Rare but not Abdicated: Status of Haemophilia in foothills of Himalaya, Uttarakhand: A cross-sectional study.

Autor: Rathaur VK; 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., Vigneshwar NKV; Junior Resident, Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India., Imran A; Assistant Professor, Department of Pediatrics, Government Doon Medical College, Dehradun, Uttarakhand, India., Pathania M; Associate Professor, Department of Internal Medicine, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India., Agrawal S; Assistant Professor, Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India., Chacham S; Additional Professor, Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India., Verma PK; Associate Professor, Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India., Bhat NK; Professor and Head of the Department, Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.
Jazyk: angličtina
Zdroj: Journal of family medicine and primary care [J Family Med Prim Care] 2021 Mar; Vol. 10 (3), pp. 1437-1442. Date of Electronic Publication: 2021 Apr 08.
DOI: 10.4103/jfmpc.jfmpc_1613_20
Abstrakt: Background: 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.
Materials and Method: 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.
Result: 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%.
Conclusion: 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.
Competing Interests: There are no conflicts of interest.
(Copyright: © 2021 Journal of Family Medicine and Primary Care.)
Databáze: MEDLINE
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