Department of Pediatrics, Children's Mercy Hospital, Kansas City, Missouri;
Department of Pediatrics, Children's Mercy Hospital, Kansas City, Missouri.
Pediatrics. 2018 May;141(5). doi: 10.1542/peds.2017-3485.
Bleeding disorders and abusive head trauma (AHT) are associated with intracranial hemorrhage (ICH), including subdural hemorrhage (SDH). Because both conditions often present in young children, the need to screen for bleeding disorders would be better informed by data that include trauma history and are specific to young children. The Universal Data Collection database contains information on ICH in subjects with bleeding disorders, including age and trauma history. Study objectives were to (1) characterize the prevalence and calculate the probabilities of any ICH, traumatic ICH, and nontraumatic ICH in children with congenital bleeding disorders; (2) characterize the prevalence of spontaneous SDH on the basis of bleeding disorder; and (3) identify cases of von Willebrand disease (vWD) that mimic AHT.
We reviewed subjects <4 years of age in the Universal Data Collection database. ICH was categorized on the basis of association with trauma. Prevalence and probability of types of ICH were calculated for each bleeding disorder.
Of 3717 subjects, 255 (6.9%) had any ICH and 206 (5.5%) had nontraumatic ICH. The highest prevalence of ICH was in severe hemophilia A (9.1%) and B (10.7%). Of the 1233 subjects <2 years of age in which the specific location of any ICH was known, 13 (1.1%) had spontaneous SDH (12 with severe hemophilia; 1 with type 1 vWD). The findings in the subject with vWD were not congruent with AHT.
In congenital bleeding disorders, nontraumatic ICH occurs most commonly in severe hemophilia. In this study, vWD is not supported as a "mimic" of AHT.
出血性疾病和虐待性头部外伤(AHT)与颅内出血(ICH)有关,包括硬膜下血肿(SDH)。由于这两种疾病通常发生在幼儿中,因此需要根据包括创伤史在内的数据来更好地筛查出血性疾病,这些数据应针对幼儿进行具体分析。通用数据收集数据库包含有关出血性疾病患者 ICH 的信息,包括年龄和创伤史。研究目的是:(1)描述患有先天性出血性疾病的儿童中 ICH 的患病率并计算其发生率,包括外伤性 ICH 和非外伤性 ICH;(2)根据出血性疾病的特征描述自发性 SDH 的患病率;(3)确定模仿 AHT 的血管性血友病(vWD)病例。
我们回顾了通用数据收集数据库中年龄<4 岁的患者。ICH 根据与创伤的关系进行分类。为每种出血性疾病计算了 ICH 类型的患病率和发生率。
在 3717 例患者中,255 例(6.9%)患有任何 ICH,206 例(5.5%)患有非外伤性 ICH。ICH 的患病率最高的是严重血友病 A(9.1%)和 B(10.7%)。在已知任何 ICH 具体位置的 1233 例<2 岁的患者中,有 13 例(1.1%)患有自发性 SDH(12 例为严重血友病;1 例为 1 型 vWD)。vWD 患者的发现与 AHT 不一致。
在先天性出血性疾病中,非外伤性 ICH 最常发生于严重血友病。在本研究中,vWD 不能作为 AHT 的“模拟”疾病。