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新生儿同种免疫性血小板减少症的产前诊断与管理

Prenatal diagnosis and management of neonatal alloimmune thrombocytopenia.

作者信息

Johnson J A, Ryan G, al-Musa A, Farkas S, Blanchette V S

机构信息

Fetal Diagnosis and Treatment Center, University of Toronto, Ontario.

出版信息

Semin Perinatol. 1997 Feb;21(1):45-52. doi: 10.1016/s0146-0005(97)80019-5.

Abstract

Neonatal alloimmune thrombocytopenia (NAIT) is an uncommon (1 in 2,000 livebirths) but serious disorder characterized by marked thrombocytopenia in the fetus and neonate. Platelet destruction is caused by a maternal antibody directed against a fetal platelet antigen inherited from the father and lacking in the mother's platelets. Intracranial hemorrhage (ICH) is the most devastating complication of NAIT, affecting approximately 20% of all proven cases, up to 50% of which occur antenatally. Because close to 100% of subsequent pregnancies will be equally or more severely affected, antenatal management directed at preventing ICH in utero has assumed great clinical importance. In recent years, considerable progress has been made in this regard, and although clinical uncertainties still exist, the natural history of this disease and its response to various antenatal interventions have become reasonably well understood. This review will focus on the diagnosis and current management of NAIT, including controversies surrounding current treatment modalities and future prospects for treatment and prevention.

摘要

新生儿同种免疫性血小板减少症(NAIT)是一种罕见的疾病(2000例活产中有1例),但病情严重,其特征是胎儿和新生儿出现明显的血小板减少。血小板破坏是由母体抗体引起的,该抗体针对从父亲遗传而来且母亲血小板中缺乏的胎儿血小板抗原。颅内出血(ICH)是NAIT最具破坏性的并发症,约20%的确诊病例会受到影响,其中高达50%发生在产前。由于几乎100%的后续妊娠会受到同等程度或更严重的影响,因此旨在预防子宫内ICH的产前管理具有重大的临床意义。近年来,在这方面取得了相当大的进展,尽管临床不确定性仍然存在,但这种疾病的自然史及其对各种产前干预措施的反应已得到较好的理解。本综述将重点关注NAIT的诊断和当前管理,包括围绕当前治疗方式的争议以及治疗和预防的未来前景。

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