Clinical Manifestations of Children with Sickle Cell Disease in the Northern and Western Regions of Yemen

Abstract

Background: Sickle cell disease (SCD) is characterized by a wide variation in the clinical manifestations and severity of the associated complications despite the molecular abnormality leading to sickle gene is similar in all haplotypes. Aim: This study aimed to evaluate the clinical manifestations patterns of children affected by homozygous SS SCD. Methods: It was a hospital-based, cross-sectional study carried out on 147 pediatric patients from the time of birth up to 12 years. It was conducted at Saudi Hospital Hajjah (SHH) over a period of a year (2020). Results: The mean age was 7.32 ± 3.5 years, 57.1% of them were males. Two children (1.3%) had their symptoms started at the first year of age. The most frequent symptoms were vaso-occlusive crises (VOC), generalized bone pain (38.0%), fever (34.0%), bone pain and fevers (9.5%), anemic crises (7.5%) and hand-foot-syndrome (6.1%). Splenomegaly was detected in 28.5% and cerebrovascular accident (CVA) in one child (0.7%). Splenomegaly as well as bone pain were significantly observed among children older than five years (P < 0.05). Conclusion: SCD is characterized by a marked variations in the clinical manifestations. The symptoms started as early as the first year of life and VOC remain the most frequent clinical presentation. Genetic counseling, premarital screening as well as early infant screening are urgently required in our country to reduce the magnitude of disease and to improve the quality of care