TY - JOUR
T1 - Mortality of patients with haemophilia in Brazil
T2 - First report
AU - Jardim, Leticia Lemos
AU - van der Bom, Johanna G
AU - Caram-Deelder, Camila
AU - Gouw, Samantha Claudia
AU - Leal Cherchiglia, Mariangela
AU - Meireles Rezende, Suely
N1 - © 2019 John Wiley & Sons Ltd.
PY - 2019/5/1
Y1 - 2019/5/1
N2 - INTRODUCTION: Brazil has the fourth largest world population of patients with haemophilia. However, mortality rates in this population are unknown.AIM: To analyse mortality and its causes in Brazilian patients with haemophilia from 2000 to 2014.METHODS: The number of deceased patients with haemophilia and causes of death were obtained from the Brazilian National Mortality Information System (SIM), according to the 10th International Classification of Diseases (ICD-10). Standardized mortality ratios (SMR) were calculated to estimate the rate of overall death of patients with haemophilia relative to that of the Brazilian general male population.RESULTS: A total of 784 deaths were identified in the period of 15 years. Mortality of patients with haemophilia was 13% higher when compared with the general male population (SMR 1.13, 95% CI: 1.01-1.16). Haemorrhage was the main cause of death (n = 254; 32.4%) of which 137 (54%) was intracranial haemorrhage. The total number of deaths due to HIV decreased over the years, and an increase in deaths due to cancer and cardiovascular disease was observed. A total of 129 deaths (16.5%) were related to hepatitis infection, of whom, 109 (86.5%) patients also presented with cirrhosis and hepatocellular carcinoma or other liver diseases.CONCLUSION: Mortality rate of Brazilian patients with haemophilia decreased over the evaluated period. Intracranial haemorrhage is still an important cause of death in these patients, which requires major effort for prevention. Death due to age-related cardiovascular disease and cancer has increased over the years, following the same tendency observed in developed countries.
AB - INTRODUCTION: Brazil has the fourth largest world population of patients with haemophilia. However, mortality rates in this population are unknown.AIM: To analyse mortality and its causes in Brazilian patients with haemophilia from 2000 to 2014.METHODS: The number of deceased patients with haemophilia and causes of death were obtained from the Brazilian National Mortality Information System (SIM), according to the 10th International Classification of Diseases (ICD-10). Standardized mortality ratios (SMR) were calculated to estimate the rate of overall death of patients with haemophilia relative to that of the Brazilian general male population.RESULTS: A total of 784 deaths were identified in the period of 15 years. Mortality of patients with haemophilia was 13% higher when compared with the general male population (SMR 1.13, 95% CI: 1.01-1.16). Haemorrhage was the main cause of death (n = 254; 32.4%) of which 137 (54%) was intracranial haemorrhage. The total number of deaths due to HIV decreased over the years, and an increase in deaths due to cancer and cardiovascular disease was observed. A total of 129 deaths (16.5%) were related to hepatitis infection, of whom, 109 (86.5%) patients also presented with cirrhosis and hepatocellular carcinoma or other liver diseases.CONCLUSION: Mortality rate of Brazilian patients with haemophilia decreased over the evaluated period. Intracranial haemorrhage is still an important cause of death in these patients, which requires major effort for prevention. Death due to age-related cardiovascular disease and cancer has increased over the years, following the same tendency observed in developed countries.
KW - Adolescent
KW - Adult
KW - Aged
KW - Aged, 80 and over
KW - Brazil/epidemiology
KW - Cause of Death
KW - Child
KW - Child, Preschool
KW - Female
KW - Hemophilia A/complications
KW - Hemophilia B/complications
KW - Humans
KW - Infant
KW - Infant, Newborn
KW - Male
KW - Middle Aged
KW - Prognosis
KW - Retrospective Studies
KW - Young Adult
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85062973258&origin=inward
UR - https://www.ncbi.nlm.nih.gov/pubmed/30875453
U2 - https://doi.org/10.1111/hae.13730
DO - https://doi.org/10.1111/hae.13730
M3 - Article
C2 - 30875453
SN - 1351-8216
VL - 25
SP - e146-e152
JO - Haemophilia
JF - Haemophilia
IS - 3
ER -