Thrombosis Unit, National Hemophilia Center, Sheba Medical Center, Tel Hashomer, Israel.
Haemophilia. 2010 Jul;16 Suppl 5:168-75. doi: 10.1111/j.1365-2516.2010.02316.x.
Bleeding disorders may present during the neonatal period, however, absent patient history along with unique physical signs, physiologically decreased levels of plasma proteins and laboratory variations of platelet function tests may render any diagnosis difficult to establish. Intra cranial haemorrhage (ICH) may be the clinical presenting symptom of a severe coagulation factor deficiency. Haemophilia in the newborn period poses unique challenges in diagnosis and management, Data presented from the UDC and similar surveillance systems world-wide can be used to further clinical research and improve management strategies. Development haemostasis should be considered as well as laboratory variations of coagulation tests while evaluating and diagnosis neonates suspected of bleeding disorders. Therapy of bleeding episodes in the neonate relies upon proper replacement and repeated haemostatic evaluation of patients' status, while dealing with underlying etiological causes. This manuscript discusses the unique aspects of clinical presentation, laboratory assessment, and treatment of various bleeding disorders in neonates.
出血性疾病可能在新生儿期出现,然而,缺乏患者病史以及独特的体征、生理性血浆蛋白水平降低和血小板功能检测的实验室差异可能使任何诊断都难以确定。颅内出血(ICH)可能是严重凝血因子缺乏的临床表现。新生儿期的血友病在诊断和管理方面带来了独特的挑战,来自 UDC 和全球类似监测系统的数据可用于进一步的临床研究和改进管理策略。在评估和诊断疑似出血性疾病的新生儿时,应考虑发育止血以及凝血检测的实验室差异。新生儿出血发作的治疗依赖于适当的替代和对患者病情的反复止血评估,同时处理潜在的病因。本文讨论了新生儿各种出血性疾病的临床表现、实验室评估和治疗的独特方面。