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Background: Several cardiac and hepatic abnormalities have been observed in patients with sickle cell anemia (SCA) which significantly increase disease-related morbidity and mortality. Objective: To determine the levels of cardiac troponin 1 (cTnI) and alpha-fetoprotein (AFP) in adults with SCA and compare with suitable controls. Subjects and Methods: A total of 95 participants, consisting of 30 heterozygous hemoglobin AS (HbAS), 30 homozygous hemoglobin SS (HbSS) (in steady state), 5 HbSS (in crisis), and 30 hemoglobin AA (HbAA), were studied. Five milliliter of venous blood was collected from each participant for cTnI and AFP level estimation (by the enzyme-linked immunosorbent assay technique) and hemoglobin phenotype confirmation (by cellulose acetate electrophoresis). Results: The mean serum level of cTnI was significantly higher in homozygous SCA participants in crisis compared HbAA controls and SCA participants in steady state (1.13 ± 0.46 ng/ml vs. 0.32 ± 0.03 ng/ml and 1.13 ± 0.46 ng/ml vs. 0.27 ± 0.04 ng/ml, respectively, P = 0.01). There were no significant differences in the mean serum levels of cTnI between SCA participant in steady state and HbAS and HbAA (P = 0.33 and 0.58, respectively). Serum AFP levels were not significantly different in SCA (both in crises and in steady state) compared with HbAS and HbAA participants (P values all > 0.05). Conclusion: Cardiac injury may underlie SCA bone pain crisis; assessment of cardiac function may be indicated, particularly in patients with recurrent crises.

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Background: Sickle cell disease is a common problem across central India, but its clinical features may differ from that in African populations. There is a need to define the features of sickle cell disease in India, and the current study addresses some features of the bone pain crisis. Objectives: The objective of the study was to describe the epidemiology of the bone pain crisis of sickle cell disease in Gujarat and explore the relationship with infection by Plasmodium vivax. Materials and Methods: This was a prospective review of all admissions in patients with sickle cell disease to a private pediatric institution in Bardoli, Gujarat, in the year 2015. Hemoglobin electrophoresis of all patients was consistent with homozygous sickle cell disease, but family studies indicated that at least seven cases had the severe sickle cell-beta + thalassemia presumed to be the common IVS1-5G>C mutation. Clinical, hematological, and parasitological features were recorded. Results: There were 914 admissions among 654 patients who had between one and seven admissions. The bone pain crisis accounted for 763 (83%) of admissions and increased between July and October coinciding with the monsoon period. Blood smears were examined for malarial parasites in 811 admissions and were positive for P. vivax in 73% patients. There was no evidence that P. vivax infections varied with the cause of admission or increased during the monsoon period. Conclusions: There was a high prevalence of P. vivax infection in hospital admissions of sickle cell patients, but the data did not support an etiological role in the bone pain crisis. A trial of malarial prophylaxis might determine its effect on the clinical features and outcome of sickle cell disease.

Background and Objectives: Inflammatory markers that influence bone pain crisis (BPC) and other complications of sickle cell anaemia (SCA) are numerous and play various roles. This study determined the plasma levels of tumour necrosis factor (TNF) - α, interleukin - 8 (IL-8), and endothelin - 1 (ET-1) in adult SCA patients during BPC and in steady state. In addition, the plasma levels of these cytokines were correlated with the severity of BPC of the patients. Methods and Materials: Sixty adult SCA patients (30 during BPC and 30 during steady state) and 30 haemoglobin A controls were enrolled for this cross-sectional study. The severity of BPC was assessed clinically, and questionnaires were filled. Plasma levels of TNF-α, IL-8 and ET-1 were quantified by ELISA, and haematological parameters were determined using a 5-part autoanalyzer. Plasma levels were correlated with the severity of bone pain crisis. Results were considered statistically significant if p<0.05. Results: Plasma TNF-α, IL-8, and ET-1 were significantly elevated in the BPC group than in the steady state group and the controls. Plasma TNF-α, IL-8 and ET-1 were markedly higher in the severe BPC groups than the steady state and control groups, There was a positive correlation between TNF-α and ET-1 in the bone pain crisis group. Conclusion: Elevated levels of plasma TNF-α, IL-8, and ET-1 further establish the chronic inflammatory state in SCA and equally affirm their significant contribution, not only to pathogenesis but also to the severity of pain in SCA. [ABSTRACT FROM AUTHOR]

Background: Sickle cell disease is a common problem across central India, but its clinical features may differ from that in African populations. There is a need to define the features of sickle cell disease in India, and the current study addresses some features of the bone pain crisis. Objectives: The objective of the study was to describe the epidemiology of the bone pain crisis of sickle cell disease in Gujarat and explore the relationship with infection by Plasmodium vivax. Materials and Methods: This was a prospective review of all admissions in patients with sickle cell disease to a private pediatric institution in Bardoli, Gujarat, in the year 2015. Hemoglobin electrophoresis of all patients was consistent with homozygous sickle cell disease, but family studies indicated that at least seven cases had the severe sickle cell-beta + thalassemia presumed to be the common IVS1-5G>C mutation. Clinical, hematological, and parasitological features were recorded. Results: There were 914 admissions among 654 patients who had between one and seven admissions. The bone pain crisis accounted for 763 (83%) of admissions and increased between July and October coinciding with the monsoon period. Blood smears were examined for malarial parasites in 811 admissions and were positive for P. vivax in 73% patients. There was no evidence that P. vivax infections varied with the cause of admission or increased during the monsoon period. Conclusions: There was a high prevalence of P. vivax infection in hospital admissions of sickle cell patients, but the data did not support an etiological role in the bone pain crisis. A trial of malarial prophylaxis might determine its effect on the clinical features and outcome of sickle cell disease. [ABSTRACT FROM AUTHOR]