Skip to main content
SpringerLink
Log in

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

  • Research Article
  • Published:
Proceedings of the National Academy of Sciences, India Section B: Biological Sciences Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig.1
Fig. 2
Fig. 3
Fig.4
Fig.5
Fig. 6
Fig. 7
Fig. 8
Fig. 9

Similar content being viewed by others

References

  1. Cighetti G, Duca L, Bortone L et al (2002) Oxidative status and malondialdehyde in beta-thalassaemia patients. Eur J Clin Investig 32:55–60

    Article  CAS  Google Scholar 

  2. Zurlo MG, De Stefano P, Borgna-Pignatti C et al (1989) Survival and causes of death in thalassaemia major. Lancet 2:27–30

    Article  CAS  Google Scholar 

  3. Yeo HC, Helbock HJ, Chyu DW, Ames BN (1994) Assay of malondialdehyde in biological fluids by gas chromatography–mass spectrometry. Ana Biochem 220:391–396

    Article  CAS  Google Scholar 

  4. Weatherall D, Clegg J (2001) Inherited haemoglobin disorders: an increasing global health problem. Bull World Health Org 79 (8): 704–712.

    CAS  PubMed  Google Scholar 

  5. Steiner LA, Gallagher PG (2007) Erythrocyte disorders in the perinatal period. Semin Perinatol 31:254–261

    Article  Google Scholar 

  6. Adewoyin AS (2015) Management of sickle cell disease: a review for physician education in Nigeria (sub-saharan Africa). Anemia 2:1–21

    Article  Google Scholar 

  7. Kotila TR (2010) Guidelines for diagnosis of hemoglobinopathies in Nigeria. Ann Ib Postgrad Med 8:1

    Google Scholar 

  8. Wenning MR, Sonati MF (2007) Hemoglobinopatiashereditarias. In: Lopes AC (ed) Diagnostico e tratamento. Manole, Sao Paulo, pp 310–314

    Google Scholar 

  9. Balgir RS (2002) The genetic burden of hemoglobinopathies with special reference to community health in India and the challenges ahead. Indian J Hematol Blood Transfus 20:2–7

    Google Scholar 

  10. Balgir RS (2000) The burden of haemoglobinopathies in India and the challenges ahead. Curr Sci 79:1536–1547

    CAS  Google Scholar 

  11. Kulozik AE, Bail S, Kar BC, Serjeant BE, Serjeant GR (1991) Sickle cell-β+ thalassemia in Orissa state, India. Br J Haematol 77:215–220

    Article  CAS  Google Scholar 

  12. Yamane T (1967) Elementary sampling theory. Human hemoglobins. Ann Rev Genet 14:145–178

    Google Scholar 

  13. Beutler E (1990) The thalassaemias. In: Weatherall DJ (ed) Williams Haematology, 5th edn. McGraw-Hills, New York, pp 581–611

    Google Scholar 

  14. Akinbami A, Dosunmu A, Adediran A, Oshinaike O, Adebola P, Arogundade O (2012) Haematological values in homozygous sickle cell disease in steady state and haemoglobin phenotypes AA controls in Lagos. Nigeria BMC Res Notes 5(1):396–402

    Article  Google Scholar 

  15. Behera S, Bulliyya G (2016) Magnitude of anemia and hematological predictors among children under 12 years in Odisha, India. Anemia 23(3):165–169

    Google Scholar 

  16. Pauling L, Itano HA, Singer SJ, Wells IC (1949) Sickle cell anemia: a molecular disease. Science II:543–548

    Article  Google Scholar 

  17. Piplani S, Manan R, Lalit M, Manjari M, Bhasin T, Bawa J (2013) NESTROFT: a valuable, cost effective screening test for Beta thalassemia trait in north Indian Punjabi population. J Clin Diagnos Res 7(12):2784–2687

    Google Scholar 

  18. Drabkin DL, Austin JH (1935) Spectrophotometric studies II. Preparation from washed blood cells; nitric oxide hemoglobin and sulfhemoglobin. J Clin Pathol 15:44–45

    Google Scholar 

  19. National Institutes of Health (1998) Clinical guidelines on the identification, evaluation, and treatment of overweight and obesity in adults: the evidence report. Obes Res 6(2):51–209

    Google Scholar 

  20. Dine MS, Gartside PS, Glueck CJ, Rheins L, Greene G, Khoury P (1981) Relationship of head circumference to length in the first 400 days of life: a mnemonic. Paediatrics 67(4):506–507

    CAS  Google Scholar 

  21. Berkley J, Mwangi I, Griffiths K et al (2005) Assessment of severe malnutrition among hospitalized children in rural Kenya: comparison of weight for height and mid upper arm circumference. JAMA 294(5):591–597

    Article  CAS  Google Scholar 

  22. Boye KR, Dimitriou T, Manz F et al (2002) Anthropometric assessment of muscularity during growth: estimating fat-free mass with 2 skin fold-thickness measurements is superior to measuring mid upper arm muscle area in healthy prepubertal children. Am J Clin Nutr 76(3):628–632

    Article  CAS  Google Scholar 

  23. Briend A, Maire B, Fontaine O, Garenne M (2012) Mid upper arm circumference and weight for height to identify high risk malnourished under five children. Matern Child Nutr 8(1):130–133

    Article  Google Scholar 

  24. Goossens S, Bekele Y, Yun O, Harczi G, Ouannes M, Shepherd S (2012) Mid-upper arm circumference based nutrition programming: evidence for a new approach in regions with high burden of acute malnutrition. PLoS ONE 7(11):e49320

    Article  CAS  Google Scholar 

  25. World Health Organization (WHO) (1983) Community control of hereditary anaemia: memorandum from a WHO meeting. Bull World Health Org 61:63–80

    Google Scholar 

  26. Berenbaum SA, Beltz AM, Corley R (2015) The importance of puberty for adolescent development: conceptualization and measurement. Adv Child Dev Behav 48:53–92

    Article  Google Scholar 

  27. Dir AL, Hummer TA, Aalsma MC, Hulvershorn LA (2019) Pubertal influences on neural activation during risky decision-making in youth with ADHD and disruptive behaviour disorders. Dev Cogn Neurosci 36:100634 (1–19).

  28. World Medical Association Medical Ethics Committee (1999) Updating the WMA declaration of Helsinki. World Med J 45:11–13

    Google Scholar 

Download references

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.

Author information

Authors and Affiliations

  1. Department of Zoology, Utkal University, Vani Vihar, Bhubaneswar, Odisha, 751004, India

    Sonali Dutta & Prafulla K. Mohanty

  2. Department of Pharmacoloy, School of Pharmaceutical Sciences, SOA University, Bhubaneswar, 751030, India

    Biswakanth Kar

  3. Department of Clinical Hematology, S.C.B. Medical College and Hospital, Cuttack, Odisha, 753007, India

    Rajeeb K. Nayak

Authors
  1. Sonali Dutta
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Prafulla K. Mohanty
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Biswakanth Kar
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Rajeeb K. Nayak
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sonali Dutta.

Ethics declarations

Conflict of Interest

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.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

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. 91, 173–184 (2021). https://doi.org/10.1007/s40011-020-01209-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40011-020-01209-8

Keywords

Search

Navigation