Nutritional rehabilitation increases the resting energy expenditure of malnourished children with severe cerebral palsy.

AIM: The aim of this study was to measure resting energy expenditure (REE) and energy intake in children with quadriplegic cerebral palsy (CP), to relate these to anthropometric measurements, and to determine the influence of nutritional rehabilitation on REE. METHODS: Fifty-six children (20 females, 36 males; age range 3y 11mo-18y; mean age 10y; SD 3y 11mo) with CP (Gross Motor Function Classification System level V) participated in this cross-sectional study. Children were excluded if they had a known metabolic disorder, genetic syndrome, or chromosomal abnormality. Thirty-three of the children were tube fed and 23 were fed orally. A comparison group comprised 111 (42 females, 69 males) healthy children who had undergone anthropometric and REE measurements and were of similar age to the children with CP (4-19y). REE was measured by indirect calorimetry and energy intake was determined from weighed food records. RESULTS: The REE in the children with CP was low (79.5%) compared with that predicted and highly variable (SD 38.4%). Fat-free mass was the strongest predictor of REE, accounting for 27% of the variation. Energy intake as a percentage of REE in was greatly overestimated in oral-fed children with CP (293%). In a subset of children with CP (n=14), an increased energy intake by gastrostomy tube feeding resulted in an increase in REE from 70.0% to 101.9% of that predicted. INTERPRETATION: The REE of children with CP is low and variable and is not strongly related to any one anthropometric measurement. Food records in oral-fed children with CP are of little value owing to their inaccuracy. This study provided support for the hypothesis that the low REE found in malnourished children with CP is partly due to a low energy intake.

Longitudinal analysis of resting energy expenditure in patients with cystic fibrosis.

OBJECTIVE: To assess whether elevated resting energy expenditure (REE) in female patients with cystic fibrosis (CF) persists longitudinally during late childhood and puberty. STUDY DESIGN: REE and body composition were measured 3 times in 2 years in 86 children with CF. Pubertal status, bacterial colonization, liver disease, pancreatic and pulmonary function, and genotype were determined, and linear mixed model analyses were used to determine predictors and changes in REE longitudinally. RESULTS: REE did not change with time allowing for fat free mass, pancreatic insufficiency (PI), or severe mutations. Pulmonary function and liver disease were not significant predictors of REE. Percentage predicted REE compared with control data was higher (P = .002) in female patients with CF (109.5%) and lower in male patients with CF (104%) and persisted with time. In post-menarchal female patients with CF, REE adjusted for fat free mass was 366 kJ/d lower than in pre-menarchal female patients, but still 112% predicted. CONCLUSIONS: This longitudinal study demonstrates that REE is elevated in patients with CF with PI and severe mutations. The elevation of percentage predicted REE was greater in female patients than male patients and persisted for 2 years, and during pubertal maturation, independent of pulmonary and liver disease. These results highlight the need for a high-energy diet throughout childhood and adolescence, particularly in female patients with PI.

Total body protein in healthy adolescent girls: validation of estimates derived from simpler measures with neutron activation analysis.

BACKGROUND: Little recent and accurate information about body protein content in healthy adolescent girls is available. OBJECTIVE: The objective was to assess the total body nitrogen (TBN) and total body protein (TBPr) contents of fat-free mass (P:FFM) in a group of healthy adolescent girls and to validate previously published TBN prediction equations. DESIGN: TBN was measured with in vivo neutron activation analysis (TBNNAA). Bone mineral density and FFM were measured with dual-energy X-ray absorptiometry (FFMDXA), total body water and FFM were measured with bioimpedance analysis, and FFM was assessed by measuring skinfold thicknesses in 51 girls with a mean (+/- SD) age of 14.7 +/- 0.7 y. The validity of the TBN prediction equations was assessed with Bland-Altman analysis. RESULTS: TBNNAA in our adolescent group was higher (1.49 kg) than values reported in earlier studies of women (1.25 and 1.31 kg), and P:FFM was slightly higher (23%) than that documented in adults (19-21%). Previously published TBN equations showed either systematic bias or wide limits of agreement. CONCLUSION: A predictive equation derived from the present study population based on FFMDXA improves the prediction of TBN for groups of young girls but may not be helpful for individuals in clinical settings.

Reduced body protein in children with spastic quadriplegic cerebral palsy.

BACKGROUND: No studies have directly measured body protein or validated skinfold-thickness anthropometry and dual-energy X-ray absorptiometry (DXA) to assess body protein in children with spastic quadriplegic cerebral palsy (SQCP). OBJECTIVE: We aimed to measure and evaluate body protein and to determine whether skinfold-thickness anthropometry and DXA can predict body protein in children with SQCP. DESIGN: This was a cross-sectional study of 59 children (22 girls, 37 boys) aged 3.9-19.5 y with SQCP. The children underwent measurements of anthropometric indexes, lean tissue mass by DXA (LTM(DXA)), and total body protein by neutron activation analysis (TBP(NAA)). In addition, TBP was estimated from both skinfold-thickness anthropometry (TBP(SKIN)) and DXA (TBP(DXA)). The agreement of TBP(SKIN) and TBP(DXA) was tested against TBP(NAA) by using Bland and Altman plot analysis. RESULTS: Height and weight SD scores (x +/- SD: -3.1 +/- 1.6 and -4.8 +/- 5.3, respectively) were significantly lower than reference data in the children with SQCP (P < 0.001). TBP(NAA) for age and height was low in the children with SQCP (P < 0.001): 56.1 +/- 17.3% and 81.5 +/- 15.7%, respectively, of the values predicted from control data. TBP(SKIN) and TBP(DXA) were both highly correlated with TBP(NAA): r = 0.90, P < 0.001, and r = 0.91, P < 0.001, respectively. Despite these significant correlations, agreement analyses showed wide variation of up to 33.3% of the mean for both methods. CONCLUSIONS: Body protein in children with SQCP is significantly reduced for age and height. Skinfold anthropometry and DXA show wide variation in estimation of body protein compared with NAA in this group of children.

Dietary management of galactosemia.

Galactosemia is detected by newborn screening in New South Wales and managed by the metabolic team at the Children's Hospital at Westmead. Infants with the Duarte variant are not treated. Management is based on the Handbook for Galactosemia prepared in 1998. This handbook provides information for the family on the dietary management, inheritance and ovarian function. The major dietary sources of galactose are milk and milk products. Breastfeeding must be ceased and replaced with a soy formula. Once solid foods are commenced certain foods should be avoided. Other foods, which may contain some free galactose are recommended in limited quantities only. There is no restriction on other fruits and vegetables. An ongoing issue with dietary management is adequate nutrient intake, particularly of calcium. Intake of milk substitutes and calcium supplements is often inadequate.

Body composition changes in female adolescents with anorexia nervosa.

BACKGROUND: Body weight provides limited information about nutritional status of patients with anorexia nervosa (AN). OBJECTIVES: Our objectives were to determine body composition (BC) changes, to find clinical predictors and endocrine correlates of total body protein (TBPr) depletion, and to compare results on fat mass (FM) obtained with anthropometry (skinfold measurements) and dual-energy X-ray absorptiometry (DXA) in patients with AN. DESIGN: Body weight, body mass index (BMI; in kg/m(2)), BC (with DXA and skinfold measurements), and TBPr [with in vivo neutron activation analysis (IVNAA)] was assessed in 50 AN patients (15.2 y) and 40 healthy sex- and age-matched controls. In 47 AN patients and 22 controls, hormone concentrations were measured. RESULTS: In AN patients, body weight (44.4 +/- 5.5 kg), BMI (16.7 +/- 1.6), and FM(DXA) (7.0 +/- 3.4 kg) were lower than in controls. Lean tissue mass by DXA (LTM(DXA)) was similar in AN patients and controls (35.7 +/- 4.3 compared with 35.8 +/- 4.5 kg), but TBPr was 87% of that of controls (8.1 +/- 1.0 compared with 9.2 +/- 1.2 kg; P < 0.001). Cortisol was high, testosterone was unchanged, and estradiol and insulin-like growth factor I were low. Severe protein depletion measured by IVNAA seen in 17 AN patients could not be identified with simpler methods. All except 1 of 26 AN patients with a BMI > 16.5 had normal TBPr. The difference in individual percentage of body fat measured with DXA and skinfold measurements came up to 9%. CONCLUSION: The severe protein depletion in 34% of AN patients was not accurately identified by LTM(DXA) or simpler methods, but a BMI > 16.5 indicated normal TBPr. Future studies need to compare DXA and skinfold measurements with a reference technique to assess FM in AN patients.

Differences in resting energy expenditure between male and female children with cystic fibrosis.

OBJECTIVES: To evaluate which factors might contribute to raised resting energy expenditure (REE) in patients with cystic fibrosis (CF). STUDY DESIGN: REE and anthropometry were measured in 134 (males = 68) children with CF and 100 (males = 51) controls (range, 3-18.7 years) in an outpatient setting. Bacterial colonization, liver disease, inhaled steroid use, pancreatic and pulmonary function, sex, and genotype were determined and regression analysis was used to determine the predictors of REE in the group with CF. RESULTS: REE for children with CF was increased on average by 7.2% compared with controls. This increase was greater for females than for males. REE in males was positively associated with fat-free mass (FFM), pancreatic insufficiency (PI), and liver disease, and negatively associated with pulmonary function, whereas in females, REE was positively associated with FFM and PI. REE (adjusted for FFM) was higher in children with a severe mutation (5495 +/- 47 kJ) compared with a mild mutation (5,176 +/- 124 kJ, P <.02). CONCLUSIONS: PI, severe mutations, and female sex are the main contributing factors to elevated REE in patients with CF with near normal pulmonary function.