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胸腔镜下心包切除术治疗患有穆利布雷侏儒症的小儿缩窄性心包炎

Thoracoscopic pericardiectomy for constrictive pericarditis in a pediatric patient with mulibrey nanism.

作者信息

Christov Georgi, Burch Michael, Andrews Rachel, Hurst Jane, Ashworth Michael, Mustafa Muhammad, Muthialu Nagarajan

机构信息

Department of Cardiology, Cardiothoracic Unit, Great Ormond Street Hospital, London, United Kingdom.

出版信息

World J Pediatr Congenit Heart Surg. 2013 Oct;4(4):442-3. doi: 10.1177/2150135113485761.

Abstract

Mulibrey nanism is a rare autosomal recessive disease with multisystem involvement. Clinical deterioration is most often related to cardiac involvement in the form of restrictive or constrictive disorders, and pericardiectomy may be required. We report a case of Mulibrey nanism in a patient of non-Finnish origin, where a thoracoscopic pericardiectomy helped in good palliation and clinical recovery.

摘要

穆利布雷侏儒症是一种罕见的常染色体隐性疾病,累及多个系统。临床恶化通常与心脏受累有关,表现为限制性或缩窄性疾病,可能需要进行心包切除术。我们报告一例非芬兰裔患者的穆利布雷侏儒症病例,其中胸腔镜心包切除术有助于良好的缓解和临床恢复。

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