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The frequency of complications in a cohort of patients diagnosed with hemophilia A and hemophilia B receiving prophylactic treatment in Colombia: A retrospective noninterventional study

INTRODUCTION: Hemophilia A and B are disorders associated with the deficit of coagulation factors VIII and IX. OBJECTIVE: Was to determine the incidence of complications in a cohort of patients diagnosed with moderate and severe hemophilia A or B under treatment in a specialized institution. METHODS...

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Autores principales: Machado Alba, Jorge E., Wilches-Gutierrez, Juan David, Arias-Osorio, Diana Rocio, Reyes, Juan Manuel, Nakandakari, Maria Lourdes, Ospina-Arzuaga, Harrison David, Gaviria-Mendoza, Andres, Castaño-Gamboa, Natalia, Valladales-Restrepo, Luis Fernando, Machado-Duque, Manuel E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10681305/
https://www.ncbi.nlm.nih.gov/pubmed/38011251
http://dx.doi.org/10.1371/journal.pone.0286187
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author Machado Alba, Jorge E.
Wilches-Gutierrez, Juan David
Arias-Osorio, Diana Rocio
Reyes, Juan Manuel
Nakandakari, Maria Lourdes
Ospina-Arzuaga, Harrison David
Gaviria-Mendoza, Andres
Castaño-Gamboa, Natalia
Valladales-Restrepo, Luis Fernando
Machado-Duque, Manuel E.
author_facet Machado Alba, Jorge E.
Wilches-Gutierrez, Juan David
Arias-Osorio, Diana Rocio
Reyes, Juan Manuel
Nakandakari, Maria Lourdes
Ospina-Arzuaga, Harrison David
Gaviria-Mendoza, Andres
Castaño-Gamboa, Natalia
Valladales-Restrepo, Luis Fernando
Machado-Duque, Manuel E.
author_sort Machado Alba, Jorge E.
collection PubMed
description INTRODUCTION: Hemophilia A and B are disorders associated with the deficit of coagulation factors VIII and IX. OBJECTIVE: Was to determine the incidence of complications in a cohort of patients diagnosed with moderate and severe hemophilia A or B under treatment in a specialized institution. METHODS: A retrospective study of a cohort of patients with replacement therapy for hemophilia A or B, evaluating treatment and complications between January/2012 and July/2019. Sociodemographic, clinical and disease management-related variables were extracted from the medical records. Time to inhibitor development and rate associated with bleeding and hospitalizations were evaluated. RESULTS: A total of 159 male patients were identified with hemophilia A (n = 140; 88.1%) and B (n = 19; 11.9%) with a mean follow-up of 5.9±2.3 years. The mean age was 23.6±16.1 years, hemophilia was reported as severe in 125 patients in hemophilia A (89.3%) and 13 patients in hemophilia B (68.4%). Primary prophylaxis was registered in 17.0% of patients, 44.7% secondary, and 38.3% tertiary, with recombinant factors (n = 84; 52.8%) followed by plasma derived factors (n = 75; 47.2%). The incidence of inhibitor development was 0.3 per 100 patients/year, with mean time to event of 509 days. The incidence of bleeding was 192 per 100 patients/year, especially at the joint (n = 99; 62.3%) and muscle (n = 25; 15.7%) level. The incidence of hospitalization was 3.7 per 100 patients/year. CONCLUSIONS: The most common complication was joint bleeding which was expected in this type of patients. Low proportion of patients developed factor inhibitors during the follow up.
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spelling pubmed-106813052023-11-27 The frequency of complications in a cohort of patients diagnosed with hemophilia A and hemophilia B receiving prophylactic treatment in Colombia: A retrospective noninterventional study Machado Alba, Jorge E. Wilches-Gutierrez, Juan David Arias-Osorio, Diana Rocio Reyes, Juan Manuel Nakandakari, Maria Lourdes Ospina-Arzuaga, Harrison David Gaviria-Mendoza, Andres Castaño-Gamboa, Natalia Valladales-Restrepo, Luis Fernando Machado-Duque, Manuel E. PLoS One Research Article INTRODUCTION: Hemophilia A and B are disorders associated with the deficit of coagulation factors VIII and IX. OBJECTIVE: Was to determine the incidence of complications in a cohort of patients diagnosed with moderate and severe hemophilia A or B under treatment in a specialized institution. METHODS: A retrospective study of a cohort of patients with replacement therapy for hemophilia A or B, evaluating treatment and complications between January/2012 and July/2019. Sociodemographic, clinical and disease management-related variables were extracted from the medical records. Time to inhibitor development and rate associated with bleeding and hospitalizations were evaluated. RESULTS: A total of 159 male patients were identified with hemophilia A (n = 140; 88.1%) and B (n = 19; 11.9%) with a mean follow-up of 5.9±2.3 years. The mean age was 23.6±16.1 years, hemophilia was reported as severe in 125 patients in hemophilia A (89.3%) and 13 patients in hemophilia B (68.4%). Primary prophylaxis was registered in 17.0% of patients, 44.7% secondary, and 38.3% tertiary, with recombinant factors (n = 84; 52.8%) followed by plasma derived factors (n = 75; 47.2%). The incidence of inhibitor development was 0.3 per 100 patients/year, with mean time to event of 509 days. The incidence of bleeding was 192 per 100 patients/year, especially at the joint (n = 99; 62.3%) and muscle (n = 25; 15.7%) level. The incidence of hospitalization was 3.7 per 100 patients/year. CONCLUSIONS: The most common complication was joint bleeding which was expected in this type of patients. Low proportion of patients developed factor inhibitors during the follow up. Public Library of Science 2023-11-27 /pmc/articles/PMC10681305/ /pubmed/38011251 http://dx.doi.org/10.1371/journal.pone.0286187 Text en © 2023 Machado Alba et al https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Research Article
Machado Alba, Jorge E.
Wilches-Gutierrez, Juan David
Arias-Osorio, Diana Rocio
Reyes, Juan Manuel
Nakandakari, Maria Lourdes
Ospina-Arzuaga, Harrison David
Gaviria-Mendoza, Andres
Castaño-Gamboa, Natalia
Valladales-Restrepo, Luis Fernando
Machado-Duque, Manuel E.
The frequency of complications in a cohort of patients diagnosed with hemophilia A and hemophilia B receiving prophylactic treatment in Colombia: A retrospective noninterventional study
title The frequency of complications in a cohort of patients diagnosed with hemophilia A and hemophilia B receiving prophylactic treatment in Colombia: A retrospective noninterventional study
title_full The frequency of complications in a cohort of patients diagnosed with hemophilia A and hemophilia B receiving prophylactic treatment in Colombia: A retrospective noninterventional study
title_fullStr The frequency of complications in a cohort of patients diagnosed with hemophilia A and hemophilia B receiving prophylactic treatment in Colombia: A retrospective noninterventional study
title_full_unstemmed The frequency of complications in a cohort of patients diagnosed with hemophilia A and hemophilia B receiving prophylactic treatment in Colombia: A retrospective noninterventional study
title_short The frequency of complications in a cohort of patients diagnosed with hemophilia A and hemophilia B receiving prophylactic treatment in Colombia: A retrospective noninterventional study
title_sort frequency of complications in a cohort of patients diagnosed with hemophilia a and hemophilia b receiving prophylactic treatment in colombia: a retrospective noninterventional study
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10681305/
https://www.ncbi.nlm.nih.gov/pubmed/38011251
http://dx.doi.org/10.1371/journal.pone.0286187
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