Epidemiological characteristics of hemophilia in the pre-primary prophylaxis era: a historical cohort

Introduction: Epidemiological studies on hemophilia in the Brazilian population are historically scarce. Despite the continuous effort made by the National Program of Inherited Bleeding Disorders to map this condition, little information is available, especially on the period prior to program concep...

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Main Authors: Dayenne Helena Catelli, Júlia Plentz Portich, Adriana Celia Luz, Giovana Fecker da Costa Diaz, Liliane Ourique de Ourique, Daniel Sander Hoffmann, Guilherme Rasia Bosi, Barbara Coiro Spessato, Leo Sekine, Lucia Mariano da Rocha Silla
Format: Article
Language:English
Published: Hospital de Clinicas de Porto Alegre ; Universidade Federal do Rio Grande do Sul (UFRGS) 2021-06-01
Series:Clinical and Biomedical Research
Subjects:
Online Access:https://seer.ufrgs.br/hcpa/article/view/107936
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author Dayenne Helena Catelli
Júlia Plentz Portich
Adriana Celia Luz
Giovana Fecker da Costa Diaz
Liliane Ourique de Ourique
Daniel Sander Hoffmann
Guilherme Rasia Bosi
Barbara Coiro Spessato
Leo Sekine
Lucia Mariano da Rocha Silla
author_facet Dayenne Helena Catelli
Júlia Plentz Portich
Adriana Celia Luz
Giovana Fecker da Costa Diaz
Liliane Ourique de Ourique
Daniel Sander Hoffmann
Guilherme Rasia Bosi
Barbara Coiro Spessato
Leo Sekine
Lucia Mariano da Rocha Silla
author_sort Dayenne Helena Catelli
collection DOAJ
description Introduction: Epidemiological studies on hemophilia in the Brazilian population are historically scarce. Despite the continuous effort made by the National Program of Inherited Bleeding Disorders to map this condition, little information is available, especially on the period prior to program conception. Therefore, the present study aims to assess the epidemiological, serological, and clinical characteristics of patients with hemophilia in the state of Rio Grande do Sul, Brazil. Methods: A total of 455 patients had their medical records reviewed from January 1, 2003 to December 31, 2007. Results: We observed a remarkable prevalence of hepatitis C virus (HCV) infection in patients with both hemophilia A and B, and this prevalence significantly increased along with age (p < 0.001). No positive anti-HCV results were observed among children younger than 5 years old. There was a significant correlation between the severity of hemophilia and the number of arthropathies in all age categories. Considering the presence of inhibitors, a significant difference was observed between age groups, as older patients had higher inhibitor titers. There was a significant correlation between mean coagulation factor consumption and the number of arthropathies in patients over 5 years old. Conclusions: This profile analysis of patients with hemophilia reflects a gradual improvement in treatment safety and efficiency, as well as the need for continued investment in this population.
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spelling doaj.art-fda0bf4a8f0e4eefac80f77340f6d13b2022-12-21T16:58:26ZengHospital de Clinicas de Porto Alegre ; Universidade Federal do Rio Grande do Sul (UFRGS)Clinical and Biomedical Research2357-97302021-06-0141146727Epidemiological characteristics of hemophilia in the pre-primary prophylaxis era: a historical cohortDayenne Helena Catelli0Júlia Plentz Portich1Adriana Celia Luz2Giovana Fecker da Costa Diaz3Liliane Ourique de Ourique4Daniel Sander Hoffmann5Guilherme Rasia Bosi6Barbara Coiro Spessato7Leo Sekine8Lucia Mariano da Rocha Silla9Universidade Federal do Rio Grande do Sul, Porto-Alegre (RS), BrazilHematology Division, Hospital de Clínicas de Porto-Alegre, Porto-Alegre (RS), BrazilHemocentro do Estado do Rio Grande do Sul, BrazilHemocentro do Estado do Rio Grande do Sul, BrazilHemocentro do Estado do Rio Grande do Sul, BrazilUniversidade Estadual do Rio Grande do Sul, BrazilUniversidade de Caxias do Sul, BrazilUniversidade Federal do Rio Grande do Sul, Porto-Alegre (RS)Hemotherapy Division, Hospital de Clínicas de Porto-Alegre, Porto-Alegre (RS), BrazilUniversidade Federal do Rio Grande do Sul, Porto-Alegre (RS)Introduction: Epidemiological studies on hemophilia in the Brazilian population are historically scarce. Despite the continuous effort made by the National Program of Inherited Bleeding Disorders to map this condition, little information is available, especially on the period prior to program conception. Therefore, the present study aims to assess the epidemiological, serological, and clinical characteristics of patients with hemophilia in the state of Rio Grande do Sul, Brazil. Methods: A total of 455 patients had their medical records reviewed from January 1, 2003 to December 31, 2007. Results: We observed a remarkable prevalence of hepatitis C virus (HCV) infection in patients with both hemophilia A and B, and this prevalence significantly increased along with age (p < 0.001). No positive anti-HCV results were observed among children younger than 5 years old. There was a significant correlation between the severity of hemophilia and the number of arthropathies in all age categories. Considering the presence of inhibitors, a significant difference was observed between age groups, as older patients had higher inhibitor titers. There was a significant correlation between mean coagulation factor consumption and the number of arthropathies in patients over 5 years old. Conclusions: This profile analysis of patients with hemophilia reflects a gradual improvement in treatment safety and efficiency, as well as the need for continued investment in this population.https://seer.ufrgs.br/hcpa/article/view/107936hemophiliaepidemiologyarthropathystate of rio grande do sulbrazil
spellingShingle Dayenne Helena Catelli
Júlia Plentz Portich
Adriana Celia Luz
Giovana Fecker da Costa Diaz
Liliane Ourique de Ourique
Daniel Sander Hoffmann
Guilherme Rasia Bosi
Barbara Coiro Spessato
Leo Sekine
Lucia Mariano da Rocha Silla
Epidemiological characteristics of hemophilia in the pre-primary prophylaxis era: a historical cohort
Clinical and Biomedical Research
hemophilia
epidemiology
arthropathy
state of rio grande do sul
brazil
title Epidemiological characteristics of hemophilia in the pre-primary prophylaxis era: a historical cohort
title_full Epidemiological characteristics of hemophilia in the pre-primary prophylaxis era: a historical cohort
title_fullStr Epidemiological characteristics of hemophilia in the pre-primary prophylaxis era: a historical cohort
title_full_unstemmed Epidemiological characteristics of hemophilia in the pre-primary prophylaxis era: a historical cohort
title_short Epidemiological characteristics of hemophilia in the pre-primary prophylaxis era: a historical cohort
title_sort epidemiological characteristics of hemophilia in the pre primary prophylaxis era a historical cohort
topic hemophilia
epidemiology
arthropathy
state of rio grande do sul
brazil
url https://seer.ufrgs.br/hcpa/article/view/107936
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