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Updated: Jul 5, 2025
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Neurological abnormalities among pediatric patients with sickle cell disease in Saudi Arabia: a single-center
Ziad T Basuni1, Dania A Monagel1,2,3, Areej Taha1
1Department of Oncology, Ministry of the National Guard- Health Affairs, Jeddah, Saudi Arabia.
Insights
Sickle cell disease (SCD) can cause serious neurological issues in children, even those with high fetal hemoglobin. Early detection and intervention are vital for managing these complications in Saudi children with SCD.
Area of Science:
- Pediatric Neurology
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) is a common inherited blood disorder.
- Neurological complications are a significant concern in SCD patients.
- Early detection and treatment are critical for managing SCD-related neurological issues.
Purpose of the Study:
- To describe neurological manifestations, radiological findings, and diagnoses in Saudi children with SCD.
- To contribute to population-based guidelines for screening and treating SCD-related neurological complications.
Main Methods:
- Descriptive retrospective study of pediatric patients (<14 years) with SCD.
- Data collected from 101 participants at KAMC, Jeddah, Saudi Arabia (Jan 2008-Jan 2022).
- Analysis of demographic, clinical, and neurological data.
Main Results:
- 101 SCD patients studied, mean age at diagnosis 23 months.
- 26.7% of patients experienced neurological sequelae (seizures, stroke, etc.).
- No significant difference in neurological onset between high HbF and other SCD phenotypes.
Conclusions:
- Children with SCD face an increased risk of brain injury and neurocognitive deficits.
- Neurological complications occur even with high fetal hemoglobin, necessitating further research and interventions.
- Large-scale, population-specific studies are needed for better management of SCD neurological complications in Saudi Arabia.
Introduction:
Sickle cell disease (SCD) is a common inherited blood disorder characterized by the production of abnormal sickle-shaped red blood cells. SCD can lead to various complications including neurological issues. Early detection and treatment are crucial for preventing these complications. This study aimed to describe the neurological manifestations, radiological findings, and neurological diagnosis related to SCD in Saudi children with the aim of contributing to the formulation of population-based guidelines for screening and treating SCD-related neurological complications.
Methods:
This descriptive retrospective study included pediatric patients aged < 14 years diagnosed with SCD who were regularly followed up at the hematology clinic in KAMC, Jeddah, Saudi Arabia, from January 2008 to January 2022. Demographic and clinical data were collected from the clinical charts of 101 participants.
Results:
This study included 101 patients with SCD with a mean age of 23 months at diagnosis. Among these, 59% had SCD and high fetal hemoglobin (HbF) levels. Neurological sequelae, including seizures, stroke, and other abnormalities, were observed in 26.7% of patients. There were no significant differences in the onset of neurological issues between the patients with SCD-high HbF and those with other SCD phenotypes.
Discussion:
This study highlights the increased risk of brain injury and neurocognitive deficits in children with SCD. The occurrence of neurological sequelae in many patients emphasizes the need for early detection and intervention. Some patients experience neurological complications despite having high HbF levels, suggesting that further interventions are needed. This study has some limitations, including its small sample size and retrospective nature.
Conclusion:
Early detection and intervention are crucial for neurological complications in patients with SCD. This study emphasizes the need for further research and effective treatment strategies considering the presence of neurological complications despite the presence of high HbF levels. Large-scale studies and population-specific guidelines are warranted for better understanding and management of SCD-related neurological complications in the Saudi population.
