Related Experiment Videos
Clinical features of sickle cell disease in eastern Saudi Arab children
M I el Mouzan1, B H al Awamy, M T al Torki
1Department of Pediatrics, College of Medicine and Medical Sciences, King Faisal University, Saudi Arabia.
Insights
Sickle cell disease (SCD) in Saudi children presents milder than previously reported. High hemoglobin F levels correlate with fewer crises and delayed symptoms in these pediatric patients.
Area of Science:
- Pediatrics
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with variable clinical manifestations.
- Understanding SCD's clinical spectrum in specific populations is crucial for tailored management.
Purpose of the Study:
- To describe the clinical features of SCD in children of eastern Saudi Arabian origin.
- To correlate clinical presentation with genotype and hemoglobin F levels.
Main Methods:
- Prospective follow-up of 173 children diagnosed with SCD at birth.
- Data collection included genotype, age at presentation, symptoms, crises, hospital admissions, and mortality.
- Correlation analysis between hemoglobin F levels and clinical outcomes.
Main Results:
- The most common genotypes were sickle cell anemia (146) and sickle beta+-thalassemia (24).
- Painful crises (60%) and dactylitis (31.6%) were the most frequent initial symptoms.
- Only 9.1% of crises required hospital admission, and no deaths occurred; high HbF levels correlated with milder disease.
Conclusions:
- SCD in eastern Saudi children exhibits a clinically milder course compared to earlier reports.
- High hemoglobin F levels are associated with a delayed onset of symptoms and a reduced number of crises.
- These findings highlight population-specific variations in SCD clinical severity.
Abstract:
The clinical features of sickle cell disease (SCD) in Saudi Arab children of eastern origin are presented. One hundred and seventy-three children were diagnosed at birth and followed prospectively from 3 months to up to 4 years of age. There were 87 boys and 86 girls. Genotype distribution included 146 sickle cell anemia, 24 sickle beta +-thalassemia, two sickle beta 0-thalassemia, and one sickle hemoglobin C disease. Of our patients, 7% presented in the first 12 months of age and 27% remained asymptomatic at 4 years. Painful crises of bones and joints were the most common initial symptoms, followed by dactylitis, abdominal crises and acute splenic sequestration (ASS), occurring in 60%, 31.6%, 6.7%, and 1.7% of the patients, respectively. None of the patients presented with severe bacterial infections. During this study, 175 sickle cell crises were documented, but only 16 (9.1%) required hospital admissions. There were no deaths in this series. High hemoglobin F levels correlated with delayed clinical presentation and reduced number of crises. We conclude that SCD in children of eastern origin is clinically milder than earlier descriptions from the Eastern Province of Saudi Arabia.