Glucose-6-Phosphate Dehydrogenase Deficiency in Children with Cleft Lip/Palate Attending Multidisciplinary Cleft Clinic at a Teaching Hospital in Ghana
Background: Cleft lip and palate are among the most common orofacial congenital anomalies worldwide known to be caused by both genetic and environmental factors. Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is an X-linked disease that affects around 400 million people globally and leads to haemolytic crisis. The main complication of these cleft surgeries is bleeding which, in a G6PD deficient child, could be prolonged and could contribute to an increase in other complications among the affected children. Aim: The aim of the study was to determine the prevalence of G6PD deficiency and its association with haemoglobin levels among children with cleft lip and palate attending the multidisciplinary cleft clinic at the Komfo Anokye Teaching Hospital in Kumasi, Ghana. Method: A cross-sectional study of consecutively selected patients attending the multidisciplinary cleft clinic at the Komfo Anokye Teaching Hospital from February-May 2025 was conducted. Consented participants’ socio-demographic and clinical information were collected. A venous blood sample (3 ml) was collected based on standard procedures. G6PD enzyme activity and complete blood count were measured using the Methemoglobin reduction test and a fully automated haematology analyzer respectively. Logistic regression and their odds ratio were used to determine the association between haemoglobin (Hb) level and G6PD deficiency. Results: A total of ninety-one (91) participants were enrolled for the study, comprising 51 (56.0%) males and 40 (44.0%) females. A higher proportion (27.5%) presented with Veau III (cleft lip and palate), 22.0% presented with Veau II (cleft palate) and 17.6% with Veau IV (bilateral cleft lip and palate). Almost 61% of the participants were anaemic with nearly 29% having mild and moderate anaemia respectively. About 44% in the 6 - 59-month-old group had mild anaemia whilst a quarter had moderate anaemia. 12.1% (95% CI = 6.2% - 20.6%) had Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency. A higher proportion of males had G6PD deficiency compared to females (OR = 4.07; 95% CI = 0.83 - 20.04). There was no statistically significant association between G6PD deficiency and anaemia [OR = 1.17 (95% CI = 0.32 - 4.31)]. Conclusion: Glucose-6-phosphate dehydrogenase deficiency was found in 12.1% of children with cleft lip and palate. Association between G6PD deficiency and anaemia was not statistically significant.
References
[1]
Richardson, S.R. and Malley, G.F.O. (2022) G6PD Deficiency. StatPearls. https://www.ncbi.nlm.nih.gov/books/NBK470315/
[2]
Luzzatto, L. and Seneca, E. (2013) G6pd Deficiency: A Classic Example of Pharmacogenetics with On-Going Clinical Implications. BritishJournalofHaematology, 164, 469-480. https://doi.org/10.1111/bjh.12665
[3]
Lee, J., Kim, T.I., Kang, J., Jun, H., Lê, H.G., Thái, T.L., et al. (2018) Prevalence of Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency among Malaria Patients in Upper Myanmar. BMC Infectious Diseases, 18, Article No. 131. https://doi.org/10.1186/s12879-018-3031-y
[4]
Nkhoma, E.T., Poole, C., Vannappagari, V., Hall, S.A. and Beutler, E. (2009) The Global Prevalence of Glucose-6-Phosphate Dehydrogenase Deficiency: A Systematic Review and Meta-Analysis. Blood Cells, Molecules, and Diseases, 42, 267-278. https://doi.org/10.1016/j.bcmd.2008.12.005
[5]
Wajcman, H. and Galactéros, F. (2004) Le déficit en glucose-6 phosphate déshydro-génase: Protection contre le paludisme et risque d’accidents hémolytiques. Comptes Rendus. Biologies, 327, 711-720. https://doi.org/10.1016/j.crvi.2004.07.010
[6]
Amoah, L.E., Opong, A., Ayanful-Torgby, R., Abankwa, J. and Acquah, F.K. (2016) Prevalence of G6PD Deficiency and Plasmodium falciparum Parasites in Asymptomatic School Children Living in Southern Ghana. Malaria Journal, 15, Article No. 388. https://doi.org/10.1186/s12936-016-1440-1
[7]
Nguetse, C.N., Meyer, C.G., Adegnika, A.A., Agbenyega, T., Ogutu, B.R., Kremsner, P.G., et al. (2016) Glucose-6-Phosphate Dehydrogenase Deficiency and Reduced Haemoglobin Levels in African Children with Severe Malaria. MalariaJournal, 15, Article No. 346. https://doi.org/10.1186/s12936-016-1396-1
[8]
Agbenorku, P., Yore, M., Danso, K.A. and Turpin, C. (2013) Incidence of Orofacial Clefts in Kumasi, Ghana. ISRNPlasticSurgery, 2013, Article ID: 280903. https://doi.org/10.5402/2013/280903
[9]
Schutte, B.C. (1999) The Many Faces and Factors of Orofacial Clefts. Human MolecularGenetics, 8, 1853-1859. https://doi.org/10.1093/hmg/8.10.1853
[10]
Adigwe, O.P., Onoja, S.O. and Onavbavba, G. (2023) A Critical Review of Sickle Cell Disease Burden and Challenges in Sub-Saharan Africa. JournalofBloodMedicine, 14, 367-376. https://doi.org/10.2147/jbm.s406196
[11]
Sims, A.M., Bonsu, K.O., Urbonya, R., Farooq, F., Tavernier, F., Yamamoto, M., et al. (2021) Diagnosis Patterns of Sickle Cell Disease in Ghana: A Secondary Analysis. BMCPublicHealth, 21, Article No. 1719. https://doi.org/10.1186/s12889-021-11794-6
[12]
Sharma, U., Mishra, S., Gautam, N. and Gupta, B.K. (2020) Qualitative and Quantitative Assay of Glucose 6 Phosphate Dehydrogenase in Patients Attending Tertiary Care Center. BMCResearchNotes, 13, Article No. 298. https://doi.org/10.1186/s13104-020-05145-8
Calihan, J. (2021). Haematology. In: Kleinman, K., McDaniel, L. and Molly, M., Eds., The Harriet Lane Handbook, 22nd Edition, Elsevier, 361.
[15]
World Health Organization (2024) Guideline on Haemoglobin Cutoffs to Define Anaemia in Individuals and Populations. Guideline Development Process. https://www.ncbi.nlm.nih.gov/books/NBK602202/
[16]
Adekunle, A., James, O., Adamson, O., Agbogidi, O., Adeyemo, W., Butali, A., et al. (2020) Management of Orofacial Cleft in Nigeria—A Retrospective Study. AnnalsofMaxillofacialSurgery, 10, 434-438. https://doi.org/10.4103/ams.ams_104_20
[17]
Tettamanti, L. (2017) Cleft Palate Only: Current Concepts. Oral&Implantology, 10, 45-52. https://doi.org/10.11138/orl/2017.10.1.045
[18]
Lasma Azaria, Y. (2023) Management in Cleft Lip and Palate Surgery: A Systematic Review. JournalofAdvanceResearchinMedical&HealthScience, 9, 28-33. https://doi.org/10.53555/nnmhs.v9i4.1646
[19]
Tadesse, S.E., Zerga, A.A., Mekonnen, T.C., Tadesse, A.W., Hussien, F.M., Feleke, Y.W., et al. (2022) Burden and Determinants of Anemia among Under-Five Children in Africa: Systematic Review and Meta-Analysis. Anemia, 2022, Article ID: 1382940. https://doi.org/10.1155/2022/1382940
[20]
Miller, C. (2011) Feeding Issues and Interventions in Infants and Children with Clefts and Craniofacial Syndromes. Seminars in Speech and Language, 32, 115-126. https://doi.org/10.1055/s-0031-1277714
[21]
de Vries, I.A.C., Breugem, C.C., van der Heul, A.M.B., Eijkemans, M.J.C., Kon, M. and van der Molen, A.B.M. (2013) Prevalence of Feeding Disorders in Children with Cleft Palate Only: A Retrospective Study. Clinical Oral Investigations, 18, 1507-1515. https://doi.org/10.1007/s00784-013-1117-x
[22]
Motshoge, T., Ababio, G., Aleksenko, L., Souda, S., Muthoga, C.W., Mutukwa, N., et al. (2018) Prevalence of G6PD Deficiency and Associated Haematological Parameters in Children from Botswana. Infection, GeneticsandEvolution, 63, 73-78. https://doi.org/10.1016/j.meegid.2018.05.014
[23]
Ettinger, R.E. and Buchman, S.R. (1990) Cleft Lip and Palate: Embryology, Principles and Treatment. In: Grabb, W.C., Smith, J.W. and Aston, S.J., Eds., GrabbandSmith’sPlasticSurgery (8th Edition), Lippincott Williams and Wilkins, 835.
[24]
da Rocha, J.E.B., Othman, H., Tiemessen, C.T., Botha, G., Ramsay, M., Masimirembwa, C., et al. (2021) G6PD Distribution in Sub-Saharan Africa and Potential Risks of Using Chloroquine/Hydroxychloroquine Based Treatments for Covid-19. The Pharmacogenomics Journal, 21, 649-656. https://doi.org/10.1038/s41397-021-00242-8
[25]
Amoah, L.E., Asare, K.K., Dickson, D., Abankwa, J., Busayo, A., Bredu, D., et al. (2021) Genotypic Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Protects against Plasmodium falciparum Infection in Individuals Living in Ghana. PLOS ONE, 16, e0257562. https://doi.org/10.1371/journal.pone.0257562
[26]
Goi, T., Shionoya, Y., Sunada, K. and Nakamura, K. (2019) General Anesthesia in a Glucose-6-Phosphate Dehydrogenase Deficiency Child: A Case Report. AnesthesiaProgress, 66, 94-96. https://doi.org/10.2344/anpr-66-01-05
[27]
Cicvarić, A., Glavaš Tahtler, J., Vukoja Vukušić, T., Bekavac, I. and Kvolik, S. (2022) Management of Anesthesia and Perioperative Procedures in a Child with Glucose-6-Phosphate Dehydrogenase Deficiency. JournalofClinicalMedicine, 11, Article 6476. https://doi.org/10.3390/jcm11216476
[28]
Valiaveedan, S., Mahajan, C., Rath, G.P., Bindra, A. and Marda, M.K. (2011) Anaesthetic Management in Patients with Glucose-6-Phosphate Dehydrogenase Deficiency Undergoing Neurosurgical Procedures. IndianJournalofAnaesthesia, 55, 68-70. https://doi.org/10.4103/0019-5049.76597
[29]
Elyassi, C.A.R. and Rowshan, M.H.H. (2009) Perioperative Management of the Glucose-6-Phosphate Dehydrogenase Deficient Patient: A Review of Literature. AnesthesiaProgress, 56, 86-91. https://doi.org/10.2344/0003-3006-56.3.86