Traivaree C, Blanchette V, Armstrong D, Floros G, Stain A M, Carcao M D
Division of Haematology/Oncology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Haemophilia. 2007 Sep;13(5):552-9. doi: 10.1111/j.1365-2516.2007.01545.x.
We conducted a review of a single institutional experience of patients with haemophilia presenting with suspected intracranial haemorrhage (ICH) who underwent computed tomographic (CT) neuro-imaging. We found that over a 9-year period (1996-2004) 43 patients with haemophilia presented 73 times with suspected ICH: 10 presented multiple times (range: 2-9 times). The median age at presentation was 3.5 years (range: 0.5-17). Preceding trauma occurred in most (62/73; 85%) episodes. ICH was confirmed in 11 of the 73 (16%) episodes in eight patients. Patients with severe haemophilia accounted for a disproportionate number of episodes of suspected (60/73; 82%) and of confirmed ICH (10/11; 91%). All ICH occurred in patients not on prophylaxis; five occurred in three inhibitor-positive patients. Altered consciousness at presentation was present in 10/11 (91%) cases of confirmed ICH but only in 5/62 (8%) (ICH-negative) episodes. The positive and negative predictive values of altered consciousness to predict/rule out an ICH was 67% and 98%, respectively. The following were associated with an increased risk of presenting with suspected ICH and of having a confirmed ICH: (i) having severe haemophilia; (ii) not being on prophylaxis; (iii) having an inhibitor; and (iv) presenting with an altered level of consciousness. Patients without any of these features may not need to undergo CT imaging when presenting with suspected ICH. Ideally a prospective study to evaluate this hypothesis should be conducted.
我们回顾了一家机构中血友病患者疑似颅内出血(ICH)并接受计算机断层扫描(CT)神经影像学检查的病例。我们发现,在9年期间(1996 - 2004年),43例血友病患者出现73次疑似ICH:10例为多次出现(范围:2 - 9次)。发病时的中位年龄为3.5岁(范围:0.5 - 17岁)。大多数发作(62/73;85%)之前有过创伤。73次发作中有11次(16%)在8例患者中确诊为ICH。重度血友病患者的疑似发作(60/73;82%)和确诊ICH发作(10/11;91%)比例过高。所有ICH均发生在未接受预防治疗的患者中;5次发生在3例有抑制物阳性的患者中。确诊ICH的11例中有10例(91%)发病时意识改变,但在62次(ICH阴性)发作中仅有5例(8%)出现意识改变。意识改变预测/排除ICH的阳性和阴性预测值分别为67%和98%。以下因素与疑似ICH发作及确诊ICH风险增加相关:(i)患有重度血友病;(ii)未接受预防治疗;(iii)有抑制物;(iv)发病时意识水平改变。无这些特征的患者在疑似ICH发作时可能无需进行CT成像。理想情况下,应进行一项前瞻性研究以评估这一假设。