Rathaur Vyas K, Vigneshwar N K V, Imran Ayesha, Pathania Monika, Agrawal Sonam, Chacham Swathi, Verma Prashant K, Bhat Nowneet K
Professor and Head of the Department, Department of Pediatrics, Veer Chandra Singh Garhwali Govt. Institute of Medical Science & Research, N. K. V. Vigneshwar, Dehradun, Uttarakhand, India.
Junior Resident, Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.
J Family Med Prim Care. 2021 Mar;10(3):1437-1442. doi: 10.4103/jfmpc.jfmpc_1613_20. Epub 2021 Apr 8.
Haemophilia is one of the bleeding disorders, which is inherited, in an xlinked recessive pattern. The diagnosis is by estimation of factor levels of 8 and 9. Timebound treatment for people living with Haemophilia (PWH) is factor replacement during bleeding manifestation. The prevalence of Haemophilia was mostly underestimated, and it is more so in hilly terrains like the state of Uttarakhand.
This is a crosssectional study by compiling the data of PWH visiting the tertiary care centre for Haemophilia in Uttarakhand. We collected data from the patients with bleeding disorder reporting to the Haemophilia centre from July 2017 to December 2018. In this manuscript, we try to describe the pattern of Haemophilia and the degree of severity and incidence of inhibitors among the sample population of PWH who represent the population of Uttarakhand. The magnitude of problems faced by PWH from this hilly terrain to assess basic treatment in case of emergency is also being depicted.
We reported Haemophilia A contributing about 80% of the PWH in our centre. Average distance a PWH has to travel to obtain treatment was about 131.5 km (SD ± 83.7 km). Incidence of inhibitors was about 5%.
We infer from our study that Hemophilia A is more common than Hemophilia B. Through this manuscript we hope to spread awareness of the Haemophilia care that is ongoing, the role of prophylaxis therapy and the future role of primary care physicians that may change the care of PWH in future.
血友病是一种遗传性出血性疾病，呈X连锁隐性遗传模式。诊断方法是测定因子Ⅷ和因子Ⅸ水平。血友病患者（PWH）的限期治疗是在出血表现时进行因子替代治疗。血友病的患病率大多被低估，在像北阿坎德邦这样的山区更是如此。
这是一项横断面研究，通过收集前往北阿坎德邦血友病三级护理中心就诊的PWH的数据进行。我们收集了2017年7月至2018年12月向血友病中心报告的出血性疾病患者的数据。在本手稿中，我们试图描述代表北阿坎德邦人群的PWH样本群体中的血友病模式、严重程度以及抑制剂的发生率。还描述了来自这个山区的PWH在紧急情况下评估基本治疗所面临问题的严重程度。
我们报告在我们中心，A型血友病约占PWH的80%。PWH为获得治疗平均要走的距离约为131.5公里（标准差±83.7公里）。抑制剂的发生率约为5%。
我们从研究中推断，A型血友病比B型血友病更常见。通过本手稿，我们希望提高对正在进行的血友病护理、预防治疗的作用以及初级保健医生未来可能改变PWH护理方式的作用的认识。
