Clinical and epidemiological profile of hemophilia in Cuba based on the National Registry of Congenital Coagulopathies. First report

Abstract

Abstract Aims The objective of the study was the description of the hemophilia in Cuba and to observe the relationship between clinical parameters and inhibitors. Methods Cross-sectional study of the clinical characterization of hemophilia in Cuba based on the National Registry of Congenital Coagulopathies.The main variables obtained were place of residence, type of hemophilia, levels of functional activity, age and age at diagnosis, skin color, family history, degree of response to inhibitors. Results The total number of subjects included was 496; statistically significant differences were founded showing a lower age for moderate patients compared to severe and mild patients. Mean difference for severe vs moderate patients 7.54 (95%CI 3.14–11.95), p < 0.0001. The number of subjects with inhibitors was 35 (8.5%). There was no statistical significance between age range and the presence of inhibitors, but differences were observed when age was analyzed as continuous variable p < 0.05 (inverse relation between age and inhibitors). For severity, statistical significance was observed in relation to the presence of inhibitors p < 0.05. Conclusions Our study showed a high percentage of subjects diagnosed in the first year of life, mainly severe cases, which implies the use of management tools adapted to the needs of patients from the very beginning, as well as continuous monitoring with treatment alternatives according to individual aspects.