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首页> 外文期刊>Case Reports in Pediatrics >Hemorrhagic Shock after Neonatal Circumcision: Severe Congenital Factor XIII Deficiency
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Hemorrhagic Shock after Neonatal Circumcision: Severe Congenital Factor XIII Deficiency

机译:新生儿割礼后出血休克:严重先天性因素XIII缺乏

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A Caucasian male infant born full term via normal spontaneous vaginal delivery was given vitamin K after birth, circumcised on day of life (DOL) 1, and discharged from the nursery on DOL 2. At the time of circumcision, oozing from the surgical site was noted and initially resolved with silver nitrate. Over the next two days, he presented to local emergency rooms multiple times for recurrent bleeding, eventually developing hemorrhagic shock resulting in admission to the neonatal intensive care unit. After extensive work up, he was ultimately diagnosed with severe congenital factor XIII deficiency. Congenital factor XIII deficiency is a rare bleeding disorder characterized by normal prothrombin time (PT) and activated partial thromboplastin time (aPTT) coagulation labs on routine screening, and has a high risk of complications, such as spontaneous intracranial hemorrhage. Although uncommon, when caring for a child with bleeding, physicians must have a high index of suspicion to make this diagnosis in order to initiate proper treatment and start prophylaxis given the risk of morbidity and mortality in untreated patients.
机译:一名白种人男性婴儿通过正常自发性阴道递送出生的全部期限在出生后获得维生素K,在生命日(DOL)1周日割礼,并从DOL的托儿所排出。在包皮环切术时,从外科部位渗出注意到并最初用硝酸盐解决。在接下来的两天里,他向当地急诊室发出多次进行复发出血,最终发展出血震动导致新生儿重症监护病房。经过广泛的努力,他最终被诊断出患有严重的先天性因素XIII缺乏症。先天性因素XIII缺乏是一种罕见的出血障碍,其特征在于正常的凝血酶原时间(Pt)和活化的部分血栓形成时间(APTT)凝血实验室在常规筛查中,并且具有很高的并发症风险,例如自发的颅内出血。虽然罕见,当关心患有出血的孩子时,医生必须具有高度怀疑的指标,以使这一诊断能够启动适当的治疗和开始预防,鉴于未处理患者的发病率和死亡率的风险。

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