Studies in Sickle-Cell Anemia&lt;subtitle&gt;VIII. Further Observations on the Clinical Manifestations of Sickle-Cell Anemia in Children&lt;/subtitle&gt;

Scott, Roland B.; Ferguson, Angella D.; Jenkins, Melvin E.; Clark, Harriette M.

doi:10.1001/archpedi.1955.04030010684004

Cited by 11 publications

(5 citation statements)

References 14 publications

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“…The effects of sickle cell disease (SCD) on weight and height in children were first described more than half a century ago. 1,2 Previous studies have revealed a consistent pattern of diminished growth among individuals with SCD from all regions of the world, with evidence linking growth failure to endocrine dysfunction, metabolic derangement, and nutrient deficiencies. 3 The Cooperative Study of Sickle Cell Disease (CSSCD) data on growth and sexual maturation of more than 2000 children and young adults with various sickle genotypes revealed that individuals with HbSS and HbSb 0 thalassemia were smaller compared with those with HbSC and HbSb + thalassemia.…”

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confidence: 95%

Hydroxyurea and Growth in Young Children With Sickle Cell Disease

et al. 2014

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BACKGROUND: Growth impairment is a known complication of sickle cell disease. Effects of hydroxyurea (HU) on growth in very young children are not known. METHODS: Height, weight, BMI, and head circumference (HC) were compared with World Health Organization (WHO) standards in BABY HUG, a multicenter, randomized, double-blinded, placebo-controlled 2-year clinical trial of HU in 193 children 9 to 18 months of age. Anthropometric data were closely monitored and converted to z scores by using WHO standardized algorithms for descriptive analyses. The treatment and placebo groups were compared longitudinally by using a mixed model analysis. RESULTS: At entry, the z scores of BABY HUG children were higher than WHO norms. After 2 years of HU or placebo treatment, there were no significant differences between the groups, except for the mean HC z scores at study exit (HU: +0.8 versus placebo: +1.0, P = .05). Baseline z scores were the best predictors of z scores at study exit. The absolute neutrophil count, absolute reticulocyte count, and total white blood cell count had significant negative correlations with growth measures. CONCLUSIONS: Both groups had normal or near normal anthropometric measures during the study. The HC z scores at study entry and exit were slightly greater than WHO norms. Higher baseline white blood cell count, absolute reticulocyte count, and absolute neutrophil count were associated with poorer growth. The significance of the slightly lower HC in the treatment group at study exit is not clear. Trends toward normalization of weight and height and effects on HC will be monitored in ongoing BABY HUG follow-up studies.

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confidence: 95%

Hydroxyurea and Growth in Young Children With Sickle Cell Disease

et al. 2014

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“…8 At the time of hospital admission, energy intake during acute illness is decreased by as much as 44% of the recommended daily amount (RDA) (SD 9%); during follow-up, intake is closer to 90% of RDA. 9 Dietary intakes can be reduced markedly prior to admission and remain sub-optimal for weeks.…”

Section: Nutritional Status and Disease Severitymentioning

confidence: 99%

Growth and nutritional status of children with homozygous sickle cell disease

Al-Saqladi

Cipolotti

Fijnvandraat

et al. 2008

Annals of Tropical Paediatrics

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Background: Poor growth and under-nutrition are common in children with sickle cell disease (SCD). This review summarises evidence of nutritional status in children with SCD in relation to anthropometric status, disease severity, body composition, energy metabolism, micronutrient deficiency and endocrine dysfunction. Methods: A literature search was conducted on the Medline/PUBMED, SCOPUS, SciELO and LILACS databases to July 2007 using the keywords sickle cell combined with nutrition, anthropometry, growth, height and weight, body mass index, and specific named micronutrients. Results: Forty-six studies (26 cross-sectional and 20 longitudinal) were included in the final anthropometric analysis. Fourteen of the longitudinal studies were conducted in North America, the Caribbean or Europe, representing 78.8% (2086/2645) of patients. Most studies were observational with wide variations in sample size and selection of reference growth data, which limited comparability. There was a paucity of studies from Africa and the Arabian Peninsula, highlighting a large knowledge gap for low-resource settings. There was a consistent pattern of growth failure among affected children from all geographic areas, with good evidence linking growth failure to endocrine dysfunction, metabolic derangement and specific nutrient deficiencies. Conclusions: The monitoring of growth and nutritional status in children with SCD is an essential requirement for comprehensive care, facilitating early diagnosis of growth failure and nutritional intervention. Randomised controlled trials are necessary to assess the potential benefits of nutritional interventions in relation to growth, nutritional status and the pathophysiology of the disease.

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“…The ndings of studies identi ed through this review are generally consistent with nutrition-related investigations involving SCD patients in other parts of the world. More than fty years ago, poor growth was rst reported in patients with SCD, and that observation has since been repeated in multiple studies involving SCD populations in Jamaica, Brazil, India, and North America [13,15,[82][83][84][85][86][87][88]. Many of these studies speci cally note that the growth faltering occurred in patients that were receiving recommended daily protein and calorie intakes.…”

Section: Discussionmentioning

confidence: 98%

Nutritional perspectives on sickle cell disease in Africa: A systematic review

Nartey

Spector

Adu‐Afarwuah

et al. 2021

Preprint

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Background: Sickle cell disease (SCD), an inherited blood disorder, predominantly affects individuals in sub-Saharan Africa. Research linking its pathophysiology and nutritional status in African patients has not been previously described. This systematic review aimed to assess the landscape of studies in sub-Saharan Africa focused on nutritional aspects of SCD 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. All original studies involving individuals with any phenotypic variant of SCD and at least one nutritional status outcome; and were conducted between January 1995 and November 2020 in Africa were included. ‘Nutritional status’ was defined by studies on dietary intake, measurements of growth/anthropometry, or nutritional biomarkers. Studies were included if they involved Databases used were Ovid Embase, Medline, Biosis and Web of Science. Results: The search returned 526 articles with 76 studies included in the final analyses. Most investigations (67%) were conducted in Nigeria. Studies were categorized into one of three main categories: descriptive studies of anthropometric characteristics (49%), descriptive studies of macro- or micronutrient intakes (41%), and interventional studies (11%). Findings consistently found growth impairment, especially among children and adolescents from sub-Saharan Africa. Studies assessing macro- and micronutrients generally had small sample sizes and were exploratory in nature. Only four randomized trials were identified, which measured the impact of lime juice, long-chain fatty acids supplementation, ready-to-use supplementary food, and oral arginine on health outcomes. Conclusions: The findings reveal a moderate number of descriptive studies, most with small sample sizes, that focused on various aspects of nutrition and SCD 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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Studies in Sickle-Cell Anemia<subtitle>VIII. Further Observations on the Clinical Manifestations of Sickle-Cell Anemia in Children</subtitle>

Cited by 11 publications

References 14 publications

Hydroxyurea and Growth in Young Children With Sickle Cell Disease

Hydroxyurea and Growth in Young Children With Sickle Cell Disease

Growth and nutritional status of children with homozygous sickle cell disease

Nutritional perspectives on sickle cell disease in Africa: A systematic review

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