产前产后静脉注射免疫球蛋白对新生儿血色素沉着症严重程度的影响:两兄弟的故事(病例报告)。

Pub Date : 2021-04-01 Epub Date: 2021-06-30 DOI:10.1055/s-0041-1731311
Veronica Mugarab-Samedi, Michelle D Ryan, Essa Hamdan Al Awad, Adel Elsharkawy
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引用次数: 2

摘要

背景新生儿血色素沉着症(NH)是一种罕见的疾病,是婴儿肝移植的主要原因。由于认识到NH是由胎儿补体介导的肝损伤引起的,静脉注射免疫球蛋白(IVIG)被成功引入治疗。我们提出两个病例NH来自同一家庭,以说明与IVIG产前治疗在减轻和可能预防这种严重的发病率的作用。结论产前治疗和产后早期给予IVIG是控制NH的有效方法,有助于减轻症状的严重程度,预防肝功能衰竭,避免肝移植的需要。
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The Effect of Prenatal and Postnatal Treatment with Intravenous Immunoglobulin on Severity of Neonatal Hemochromatosis: The Tale of Two Brothers (Case Report).

Background  Neonatal hemochromatosis (NH) is a rare condition that was the main reason for liver transplantation in infants. With the realization that NH results from the fetal complement-mediated liver injury, intravenous immunoglobulins (IVIG) were successfully introduced for the treatment. Case Presentation  We present two cases of NH from the same family to illustrate the role of antenatal treatment with IVIG in alleviation and possible prevention of this serious morbidity. Conclusion  A prenatal treatment and early postnatal administration of IVIG are effective ways to manage NH that help to reduce the severity of the symptoms, prevent liver failure, and avoid the need for liver transplantation.

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