Epidemiological and clinical profile of sickle cell disease in a series of Moroccan children
View abstract on PubMed
Summary
This summary is machine-generated.Sickle cell disease (SCD) significantly impacts Moroccan children, with rural prevalence and consanguinity noted. Higher fetal hemoglobin offers protection, underscoring the need for national management strategies.
Area Of Science
- Pediatrics
- Hematology
- Genetics
Background
- Sickle cell disease (SCD) is a prevalent global genetic disorder.
- It stems from abnormal hemoglobin (HbS) in red blood cells, causing severe complications.
- Understanding SCD's characteristics in specific populations is crucial for targeted interventions.
Purpose Of The Study
- To delineate the epidemiological and clinical features of SCD in Moroccan children.
- To identify distinct clinical profiles and associated factors within this cohort.
Main Methods
- A prospective descriptive study involving 194 Moroccan children (97 SCD patients, 97 controls).
- Data collection via medical records and parental interviews.
- Cluster analysis to define clinical profiles; statistical analysis for parameter comparisons.
Main Results
- SCD patients showed a mean age of 7.59 years, with female predominance and high rural residence (77.3%).
- Parental consanguinity was reported in 54.6% of cases.
- Three clinical profiles emerged: mild anemia, recurrent vaso-occlusive crises (VOC), and acute severe anemia with infections. Higher fetal hemoglobin levels correlated with protection against acute complications and reduced transfusion needs.
Conclusions
- SCD poses a significant health burden in the studied Moroccan region.
- Clinical heterogeneity necessitates tailored management approaches.
- Enhanced nationwide strategies including awareness, education, genetic counseling, and screening are vital for improving patient outcomes.
Related Concept Videos
The Concept of Multiple Allelism
Multiple allelism describes genes that exist in three or more allelic forms. Although diploid organisms, like humans, normally possess only two alleles of each gene, there are multiple alleles of many (if not most) human genes present in a population. Blood type is one example of multiple allelism. There are three alleles for blood type (HBB gene) in humans: IA, IB, and i.
Incomplete Dominance
Sickle cell anemia, which is caused by a mutation in the gene...
Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...
In statistical epidemiology and health sciences, two essential metrics—prevalence and incidence—are fundamental for understanding disease dynamics within a population. These measures enable public health officials, epidemiologists, and researchers to assess the burden of diseases, allocate resources effectively, and design impactful public health policies and interventions.
Prevalence indicates the proportion of individuals in a population who have a specific disease or health...
Drug distribution in the pediatric population exhibits unique challenges and considerations due to the physiological differences between children, particularly neonates and infants, and adults. A crucial aspect of pediatric pharmacology is understanding how these differences impact the pharmacokinetics of various drugs, necessitating age-specific dosing strategies to ensure efficacy and safety.Neonates and infants have a higher total body water content, ~75%–90% of their body weight,...
In pediatric medicine, understanding the renal function and drug elimination nuances is crucial for administering safe and effective treatments. Newborns, in particular, display markedly slower renal functions than adults, profoundly affecting how drugs are cleared from their bodies. This slower drug clearance requires clinicians to extend the dosing intervals for many medications to prevent drug accumulation and toxicity while ensuring therapeutic efficacy.One key area where these adjustments...
Assessing and diagnosing Chronic Obstructive Pulmonary Disease (COPD) involves a detailed approach that includes a comprehensive review of medical history, physical examination, and a variety of diagnostic tests. This thorough evaluation is essential to ensure an accurate diagnosis and guide effective management strategies.
Medical History
• Symptom Review: Chronic symptoms such as persistent cough, sputum production, shortness of breath (dyspnea), and episodes of exacerbation are...

