Preprint / Version 2

Nutritional Status of Sickle Cell Patients: A Literature Review

##article.authors##

  • Minjoon Hur Raffles Christian School Pondok Indah
  • Boluwatiwi Durojaye

DOI:

https://doi.org/10.58445/rars.164

Keywords:

Sickle Cell Disease, Nutrient Deficiency, Vitamins, Minerals

Abstract

Sickle cell disease (SCD) is a lethal, life-long condition characterized by a mutation in the gene that codes for hemoglobin. To alleviate the pain experienced by sickle cell (SC) patients, adequate nutrition levels are vital as deficiencies of vitamins or minerals may cause other symptoms. The objective of this review is to outline the nutrient status of SC patients and propose research areas where further study is needed. This review summarizes twelve primary research papers that measured the level of vitamins A, C, D, E, B-2, B-6, B-12, folate, magnesium, iron, zinc, and copper in SC patients. The majority of SC patients had suboptimal vitamin A levels and were vitamin D deficient. Their zinc and vitamins B-6, C, and E levels were adequate, while their folate, copper, and iron levels were elevated. There were notable differences in some of the measurement methods used for the same nutrient levels. There is more research needed to find the optimal amount of vitamin A supplement for SC patients, and the effect of zinc supplementation on copper levels and the cause of low zinc levels in SC patients remains unexplored.

References

Kavanagh, P.L.; Fasipe, T.A.; Wun, T. Sickle Cell Disease: A review. JAMA. 2022, 328 (1), 57-68. DOI: 10.1001/jama.2022.10233

Tebbi, C.K. Sickle Cell Disease, a Review. Hemato. 2022, 3 (2), 341-366. DOI: 10.3390/hemato3020024

Xu, J.Z. and Thein, S.L. The carrier state for sickle cell disease is not completely harmless. Haematologica. 2019, 104 (6), 1106-1111. DOI: 10.3324/haematol.2018.206060

Lubeck, D.; Agodoa, I.; Bhakta, N.; et al. Estimated Life Expectancy and Income of Patients with Sickle Cell Disease Compared with Those Without Sickle Cell Disease. JAMA Network Open. 2019, 2 (11), e1915374. DOI: 10.1001/jamanetworkopen.2019.15374

Hyacinth, H.I.; Gee B.E.; Hibbert J.M. The Role of Nutrition in Sickle Cell Disease. Nutr. Metab. Insights. 2010, 3, 57-67. DOI: 10.4137/NMI.S5048

Koehler, K. and Drenowatz, C. Integrated Role of Nutrition and Physical Activity for Lifelong Health. Nutrients. 2019, 11 (7), 1437. DOI: 10.3390/nu11071437

Masood, W.; Annamaraju, P.; Uppaluri, K.R. Ketogenic Diet, In StatPearls [Internet], Year 2022 ed.; StatPearls Publishing, 2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK499830/

World Health Organization-Healthy diet. https://www.who.int/news-room/fact-sheets/detail/healthy-diet. Accessed on 19th January 2023

Hambidge, M. Human zinc deficiency. J. Nutr. 2000, 130 (5S Suppl), 1344S-9S. DOI: 10.1093/jn/130.5.1344S

Wasnik, R.R. and Akarte, N.R. Evaluation of Serum Zinc and Antioxidant Vitamins in Adolescent Homozygous Sickle Cell Patients in Wardha, District of Central India. J. Clin. Diagn. Res. 2017, 11 (8), BC01-BC03. DOI: 10.7860/JCDR/2017/30855.10320

Yokokawa, H.; Fukuda, H.; Saita, M.; et al. Serum zinc concentrations and characteristics of zinc deficiency/marginal deficiency among Japanese subjects. J. Gen. Fam. Med. 2020, 21 (6), 248-255. DOI: 10.1002/jgf2.377

Hasanato, R.M.W. Zinc and antioxidant vitamin deficiency in patients with severe sickle cell anemia. Ann. Saudi. Med. 2006, 26 (1), 17-21. DOI: 10.5144/0256-4947.2006.17

Dougherty, K.A.; Schall, J.I.; Kawchak, D.A.; et al. No Improvement in suboptimal vitamin A status with a randomized, double-blind, placebo-controlled trial of vitamin A supplementation in children with sickle cell disease. Am. J. Clin. Nutr. 2012, 96 (4), 932-940. DOI: 10.3945/ajcn.112.035725

World Health Organization-Vitamin A deficiency. https://www.who.int/data/nutrition/nlis/info/vitamin-a-deficiency. Accessed on 23rd December 2022

Rizvi, S.; Raza, S.T.; Ahmed, F.; et al. The Role of Vitamin E in Human Health and Some Diseases. Sultan Qaboos Univ. Med. J. 2014, 14 (2), e157-e165

Miyazawa, T.; Bureaus, G.C.; Itaya, M.; et al. Vitamin E: Regulatory Redox Interactions. IUBMB Life. 2019, 71 (4), 430-441. DOI: 10.1002/iub.2008

Traber, M.G. Vitamin E inadequacy in humans: causes and consequences. Adv. Nutr. 2014, 5 (5), 503-514. DOI: 10.3945/an.114.006254

National Institutes of Health-Vitamin A and Carotenoids. https://ods.od.nih.gov/factsheets/VitaminA-HealthProfessional/. Accessed on 13th February 2023

Carr, A.C. and Maggini, S. Vitamin C and Immune Function. Nutrients. 2017, 9 (11), 1211. DOI: 10.3390/nu9111211

McCall, S. J.; Clark, A. B.; Luben, R. N.; et al. Plasma Vitamin C and Functional Health: Results from the European Prospective Investigation into Cancer-Norfolk. Nutrients. 2019, 11 (7), 1552. DOI: 10.3390/nu11071552

Rowe, S.; Carr, A. C. Global Vitamin C Status and Prevalence of Deficiency: A Cause for Concern? Nutrients. 2020, 12 (7), 2008, DOI: 10.3390/nu12072008

Antwi-Boasiako, C.; Dankwah, G. B.; Aryee, R.; et al. Serum Iron Levels and Copper-to-Zinc Ratio in Sickle Cell Disease. Medicina. 2019, 55 (5), 180. DOI: 10.3390/medicina55050180

Kennelly, P.J.; Murray, R.K.; Jacob, M. Plasma Proteins and Immunoglobulins. Harper’s Illustrated Biochemistry, 30th ed.; Cenveo Publisher Services. 2011. Available from: https://accessmedicine.mhmedical.com/content.aspx?bookid=1366&sectionid=73247095#1106060414

Royer, A. and Sharman, T. Copper Toxicity. In StatPearls [Internet], Year 2022 ed.; StatPearls Publishing, 2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK557456/

Lee, H.C.; Chang, S.W. Vitamin D and health - The missing vitamin in humans. Pediatr. Neonatol. 2019, 60 (3), 237-244

AlJama, A.; AlKhalifah, M.; Al-Dabbous, I.A.; Alqudaihi, G. Vitamin D deficiency in sickle cell disease patients in the Eastern Province of Saudi Arabia. Ann. Saudi. Med. 2018, 38 (2), 130-136. DOI: 10.5144/0256-4947.2018.130

Williams, B.A.; Mayer, C.; McCartney, H.; et al. Detectable Unmetabolized Folic Acid and Elevated Folate Concentrations in Folic Acid-Supplemented Canadian Children With Sickle Cell Disease. Front. Nutr. 2021. DOI: 10.3389/2021/642306

Blaszczyk, U.; Duda-Chodak, A. Magnesium: its role in nutrition and carcinogenesis. Rocz. Panstw. Zakl. Hig. 2013, 64 (3), 165-71.

Rosanoff, A.; West, C.; Elin, R.J.; et al. Recommendation on an updated standardization of serum magnesium reference ranges. Eur. J. Nutr. 2022, 61 (7), 3697-3706. DOI: 10.1007/s00394-022-02916-w

De Franceschi, L.; Bachir, D.; Galacteros, F.; et al. Oral magnesium supplements reduce erythrocyte dehydration in patients with sickle cell disease. J. Clin. Invest. 1997, 100 (7), 1847-52. DOI: 10.1172/JCI119713

Arnaud, M.J. Update on the assessment of magnesium status. Br. J. Nutr. 2008, 99 (3S Suppl), S24-36. DOI: 10.1017/S000711450800682X

Antwi-Boasiako, C.; Kusi-Mensah, Y.A.; Hayfron-Benjamin, C.; et al. Total Serum Magnesium Levels and Calcium-To-Magnesium Ratio in Sickle Cell Disease. Medicina. 2019, 55 (9), 547. DOI: 10.3390/medicina55090547

Gröber, U.; Schmidt, J.; Kisters, K. Magnesium in Prevention and Therapy. Nutrients. 2015, 7 (9), 8199-226. DOI: 10.3390/nu7095388

Naigamwalla, D.Z.; Webb, J.A.; Giger, U. Iron deficiency anemia. Can. Vet. J. 2012, 53(3), 250-256.

Pagana, K.D.; Pagana, T.J.; Pagana, T.N. Mosby’s Diagnostic & Laboratory Test Reference, 14th ed.; Elsevier. 2019.

Sani, M.A.; Adewuyi, J.O.; Babatunde, A.S.; et al. The Iron Status of Sickle Cell Anaemia Patients in Ilorin, North Central Nigeria. Adv. Hematol. 2015. DOI: 10.1155/2015/386451.

Lanier, J.B.; Park, J.J.; Callahan, R.C. Anemia in Older Adults. Am. Fam. Physician. 2018, 98 (7), 437-442.

Lopez-Sall, P.; Diop, P.A.; Diagne, I.; et al. Transferrine soluble receptors' contribution to the assessment of iron status in homozygous drepanocytic anemia. Ann. Biol. Clin (Paris). 2004, 62 (4), 415-421.

Comstock, G.W.; Alberg, A.J.; Helzlsouer, K.J. Reported effects of long-term freezer storage on concentrations of retinol, beta-carotene, and alpha-tocopherol in serum or plasma summarized. Clin. Chem. 1993, 39 (6), 1075-1078.

Jargar, J.G.; Hattiwale, S.H.; Das, S.; et al. A modified simple method for determination of serum α-tocopherol (vitamin E). J. Basic Clin. Physiol. Pharmacol. 2012, 23 (1), 45-48. DOI: 10.3390/ma14133691

Olivares, M.; Méndez, M.A.; Astudillo, P.A.; et al. Present situation of biomarkers for copper status. Am. J. Clin. Nutr. 2008, 88 (3), 859S-862S. DOI: 10.1093/ajcn/88.3.859S

Sizar, O.; Khare, S.; Goyal, A.; et al. Vitamin D Deficiency. In StatPearls [Internet], Year 2022 ed.; StatPearls Publishing, 2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK532266/

Lieu, P.T.; Heiskala, M.; Peterson, P.A.; et al. The roles of iron in health and disease. Mol. Aspects Med. 2001, 22 (1-2), 1-87. DOI: 10.1016/s0098-2997(00)00006-6

Smith, O.S.; Ajose, O.A.; Adegoke, S.A.; et al. Plasma level of antioxidants is related to frequency of vaso-occlusive crises in children with sickle cell anaemia in steady state in Nigeria. Pediatric Hematology Oncology Journal. 2019, 4 (1) 17-22. DOI: 10.1016/j.phoj.2019.03.003

Emokpae, M.A. and Fatimehin, E.B. Copper-To-Zinc Ratio as an Inflammatory Marker in Patients with Sickle Cell Disease. Sci. 2020, 2 (4), 89. DOI: 10.3390/sci2040089

Tsang, B.; Sandalinas, F.; De-Regil, L.M.; Folate supplementation in women of reproductive age. Cochrane Database Syst Rev. 2015, 2015 (6), CD011766. DOI: 10.1002/14651858.CD011766

Bermejo, F. and García-López, S. A guide to diagnosis of iron deficiency and iron deficiency anemia in digestive diseases. World J Gastroenterol. 2009, 15 (37), 4638-4643. DOI: 10.3748/wjg.15.4638

Oustamanolakis, P.; Koutroubakis, I.E.; Messaritakis, I.; et al. Soluble transferrin receptor-ferritin index in the evaluation of anemia in inflammatory bowel disease: a case-control study. Ann. Gastroenterol. 2011, 24 (2), 108-114.

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2023-03-31 — Updated on 2024-05-07

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