Body composition in children with sickle cell disease

BACKGROUND: Impaired growth, poor nutritional status, and delayed skeletal and sexual maturation are common in children with sickle cell disease (SCD), yet the nature of associated body-composition deficits has not been fully described. OBJECTIVE: The objective was to assess growth, nutritional status, and body composition in 36 African American children with type SS SCD (20 females and 16 males) and 30 healthy control children (15 females and 15 males) of similar age (5-18 y) and ethnicity. DESIGN: Height, weight, bone age, pubertal status, skinfold thickness, and arm circumference were assessed. Height and weight were converted to z scores by comparison with national reference data and skinfold-thickness measurements were converted to z scores by comparison with African American- specific reference data. Fat-free mass (FFM) and fat mass (FM) were estimated by using 4 methods. Prepubertal children, pubertal males, and pubertal females were analyzed separately. RESULTS: Relative to the control subjects and to a national sample, children with SCD had significantly lower z scores for weight, height, arm circumference, and upper arm fat and muscle areas. Relative skeletal maturation was significantly delayed. After adjustment for age, children with SCD had significantly lower FM (prepubertal children and pubertal males only) and FFM (all 3 groups). CONCLUSIONS: Children with SCD have impaired growth, delayed puberty, and poor nutritional status. Low z scores for upper arm fat area indicate deficits in fat (energy) stores, and low FFM coupled with low upper arm muscle area indicate muscle wasting and low protein stores. These body-composition abnormalities suggest that the nutritional needs of the African American children with SCD were not being met.     

Influenza immunization coverage of children with sickle cell disease

ObjectivesTo assess receipt of annual flu immunization among children living with sickle cell disease (SCD).MethodsReceipt of flu immunization (2014–2019) by SCD status was assessed among all Michigan children <18 years of age using the statewide immunization registry. Logistic regression was used to estimate the odds of annual flu immunization by SCD status and age.ResultsAnnual flu immunization coverage was higher among children with SCD (46.9%; n = 751) than without (23.2%; n = 2,012,846). The annual adjusted odds of flu immunization for those with SCD were 2.8 (95% CI: 2.5–3.1) times higher than for those without SCD; there were no significant differences by age among children with SCD. Among those without SCD, adolescents aged 13–17 were 2.2 (95% CI: 2.2–2.2) times less likely to receive annual flu immunization than children 6–35 months.ConclusionsChildren with SCD had higher annual flu immunization rates than those without SCD, but >50% remain unimmunized.     

Impact of acute illness on nutritional status of infants and young children with sickle cell disease

OBJECTIVE: To evaluate changes in growth, nutritional status, body composition, and energy and nutrient intakes during illness and usual state of health in infants and young children with sickle cell disease. DESIGN: Sixteen children, aged 0.4 to 5.6 years, with SS type sickle cell disease (SCD-SS) were assessed at the time of hospital admission for an acute illness episode and during an 18-hour overnight follow-up visit 2 to 6 weeks after the acute illness episode when in a state of usual health. Main outcome measures included growth in height and weight compared with reference standards, body composition determined by the skinfold thickness technique and total body electrical conductivity, and dietary intake determined by 24-hour recall during hospital admission and at follow-up. RESULTS: Height, weight, and weight-for-height z scores did not differ from national reference data; triceps skinfold thickness and arm fat area z scores were less. Mean +/- standard error body fat was 15.6 +/- 2.1% at the time of hospital admission, as measured by total body electrical conductivity, and was not significantly different from the follow-up value (16.2 +/- 2.2%). Mean energy intake was 44 +/- 9% of Recommended Dietary Allowances at the time of admission and differed significantly from the follow-up value of 90 +/- 9% (P < .05). APPLICATIONS: Infants and children with sickle cell disease appear to be at nutritional risk during an acute illness episode, as indicated by body fat measures and inadequate intakes of energy and macronutrients. Energy intake may be suboptimal for several days surrounding an admission for an acute illness in children with sickle cell disease. Physicians and other health practitioners should be alert to inadequate nutrient intakes of their patients during this time period and may consider supplemental energy to avoid a potential net negative energy balance.     

Recognition of children with sickle cell disease in The Netherlands

Timely recognition of clinical signs and symptoms of sickle cell disease remains of great importance because the neonatal PKU screening program in The Netherlands that was introduced in January 1st 2007 will not reach all children with this disease. Of children that have been diagnosed in the Emma Children's Hospital AMC, Amsterdam, 20% would not have been reached by this new program: immigrant's children born abroad and adopted children. It goes without saying that also in children that have been born in the Netherlands before January 1st 2007 the diagnosis sickle cell disease should be considered in cases of disease-specific clinical symptoms. The initial clinical manifestation of sickle cell disease in children born in the Netherlands is potentially life-threatening in 8% (7/88), e.g. a pneumococcal infection or an acute splenic sequestration. Painful crisis, paleness and jaundice are the most common presenting symptoms. The median age at diagnosis of the group of Amsterdam children was 25 months. In view of the potential health benefit it is advised to test children from populations at risk, that are under the medical attention of a hospital for any reason, for the presence of sickle cell disease. This applies especially to children with a pneumococcal infection.     

Predictors of employment status among African Americans with sickle cell disease

Adults living with sickle cell disease (SCD) have extremely high rates of unemployment; however, very little is known about factors that contribute to their vocational outcomes. This study examined demographic, illness perception, and psychological variables as predictors of employment status among 115 adult respondents who completed a cross-sectional survey as part of the Cooperative Study of Sickle Cell Disease. Logistic regression analysis indicated that gender, assertiveness, and perceived impact of SCD were unique predictors of employment status. Women were 2.88 times more likely to be employed than men, and the odds of being employed increased by a factor of 2.47 for each one unit decrease in assertiveness. More favorable perceptions of SCD were also associated with a two-fold increase in employment. The results suggest that demographic and psychosocial factors may play a more important role in predicting employment outcomes in adults with SCD than previously recognized.     

Relationships in families of children and adolescents with sickle cell disease

This study represents the findings from interviews and assessments of children and adolescents with sickle cell disease and their parents regarding the quality of family relations, the degree to which sickle cell disease has impacted on these relations, and the variables which contribute to these relations. With 70 families as respondents, the data reveal a wide variance in quality of relations, but a broad base of positive relations. Parent report tends to be more positive than child report, both in terms of quality of relations and the impact of sickle cell disease on relations. Social support and knowledge about the disease are significant contributors to positive relations, while socioeconomic status, family structure, and illness severity are not predictive of quality of relations. With age and gender as covariates, results indicate that the families of girls tended to have more positive relations. The data suggest approaches to family and community support in order to help families maintain and build relations in the face of the stresses which sickle cell disease imposes.     

Depiction of pain in the self-drawings of children with sickle cell disease

In an attempt to explore the perception of pain in children, 30 children with sickle cell disease were asked to make two drawings; one of themselves and one of themselves in pain. It was hypothesized that the cognitive ability and the emotional state of the child would be affected by the pain experience. Children and their parents were interviewed on the incidence of pain and on the child's control over it. The cognitive ability of the child was related to the kind of control he/she exercised over the pain. An analysis of the drawings concerning their thematic representations, colour and size is also presented. The mental age of the child dropped in the drawing of pain as compared to the non-pain drawing but it was found to be irrelevant to the kind of control the child exercised on the pain.     

Understanding the social networks of parents of children with sickle cell disease

Although there is substantial literature documenting the challenges of pediatric sickle cell disease (SCD) for children and their parents, there is limited research identifying how parents prioritize their needs and use their social networks to manage information regarding their child's SCD in terms of physical and mental health. We examined parents' perceived needs regarding child health issues as they relate to SCD; who and what sources of information are utilized by parents regarding SCD; the frequency with which they consult these resources; and the level at which they trust them. Parents in this study reported that mothers, physicians, the Internet, and books were key sources of support, guidance, and counsel regarding the health needs of children with SCD. These three sources were rated high in importance, trust, frequency of contact, and perceived supportiveness toward mental and physical health needs.     

Adequacy of dietary intake declines with age in children with sickle cell disease

Dietary intake (24-hour recall) was evaluated prospectively over four annual visits in 97 children and adolescents (53 female), aged 1.5 to 18.7 years, with sickle cell disease, type SS. Macro- and micronutrient intakes were compared to Dietary Reference Intakes (DRI) and expressed as %DRI. z scores for height, weight, and body mass index were calculated to assess growth status. Both t tests and Mann-Whitney U tests were used for year 1 comparisons, and longitudinal mixed effects analysis was used for the longitudinal data. Intake of vitamins E and D, folate, calcium, and fiber as %DRI was low for children of all ages. Intake of protein, vitamin C, riboflavin, vitamin B-12, and magnesium was lower by at least 28% DRI in the oldest (aged 14 to 18 years) compared to the youngest children (aged 1 to 3 years), and intake of vitamin A, magnesium, and phosphorus was suboptimal in children older than 9 years. After adjusting for initial age and sex, intake of riboflavin, zinc, calcium, magnesium, and phosphorus declined steeply (8% to 16% DRI annually) across the 3 years. Weight and body mass index z scores also declined over time. Dietary intake was particularly poor in adolescents. Efforts are needed to ensure dietary adequacy in children with sickle cell disease, type SS and to understand the etiology of poor dietary intake.     

High risk of vitamin D deficiency in children with sickle cell disease

Vitamin D is a particularly concerning nutrient for children with homozygous SS sickle cell disease (SCD-SS) due to their increased skin melanin concentrations, reduced levels of physical activity, and poor vitamin D intake. The goal of this study was to compare the vitamin D status of children with SCD-SS to healthy African-American children living in the same geographic area. Growth, dietary intake, serum 25-hydroxyvitamin D [25(OH)D], and intact parathyroid hormone (iPTH) concentrations were measured in 61 African-American subjects with SCD-SS and 89 healthy African-American control subjects age 5 to 18 years from the Philadelphia, PA, region (latitude 39.95 degrees N). Median serum 25(OH)D concentrations were 15 ng/mL (95% confidence interval [CI]: 13, 17) in subjects with SCD-SS and 21 ng/mL (95% CI: 18, 22) in healthy control subjects (P<0.0002). Vitamin D deficiency [25(OH)D<11 mg/mL] was found in 33% of subjects with SCD-SS and 9% of healthy control subjects (P<0.001); 25% of subjects with SCD-SS and 17% of healthy control subjects had elevated iPTH [(>59 rhog/mL), P<0.05]. Ninety-three percent of subjects with SCD-SS and 90% of healthy subjects had vitamin D insufficiency [25(OH)D<30 mg/mL]. The risk of vitamin D deficiency among subjects with SCD-SS was 5.3 (95% CI: 2.5, 8.2) times greater than control subjects, adjusted for season and age. Poor vitamin D status was prevalent in children with SCD-SS and healthy African-American children living in the same geographic area. However, children with SCD-SS were at greater risk for vitamin D deficiency than healthy African-American children.     

Plasma zinc is an insensitive predictor of zinc status: use of plasma zinc in children with sickle cell disease.

BACKGROUND: This study was designed to explore the use of plasma zinc in determining zinc deficiency in children with sickle cell disease-SS (SCD-SS) as indicated by a growth response to zinc supplementation. METHODS: Fasting plasma zinc was assessed in children with SCD-SS (ages 4 to 10 years) who were randomly assigned to receive 10 mg zinc/d in cherry syrup (zinc) or cherry syrup alone (placebo) for 12 months. Evaluations for growth, dietary intake, and other biochemical parameters were made at baseline and 3, 6, and 12 months. Longitudinal mixed effects analysis evaluated differences between groups over 12 months. RESULTS: A total of 38 prepubertal children (20 male and 18 female; 18 zinc and 20 placebo) completed the study (7.1 +/- 1.7 years old). At baseline, plasma zinc was low (< or = 70 microg/dL) in 7 male subjects. Despite the significant increase in height over 12 months (+0.7 cm) with zinc supplementation (p = .019), plasma zinc did not change over the 12 months of study, and there was no association between plasma zinc and linear growth. Those children with low plasma zinc who received zinc supplementation showed improved linear growth. CONCLUSIONS: These findings suggest that plasma zinc is an insensitive indicator of zinc status in children with SCD-SS. Children with low plasma zinc will benefit from zinc supplementation. However, some children with normal plasma zinc and poor growth may also have growth-limiting zinc deficiency and exhibit a growth response to zinc supplementation. Although this study focused on children with SCD-SS, results may be generalized to other pediatric clinical illnesses where nutrition-related growth failure is investigated.     

Energy expenditure and intake in children with sickle cell disease during acute illness

BACKGROUND: Children with sickle cell disease have frequent bouts of pain and infection which may increase energy expenditure, decrease energy intake and lead to a subsequent energy deficit. METHODS: Two groups of African-American children with sickle cell disease-SS genotype were enrolled in this study upon hospital admission for a sickle cell disease related illness: a younger (<6 years, n=14, 7 M) and older group (> or =6 years, n=17, 8 M). Body composition and dietary intake were assessed, and sleeping (younger) or resting energy expenditure (older) were measured by indirect calorimetry at admission and one month later at steady state. RESULTS: Energy expenditure was not different between the two timepoints for younger children, but was slightly elevated at steady state (+50 kcal/d, P=0.049) in the older group. After controlling for gender, changes in fat-free mass and dietary intake, the significance disappeared. Energy intake in both groups was significantly depressed at admission compared to follow-up (P<0.01). CONCLUSIONS: These children and adolescents did not expend excess energy during their acute illness, however, an energy deficit was observed secondary to poor energy intake. Since 20% of patients with sickle cell disease have multiple hospitalizations per year, these results provide justification for the development and evaluation of nutrition care protocols to maintain adequate caloric intake during hospitalization and recovery.     

The importance of grandparents in extended-kin caregiving to black children with sickle cell disease

This study examined support and care the black extended-kin system provided to 34 chronically ill black children with sickle cell anemia. Findings show that grandparents played important roles in helping provide support to the sick children's primary caregivers and to the children themselves. Grandparents provided support that complemented that of fathers, and they remained in the system of care longer than any other relative in the family. The data indicate a need to design family interventions for sick children and their parent to include grandparents.     

MRI contribution in diagnosis of acute bone infarcts in children with sickle cell disease

We analyse MRI findings of nine children with sickle cell disease having painful osseous attacks. The diagnosis of vaso-occlusive crisis was confirmed by negative bacteriology. MRI is useful for determining the anatomic site and the extent of acute infarcts. It gives a global view of osseous abnormalities, and contributes to differentiating acute infarcts from acute osteomyelitis.     

Antibody persistence after serogroup C meningococcal conjugate vaccine in children with sickle cell disease

BackgroundA decline of protective antibody titers after MCC vaccine has been demonstrated in healthy children, this may be an issue of concern for risk groups. The aim of this study was to evaluate the persistence of bactericidal antibodies after MCC vaccine in sickle cell disease (SCD) patients. The type of vaccine used and booster response were also analyzed.MethodsSCD patients (n = 141) previously immunized with MCC vaccines had blood drawn 2–8 years after the last priming dose. They were distributed according to age at primary immunization into groups: <2 years and 2–13 years and evaluated by years since vaccination (2–3, 4–5 and 6–8). Serum bactericidal antibodies with baby rabbit complement (rSBA) and serogroup C-specific IgG concentrations were measured. The correlate of protection was rSBA titer ⩾8. Subjects with rSBA <8 received a booster dose and antibody levels re-evaluated after 4–6 weeks.ResultsFor children primed under 2 years of age rSBA titer ⩾8 was demonstrated in 53.3%, 21.7% and 35.0%, 2–3, 4–5, 6–8 years, respectively, after vaccination, compared with 70.0%, 45.0% and 53.5%, respectively, for individuals primed at ages 2–13 years. rSBA median titers and IgG median levels were higher in the older group. Six to eight years after vaccination the percentage of patients with rSBA titers ⩾8 was significantly higher in the group primed with MCC-TT (78.5%) compared with those primed with MCC-CRM₁₉₇ [Menjugate® (33.3%) or Meningitec® (35.7%)] (p = 0.033). After a booster, 98% achieved rSBA titer ⩾8.ConclusionImmunity to meningococcal serogroup C in SCD children declines rapidly after vaccination and is dependent on the age at priming. Booster doses are needed to maintain protection in SCD patients. Persistence of antibodies seems to be longer in individuals primed with MCC-TT vaccine comparing to those immunized with MCC-CRM₁₉₇.     

Levels of high-density lipoprotein cholesterol (HDL-C) among children with steady-state sickle cell disease

Background  

The search for sickle cell disease (SCD) prognosis biomarkers is a challenge. These markers identification can help to establish further therapy, later severe clinical complications and with patients follow-up. We attempted to study a possible involvement of levels of high-density lipoprotein cholesterol (HDL-C) in steady-state children with SCD, once that this lipid marker has been correlated with anti-inflammatory, anti-oxidative, anti-aggregation, anti-coagulant and pro-fibrinolytic activities, important aspects to be considered in sickle cell disease pathogenesis.     

Effect of zinc supplementation on growth and body composition in children with sickle cell disease

BACKGROUND: Poor growth and delayed maturation in children with sickle cell disease (SCD) may be due, in part, to mild zinc deficiency. OBJECTIVE: The objective was to determine the effects of zinc supplementation on growth and body composition in children with SCD. DESIGN: Forty-two prepubertal children (20 girls and 22 boys) aged 4-10 y with SCD-SS were randomly assigned to receive 10 mg elemental Zn/d in cherry syrup (zinc group) or cherry syrup alone (control group). The 2 groups were stratified by sex and initial height status. Dietary intakes were evaluated and anthropometric, high-precision knee-height, and plasma zinc measurements were made at baseline and at 3, 6, and 12 mo. Body composition was determined every 6 mo with dual-energy X-ray absorptiometry, and z scores for anthropometric variables were computed from national reference data. Longitudinal-mixed-effects analysis was used to test for differences between the groups over the 12-mo observation period. RESULTS: Thirty-eight children completed the study. No significant differences were observed at baseline. After 12 mo, the zinc group had significantly greater mean (+/- SE) increases in height (0.66 +/- 0.29 cm/y), sitting height (0.97 +/- 0.40 cm/y), knee height (3.8 +/- 1.2 mm/y), and arm circumference z scores (0.27 +/- 0.12 cm/y). Height-for-age and weight-for-age z scores decreased significantly by 0.11 +/- 0.04 and 0.13 +/- 0.05, respectively, in the control group but did not change significantly in the zinc group. CONCLUSIONS: Prepubertal children with SCD-SS may have zinc deficiency and may benefit from zinc supplementation to improve linear growth and weight gain.