Ay Tuncel Defne, Şaşmaz Hatice İlgen, Antmen Bülent
Department of Pediatric Hematology/Oncology and Bone Marrow Transplantation Unit, University of Health Sciences, Adana City Education and Research Hospital, 01100 Adana, Turkey.
Department of Pediatric Hematology, Faculty of Medicine, Balcali Hospital, Cukurova University, 01110 Adana, Turkey.
J Clin Med. 2025 Jan 22;14(3):689. doi: 10.3390/jcm14030689.
Childhood hemophilia, a hereditary bleeding disorder predominantly affecting males, arises due to gene mutations encoding clotting factors VIII or IX. Intracranial hemorrhage represents a significant and life-threatening complication in pediatric patients with hemophilia. The incidence of intracranial hemorrhage in children with hemophilia, although relatively low, is notably higher compared to the general pediatric population. : In this study, the objective is to examine patients with hemophilia who have experienced intracranial hemorrhage retrospectively. This study is a multicenter, retrospective analysis using data from three tertiary care centers in a provincial city in Turkey. Data were obtained from the participants' hospital records. The presence of inhibitors against FVIII in the participants and the prophylaxis used against them were included in the analysis. Trauma history was queried, with types of traumas examined, including traffic accidents, falls, and a traumatic vaginal delivery. The duration and causes of complaints among the participants were investigated. The causes of complaints were categorized as fever, hematoma, convulsions, loss of consciousness, and hemiparesis. The participants' Physical Examination Findings were classified as fever, hematoma, and loss of consciousness. The duration of hospital stays was evaluated. The hemorrhage location was classified into five groups: parenchymal, subdural, scalp, subarachnoid, and multiple hemorrhagic foci. The recurrence of bleeding, the need for transfusion, surgical intervention, and mortality were also examined. : A significant difference was identified between the participants' survival rates and age variables, as well as transfusion in <36 months. A total of 9 participants had spontaneous intracranial bleeding, 2 experienced cranial trauma as a result of traffic accidents, and 25 participants were exposed to head trauma due to falls. Of the remaining individuals, one suffered head trauma from a severe impact, and one had cranial trauma following a traumatic vaginal delivery. Fourteen participants required transfusion, and three underwent surgical intervention. : According to the results of the statistical analyses, the variables Factor Level, Physical Examination Findings, Transfusion, Recurrent Bleeding, Inhibitor, and Prophylaxis were found to affect survival significantly. No significant relationship was determined between the other analyzed variables and survival. During our study, five of the participants examined died. Accordingly, the mortality rate identified in our study is 13.1%.
儿童血友病是一种主要影响男性的遗传性出血性疾病，由编码凝血因子VIII或IX的基因突变引起。颅内出血是血友病患儿严重的、危及生命的并发症。血友病患儿颅内出血的发生率虽然相对较低，但明显高于普通儿科人群。：本研究旨在回顾性研究发生颅内出血的血友病患者。本研究是一项多中心回顾性分析，使用了土耳其一个省会城市三家三级医疗中心的数据。数据从参与者的医院记录中获取。分析包括参与者中针对FVIII的抑制剂的存在情况以及针对它们使用的预防措施。询问了创伤史，检查了创伤类型，包括交通事故、跌倒和创伤性阴道分娩。调查了参与者的主诉持续时间和原因。主诉原因分为发热、血肿、惊厥、意识丧失和偏瘫。参与者的体格检查结果分为发热、血肿和意识丧失。评估了住院时间。出血部位分为五组：实质内、硬膜下、头皮、蛛网膜下和多个出血灶。还检查了出血复发情况、输血需求、手术干预和死亡率。：在参与者的生存率与年龄变量以及36个月以下儿童的输血情况之间发现了显著差异。共有9名参与者发生自发性颅内出血，2名因交通事故导致颅脑创伤，25名参与者因跌倒而头部受伤。其余个体中，1人因严重撞击导致头部创伤，1人在创伤性阴道分娩后发生颅脑创伤。14名参与者需要输血，3人接受了手术干预。：根据统计分析结果，发现因子水平、体格检查结果、输血、出血复发、抑制剂和预防措施等变量对生存有显著影响。在其他分析变量与生存之间未确定显著关系。在我们的研究过程中，接受检查的参与者中有5人死亡。因此，我们研究中确定的死亡率为13.1%。
