Abstract
Hematological and biochemical reference values in sickle cell disease (SCD) are crucial for patient management and the evaluation of interventions. This study was conducted at Muhimbili National Hospital (MNH) in Dar es Salaam, Tanzania, to establish laboratory reference ranges among children and adults with SCD at steady state. Patients were grouped into five age groups and according to their sex. Aggregate functions were used to handle repeated measurements within the individual level in each age group. A nonparametric approach was used to smooth the curves, and a parametric approach was used to determine SCD normal ranges. Comparison between males and females and against the general population was documented. Data from 4422 patients collected from 2004–2015 were analyzed. The majority of the patients (35.41%) were children aged between 5–11 years. There were no significant differences (p ≥ 0.05) in mean corpuscular hemoglobin concentration (MCHC), lymphocytes, basophils, and direct bilirubin observed between males and females. Significant differences (p < 0.05) were observed in all selected parameters across age groups except with neutrophils and MCHC in adults, as well as platelets and alkaline phosphatase in infants when the SCD estimates were compared to the general population. The laboratory reference ranges in SCD at steady state were different from those of the general population and varied with sex and age. The established reference ranges for SCD at steady state will be helpful in the management and monitoring of the progress of SCD.
Highlights
Sickle cell disease (SCD) is the most common genetic disorder globally, with an estimated 330,000 babies born with the condition annually [1]
The mean hemoglobin, HbF, mean corpuscular hemoglobin (MCH), red blood cell (RBC), mean corpuscular volume (MCV), red cell distribution width (RDW), eosinophils, bilirubin total, alkaline phosphatase (ALP), and creatinine were different between females and males, with most deviations happening after the age of 15 years
We reported an increase in the means of MCH, MCV, and neutrophils with significantly higher values in females
Summary
Sickle cell disease (SCD) is the most common genetic disorder globally, with an estimated 330,000 babies born with the condition annually [1]. Individuals with SCD go through alternating periods of stable health (steady state) and crises associated with complications such as anemia and pain [10]. These are generally associated with alteration in the quantity and function of the cellular elements in the blood. Research has shown that patients with a high leukocyte count, low steady state hemoglobin, and lower fetal hemoglobin level (HbF) have increased risks for sickle cell crises, including stroke [12]
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