Advanced search
Publication Cover
Hematology Volume 28, 2023 - Issue 1
Submit an article Journal homepage
1,852
Views
0
CrossRef citations to date
0
Altmetric
Research Article

Clinical and biological profile of Sickle Cell Anemia children in a rural area in Central Africa

Gloire Mbayaboa Department of Pediatrics, University of Kinshasa, Kinshasa, Democratic Republic of the Congo;b Center for Human Genetics, KU Leuven and University Hospitals Leuven, Leuven, Belgium;d Center of Human Genetics, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of the CongoORCID Iconhttps://orcid.org/0000-0002-6887-4092View further author information
ORCID Icon,
Mamy Ngoleb Center for Human Genetics, KU Leuven and University Hospitals Leuven, Leuven, Belgium;c Department of Clinical Biology, University of Kinshasa, Kinshasa, Democratic Republic of the Congo;d Center of Human Genetics, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of the CongoORCID Iconhttps://orcid.org/0000-0002-1041-9257View further author information
ORCID Icon,
Paul Kabuyi Lumbalaa Department of Pediatrics, University of Kinshasa, Kinshasa, Democratic Republic of the Congo;b Center for Human Genetics, KU Leuven and University Hospitals Leuven, Leuven, Belgium;d Center of Human Genetics, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of the CongoORCID Iconhttps://orcid.org/0000-0003-2501-6765View further author information
ORCID Icon,
Aimé Lumakaa Department of Pediatrics, University of Kinshasa, Kinshasa, Democratic Republic of the Congo;d Center of Human Genetics, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of the Congo;e Service de Génétique, CHU de Liège, Liège, BelgiumORCID Iconhttps://orcid.org/0000-0002-5468-8678View further author information
ORCID Icon,
Valerie Raceb Center for Human Genetics, KU Leuven and University Hospitals Leuven, Leuven, BelgiumORCID Iconhttps://orcid.org/0000-0002-0012-0219View further author information
ORCID Icon,
Gert Matthijsb Center for Human Genetics, KU Leuven and University Hospitals Leuven, Leuven, BelgiumORCID Iconhttps://orcid.org/0000-0001-6710-1912View further author information
ORCID Icon,
Tite Minga Mikobid Center of Human Genetics, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of the Congo;f Département des sciences de base, Laboratory of biochemistry and molecular biology; Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of the CongoORCID Iconhttps://orcid.org/0000-0003-0809-3304View further author information
ORCID Icon,
Koenraad Devriendtb Center for Human Genetics, KU Leuven and University Hospitals Leuven, Leuven, BelgiumORCID Iconhttps://orcid.org/0000-0002-3651-2548View further author information
ORCID Icon,
Chris Van Geetg Department of Cardiovascular Sciences and Pediatrics (Hemato-oncology), KU Leuven and University Hospitals Leuven, Leuven, BelgiumORCID Iconhttps://orcid.org/0000-0003-1342-6265View further author information
ORCID Icon &
Prosper Tshilobo Lukusaa Department of Pediatrics, University of Kinshasa, Kinshasa, Democratic Republic of the Congo;b Center for Human Genetics, KU Leuven and University Hospitals Leuven, Leuven, Belgium;d Center of Human Genetics, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of the CongoCorrespondence[email protected]
ORCID Iconhttps://orcid.org/0000-0002-5414-2915View further author information
ORCID Icon show all
Article: 2193770 | Received 25 Aug 2022, Accepted 17 Mar 2023, Published online: 04 Apr 2023

References

  • Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018–2031. doi:10.1016/S0140-6736(10)61029-X.
  • Weatherall D, Akinyanju O, Fucharoen S, et al Chapter 34. inherited disorders of hemoglobin. In Disease control priorities in developing countries (2nd Ed). The International Bank for Reconstruction and Development / The World Bank 2006;663–680.
  • Agasa B, Bosunga K, Opara A, et al. Prevalence of sickle cell disease in a northeastern region of the Democratic Republic of Congo: What impact on transfusion policy? Transfus Med. 2010;20(1):62–65.
  • Tshilolo L, Kafando E, Sawadogo M, et al. Neonatal screening and clinical care programmes for sickle cell disorders in sub-Saharan Africa: Lessons from pilot studies. Public Health. 2008;122(9):933–941. doi:10.1016/j.puhe.2007.12.005.
  • Tshilolo L, Aissi LM, Lukusa D, et al. Neonatal screening for sickle cell anaemia in the Democratic Republic of the Congo: Experience from a pioneer project on 31 204 newborns. J Clin Pathol. 2009;62(1):35–38. doi:10.1136/jcp.2008.058958.
  • Grosse SD, Odame I, Atrash HK, et al. Sickle cell disease in Africa. Am J Prev Med. 2011;41(6):S398398–S39S405. doi:10.1016/j.amepre.2011.09.013.
  • Kumar AA, Chunda-Liyoka C, Hennek JW, et al. Evaluation of a density-based rapid diagnostic test for sickle cell disease in a clinical setting in Zambia. PLoS One. 2014;9(12):1–24.
  • Piel FB, Hay SI, Gupta S, et al. Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions. PLoS Med. 2013;10(7). e1001484. doi:10.1371/journal.pmed.100148.
  • Inusa BPD, Hsu LL, Kohli N, et al. Sickle cell disease—genetics, pathophysiology, clinical presentation and treatment. Int J Neonatal Screen. 2019;5(2):20. doi:10.3390/ijns5020020.
  • Ballas SK, Kesen MR, Goldberg MF, et al. Beyond the definitions of the phenotypic complications of sickle cell disease: An update on management. Sci World J. 2012;: 94953. doi:10.1100/2012/949535.
  • Makani J, Ofori-Acquah SF, Nnodu O, et al. Sickle cell disease: New opportunities and challenges in Africa. Sci World J. 2013: 1–16. doi:10.1155/2013/193252.
  • El-hazmi MAF. Clinical and haematological diversity of sickle cell disease in Saudi children. J Trop Pediatr. 1992;38(3):106–112. https://pubmed.ncbi.nlm.nih.gov/1380566/
  • Sebastiani P, Nolan VG, Baldwin CT, Abad-Grau MM, Wang L, Adewoye AH, et al. A network model to predict the risk of death in sickle cell disease. Blood. 2007;110(7):2727–2735. doi:10.1182/blood-2007-04-084921
  • Van Den Tweel XW, Van Der Lee JH, Heijboer H, et al. Development and validation of a pediatric severity index for sickle cell patients. Am J Hematol. 2010;85(10):746–751. doi:10.1002/ajh.21846.
  • Samuel AA, Peter Kuti B. J Appl Hematol. 2013;4:58–64.
  • Antwi-Boasiako C, Ekem I, Abdul-Rahman M, et al. Hematological parameters in Ghanaian sickle cell disease patients. J Blood Med. 2018;Volume 9:203–209. doi:10.2147/JBM.S169872.
  • Uche E, Adelekan O, Akinbami A, et al. Serum homocysteine and disease severity in sickle cell anemia patients in Lagos. J Blood Med. 2019;10:127–134. doi:10.2147/JBM.S198316.
  • Oladimeji OI, Adeodu OO, Onakpoya OH, et al. Prevalence of ocular abnormalities in relation to sickle cell disease severity among children in South-western, Nigeria. Eur J Ophthalmol. 2021;31(5):2659–2665. doi:10.1177/1120672120957615.
  • Ezenwosu O, Chukwu B, Ezenwosu I, et al. Clinical depression in children and adolescents with sickle cell anaemia: influencing factors in a resource-limited setting. BMC Pediatr. 2021;21(1):1–8. doi:10.1186/S12887-020-02457-3.
  • Okocha CE, Manafa PO, Igwe CN, et al. Adiponectin and disease severity in sickle cell anemia patients attending a tertiary health institution in Nnewi, Southeast Nigeria. Front Genet. 2022;13(February):1–5.
  • Hyacinth HI, Gee BE, Hibbert JM. The role of nutrition in sickle cell disease. Nutr Metab Insights. 2010;3:NMI.S5048.
  • Reid M. Nutrition and sickle cell disease. Comptes Rendus Biol. 2013;336(3):159–163. doi:10.1016/j.crvi.2012.09.007.
  • Mandese V, Marotti F, Bedetti L, et al. Effects of nutritional intake on disease severity in children with sickle cell disease. Nutr J. 2016;15(1):1–6. doi:10.1186/s12937-016-0159-8.
  • Mikobi TM, Lukusa Tshilobo P, Aloni MN, et al. Clinical phenotypes and the biological parameters of Congolese patients suffering from sickle cell anemia: A first report from Central Africa. J Clin Lab Anal. 2017;31(6):1–6.
  • Carrocini GC de S, Zamaro PJA, Bonini-Domingos CR. What influences Hb fetal production in adulthood? Rev Bras Hematol Hemoter. 2011;33(3):231–236. doi:10.5581/1516-8484.20110059.
  • Dover GJ, Smith KD, Chang YC, et al. Fetal hemoglobin levels in sickle cell disease and normal individuals are partially controlled by an X-linked gene located at Xp22.2. Blood. 1992;80(3):816–824. doi:10.1182/blood.V80.3.816.816.
  • Green NS, Ender KL, Pashankar F, et al. Candidate sequence variants and fetal hemoglobin in children with sickle cell disease treated with hydroxyurea. PLoS One. 2013;8(2):1–7.
  • Garner C, Mitchell J, Hatzis T, et al. Haplotype mapping of a major quantitative-trait locus for fetal hemoglobin production, on chromosome 6q23. Am J Hum Genet. 1998;62(6):1468–1474. doi:10.1086/301859.
  • Fogarty H, Gaul A, Syed S, Aleksejenko N, Geoghegan R, Conroy H, et al. Adherence to hydroxyurea, health-related quality of life domains and attitudes towards a smartphone app among Irish adolescents and young adults with sickle cell disease. Ir J Med Sci 2022;191(2):809–816. doi:10.1007/s11845-021-02588-1
  • Badawy SM, Thompson AA, Penedo FJ, et al. Barriers to hydroxyurea adherence and health-related quality of life in adolescents and young adults with sickle cell disease. Eur J Haematol. 2017;98(6):608–614. doi:10.1111/ejh.12878.

Related research

People also read lists articles that other readers of this article have read.

Recommended articles lists articles that we recommend and is powered by our AI driven recommendation engine.

Cited by lists all citing articles based on Crossref citations.
Articles with the Crossref icon will open in a new tab.