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Assessing Major Organ Dysfunction in Children With Sickle Cell Disease: Protocol for a Cross-Sectional Study
Vivian Paintsil1,2, Sandra Kwarteng Owusu1,2, Samuel Blay Nguah1,2
1Department of Child Health, School of Medical Sciences Kwame Nkrumah University of Science and Technology Kumasi Ghana.
Insights
Sickle cell disease (SCD) causes organ damage in children. This study in Ghana assesses organ dysfunction and its factors in pediatric SCD patients to improve care and policies.
Area of Science:
- Pediatric Hematology
- Genetic Disorders
- Public Health
Background:
- Sickle cell disease (SCD) is a prevalent global genetic disorder, affecting approximately 300,000 newborns annually, with a 1.8% prevalence in Ghana.
- Increasing life expectancy in SCD patients necessitates evaluating the impact of progressive organ damage on quality of life.
- Key organs affected by SCD include the lungs, heart, brain, kidneys, and bones.
Purpose of the Study:
- To describe major organ dysfunction in children aged 6-16 years with SCD.
- To identify factors associated with organ dysfunction in pediatric SCD patients at the Kumasi Centre for Sickle Cell Disease (KC-SCD).
- To inform recommendations and hospital policies for screening and managing end-organ dysfunction in pediatric SCD.
Main Methods:
- A cross-sectional study conducted over 1 year and 8 months at the Komfo Anokye Teaching Hospital's pediatric sickle cell clinic.
- Data collection included sociodemographics, medical history, anthropometry, pubertal stage, full blood count, reticulocyte counts, lactate dehydrogenase, lung function tests, electrocardiogram, echocardiogram, and mental health assessment.
- Statistical analysis using STATA to determine the prevalence of end-organ dysfunction and its relationship with clinical and laboratory covariates.
Main Results:
- The study will determine the prevalence of specific end-organ dysfunctions in pediatric SCD patients.
- Inferential analysis will identify associations between various end-organ dysfunctions and clinical/laboratory factors.
- Results will quantify the burden of end-organ damage in the pediatric SCD population in Ghana.
Conclusions:
- Findings will elucidate the extent of end-organ damage in children with SCD in Ghana.
- The results will guide the development of targeted screening protocols for end-organ dysfunction.
- This research will contribute to evidence-based recommendations and hospital policies for improved pediatric SCD management.
Background And Aims:
Sickle cell disease (SCD) is the most common clinically significant genetic disorder affecting about 300,000 newborns globally each year. In Ghana, a prevalence of 1.8% exists among newborns. With increasing life expectancy, assessment of the effect of progressive organ damage on quality of life becomes essential. Organs involved could be the lungs, heart, brain, kidney, and bones. This study aims to describe major organ dysfunction and its associated factors in children aged 6-16 years with SCD in the Kumasi Centre for Sickle Cell Disease (KC-SCD) patient cohort in Kumasi, Ghana.
Methods:
It will be a cross-sectional study conducted over a 1-year 8-month period at the pediatric sickle cell clinic of the Komfo Anokye Teaching Hospital (KATH). All patients, except those coming for acute care or emergency visits, will be eligible for enrollment. Data on sociodemographic details, medical history, and previous complications, anthropometry, and pubertal stage will be collected. Full blood count, reticulocyte counts, lactate dehydrogenase counts, lung function tests, electrocardiogram, echocardiogram, mental health status assessment and pubertal staging will be conducted to assess end organ dysfunctions. Data will be entered into a REDCap database, cleaned, and exported to the STATA statistical software for analysis. The prevalence of specific end-organ dysfunction will be determined based on predefined criteria. Subsequently, the relationship between the various end-organ dysfunctions and clinical and laboratory covariates will be determined using inferential analysis.
Conclusion:
These results will help to know the burden of end organ damage in our pediatric population and subsequently feed into the recommendations and hospital policies for screening for end organ dysfunction.
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