Growth Deficits in Children with Sickle Cell Disease

Silva, Célia Maria; Viana, Marcos Borato

doi:10.1016/s0188-4409(01)00360-5

Cited by 51 publications

(51 citation statements)

References 13 publications

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“…In most cases, similar to our study, nutritional status was worse in males compared to females 11,[26][27][28] although one study reported the contrary. 29 However, in the one study that assessed disease severity in relation to growth, effect was limited to females.…”

Section: Prevalence Of Malnutrition In Sca By Age and Sexsupporting

confidence: 90%

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

Cox¹,

Makani²,

Fulford³

et al. 2011

Haematologica

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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 controls who attended screening (siblings, walk-ins and referrals) but who were found not to have sickle cell anemia (n=717; ages 0.5-64 years). ResultsSickle cell anemia was associated with stunting (OR=1.92, P<0.001, 36.2%) and wasting (OR=1.66, P=0.002, 18.4%). The greatest growth deficits were observed in adolescents and in boys. Independent of age and sex, lower hemoglobin concentration was associated with increased odds of malnutrition in sickle cell patients. Of the 1,041 sickle cell anemia patients with a body mass index z-score at enrolment, 92% were followed up until September 2009 (n=908) or death (n=50). Body mass index and weight-for-age z-score predicted hospitalization (hazard ratio [HZR]=0.90, P=0.04 and HZR=0.88, P=0.02) but height-for-age z-score did not (HZR=0.93, NS). The mortality rate of 2.5 per 100 person-years was not associated with any of the anthropometric measures. ConclusionsIn this non-birth-cohort of sickle cell anemia with significant associated undernutrition, wasting predicted an increased risk of hospital admission. Targeted nutritional interventions should prioritize treatment and prevention of wasting.Key words: sickle cell anemia, nutrition, growth, anthropometry, Africa, mortality, morbidity. Citation

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Section: Prevalence Of Malnutrition In Sca By Age and Sexsupporting

confidence: 90%

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

Cox¹,

Makani²,

Fulford³

et al. 2011

Haematologica

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“…Since publication of the CSSCD data, several other studies have revealed that children and adolescents with SCD had significantly lower height, weight, and BMI compared with reference growth curves. [9][10][11][12][13][14][15][16] In general the adverse influence of SCD has been more pronounced for weight than height. Endocrine dysfunction, poor nutrient intake, micronutrient deficiencies, hypermetabolism, and high protein turnover have been described in individuals with SCD and growth failure.…”

Section: Discussionmentioning

confidence: 99%

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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“…In addition, there may be differences in chest wall and vertebral structure among adults with Hb-SS contributing to the restrictive physiology. This could be due to repeated bony infarctions during growth and development and the spinal osteoporosis and osteomalacia typical of Hb-SS (32)(33)(34). Non-SCD-specific etiologies of extrapulmonary restriction such as obesity may be playing a role in the restrictive physiology observed in a subset of the subjects observed.…”

Section: Discussionmentioning

confidence: 99%

Abnormal Pulmonary Function in Adults with Sickle Cell Anemia

Klings

Wyszynski

Nolan

et al. 2006

Am J Respir Crit Care Med

187

166

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Rationale: Pulmonary complications of sickle cell anemia (Hb-SS) commonly cause morbidity, yet few large studies of pulmonary function tests (PFTs) in this population have been reported. Objectives: PFTs (spirometry, lung volumes, and diffusion capacity for carbon monoxide [DL CO ]) from 310 adults with Hb-SS were analyzed to determine the pattern of pulmonary dysfunction and their association with other systemic complications of sickle cell disease. Methods: Raw PFT data were compared with predicted values. Each subject was subclassified into one of five groups: obstructive physiology, restrictive physiology, mixed obstructive/restrictive physiology, isolated low DL CO , or normal. The association between laboratory data of patients with decreased DL CO Sickle cell anemia (Hb-SS) results from homozygosity for a point mutation in the ␤-globin gene (HBB; Glu6Val) causing the resultant sickle hemoglobin (Hb S) to be less soluble when deoxygenated than normal hemoglobin (1). Even with improved treatment, including the early use of prophylactic antibiotic regimens, judicious transfusions, and the administration of hydroxyurea in selected patients, mortality remains high for this population. The median age at death is 42 yr for males and 48 yr for females with Hb-SS. Pulmonary complications, including acute chest syndrome (ACS), pulmonary hypertension (PH), and pulmonary fibrosis, account for 20-30% of deaths in the Hb-SS population and are often underrecognized by the health care community (2, 3).Dyspnea is a frequent complaint amongst patients with sickle cell disease, the etiology of which is unclear and likely multifacto- 5). Studies of lung function to date in this population have been of modest size, often involving fewer than 50 patients (4, 6-11) and largely inconclusive. Their results have yielded a spectrum of abnormalities, including restrictive lung disease, abnormal diffusion capacity for carbon monoxide (Dl CO ), obstructive disease, and hypoxemia (4,6,7,9,12). No definitive profile for pulmonary function in sickle cell disease has emerged. As a result, clinicians find pulmonary function tests (PFTs) difficult to interpret in this population and their clinical utility for directing further investigation and therapy has not been well evaluated.Of growing concern is the link between obstructive lung disease and ACS, particularly in children (7,13,14). The few published studies suggest that obstructive lung disease, in some cases, plays a role in the pathogenesis of ACS (7,13,15). Moreover, obstructive lung disease could be a long-term sequela of recurrent episodes of ACS (6, 9). Larger scale studies are necessary to elucidate more clearly the interaction between lung function and ACS. In addition, certain findings on PFTs might be a marker of other complications of sickle vasculopathy. For example, isolated decreased Dl CO is a well-established finding associated with PH (16, 17). However, its role as a marker or predictor of PH in the Hb-SS population is unknown. The purpose of this study is to evalua...

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Growth Deficits in Children with Sickle Cell Disease

Cited by 51 publications

References 13 publications

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

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

Hydroxyurea and Growth in Young Children With Sickle Cell Disease

Abnormal Pulmonary Function in Adults with Sickle Cell Anemia

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