Caracterización clínica de las crisis drepanocíticas en pacientes pediátricos con anemia falciforme del Hospital del niño “Dr. Francisco De Icaza Bustamante” durante el período del 2016 al 2022.

Abstract

Introduction: Sickle cell disease is a genetic disorder that primarily affects people of African descent and encompasses a group of hemoglobinopathies; the main one being sickle cell anemia which causes hemolysis, pain crises, and chronic organ damage. Vaso-occlusive crises lead to episodes of intense acute pain in different parts of the body. Objectives: To clinically characterize sickle cell crises in pediatric patients with sickle cell anemia, identifying triggering factors and associated complications. Methodology: An observational, retrospective, cross-sectional, and descriptive study was carried out based on the review of medical records from the "Dr. Francisco De Icaza Bustamante" Children's Hospital. Pediatric patients with sickle cell anemia who presented at least one sickle cell crisis from 2016 to 2022 were included. Data were analyzed using descriptive statistics through frequencies and percentages. Results: Out of 165 pediatric patients with sickle cell anemia, 103 presented crises; of these, 50.5% were male. 76.6% identified themselves as mixed race. 69.9% had at least one family history of sickle cell anemia. The most frequent symptoms were pain (100%), paleness (77.3%) and jaundice (66%). The most prevalent site of pain was at the abdominal level (62.1%) and lower limbs (52.4%). The main triggering factor was fever (59.2%). Among the complications, pneumonia (21.4%) and acute chest syndrome (13.6%) stood out. Conclusion: Vaso-occlusive crises are common in children with sickle cell anemia and manifest mainly with intense pain that can vary in location and be associated with various complications that can originate high morbidity and mortality.