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红细胞生成性原卟啉症患儿行体外循环心肺转流术。

Cardiopulmonary bypass in a child with erythropoietic protoporphyria.

机构信息

The Royal Children's Hospital, Melbourne, Australia.

Children's National Hospital, Washington, DC, USA.

出版信息

Australas J Dermatol. 2022 Feb;63(1):e60-e62. doi: 10.1111/ajd.13751. Epub 2021 Nov 24.

Abstract

Children with erythropoietic porphyria are generally under the care of paediatric dermatologists. When these children undergo major surgery, they are at risk of unusual complications due to their photosensitivity. Dermatologists may be consulted prior to surgery for advice. We describe a case of a child with erythropoietic porphyria undergoing open heart surgery, utilising an exchange transfusion alongside other strategies to minimise the risk of photosensitivity-induced haemolysis.

摘要

患有红细胞生成性血卟啉症的儿童通常由儿科皮肤科医生负责治疗。当这些儿童接受重大手术时,由于其光敏性,他们可能会面临异常并发症的风险。在手术前,皮肤科医生可能会被咨询以获取建议。我们描述了一例红细胞生成性血卟啉症患儿接受心脏直视手术的病例,采用了换血疗法和其他策略来最大程度地降低光敏性溶血的风险。

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