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

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...

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Autores principales: Rathaur, Vyas K., Vigneshwar, N. K. V., Imran, Ayesha, Pathania, Monika, Agrawal, Sonam, Chacham, Swathi, Verma, Prashant K., Bhat, Nowneet K.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8140229/
https://www.ncbi.nlm.nih.gov/pubmed/34041191
http://dx.doi.org/10.4103/jfmpc.jfmpc_1613_20
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author Rathaur, Vyas K.
Vigneshwar, N. K. V.
Imran, Ayesha
Pathania, Monika
Agrawal, Sonam
Chacham, Swathi
Verma, Prashant K.
Bhat, Nowneet K.
author_facet Rathaur, Vyas K.
Vigneshwar, N. K. V.
Imran, Ayesha
Pathania, Monika
Agrawal, Sonam
Chacham, Swathi
Verma, Prashant K.
Bhat, Nowneet K.
author_sort Rathaur, Vyas K.
collection PubMed
description 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.
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spelling pubmed-81402292021-05-25 Rare but not Abdicated: Status of Haemophilia in foothills of Himalaya, Uttarakhand: A cross-sectional study Rathaur, Vyas K. Vigneshwar, N. K. V. Imran, Ayesha Pathania, Monika Agrawal, Sonam Chacham, Swathi Verma, Prashant K. Bhat, Nowneet K. J Family Med Prim Care Original Article 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. Wolters Kluwer - Medknow 2021-03 2021-04-08 /pmc/articles/PMC8140229/ /pubmed/34041191 http://dx.doi.org/10.4103/jfmpc.jfmpc_1613_20 Text en Copyright: © 2021 Journal of Family Medicine and Primary Care https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Original Article
Rathaur, Vyas K.
Vigneshwar, N. K. V.
Imran, Ayesha
Pathania, Monika
Agrawal, Sonam
Chacham, Swathi
Verma, Prashant K.
Bhat, Nowneet K.
Rare but not Abdicated: Status of Haemophilia in foothills of Himalaya, Uttarakhand: A cross-sectional study
title Rare but not Abdicated: Status of Haemophilia in foothills of Himalaya, Uttarakhand: A cross-sectional study
title_full Rare but not Abdicated: Status of Haemophilia in foothills of Himalaya, Uttarakhand: A cross-sectional study
title_fullStr Rare but not Abdicated: Status of Haemophilia in foothills of Himalaya, Uttarakhand: A cross-sectional study
title_full_unstemmed Rare but not Abdicated: Status of Haemophilia in foothills of Himalaya, Uttarakhand: A cross-sectional study
title_short Rare but not Abdicated: Status of Haemophilia in foothills of Himalaya, Uttarakhand: A cross-sectional study
title_sort rare but not abdicated: status of haemophilia in foothills of himalaya, uttarakhand: a cross-sectional study
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8140229/
https://www.ncbi.nlm.nih.gov/pubmed/34041191
http://dx.doi.org/10.4103/jfmpc.jfmpc_1613_20
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