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The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia: Results from the Saudi national hemophilia screening program

Hemophilia A and B are X-linked diseases that predominantly affect male patients. Patients can develop coagulation factor inhibitors, which exponentially increases the treatment cost. However, the prevalence of factor VIII and IX inhibitors in Saudi Arabia is unclear. This study aimed to determine t...

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Autores principales: Owaidah, Tarek, Momen, Abdulkareem Al, Alzahrani, Hazzaa, Almusa, Abdulrahman, Alkasim, Fawaz, Tarawah, Ahmed, Nouno, Randa Al, Batniji, Fatima Al, Alothman, Fahad, Alomari, Ali, Abu-Herbish, Saud, Abu-Riash, Mahmoud, Siddiqui, Khawar, Ahmed, Mansor, Mohamed, SY, Saleh, Mahasen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266150/
https://www.ncbi.nlm.nih.gov/pubmed/28079788
http://dx.doi.org/10.1097/MD.0000000000005456
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author Owaidah, Tarek
Momen, Abdulkareem Al
Alzahrani, Hazzaa
Almusa, Abdulrahman
Alkasim, Fawaz
Tarawah, Ahmed
Nouno, Randa Al
Batniji, Fatima Al
Alothman, Fahad
Alomari, Ali
Abu-Herbish, Saud
Abu-Riash, Mahmoud
Siddiqui, Khawar
Ahmed, Mansor
Mohamed, SY
Saleh, Mahasen
author_facet Owaidah, Tarek
Momen, Abdulkareem Al
Alzahrani, Hazzaa
Almusa, Abdulrahman
Alkasim, Fawaz
Tarawah, Ahmed
Nouno, Randa Al
Batniji, Fatima Al
Alothman, Fahad
Alomari, Ali
Abu-Herbish, Saud
Abu-Riash, Mahmoud
Siddiqui, Khawar
Ahmed, Mansor
Mohamed, SY
Saleh, Mahasen
author_sort Owaidah, Tarek
collection PubMed
description Hemophilia A and B are X-linked diseases that predominantly affect male patients. Patients can develop coagulation factor inhibitors, which exponentially increases the treatment cost. However, the prevalence of factor VIII and IX inhibitors in Saudi Arabia is unclear. This study aimed to determine the Saudi prevalence of factor VIII and IX inhibitors. This 4-year, 7-center, cross-sectional study evaluated the Saudi prevalences of hemophilia A and B. We collected the patients’ clinical data, evaluated their disease, and tested for factor inhibitors. We included 202 patients with hemophilia (median age at diagnosis: 0.13 years, range: birth–34.8 years). The patients included 198 male patients (98%), 148 patients with hemophilia A (73.3%), and 54 patients with hemophilia B (26.7%). The patients exhibited severe factor VIII activity (<1%; 121 patients; 5.2%), moderate activity (1–5%; 7 patients; 4.9%), and mild activity (14 patients; 9.9%). Among the patients with care-related data, most patients were treated for episodic bleeding (76.8%) or received prophylaxis (22.6%); 1 patient received both treatments. Among the patients with source-related data, the factor replacements were derived from plasma (48.4%), recombinant concentrates (22.9%), both sources (14.6%), or fresh frozen plasma (14.1%). Factor VIII inhibitors were observed in 43 (29.3%) of the 147 patients, and only 1 of the 54 patients developed factor IX inhibitors. Most patients who developed inhibitors had severe hemophilia (40/44; 90.9%), and inhibitors were also common among patients who received recombinant products (14/43; 32.6%). The Saudi prevalence of factor inhibitors was similar to those among other ethnic populations.
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spelling pubmed-52661502017-02-07 The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia: Results from the Saudi national hemophilia screening program Owaidah, Tarek Momen, Abdulkareem Al Alzahrani, Hazzaa Almusa, Abdulrahman Alkasim, Fawaz Tarawah, Ahmed Nouno, Randa Al Batniji, Fatima Al Alothman, Fahad Alomari, Ali Abu-Herbish, Saud Abu-Riash, Mahmoud Siddiqui, Khawar Ahmed, Mansor Mohamed, SY Saleh, Mahasen Medicine (Baltimore) 4800 Hemophilia A and B are X-linked diseases that predominantly affect male patients. Patients can develop coagulation factor inhibitors, which exponentially increases the treatment cost. However, the prevalence of factor VIII and IX inhibitors in Saudi Arabia is unclear. This study aimed to determine the Saudi prevalence of factor VIII and IX inhibitors. This 4-year, 7-center, cross-sectional study evaluated the Saudi prevalences of hemophilia A and B. We collected the patients’ clinical data, evaluated their disease, and tested for factor inhibitors. We included 202 patients with hemophilia (median age at diagnosis: 0.13 years, range: birth–34.8 years). The patients included 198 male patients (98%), 148 patients with hemophilia A (73.3%), and 54 patients with hemophilia B (26.7%). The patients exhibited severe factor VIII activity (<1%; 121 patients; 5.2%), moderate activity (1–5%; 7 patients; 4.9%), and mild activity (14 patients; 9.9%). Among the patients with care-related data, most patients were treated for episodic bleeding (76.8%) or received prophylaxis (22.6%); 1 patient received both treatments. Among the patients with source-related data, the factor replacements were derived from plasma (48.4%), recombinant concentrates (22.9%), both sources (14.6%), or fresh frozen plasma (14.1%). Factor VIII inhibitors were observed in 43 (29.3%) of the 147 patients, and only 1 of the 54 patients developed factor IX inhibitors. Most patients who developed inhibitors had severe hemophilia (40/44; 90.9%), and inhibitors were also common among patients who received recombinant products (14/43; 32.6%). The Saudi prevalence of factor inhibitors was similar to those among other ethnic populations. Wolters Kluwer Health 2017-01-13 /pmc/articles/PMC5266150/ /pubmed/28079788 http://dx.doi.org/10.1097/MD.0000000000005456 Text en Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0
spellingShingle 4800
Owaidah, Tarek
Momen, Abdulkareem Al
Alzahrani, Hazzaa
Almusa, Abdulrahman
Alkasim, Fawaz
Tarawah, Ahmed
Nouno, Randa Al
Batniji, Fatima Al
Alothman, Fahad
Alomari, Ali
Abu-Herbish, Saud
Abu-Riash, Mahmoud
Siddiqui, Khawar
Ahmed, Mansor
Mohamed, SY
Saleh, Mahasen
The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia: Results from the Saudi national hemophilia screening program
title The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia: Results from the Saudi national hemophilia screening program
title_full The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia: Results from the Saudi national hemophilia screening program
title_fullStr The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia: Results from the Saudi national hemophilia screening program
title_full_unstemmed The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia: Results from the Saudi national hemophilia screening program
title_short The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia: Results from the Saudi national hemophilia screening program
title_sort prevalence of factor viii and ix inhibitors among saudi patients with hemophilia: results from the saudi national hemophilia screening program
topic 4800
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266150/
https://www.ncbi.nlm.nih.gov/pubmed/28079788
http://dx.doi.org/10.1097/MD.0000000000005456
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