Growth and nutritional status of children with homozygous sickle cell disease

Abstract

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.

Baixa estatura e magreza em crianças e adolescentes com doença falciforme

“…Os que utilizaram pontos de corte superiores ao recomendado, como -1 EZ ou percentil 10, superestimaram a prevalência de deficit nutricional. Além disso, é possível que a frequência de outros fatores envolvidos na etiologia do deficit de crescimento, como fatores hematológicos, cardiovasculares, socioeconômicos, função endócrina e metabólica, justifique as diferenças encontradas 26 .…”

“…O consumo dietético torna-se marcadamente reduzido no período que precede as crises vaso--oclusivas e permanece em níveis subótimos durante dias ou semanas 26 . A elevada demanda metabólica deve-se, em grande parte, à necesTabela 3.…”

“…Normal Alto o atraso na maturação sexual observado nesses indivíduos está relacionado com a reduzida massa corporal, mostrando que a nutrição inadequada, além do componente endócrino, tem relação com o atraso puberal 26 .…”

Nutritional status, hospitalization and mortality among patients with sickle cell anemia in Tanzania

“…In the UK East London Cohort with a very high rate of survival, 25 malnutrition is uncommon with only 6.5% of 2-15 year olds having a zscore of less than -2 for weight-for-age and 4.2% for height-for-age or BMI 13 suggesting that improved nutritional status may have increased survival or that increased care has resulted in milder disease and improved nutritional status. Supporting the latter possibility, hydroxyurea therapy has been associated with a decrease in resting energy expenditure, correlated with increased HbF expression, and decreased wasting.…”

“…[10][11][12] However, the causes and clinical significance are not well understood. 13 In Africa, where dietary intakes are sub-optimal for many populations, we hypothesized that nutrition is an important modifiable risk factor for SCA morbidity and that poor nutritional status is associated with increased mortality and morbidity. Here we describe anthropometric measures of nutritional status in a large cohort of Tanzanian SCA patients at enrolment.…”