Bimal Prasad Jit1, Pradeep Kumar Mohanty2, Prasanta Purohit3, Kishalaya Das4, Siris Patel4, Satyabrata Meher4, Jyoti Ranjan Mohanty5, Shalini Sinha5, Rajendra Kumar Behera6, Padmalaya Das7. 1. School of Life Sciences, AIPH University, Bhubaneswar, Odisha, India; Sickle Cell Clinic and Molecular Biology Laboratory, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur, Odisha, India. 2. Sickle Cell Clinic and Molecular Biology Laboratory, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur, Odisha, India; Department of Medicine, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur, Odisha, India. 3. Sickle Cell Clinic and Molecular Biology Laboratory, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur, Odisha, India; Multidisciplinary Research Unit, Maharaja Krishna Chandra Gajapati Medical College, Berhampur, Odisha, India. 4. Sickle Cell Clinic and Molecular Biology Laboratory, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur, Odisha, India. 5. School of Life Sciences, AIPH University, Bhubaneswar, Odisha, India. 6. School of Life Sciences, Sambalpur University, Jyoti Vihar, Burla, Sambalpur, Odisha, India. 7. School of Life Sciences, AIPH University, Bhubaneswar, Odisha, India. Electronic address: pdas@aiph.ac.in.
Abstract
BACKGROUND: Sickle cell disease (SCD) is a Mendelian single gene disorder with highly variable phenotypic expression. In the present study, we analyzed the influence of HbF, alpha thalassemia and other hematological indices to determine their association with acute pain episodes. METHOD: This case control study consisted of SCD subjects with HbS phenotype experiencing three or more acute pain episodes in last twelve months (cases) and without any episode of acute pain during last twelve months (controls). Hematological parameters, HbF, and presence of alpha thalassemia were assessed in all subjects. RESULTS: A statistically significant difference between HbF levels (P < 0.025, χ2 test) and alpha thalassemia (P < 0.008, χ2 test) was observed between controls and cases group. Univariate analysis indicated that increased HbF levels > 25% (OR: 0.37, 95% CI: 0.18-0.77, P < 0.008) and presence of alpha thalassemia (OR: 0.53, 95% CI: 0.33-0.85, P < 0.009) provided protection, while multivariate analysis revealed significant protection was attributable only by higher HbF levels (OR: 0.39, 95% CI: 0.17-0.88, P < 0.025). Significantly higher HbF levels were observed only in the 11-20 age group of cases in comparison to controls (Student's t-test, P < 0.001). CONCLUSION: Higher concentrations of HbF are associated with protection against frequent episodes of acute pain crisis in SCD patients.
BACKGROUND:Sickle cell disease (SCD) is a Mendelian single gene disorder with highly variable phenotypic expression. In the present study, we analyzed the influence of HbF, alpha thalassemia and other hematological indices to determine their association with acute pain episodes. METHOD: This case control study consisted of SCD subjects with HbS phenotype experiencing three or more acute pain episodes in last twelve months (cases) and without any episode of acute pain during last twelve months (controls). Hematological parameters, HbF, and presence of alpha thalassemia were assessed in all subjects. RESULTS: A statistically significant difference between HbF levels (P < 0.025, χ2 test) and alpha thalassemia (P < 0.008, χ2 test) was observed between controls and cases group. Univariate analysis indicated that increased HbF levels > 25% (OR: 0.37, 95% CI: 0.18-0.77, P < 0.008) and presence of alpha thalassemia (OR: 0.53, 95% CI: 0.33-0.85, P < 0.009) provided protection, while multivariate analysis revealed significant protection was attributable only by higher HbF levels (OR: 0.39, 95% CI: 0.17-0.88, P < 0.025). Significantly higher HbF levels were observed only in the 11-20 age group of cases in comparison to controls (Student's t-test, P < 0.001). CONCLUSION: Higher concentrations of HbF are associated with protection against frequent episodes of acute pain crisis in SCDpatients.
Authors: Giulia Ceglie; Margherita Di Mauro; Isabella Tarissi De Jacobis; Francesca de Gennaro; Martina Quaranta; Carlo Baronci; Alberto Villani; Giuseppe Palumbo Journal: Front Mol Biosci Date: 2019-12-05