Abstract:BackgroundReduced growth is common in children with sickle cell anemia, but few data exist on associations with long-term clinical course. Our objective was to determine the prevalence of malnutrition at enrolment into a hospital-based cohort and whether poor nutritional status predicted morbidity and mortality within an urban cohort of Tanzanian sickle cell anemia patients.
Design and MethodsAnthropometry was conducted at enrolment into the sickle cell anemia cohort (n=1,618; ages 0.5-48 years) and in control… Show more
“…Endocrine dysfunction, poor nutrient intake, micronutrient deficiencies, hypermetabolism, and high protein turnover have been described in individuals with SCD and growth failure. [26][27][28][29][30][31] Growth hormone deficiency may account for a small percentage of children with SCD who have severe growth delay. 32 Hemolysis, chronic anemia, and a high metabolic rate appear to be the most significant adverse factors for growth in SCD.…”
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.
“…Endocrine dysfunction, poor nutrient intake, micronutrient deficiencies, hypermetabolism, and high protein turnover have been described in individuals with SCD and growth failure. [26][27][28][29][30][31] Growth hormone deficiency may account for a small percentage of children with SCD who have severe growth delay. 32 Hemolysis, chronic anemia, and a high metabolic rate appear to be the most significant adverse factors for growth in SCD.…”
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.
“…This prevalence of acute chest syndrome is twice as high as the USA, 30 and may be due to lack of immunization, late presentation, and concomitant malnutrition. 31 The incidence of stroke in children with SCA is much lower in the USA, with only 11% having stroke before age 20 and 24% before age 45. 16 Despite the high prevalence of stroke in our study, no one had undergone screening transcranial Doppler to identify a higher risk of stroke.…”
Objectives
Tanzania has the 3rd highest birth rate of sickle cell anaemia (SCA) in Africa, but few studies describe severity of complications or available treatments, especially in North West Tanzania around Lake Victoria where the sickle gene is most prevalent. This is a report of the spectrum of clinical disease and range of interventions available at Bugando Medical Centre (Bugando) in North West Tanzania in Africa.
Methods
A cross-sectional study was carried out in Bugando between August 1, 2012 and September 30, 2012. Children (< 15 years old) with SCA attending Bugando were sequentially enrolled. A trained research assistant completed a Swahili questionnaire with the parent or guardian of each participant concerning demographic information, clinical features of disease, and treatments received.
Results
Among the 124 participants enrolled, the median age was 6 years [IQR 4-8.5], and only 13 (10.5%) were < 3 years old. Almost all participants (97.6%) had a prior history of a vaso-occlusive episode, 83 (66.9%) had prior acute chest syndrome, and 21 (16.9%) had prior stroke. In the preceding 12 months, 120 (96.8%) had been hospitalized, and a vaso-occlusive episode was the most common reason for hospitalization (35.5%). Prescriptions for folic acid (92.7%) and malaria prophylaxis (84.7%) were common, but only one had received a pneumococcal vaccine, and none had received hydroxyurea or prophylactic penicillin.
Conclusion
Children with SCA receiving care in Tanzania are diagnosed late, hospitalized frequently, and have severe complications. Opportunities exist to improve care through wider access to screening and diagnosis as well as better coordination of comprehensive care.
“…Finally, we did not capture data on immigration status on the children included in this study, yet the participating institutions are located in urban areas with large immigrant populations from sub-Saharan Africa, the Caribbean, and Central and South America. Children with SCD from developing countries are often underweight for age and gender5,39,40 ; therefore, our results may underestimate the prevalence of elevated BMI percentiles among US-born children with SCD.CONCLUSIONSA significant proportion of children and adolescents with SCD in New England are overweight or obese, which is associated with higher baseline Hb levels.Longitudinal studies are needed to determine if there is a causal relationship between increased Hb and weight status.…”
Nearly one-quarter of children and adolescents with SCD in New England are overweight or obese. Longitudinal studies are needed to determine the impact of elevated BMI on the morbidity and mortality of both children and adults with SCD.
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