Nutritional Status of Young Children with Inherited Blood Disorders in Western Kenya

Tsang, Becky L.; Sullivan, Kevin M.; Ruth, Laird J.; Williams, Thomas N.; Suchdev, Parminder S.

doi:10.4269/ajtmh.13-0496

Cited by 10 publications

(18 citation statements)

References 39 publications

(52 reference statements)

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“…In a previous trial in the same area as the present study, α + -thalassaemia occurred in 48.8% of pregnant women (heterozygotes: 41.3%; homozygotes: 7.5%), but there was no evidence that it was associated with ZPP [ 4 ]. In a nearby area, the prevalence of sickle cell trait and sickle cell disease in preschool children was 17.1% and 1.6%, respectively; genotypes indicating G6PD deficiency occurred in 8.2% of males and 6.8% of children overall, whilst the prevalence of haptoglobin 2–2 genotype was 20.4% [ 17 , 18 ] A study in Gambian children aged 2–6 years, however, failed to find an association between haptoglobin genotype and ZPP [ 19 ].…”

Section: Discussionmentioning

confidence: 99%

Diagnostic utility of zinc protoporphyrin to detect iron deficiency in Kenyan preschool children: a community-based survey

et al. 2017

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BackgroundZinc protoporphyrin (ZPP) has been used to screen and manage iron deficiency in individual children, but it has also been recommended to assess population iron status. The diagnostic utility of ZPP used in combination with haemoglobin concentration has not been evaluated in pre-school children. We aimed to a) identify factors associated with ZPP in children aged 12–36 months; b) assess the diagnostic performance and utility of ZPP, either alone or in combination with haemoglobin, to detect iron deficiency.MethodsWe used baseline data from 338 Kenyan children enrolled in a community-based randomised trial. To identify factors related to ZZP measured in whole blood or erythrocytes, we used bivariate and multiple linear regression analysis. To assess diagnostic performance, we excluded children with elevated plasma concentrations of C-reactive protein or α 1-acid glycoprotein, and with Plasmodium infection, and we analysed receiver operating characteristics (ROC) curves, with iron deficiency defined as plasma ferritin concentration < 12 μg/L. We also developed models to assess the diagnostic utility of ZPP and haemoglobin concentration when used to screen for iron deficiency.ResultsWhole blood ZPP and erythrocyte ZPP were independently associated with haemoglobin concentration, Plasmodium infection and plasma concentrations of soluble transferrin receptor, ferritin, and C-reactive protein. In children without inflammation or Plasmodium infection, the prevalence of true iron deficiency was 32.1%, compared to prevalence of 97.5% and 95.1% when assessed by whole blood ZPP and erythrocyte ZPP with conventional cut-off points (70 μmol/mol and 40 μmol/mol haem, respectively). Addition of whole blood ZPP or erythrocyte ZPP to haemoglobin concentration increased the area-under-the-ROC-curve (84.0%, p = 0.003, and 84.2%, p = 0.001, respectively, versus 62.7%). A diagnostic rule (0.038689 [haemoglobin concentration, g/L] + 0.00694 [whole blood ZPP, μmol/mol haem] >5.93120) correctly ruled out iron deficiency in 37.4%–53.7% of children screened, depending on the true prevalence, with both specificity and negative predictive value ≥90%.ConclusionsIn young children, whole blood ZPP and erythrocyte ZPP have added diagnostic value in detecting iron deficiency compared to haemoglobin concentration alone. A single diagnostic score based on haemoglobin concentration and whole blood ZPP can rule out iron deficiency in a substantial proportion of children screened.Trial registration ClinicalTrials.gov NCT02073149 (25 February 2014).Electronic supplementary materialThe online version of this article (doi:10.1186/s12878-017-0082-z) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

Diagnostic utility of zinc protoporphyrin to detect iron deficiency in Kenyan preschool children: a community-based survey

et al. 2017

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“…Information on SCD has mainly been reported among US population, India and Jamaica, with less information in Africa where the gene is highly predominant. Although some studies [6,7] have reported on nutritional status of children with SCD in parts of Africa, there has been paucity of studies designed to identify the dietary intake of children with SCD in Ghana. A study in Ghana [8] reported on high prevalence of malnutrition (61.3%) among children with SCD but did not consider their dietary intake.…”

Section: Introductionmentioning

confidence: 99%

Dietary intakes and nutritional status of children with sickle cell disease at the Princess Marie Louise Hospital, Accra – a survey

2018

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Background: Sickle cell disease (SCD) is a chronic genetic blood disorder common among people of African descent, which places nutritional burden among affected individuals. The aim of the study was to determine the dietary intake and nutritional status of children with SCD. Method: This was a cross sectional study that involved 120 children with SCD aged 3-12 years at the paediatric outpatient department of Princess Marie Louise Hospital (PML) in Accra. A semi-structured questionnaire was used to take information on participant's demographic characteristics and clinical data were obtained from their medical records. Dietary data were obtained using 24-h dietary recall and food frequency questionnaire. Height and weight were measured for participants and their haemoglobin levels were determined to assess their anaemia status. Results: Intakes of participants were below recommendations for most nutrients, particularly for calcium and antioxidant vitamins (vitamin C and E). Adequacy of nutrient intake declined with age and children with genotype HbSS had a lower tendency to meet dietary recommendations (aOR = 0.45, 95% CI: 0.18, 1.10; p = 0.084). More than a third of the study children (38%) were malnourished, with older children having a tendency to be malnourished compared to the younger ones. Children with the SS genotype were more likely to be stunted (aOR = 3.48, 95% CI: 1.10, 11.01), compared to other genotypes. Conclusion: Malnutrition is prevalent among children with SCD and hence there is the need to develop comprehensive management coupling nutritional therapy to medical care to improve their nutritional status. Nutritional management should focus much on calcium-rich foods and antioxidants nutrients particularly vitamin C and E to reduce rapid erythrocyte haemolysis and chronic anaemia.

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“…For example, a study that involved young patients aged 6-35 months showed no association with wasting, stunting, or underweight status. [34] A study of 233 children aged 2-17 years with SCD in Lagos, Nigeria found that the factor most signi cantly associated with both wasting and stunting was older age.…”

Section: Resultsmentioning

confidence: 99%

“…The second most common group of studies identi ed involved assessment of biomarkers from serum samples for macronutrients or micronutrients. Most studies were conducted in Nigeria (17/22, 77%); other studies took place in DRC, [49] Tanzania, [25] Egypt, [50] Kenya, [34] and Malawi. [51] Nutritional parameters measured included proteins/amino acids, fatty acids, vitamins, and minerals.…”

Section: Resultsmentioning

confidence: 99%

“…One study of 14 SCD patients in Kenya found no association of HbSS genotype and low concentrations vitamin A. [34] Three reports of a related study population in Ilese, Nigeria, investigated vitamin D status of young SCD patients. [57][58][59] When compared to healthy controls, mean 25-hydroxyvitamin D levels were signi cantly lower in SCD patients and suboptimal vitamin D levels were seen in greater than 10% of patients.…”

Section: Resultsmentioning

confidence: 99%

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Nutritional Perspectives on Sickle Cell Disease in Africa: A Systematic Review

Nartey

Spector

Adu‐Afarwuah

et al. 2020

Preprint

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Background: Sickle cell disease (SCD), an inherited blood disorder, predominantly affects individuals in sub-Saharan Africa. While there are established studies that links its pathophysiology and nutritional status, the extent of research in this area involving African patients has not been previously described. This systematic review aimed to assess the nutrition-focused landscape of SCD studies in Africa, summarize findings, and highlight gaps in knowledge that could inform priority-setting for future research.Methods: The study was conducted using the Preferred Reporting items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. We included all original studies, conducted between 1995 and 2018, on nutritional status of individuals with SCD in Africa. ‘Nutritional status’ was defined by studies on dietary intake, measurements of growth/anthropometry, and nutritional biomarkers. Studies of anemia in non-SCD individuals and those with sickle cell traits were excluded. Databases used were Ovid Embase, Medline, Biosis and Web of Science. Results: The search returned 366 articles from which 62 studies were included in the final analyses. Seventy percent of the investigations were conducted in Nigeria. Studies were categorized into one of three main categories: descriptive studies of anthropometric characteristics (50%), descriptive studies of macro- or micronutrient levels (35%), and interventional studies (15%). Findings consistently found growth impairment, especially among children and adolescents. Studies assessing macro- and micro-nutrients generally had small sample sizes and were exploratory in nature. Only two randomized placebo-controlled trials were identified, which measured the impact of fatty acid supplementation and lime juice on health outcomes. Conclusions: The findings reveal a moderate number of descriptive studies, with small sample size, that focused on various aspects of nutrition and sickle cell disease in African patients. There was a stark lack of interventional studies that could be used to inform evidence-based changes in clinical practice. Findings of investigations were generally consistent with data from other regional settings, describing a significant risk of growth faltering and malnutrition among individuals with SCD. There is an unmet need for clinical research to better understand the potential benefits of nutritional-related interventions for patients with SCD in sub-Saharan Africa in order to promote optimal growth and improve health outcomes.

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Nutritional Status of Young Children with Inherited Blood Disorders in Western Kenya

Cited by 10 publications

References 39 publications

Diagnostic utility of zinc protoporphyrin to detect iron deficiency in Kenyan preschool children: a community-based survey

Diagnostic utility of zinc protoporphyrin to detect iron deficiency in Kenyan preschool children: a community-based survey

Dietary intakes and nutritional status of children with sickle cell disease at the Princess Marie Louise Hospital, Accra – a survey

Nutritional Perspectives on Sickle Cell Disease in Africa: A Systematic Review

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