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《血友病胎儿与新生儿管理实用指南》

A Practical Guide to the Management of the Fetus and Newborn With Hemophilia.

机构信息

Section of Pediatric Hematology/Oncology, Janeway Children's Health and Rehabilitation Centre, St. John's, Canada.

Discipline of Pediatrics, Faculty of Medicine, Memorial University, St. John's, Canada.

出版信息

Clin Appl Thromb Hemost. 2018 Dec;24(9_suppl):29S-41S. doi: 10.1177/1076029618807583. Epub 2018 Oct 29.

Abstract

Newborns with hemophilia are at risk of intracranial hemorrhage, extracranial hemorrhage, and other bleeding complications. The safe delivery of a healthy newborn with hemophilia is a complex process that can begin even before conception, and continues throughout pregnancy, birth, and the newborn period. This process involves the expectant parents and a wide variety of health-care professionals: genetic counselors, obstetricians, neonatologists, pediatricians, radiologists, adult and pediatric hematologists, and nurses with expertise in hemophilia. Because of this multidisciplinary complexity, the relative rarity of births of newborns with hemophilia, and the lack of high-quality evidence to inform decisions, there is considerable variation in practice in this area. We present a comprehensive multidisciplinary approach, from preconception counseling to discharge planning after birth, and describe available options for management decisions. We highlight a number of areas of important uncertainty and controversy, including the preferred mode of delivery, the appropriate use and timing of neuroimaging tests, and the appropriate use of clotting factor concentrates in the newborn period. While the approach presented here will aid clinicians in planning and providing care, further research is required to optimize the care of newborns with hemophilia.

摘要

新生儿血友病患者有颅内出血、颅外出血和其他出血并发症的风险。安全分娩健康的血友病新生儿是一个复杂的过程,甚至可以在受孕前开始,并贯穿整个妊娠、分娩和新生儿期。这一过程涉及到准父母和各种医疗保健专业人员:遗传咨询师、产科医生、新生儿科医生、儿科医生、放射科医生、成人和儿科血液病医生以及血友病专业护士。由于这种多学科的复杂性、血友病新生儿出生率相对较低以及缺乏高质量的证据来指导决策,在这一领域的实践中存在着相当大的差异。我们提出了一种全面的多学科方法,从孕前咨询到出生后的出院计划,并描述了管理决策的可用选择。我们强调了一些重要的不确定性和争议领域,包括首选的分娩方式、神经影像学检查的适当使用和时机,以及在新生儿期适当使用凝血因子浓缩物。虽然这里提出的方法将有助于临床医生计划和提供护理,但需要进一步的研究来优化血友病新生儿的护理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f20/6714852/fc62f3aabce4/10.1177_1076029618807583-fig1.jpg

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