII. Haemorrhage in various sites may occur spontaneously or secondary to trauma depending on the severity of the deficiency. Common manifestations include haemarthrosis, epistaxis, gastrointestinal haemorrhage and haematuria. Spontaneous haemothorax has rarely been reported both in children and adults1,2. We report the case of a haemophiliac child presenting with spontaneous haemothorax due to the rarity of this clinical presentation in order to raise the awareness among clinicians.

摘要

甲型血友病是一种由凝血因子 VIII 缺乏引起的 X 连锁疾病。根据缺乏的严重程度,不同部位的出血可能自发发生或继发于创伤。常见表现包括关节积血、鼻出血、胃肠道出血和血尿。儿童和成人自发性血胸均鲜有报道。我们报告了一例血友病患儿出现自发性血胸的病例,由于这种临床表现罕见,旨在提高临床医生的认识。

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引用本文的文献

本文引用的文献

1
Haemothorax in children with congenital coagulopathy.
Haemophilia. 2010 Jul 1;16(4):688-91. doi: 10.1111/j.1365-2516.2010.02209.x. Epub 2010 Apr 7.
6
Central venous access devices in haemophilia.血友病中的中心静脉通路装置
Haemophilia. 2004 Mar;10(2):134-46. doi: 10.1046/j.1365-2516.2003.00840.x.
7
Spontaneous hemopneumothorax in a hemophiliac.血友病患者的自发性血气胸
J Am Med Assoc. 1959 Apr 4;169(14):1623-5. doi: 10.1001/jama.1959.73000310006015b.
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[Conservative treatment of hemophiliac bleeding].
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