Comparative study of the growth and nutritional status of Brazilian and Nigerian school-aged children with sickle cell disease

Comparative study of the growth and nutritional status of Brazilian and Nigerian school-aged children with sickle cell disease Samuel A. Adegokea,b,*, Maria S. Figueiredoa, Adekunle D. Adekilec and Josefina A.P. Bragad a *Corresponding author: Tel: +2348–35037560; E-mail: Received 13 January 2017; revised 4 May 2017; editorial decision 11 August 2017; accepted 31 August 2017 Background: Comparative studies of patients in different sociogeographic/ecological zones may unravel potential environmental and nutritional factors influencing disease phenotype. In sickle cell disease (SCD), differential access to comprehensive care may influence their growth and nutritional status. Methods: From June 2015 to February 2016, steady-state nutritional parameters of 109 Brazilian and 95 Nigerian children with SCD attending routine clinic visits at Universidade Federal de São Paulo, Brazil and Obafemi Awolowo University Teaching Hospital, Ile-Ife (Ilesa unit), respectively, were compared. Results: A relatively high proportion of the children in both centres (23.5%) were wasted [body-mass index (BMI)-for-age z-score<−2). BMI-for-age z-score, height-for-age z-score, upper arm fat area and fat percentage were lower in the Nigerian cohorts. More Nigerians, 29.5% (28/95) against 18.3% (20/109) were wasted, and had short stature, [12.6% (12/95) vs. 3.7% (4/109)] than Brazilians. A higher proportion of Brazilian patients were overweight or obese (9.2 vs. 4.3%), and taller for age (15.6 vs. 8.4%). None of the Nigerian patients had severe vitamin D deficiency, only 12.6% (12/95) had suboptimal vitamin D and 1.1% (1/95) had low serum zinc levels, unlike 79.8% (87/109) of the Brazilian patients with suboptimal vitamin D and 10.1% (11/109) with low zinc. Conclusion: Undernutrition is still prevalent among the two cohorts. Nigerian patients were thinner and had reduced linear growth for age. This observation justifies the continued need for specialized nutritional care for children with SCD. In addition to hydroxyurea therapy, research is needed to determine appropriate nutritional intervention and exercise regimens for these children. Keywords: Brazilian, Children, Growth, Nigerian, Nutritional status, Sickle cell disease Introduction SCD is the most common haematological hereditary disease in Brazil and Nigeria.1,2 It is estimated that about 4% (2–8%) of the world population carry an abnormal haemoglobin gene, with sickle cell anaemia (SCA) being the most common form of haemoglobinopathy.3 Approximately 20–25 million people live with SCA worldwide, out of which about 12–15 million reside in sub-Saharan Africa, and annually about 300 000 children with the gene are born globally.4 The disease affects people across the globe particularly sub-Saharan Africa, India, Mediterranean and Southern European countries.5 Ancient and recent population migrations have also carried the disease to the Caribbean, South and North America, and Northern Europe.5 Reduced growth and delayed development are common in children with SCD to the extent that some researchers are advocating the use of modified growth charts when monitoring their nutritional state.6 Usually, these patients experience a progressive decrease in growth velocity up to adolescence. In addition, they have a delay in bone maturation, epiphysis fusion during puberty and sexual development.5 Micronutrients, such as serum zinc, 25-hydroxyvitamin D (25-OHD), selenium and retinol, are suboptimal in SCD due to reduced appetite and poor dietary intake, increased energy and metabolic requirements, and reduced physical activity.7,8 SCD-directed therapies, such as hydroxyurea (HU) and chronic blood transfusion have been © The Author(s) 2017. Published by Oxford University Press on behalf of Royal Society of Tropical Medicine and Hygiene. All rights reserved. For permissions, please e-mail: . 327 Haematology and Blood Transfusion Division, Escola Paulista de Medicina, Universidade, Federal de São Paulo, Brazil; bDepartment of Pediatrics and Child Health, Obafemi Awolowo University, Ile-Ife, Nigeria; cDepartment of Paediatrics, Faculty of Medicine, Kuwait University, Kuwait; dDepartment of Pediatrics, Escola Paulista de Medicina, Universidade Federal de São Paulo, Brazil ORIGINAL ARTICLE Int Health 2017; 9: 327–334 doi:10.1093/inthealth/ihx035 Advance Access publication 28 September 2017 S. A. Adegoke et al. Methods This is a descriptive cross-sectional study in which demographic, clinical, nutritional and laboratory data of paediatric patients with SCD in steady state were collected. Study location The study was carried out at the paediatric haematology clinics of the Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), Brazil and Obafemi Awolowo University Teaching Hospital (OAUTH), Ilesa Unit, Nigeria. Nigeria, the largest country in West Africa with an estimated population of about 170 million people, is located on latitude 10°N of equator and longitude 8°E of Greenwich Meridian. It has an abundance sunlight throughout the year. The Nigerian study participants are predominantly Yoruba, of Ife-Ijesa extraction, in the southwestern part of the country. Ilesa, the study centre is an urban city and is located about 250 km north-east of Lagos, the former Nigerian capital. A typical Yoruba diet is high in carbohydrate. The paediatric haematology clinic of OAUTH runs weekly and sees an average of 25 patients/week. A total of about 400 children with SCD are registered in the clinic. The clinic offers daily chemoprophylaxis, such as folate and preventive antimalaria 328 treatment (proguanil). Hydroxyurea is prescribed only when indicated and the patient’s family can afford the cost. Although oral penicillin prophylaxis is not currently routinely prescribed, all children are offered Pneumococcal and Haemophilus influenza type B vaccination. Brazil, the largest country in South America is located on latitude 10°S and longitude 55°W of Equator. São Paulo city, like other cities in south-east Brazil, experiences a prolonged annual period of cold weather and reduced exposure to sunlight. The population is heterogenous comprising diverse ethnic groups, including native Latin Americans, African, Asian, Arabs, Jewish, North American and Europeans, particularly Italians. The paediatric haematology clinic of UNIFESP also runs every week. The clinic offers comprehensive SCD care, including hydroxyurea and chronic blood transfusion therapy, transcranial Doppler ultrasonography for stroke prevention, and daily folate chemoprophylaxis, oral penicillin, Pneumococcal and Haemophilus influenza type B vaccination are also routinely given. Patients The study included 109 Brazilian and 95 Nigerian children aged 4–11 years who had been diagnosed with SCA. They routinely attend the paediatric haematology clinics of the respective hospitals. All the study participants were in steady state at the time of recruitment, i.e. they had been free of crisis (pain (...truncated)