RESULTS AND DISCUSSION: Ninety-six haemophilia patients were identified - 79(82.3%) haemophilia A(HA) and 17(17.7%) haemophilia B(HB). Severe haemophilia patients were noted in 45.6% (36/79) of HA and 64.7% (11/17) of HB. In all 44.3% of the HA and 52.9% of the HB population had no identifiable family history of haemophilia. Two-thirds of the patients with severe HA were on prophylaxis [24/36 (66.7%)] and only onethird [4/11 (36.4%)] in severe HB. Inhibitors developed in 9/79 (11.4%) of the HA population [3/79 (3.8%) high responders]. The median inhibitor titre was not significantly different between the different treatment groups - on demand versus prophylaxis (1.0BU versus 2.0BU; z statistic -1.043, p-value 0.297, Mann-Whitney test). None of the patients developed inhibitory alloantibodies to factor IX. Four HA patients (5.1%) underwent immune tolerance induction where one case had a successful outcome. Three severe HA patients received emicizumab prophylaxis and showed remarkable reduction in bleeding events with no thromboembolic events being reported. One female moderate HA patient received PEGylated recombinant anti-haemophilic factor. Eleven patients underwent radiosynovectomy. One mild HB patient succumbed to traumatic intracranial bleeding. Our data reported a prevalence (per 100,000 males) of 5.40 cases for all severities of HA, 2.46 cases for severe HA; 1.16 cases for all severities of HB, and 0.75 cases for severe HB. The overall incidence of HA and HB was 1 in 11,500 and 1 in 46,000, respectively.
CONCLUSION: This study outlines the Sarawakian haemophilia landscape and offers objective standards for forward planning. Shared responsibilities among all parties are of utmost importance to improve the care of our haemophilia population.
METHODS: A 5-year retrospective chart review was carried out at 2 university hospitals. All patients with acute blunt traumatic spinal or spinal cord injuries transported directly from the injury site to the hospital were entered. None of the 120 patients seen at the University of Malaya had spinal immobilization during transport, whereas all 334 patients seen at the University of New Mexico did. The 2 hospitals were comparable in physician training and clinical resources. Neurologic injuries were assigned to 2 categories, disabling or not disabling, by 2 physicians acting independently and blinded to the hospital of origin. Data were analyzed using multivariate logistic regression, with hospital location, patient age, gender, anatomic level of injury, and injury mechanism serving as explanatory variables.
RESULTS: There was less neurologic disability in the unimmobilized Malaysian patients (OR 2.03; 95% CI 1.03-3.99; p = 0.04). This corresponds to a <2% chance that immobilization has any beneficial effect. Results were similar when the analysis was limited to patients with cervical injuries (OR 1.52; 95% CI 0.64-3.62; p = 0.34).
CONCLUSION: Out-of-hospital immobilization has little or no effect on neurologic outcome in patients with blunt spinal injuries.