Late-Onset Molybdenum Cofactor Deficiency Type A: A Treatable Cause of Developmental Delay.

Molybdenum cofactor deficiency classically presents in neonates with intractable seizures; however, milder cases generally present before age 2 years with developmental delays and may go undiagnosed. Early diagnosis, and safe, US Food and Drug Administration-approved substrate replacement are critical to preserve neurologic function. This article discusses 2 children who presented with late-onset molybdenum cofactor deficiency type A.

Associations between different weight-related anthropometric traits and lifestyle factors in Norwegian children and adolescents: A case for measuring skinfolds.

OBJECTIVES: The purpose of this study was to investigate the association between weight-related anthropometric measures and children's eating habits, physical activity and sedentary lifestyle at a population level. METHODS: Data from the Bergen Growth Study were used to study the association of z-scores of waist circumference (WC), weight-to-height ratio (WHtR), subscapularis (SSF) and triceps (TSF) skinfolds and BMI, with lifestyle factors in 3063 Norwegian children (1543 boys) aged 4-15 years, using linear regression analysis. Each sex was analyzed separately. RESULTS: In a fully adjusted model with additional correction for BMI z-scores, the consumption of vegetables was associated with higher WC (b = 0.03) and TSF (b = 0.05) z-scores in girls. Sedentary behavior was not associated with any of the anthropometric measures. Physical activity was negatively associated with SSF (b = -0.07) and TSF (b = -0.07) z-scores in boys, while a significant negative association was observed with WC (b = -0.02), WHtR (b = -0.03), SSF (b = -0.04) and TSF (b = -0.06) in girls. CONCLUSION: Physical activity was negatively associated with skinfolds in both sexes. The BMI was not related to the level of physical activity, and should be complemented with direct measures of fat tissue, like skinfolds, when studying the effect of physical activity on body composition in children.

Timing of menarche in Norwegian girls: associations with body mass index, waist circumference and skinfold thickness.

BACKGROUND: Research studies show conflicting results regarding the association between menarche and body weight. The purpose of the present study was to investigate if anthropometric indicators of body composition, body mass index (BMI), waist circumference (WC), triceps (TSF) and subscapular skinfold (SSF) thicknesses, were differentially associated with age at menarche in Norwegian girls. METHODS: The association between menarche and BMI, WC, TSF and SSF was investigated in 1481 girls aged 8-15.5 years, and in a subgroup of 181 girls with menarche during the 12 months prior to examination. Anthropometric measures were categorized as low (< -1SDS), average (-1 ≤ SDS ≤ +1) or high (> 1SDS), and menarche according to this classification was analysed with Kaplan-Meier curves and unadjusted and adjusted Cox regression. RESULTS: The median age at menarche in the total sample was 13.1 years. In the unadjusted models, low categories of all traits were associated with later menarche, and high categories with earlier menarche. When adjusted for other covariates, earlier menarche was only related with a high BMI (Hazard Ratio 1.41, 95% confidence interval (CI) 1.07, 1.85), and later menarche with a low BMI (HR 0.53, 95%CI 0.38, 0.75) and low SSF (HR 0.54, 95%CI 0.39, 0.75). In girls with recent menarche, early menarche was significantly associated with a high BMI in the final model (HR 1.79, 95%CI 1.23, 2.62). CONCLUSIONS: The timing of menarche was associated with the BMI, WC, TSF and SSF, but more strongly so with the BMI. These associations may be related to a common tempo of growth, as the mean age at menarche has remained stable during the last decades during a time period while the prevalence of overweight and obesity has increased significantly.

BMI and BMI SDS in childhood: annual increments and conditional change.

Background Early detection of abnormal weight gain in childhood may be important for preventive purposes. It is still debated which annual changes in BMI should warrant attention. Aim To analyse 1-year increments of Body Mass Index (BMI) and standardised BMI (BMI SDS) in childhood and explore conditional change in BMI SDS as an alternative method to evaluate 1-year changes in BMI. Subjects and methods The distributions of 1-year increments of BMI (kg/m2) and BMI SDS are summarised by percentiles. Differences according to sex, age, height, weight, initial BMI and weight status on the BMI and BMI SDS increments were assessed with multiple linear regression. Conditional change in BMI SDS was based on the correlation between annual BMI measurements converted to SDS. Results BMI increments depended significantly on sex, height, weight and initial BMI. Changes in BMI SDS depended significantly only on the initial BMI SDS. The distribution of conditional change in BMI SDS using a two-correlation model was close to normal (mean = 0.11, SD = 1.02, n = 1167), with 3.2% (2.3-4.4%) of the observations below -2 SD and 2.8% (2.0-4.0%) above +2 SD. Conclusion Conditional change in BMI SDS can be used to detect unexpected large changes in BMI SDS. Although this method requires the use of a computer, it may be clinically useful to detect aberrant weight development.

Should children with overweight or obesity be excluded from height references?

BACKGROUND AND OBJECTIVES: Growth reference charts are usually based on measurements of children free from a medical condition that affects growth. However, samples collected during the past decades often contain a large proportion of overweight or obese children. Because obesity increases linear growth, the question arises to what extent the percentiles curves for length/height are affected by the presence of children with overweight or obesity. METHODS: Data from two cross-sectional samples of 2-year-old to 18-year-old children were analysed: 12,252 Belgian children, measured in 2002-2004, and 6159 Norwegian children, measured in 2003-2006. The LMS method was used to estimate height-for-age curves with and without children considered overweight or obese according to the International Obesity Task Force thresholds. RESULTS: The prevalence of overweight (including obesity) and obesity was 13.0% and 2.8% in the Belgian and 13.8% and 2.3% in the Norwegian sample. Children were taller when overweight (+0.49 and 0.43 SD, in the Belgian and Norwegian sample, respectively) or obese (+0.73 and 0.72 SD in the Belgian and Norwegian sample, respectively). Effect sizes were smaller in younger and older children, which points to an advanced age of maturation as a possible cause. Excluding overweight and obese children had only a minor impact on the growth curves with largest difference in mean height SD scores -0.09 in the Belgian and -0.12 in the Norwegian sample with a corresponding increase of up to 0.5% and 1.2% in number of children >+2 SD. CONCLUSIONS: Current Belgian and Norwegian growth references for length/height were found to be largely unaffected by the current proportion of overweight and obese children. There is, therefore, no need for revised height charts that exclude overweight or obese children.

Assessment of body composition in children with cerebral palsy: a cross-sectional study in Norway.

AIM: The assessment of growth and body composition is challenging in children with cerebral palsy (CP). The aim of this study was to compare clinical assessments of body composition with measurements obtained using dual-energy X-ray absorptiometry (DXA) in this population. METHOD: Knee height, weight, and triceps and subscapular skinfold thickness (SFT) were measured in 47 children with CP (age range 8-18y; 18 females, 29 males). Height was estimated from knee height, and used to calculate body mass index (BMI). Using SFT measurements, body fat percentage was calculated by standard ('Slaughter') and CP-modified ('Gurka') equations and compared with results obtained using DXA. RESULTS: Children with severe gross motor function impairments (Gross Motor Function Classification System [GMFCS] level III or IV) exhibited stunted growth and had higher fat percentages and lower lean body mass than children classified in GMFCS level I or II. In 10 children classified as 'thin' according to their BMI (five of whom were assigned thinness grade of 2 or lower), percentage of body fat, as determined by DXA, was normal or high. The Slaughter equations significantly underestimated body fat percentages, whereas the precision of the CP-modified Gurka equations was excellent. INTERPRETATION: In this study, children with CP and severe motor impairments displayed stunted growth, but were not undernourished. Relying solely upon BMIs may be misleading in children with CP. Therefore, clinicians should be encouraged to measure SFT and to calculate body fat percentages using the CP-modified version of the Slaughter equation.

Interrelationships between anthropometric variables and overweight in childhood and adolescence.

OBJECTIVES: To answer the questions: how does body mass index (BMI) correlate to five overweight related anthropometric variables during different ages in childhood, and which anthropometric variables contribute most to variation in BMI during childhood? METHODS: Data on BMI, height (H), sitting height (SH), waist circumference (WC), waist to height ratio (WHtR), waist to sitting height ratio (WSHtR), subscapular skinfold (SSF), and triceps skinfold (TSF), from 4,576 Norwegian children 4.00-15.99 years of age, were transformed to standard deviation scores (SDS) and studied using correlation and multiple regression analyses. RESULTS: The correlations between BMI SDS and the standardized anthropometric variables were in general strong and positive. For all variables, the correlations were weakest in the youngest age group and highest between 7 and 12 years. WC SDS and WHtR SDS were most strongly correlated with BMI SDS through all ages and in both sexes. A model with seven anthropometric variables adjusted for age and sex explained 81.4% of the variation in BMI SDS. When adjusted for all other variables, WC SDS contributed most to the variation in BMI SDS (b = 0.467, CI [0.372, 0.562]). Age group, but not sex, contributed significantly to variation in BMI SDS. CONCLUSION: The interrelationships between BMI SDS and five standardized overweight related anthropometric variables were dependent on age, being weakest in the youngest age group. Independent of sex and age, WC SDS was in this study superior to other anthropometric variables in contributing to variation in BMI SDS during childhood.