Moreira Alvaro, Das Hrishikesh
University of Texas Health Science Center at San Antonio, TX, USA.
J Investig Med High Impact Case Rep. 2018 Sep 18;6:2324709618800349. doi: 10.1177/2324709618800349. eCollection 2018 Jan-Dec.
Hemorrhagic shock is a rare, emergent condition that is often fatal in newborns. In this article, we report cases of 3 neonates presenting with acute, life-threatening hemorrhage who were subsequently diagnosed with severe hemophilia (<1% factor VIII). The first infant was tachycardic, pale, and had a precipitous drop in his hemoglobin secondary to a subgaleal hemorrhage. The second patient sustained a splenic rupture, a sequela that has been reported in only 4 other neonatal cases. The last infant presented with tonic-clonic seizures and respiratory distress. Head imaging demonstrated extracranial and intracranial hemorrhage, complications that can result in 20% mortality. All 3 patients were successfully treated with clotting factor concentrate and blood products. After normalization of factor VIII levels, the newborns did not develop any new hemorrhages and were discharged home within 3 weeks of birth. Pediatric providers should be aware that these signs and symptoms may be potentially lethal complications in neonates with severe factor VIII deficiency.
失血性休克是一种罕见的紧急情况，在新生儿中往往是致命的。在本文中，我们报告了3例新生儿病例，这些新生儿出现急性、危及生命的出血，随后被诊断为严重血友病（因子VIII<1%）。第一名婴儿心动过速、面色苍白，因帽状腱膜下出血导致血红蛋白急剧下降。第二名患者发生脾破裂，这一后遗症仅在其他4例新生儿病例中报道过。最后一名婴儿出现强直阵挛性癫痫发作和呼吸窘迫。头部影像学检查显示颅外和颅内出血，这些并发症可导致20%的死亡率。所有3例患者均成功接受了凝血因子浓缩物和血液制品治疗。因子VIII水平恢复正常后，这些新生儿未出现任何新的出血情况，并在出生后3周内出院回家。儿科医护人员应意识到，这些体征和症状在严重因子VIII缺乏的新生儿中可能是潜在致命的并发症。
