Ohga S, Kajiwara M, Toubo Y, Takeuchi T, Ohtsuka M, Sano Y, Ishii E, Ueda K
Department of Pediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Am J Pediatr Hematol Oncol. 1988 Fall;10(3):244-8. doi: 10.1097/00043426-198823000-00012.
It is uncommon for infants with hemophilia to have excessive bleeding during the neonatal period. Even if bleeding occurs, it rarely becomes life-threatening, such as in intracranial hemorrhage (ICH). We here report a case of a 4-day-old boy who had intracranial hemorrhage as the first complication of hemophilia B. Computerized axial tomography (CT scan) and ultrasonography were very useful for early diagnosis. Only a few cases of neonatal hemophilia with intracranial hemorrhage have been reported, but the occurrence of this complication is probably more frequent. We reviewed seven cases (including our case) with intracranial hemorrhage as the first manifestation of neonatal hemophilia. Although these infants showed good prognosis as to survival, permanent residual neurological deficits remained in all of them. It is emphasized that intracranial hemorrhage due to hemophilia may occur in neonates even without a family history. Urgent neuroimaging and coagulation studies are necessary for an early and adequate diagnosis.
血友病婴儿在新生儿期出现大量出血的情况并不常见。即使发生出血,也很少会危及生命,比如颅内出血(ICH)。我们在此报告一例4日龄男婴,其颅内出血是乙型血友病的首发并发症。计算机断层扫描(CT扫描)和超声检查对早期诊断非常有用。仅有少数新生儿血友病伴颅内出血的病例被报道过,但这种并发症的发生率可能更高。我们回顾了7例(包括我们的病例)以颅内出血为新生儿血友病首发表现的病例。尽管这些婴儿在生存方面预后良好,但他们都遗留了永久性的神经功能缺损。需要强调的是,即使没有家族病史,新生儿也可能因血友病发生颅内出血。为了早期做出充分诊断,紧急进行神经影像学检查和凝血研究是必要的。