Abstract

BackgroundSeveral studies have shown that the pathophysiology of homozygous sickle cell anaemia (SCA) results in a myriad of metabolic, nutritional, haematological and clinical effects that interact with other co-morbid factors to determine the quality of life and life expectancy of afflicted patients. Because of its critical roles in nutrition and metabolism, inflammation, haematopoiesis and cellular immunity, this study determined the plasma levels of leptin in steady and unsteady states of HbSS in Nigerian patients.MethodsA total of 51 SCA patients aged 5 - 35 years with 34 (61.8%) being females who were either on admission or visiting four medical centres in Lagos, Nigeria together with 22 non-SCD controls aged 5 -30 years comprising 12 (54.5%) females were enrolled after obtaining their informed consent and ethical approval. Patients were further stratified into steady and unsteady cases of SCA based on clinical presentations, while blood samples collected by venipuncture from each of the study participants were analyzed haematologically for full blood count and HbF level and microscopically for malaria, while plasma leptin was assayed using ELISA method. Body composition defined by weight, fat mass and body mass index (BMI) was determined using standard methods. Data obtained for cases and controls were analyzed statistically.ResultsTwenty - one patients had unsteady HbSS and elicited greater and significant (P < 0.05) reduction in fat mass, BMI, HbF and eosinophil count but elevated mean total leukocyte, count, level of irreversibly sickled cells and P. falciparum parasitaemia (4613.7 vs. 749.6 - 1078.4 parasites/uL), pyrexia rate (58.3 vs. 25.8%) when compared with steady state patients or non-SCD controls. Compared to the control, significant decreases in plasma leptin before and after controlling for body fat that was worsened by crisis were observed among the SCD patients. Unlike the non-SCD controls, leptin correlated non-significantly (P > 0.05) with all body composition indices measured in the patients except for fat mass in unsteady cases. Multivariate regression analysis identified ESR and RC as independent predictor of low plasma leptin concentration in the SCA patients.ConclusionsBase on these findings, we conclude that plasma level of leptin is further decreased in the unsteady state of HbSS, shows poor correlation with adiposity and malarial infection but has inflammation and poor reticulocyte response as independent predictors among Nigerian patients.

Highlights

  • Several studies have shown that the pathophysiology of homozygous sickle cell anaemia (SCA) results in a myriad of metabolic, nutritional, haematological and clinical effects that interact with other co-morbid factors to determine the quality of life and life expectancy of afflicted patients

  • In comparison with the non-SCD control (n = 24), significant (P

  • When compared with stable patients, those in unsteady state were found to elicit further significant (P < 0.05) elevations of these parameters including the level of irreversible sickle cells except for HbF and reticulocytes count

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Summary

Introduction

Several studies have shown that the pathophysiology of homozygous sickle cell anaemia (SCA) results in a myriad of metabolic, nutritional, haematological and clinical effects that interact with other co-morbid factors to determine the quality of life and life expectancy of afflicted patients. In the steady state of HbSS, patients are clinically stable but endothelial activation and subclinical vasoocclusions due to inherent microvascular abnormalities are an ongoing process coupled with chronic but slow red cell haemolysis, platelet activation and moderate cytokine and acute phase response [4] These pathophysiological events are generally insufficient to induce painful crisis [4,5]. In the unsteady state of sickle cell anaemia (SCA), these pathognomic events are exaggerated and the consequence is severe vaso-occlusion and blood flow that leads to organ damage and painful crisis with complications such as bone and joint infarction, acute chest syndrome, severe anaemia, aplastic anaemia and acute renal failure [6] These complications have well been reported as the causes of hospitalization and deaths in afflicted patients in Nigeria and other countries [7,8]

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