Revista de Ciencias Médicas de Pinar del Río (Mar 2018)
Clinical behavior and complications of hemophilia in the pediatric population
Abstract
Introduction: hemophilia is an inherited disease linked to sex, caused by the decreased coagulant activity of factor VIII (hemophilia A), factor IX (hemophilia B) and factor XI (hemophilia C). Its treatment represents a high economic cost for the country and it is associated with great frequency to serious disabilities of the afflicted patient, negatively impacting its bio-psychosocial development, as well as an adequate familiar functionality. Objective: to characterize hemophilia clinically and epidemiologically in the pediatric population of Pinar del Río province, in the period from January 1985 to June 2016 Method: an observational, descriptive and cross-sectional study was conducted in pediatric patients diagnosed with hemophilia. The data were extracted from the clinical files. The diagnosis was established by coagulogram, dosage of plasma factors VIII, IX and XI and dosage of inhibitors. Descriptive statistics was used. Results: out of a total of 9 hemophiliac individuals, a clear predominance of A (n = 8) and moderate form (n = 5) was observed. The diagnoses were made before one year of age (66 %), where traumatic hemorrhages (intracranial bleedings) were the most frequent (22.1 %). All patients with moderate or severe behavior had muscle-skeletal bleeding. The main complications of the disease were secondary epilepsy and mental retardation as a consequence of neonatal intracranial hemorrhage; therapeutic complications were related to alloimmune processes. Conclusions: Traumatic hemorrhages in the early stages of life were the most frequent cause of the diagnosis of the disease, with bleeding from the central nervous system being the main cause of sequelae in these patients.
