Skip to content
Licensed Unlicensed Requires Authentication Published by De Gruyter July 19, 2021

The effect of sickle cell anemia on the linear growth of Nigerian children

Ugo Nnenna Chikani ORCID logo, Adaobi Bisi-Onyemaechi, Ijeoma Ohuche, Justus Onu ORCID logo, Shalewa Ugege, Chinwe Ogugua, Ngozi Mbanefo, Paschal Chime and Ifeoma Emodi

Abstract

Objectives

Despite the high prevalence of children with sickle cell anaemia (SCA) in West Africa, there is paucity of data on the height velocity and prevalence of growth failure in SCA patients. With advances in clinical care of SCA patients, could there be a spatial and secular trend in the growth pattern of these children? Hence, the compelling needs to embark on this study. The objectives of the study were to determine the prevalence of growth failure among patients with SCA and its correlation with age, gender and age at diagnosis.

Methods

A Prospective longitudinal study of a cohort of sickle cell anaemic paediatric patients from Pediatrics SCA Clinic, University of Nigeria Teaching Hospital, Ituku Ozalla. Patients were enrolled over a period of two years using a non-parametric convenient sampling method. Their heights were measured at baseline, three months, six months and at 12 months intervals and subsequently plotted on a standard WHO growth chart. The height velocities at different monthly intervals were calculated and compared with the WHO standard normal linear growth rates) for children (used as control) to identify those with GF. (i.e. <10th percentile). The main outcome measures were the mean height velocities at different months' intervals calculated and compared using the repeated measurement analysis of variance (ANOVA) and the Wilcoxon signed test.

Results

A cohort of 316 children aged 1–18 years with SCA was evaluated with a male preponderance of 161 (57.4%). The mean age and age at diagnosis were 11.04 ± 5.56 and 4.2 ± 1.7 years, respectively. The prevalence of growth failure and short stature was 84.7%. The burden of GF was highest among post-pubertal participants (94.1%). The most important predictor of growth velocity deficit was age (R2=0.045, standard β coefficient = −0.22, t=−03.51, p=0.001).

Conclusions

The study demonstrated high prevalence of growth failure in children and adolescents with SCA which intensified with advancement in age and older age at diagnosis.


Corresponding author: Dr Ugo Nnenna Chikani, MBBS, FMC Paed, FESPE, Senior Lecturer/Paediatric Endocrinologist, Department of Paediatrics, University of Nigeria Ituku Ozalla Campus, PMB 01129, Enugu 40001, Nigeria, E-mail:

Acknowledgments

I wish to thank the Almighty God, my mentor Prof Alan Rogol for reviewing the earlier version of the manuscript, and all participants/caregivers for immense help and cooperation.

  1. Research funding: None declared.

  2. Author contributions: All authors have accepted responsibility for the entire content of this manuscript and approved its submission.

  3. Competing interests: Authors state no conflict of interest.

  4. Informed consent: Written informed consent was obtained from parents/caregivers of participating children and assent from children older than or equal to seven years.

  5. Ethical approval: The Health and Ethics and Research Committee of the University of Nigeria Teaching Hospital approved the study.

References

1. Galadancia, N, Wudilb, BJ, Balogunc, TM, Ogunrinded, GO, Akinsuliee, A, Hasan-Hangaa, F, et al.. Current sickle cell disease management practices in Nigeria. Int Health 2014;6:23–8. https://doi.org/10.1093/inthealth/iht022.Search in Google Scholar

2. Fleming, AF, Storey, J, Molineaux, L, Iroko, EA, Attai, ED. Abnormal haemoglobins in the Sudan savanna of Nigeria. I. Prevalence of haemoglobins and relationships between sickle cell trait, malaria and survival. Ann Trop Med Parasitol 1979;73:161–72. https://doi.org/10.1080/00034983.1979.11687243.Search in Google Scholar

3. AL-Saqladi, A-WM, Cipolotti, R, Fijnvandaat, K. Growth and nutritional status of children with homozygous sickle cell disease. Ann Trop Paediatr 2008;28:165–89. https://doi.org/10.1179/146532808x335624.Search in Google Scholar

4. Nicol, LE, Allen, DB, Czernichow, P. Zeitler. Normal growth and growth disoders. In: Kappy, MS, Alten, DB, Geffner, ME, editors. Pediatric endocrinology, 5th ed. New York: Informal Healthcare; 2007:108–29 pp.Search in Google Scholar

5. WHO Multicentre Growth Reference Study Group. WHO child growth standards: growth velocity based on weight, length and head circumference: methods and development. Geneva: World Health Organization; 2009:242 p. [Accessed 29 May 2020].Search in Google Scholar

6. Soliman, AT, El-Banna, N, Al-Salmi, I, De Silva, V, Craig, A, Asfour, M. Growth hormone secretion and circulating insulin-like growth factor-I (IGF-I) and IGF binding protein-3 concentrations in children with sickle cell disease. Metabolism 1997;46:1241–5. https://doi.org/10.1016/s0026-0495(97)90224-9.Search in Google Scholar

7. Soliman, AT, Darwish, A, Mohammed, SH, Bassiony, MR, el Banna, N, Asfour, M. Circulating growth hormone (GH), insulin-like growth factor-I (IGF-I) and free thyroxine, GH response to clonidine provocation and CT scanning of the hypothalamic-pituitary area in children with sickle cell disease. J Trop Pediatr 1995;41:285–9. https://doi.org/10.1093/tropej/41.5.285.Search in Google Scholar

8. Collett-Solberg, PF1, Fleenor, D, Schultz, WH, Ware, RE. Short stature in children with sickle cell anemia correlates with alterations in the IGF-I axis. J Pediatr Endocrinol Metab 2007;20:211–8. https://doi.org/10.1515/jpem.2007.20.2.211.Search in Google Scholar

9. Nunlee-Bland, G, Rana, SR, Houston-Yu, PE, Odonkor, W. Growth hormone deficiency in patients with sickle cell disease and growth failure. J Pediatr Endocrinol Metab 2004;17:601–6. https://doi.org/10.1515/jpem.2004.17.4.601.Search in Google Scholar

10. Bennett, EL. Understanding growth failure in children with homozygous sickle cell disease. J Pediatr Oncol Nurs 2010;28:67–74. https://doi.org/10.1177/1043454210382421.Search in Google Scholar

11. Luporini, SM, Bendit, I, Manhani, R, Brrracco, OL, Manzella, L, Giannella-Neto, D. Growth hormone and insulin-like growth factor I axis and growth of children with different sickle cell anaemia haplotypes. J Pediatr Hematol Oncol 2001;23:357–63. https://doi.org/10.1097/00043426-200108000-00007.Search in Google Scholar

12. Thuilliez, V, Ditsambou, V, Mba, JR, Mba Meyo, S, Kitengue, J. Current aspects of sickle cell disease in children in Gabon. Arch Pediatr 1996;3:668–74. https://doi.org/10.1016/0929-693x(96)87087-4.Search in Google Scholar

13. Oredugba, FA, Savage, KO. Anthropometric finding in Nigerian children with sickle cell disease. Pediatr Dent 2002;24:321–5.Search in Google Scholar

14. Oyedeji, GA. The health, growth and educational performance of sickle cell disease children. East Afr Med J 1991;68:181–9.Search in Google Scholar

15. Modebe, O, Ifenu, SA. Growth retardation inhomozygous sickle cell disease: role of calorie intake and possible gender-related differences. Am J Hematol 1993;44:149–54. https://doi.org/10.1002/ajh.2830440302.Search in Google Scholar

16. Otite, O. Ethic pluralism and ethnicity in Nigeria. Niger J Democr 1990;2:35–6.Search in Google Scholar

17. Whitten, CF. Growth status of children with sickle cell anemia. Am J Dis Child 1961;102:355–64. https://doi.org/10.1001/archpedi.1961.02080010357009.Search in Google Scholar

18. Ashcroft, MT, Serjeant, GR, Desai, P. Heights, weights, and skeletal age of Jamaica adolescents with sickle cell anaemia. Arch Dis Child 1972;47:519–24. https://doi.org/10.1136/adc.47.254.519.Search in Google Scholar

19. Mann, JR. Sickle cell haemoglobinopathies in England. Arch Dis Child 1981;56:676–83. https://doi.org/10.1136/adc.56.9.676.Search in Google Scholar

20. Souza, NM, Tone, LG, Collares, EF, Souza, IM. Serum zinc levels in children with homoglobinopathy (sickle cell, beta-thalassemia, S-thalassemia). J Pediatr 1983;55:385–8.Search in Google Scholar

21. Williams, R, George, EO, Wang, W. Nutrition assessment in children with sickle cell disease. J Assoc Acad Minor Phys 1997;8:44–8.Search in Google Scholar

22. Henderson, RA, Saavedra, JM, Dover, GJ. Prevalence of impaired growth in children with homozygous sickle cell anaemia. Am J Med Sci 1994;307:405–7. https://doi.org/10.1097/00000441-199406000-00004.Search in Google Scholar

23. Telfer, P, Coen, P, Chakravorty, S, Wilkey, O, Evans, J, Newell, H, et al.. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica 2007;92:905–12. https://doi.org/10.3324/haematol.10937.Search in Google Scholar

24. Hankins, JS, Ware, RE, Rogers, ZR, Wynn, LW, Peter, AL, Scott, JP, et al.. Long-term hydroxyurea therapy for infants with sickle cell anaemia: the HUSOFT extension study. Blood 2005;106:2269–75. https://doi.org/10.1182/blood-2004-12-4973.Search in Google Scholar

25. Wang, WC, Morales, KH, Scher, CD, Styles, L, Olivieri, N, Adams, R, et al.. Effect of long-term transfusion on growth in children with sickle cell anaemia: results of the STOP trial. J Pediatr 2005;147:244–7. https://doi.org/10.1016/j.jpeds.2005.02.030.Search in Google Scholar

26. Perrine, RP, John, P, Pembrey, M, Perrine, S. Sickle cell disease in Saudi Arabs in early childhood. Arch Dis Child 1981;56:187–92. https://doi.org/10.1136/adc.56.3.187.Search in Google Scholar

27. Lowry, MF, Desai, P, Ashcroft, MT, Serjeant, BF, Serjeant, GR. Heights and weights of Jamaican children with homozygous sickle cell disease. Hum Biol 1977;49:429–36.Search in Google Scholar

28. Odetunde, O, Chinawa, JM, Achigbu, KI, Achigbu, EO. Body mass index and other anthropometric variables in children with sickle cell anaemia. Pak J Med Sci 2016;32:341–6. https://doi.org/10.12669/pjms.322.9046.Search in Google Scholar

29. Animasahun, BA, Temiye, EO, Ogunkunle, OO, Izuora, AN, Njokanma, OF. The influence of socioeconomic status on the hemoglobin level and anthropometry of sickle cell anemia patients in steady state at the Lagos University Teaching Hospital. Niger J Clin Pract 2011;14:422–7. https://doi.org/10.4103/1119-3077.91748.Search in Google Scholar

30. Kopp-Hoolihan, LE, van Loan, MD, Mentzer, WC, Heyman, MB. Elevated resting energy expenditure in adolescents with sickle cell anaemia. J Am Diet Assoc 1999;99:195–9. https://doi.org/10.1016/s0002-8223(99)00047-4.Search in Google Scholar

31. Djamila, G, Abdullahi, SU, Jabir, BW, Gambo, S. World Health Organization’s growth reference overestimates the prevalence of severe malnutrition in children with sickle cell anemia. Afr J Clin Med 2020;9:119.10.3390/jcm9010119Search in Google Scholar

32. Tanner, JM, Davis, SW. Clinical longitudinal standards for height and height velocity for North American children. J Paediatr 1985;107:317–29. https://doi.org/10.1016/s0022-3476(85)80501-1.Search in Google Scholar

33. Bozzola, M, Meazza, C, Preedy, VR, editors. Handbook of growth and growth monitoring. In: Preedy, VR, editor. Health and disease. Springer Science+Business Media, LLC; 2011:1795–9 pp.Search in Google Scholar

34. Kramer, MS, Rooks, Y, Washington, LA, Pearson, HA. Pre- and postnatal growth and development in sickle cell anaemia. J Pediatr 1980;96:857–60. https://doi.org/10.1016/s0022-3476(80)80557-9.Search in Google Scholar

35. Booker, CR, Scott, RB, Ferguson, AD. Studies insickle cell anaemia. Xxii. Clinical manifestations ofsickle cell anaemia during the first two years of life. Clin Pediatr 1964;3:111–5. https://doi.org/10.1177/000992286400300214.Search in Google Scholar

36. Thomas, PW, Singhal, A, Hemmings-Kelly, M, Serjeant, GR. Height and weight reference curves for homozygous sickle cell disease. Arch Dis Child 2000;82:204–8. https://doi.org/10.1136/adc.82.3.204.Search in Google Scholar

37. Stevens, MC, Maude, GH, Cupidore, L, Jackson, H, Hayes, RJ, Serjeant, GR. Prepubertal growth and skeletal maturation in children with sickle cell disease. Pediatrics 1986;78:124–32.10.1542/peds.78.1.124Search in Google Scholar

38. Ashcroft, MT, Serjeant, GR, Desai, P. Heights, weights, and skeletal age of Jamaica adolescents with sickle cell anaemia. Arch Dis Child 1972;47:519–24. https://doi.org/10.1136/adc.47.254.519.Search in Google Scholar

39. Zago, MA, Kerbauy, J, Souza, HM, Figueiredo, MS, Costa, FF, Cruz, SM, et al.. Growth and sexual maturation of Brazilian patients with sickle cell diseases. Trop Geogr Med 1992;44:317–21.Search in Google Scholar

40. Zemel, BS, Kawchak, DA, Ohene-Frempong, K, Schall, JI, Stallings, VA. Effects of delayed pubertal development, nutritional status, and disease severity on longitudinal patterns of growth failure in children with sickle cell disease. Pediatr Res 2007;61:607–13. https://doi.org/10.1203/pdr.0b013e318045bdca.Search in Google Scholar

41. Rhodes, M, Akohoue, SA, Shankar, SM, Fleming, I, An, A, Yu, C, et al.. Growth patterns in children with sickle cell anemia during puberty. Pediatr Blood Canc 2009;53:635–41. https://doi.org/10.1002/pbc.22137.Search in Google Scholar

42. Akodu, SO, Diaku-Akinwumi, IN, Njokanma, OF. Age at diagnosis of sickle cell anaemia in Lagos, Nigeria. Mediterr J Hematol Infect Dis 2013;5:e2013001. https://doi.org/10.4084/mjhid.2013.001.Search in Google Scholar

43. Chukwu, BF, Ezenwosu, OU, Eke, CB, Chinawa, JM, Ikefuna, AN, Emodi, IJ. What factors influence the age at diagnosis of sickle cell anemia in Enugu Nigeria? J Blood Disord Transfus 2014;5:233.Search in Google Scholar

44. Serjeant, GR. The natural history of sickle cell disease. Cold Spring Harb Perspect Med 2013;3:11783. https://doi.org/10.1101/cshperspect.a011783.Search in Google Scholar

45. Onukwuli, VO, Ikefuna, AN, Nwokocha, AR, Emodi, IJ, Eke, CB. Relationship between zinc levels and anthropometric indices among school-aged female children with sickle cell anemia in Enugu, Nigeria. Niger J Clin Pract 2017;20:1461–7. https://doi.org/10.4103/njcp.njcp_104_17.Search in Google Scholar

46. Heyman, MB, Vichinsky, E, Katz, R, Gaffield, B, Hurst, D, Castillo, R, et al.. Growth retardation in sickle-cell disease treated by nutritional support. Lancet 1985;1:903–6. https://doi.org/10.1016/s0140-6736(85)91677-0.Search in Google Scholar

Received: 2021-03-31
Accepted: 2021-06-20
Published Online: 2021-07-19
Published in Print: 2021-10-26

© 2021 Walter de Gruyter GmbH, Berlin/Boston