Clinical behavior and complications of hemophilia in the pediatric population

Authors

  • Jorge Luis Hernández Gonzalez. Hospital Pediátrico Provincial Pepe Portilla
  • Mirta Campo Díaz Hospital Pediátrico Provincial Pepe Portilla
  • Cesar Valdés Sojo Hospital Pediátrico Provincial Pepe Portilla
  • Grettel Borrego Cordero Hospital Pediátrico Provincial Pepe Portilla
  • Claudia Cabrera Morales Hospital General Docente Abel Santamaría Cuadrado

Keywords:

HEMOPHILIA A, CHILD, EPIDEMIOLOGY.

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.

 

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Author Biographies

Jorge Luis Hernández Gonzalez., Hospital Pediátrico Provincial Pepe Portilla

Doctor en Medicina. Especialista de
Primer Grado en Hematología. Máster en Atención Integral al Niño. Profesor
asistente.

Mirta Campo Díaz, Hospital Pediátrico Provincial Pepe Portilla

Doctora en Medicina. Especialista de Primer y
Segundo Grado en Hematología, especialista de Segundo Grado en Pediatría.
Máster en Atención Integral al Niño. Profesora auxiliar. Investigadora  auxiliar. Profesora consultante.

Cesar Valdés Sojo, Hospital Pediátrico Provincial Pepe Portilla

Doctor en Medicina. Especialista de Primer Grado en
Medicina General Integral y Hematología. Máster en Atención Integral al
Niño con Enfermedades Hematológicas Crónicas. Instructor.

Grettel Borrego Cordero, Hospital Pediátrico Provincial Pepe Portilla

Doctora en Medicina. Especialista de Primer
Grado en Medicina General Integral y Hematología. Máster en Atención Iintegral al Niño con Enfermedades Hematológicas Crónicas.

Claudia Cabrera Morales, Hospital General Docente Abel Santamaría Cuadrado

Doctora en Medicina. Residente de primer año en
Hematología. Instituto de Hematología e Inmunología. Servicio de Hematología.

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Published

2018-03-02

How to Cite

1.
Hernández Gonzalez. JL, Campo Díaz M, Valdés Sojo C, Borrego Cordero G, Cabrera Morales C. Clinical behavior and complications of hemophilia in the pediatric population. Rev Ciencias Médicas [Internet]. 2018 Mar. 2 [cited 2025 Aug. 30];22(2):226-33. Available from: https://revcmpinar.sld.cu/index.php/publicaciones/article/view/3334

Issue

Vol. 22 No. 2 (2018)

Section

ORIGINAL ARTICLES

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