الفهرس 
SICKLE CELL ANEMIAOnly 14 pages are availabe for public view
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Abstract
Sickle cell disease (SCD) is a Mendelian genetic diseaseencompassing a wide spectrum of disorders and results from a singlenucleotide substitution in the DNA of adenine with thymine at codon 6 ofthe beta-globin gene on chromosome 11 causing a point mutation, withhydrophobic valine replacing hydrophilic glutamic acid in the
polypeptide of the beta-globin chain of haemoglobin. The HbS mutation
can be inherited in homozygosis or in heterozygosis with other β-globin
qualitative or quantitative defects. It is characterized by a chronic hemolytic
anemia, endothelial dysfunction and vaso-occlusive crisis that promote acute
and chronic complications.Survival rate among children with SCD has increased especially in therecent decades, due to an earlier diagnosis and a better quality of care.
Consequently, the incidence of long-term complications, such as metabolicand endocrine disorders, is increasing in SCD population.Our cross sectional comparative study was carried out on forty sicklecell patients aged 5-16 years and 40 healthy children with matched age and sex at Tanta UniversityHospitals,PediatricsDepartment in the period fromApril 2022 to October 2023 aiming to evaluate insulin resistance and defects in glucose homeostasis in Egyptian children with sickle cell anemia in thesteady state. Patients with vasoocclusive painful crisis, high BMI, or on drugs affect.glycemic.status were excluded from the study. Most sickle cell patients
kept normal glucose homeostasis with comparable fasting blood glucose(FBG) level to controls with low incidence of impaired fasting glucose (7.5%) and hypoglycemia (2.5%). Homeostatic Model Assessment for InsulinResistance (HOMA-IR) was used as a marker for insulin resistance. (HOMAIR)has been frequently validated in children and adolescents (via comparison
.