Comparative Analysis of Haematological, Anthropometric and Puberty Indices in Sickle-β-Thalassemic Variants of Tertiary Care Hospital of Odisha

Abstract

During neonatal period, the most common genetic disorder is haemoglobinopathy which leads to sickle cell disease or thalassemia. In the present study, we enumerate the haematological, anthropometric and puberty indices of three different variants of sickle-β-thalassemia such as HbS-β0-thalassemic, HbS-β+-thalassemic and HbS-β++-thalassemic with respect to severity of mutation of tertiary care hospital of Odisha. Standardised procedures were followed to analyse haematological and anthropometric parameters. For analysing puberty indices, Tanner staging chart was used. In the result, it is observed that there was a significant decrease in haematological parameter in the HbF, HbA2 (%) and HbS. Delay in growth (height for age and weight for age) also fluctuates according to the variant of HbS-β-thalassemia inherited by the participant. However, when puberty indices were analysed, significant delay of sexual maturation in stage-I scale in Tanner sexual maturity index chart was observed. Hence, out of the three variants, less growth was seen in the male and female participants of HbS-β0 thalassemia, decrease in BMI, thin frame size, decrease in body size, decrease growth of head and chest circumference than normal individual due to the marrow hyperplasia occurred in β-mutation in the locus control region of HBB gene.

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Acknowledgements

The authors express their gratefulness to Postgraduate Department of Zoology, Utkal University, Vani Vihar, Bhubaneswar-751 004, for providing laboratory facilities and Department of Hematology, Sri Ram Chandra Bhanja Medical college and hospital, Cuttack-753 007, for providing samples and patient data by following all medical regulations.

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Correspondence to Sonali Dutta.

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The authors declare that they have no conflict of interest.

Ethics Approval

This study confirms the ethical principles of medical research developed by World Medical Association, Declaration by Helsinki (1999) [28]. Ethical clearance was given by the Committee on Human Research Publication and Ethics (CHRPE) of the school of medical sciences and Institutional Ethical Committee of SCB Medical College, Cuttack, to undertake the investigation in Department of Clinical Hematology and Department of Paediatrics, SCB Medical College, Cuttack vide reference number IEC/IRB No. 824/11.03.2019. This study also received approval from Human Ethics Committee, Utkal University, Vani Vihar, Bhubaneswar, Odisha. All participants signed an informed consent form in accordance with the CHRPE and HEC regulations before participating in the study.

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Significance Statement The comparative analysis of haematological, anthropometric and puberty indices in sickle-β-thalassemic variants will useful for health assessment of the patients and also provide a baseline or future research in Odisha.

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Dutta, S., Mohanty, P.K., Kar, B. et al. Comparative Analysis of Haematological, Anthropometric and Puberty Indices in Sickle-β-Thalassemic Variants of Tertiary Care Hospital of Odisha. Proc. Natl. Acad. Sci., India, Sect. B Biol. Sci. (2021). https://doi.org/10.1007/s40011-020-01209-8

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Keywords

  • HbS-β-thalassemia
  • BMI
  • Puberty indices
  • Tanner staging
  • Haematology