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Mortality of patients with haemophilia in Brazil: First report
Author(s) -
Jardim Leticia Lemos,
van der Bom Johanna G.,
CaramDeelder Camila,
Gouw Samantha Claudia,
Leal Cherchiglia Mariangela,
Rezende Suely Meireles
Publication year - 2019
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/hae.13730
Subject(s) - medicine , haemophilia , mortality rate , cause of death , haemophilia a , population , pediatrics , standardized mortality ratio , hepatocellular carcinoma , disease , liver disease , environmental health
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.