Resting energy expenditure in females with cystic fibrosis: is it affected by puberty?

OBJECTIVE: The aims of this study were to determine the effect of puberty and the menstrual cycle on resting energy expenditure (REE) in females with cystic fibrosis (CF). DESIGN: Cross-sectional study. All participants had measurements of REE, anthropometry and pubertal staging. The measurements in the postmenarche group were carried out both in the follicular and luteal phases of their menstrual cycle. SETTING: CF outpatient clinic at the Children's Hospital at Westmead. SUBJECTS: Fifty-six females with CF and pancreatic insufficiency (13 postmenarche) were recruited from the hospital clinic and 63 controls (21 postmenarche) were recruited through families and friends of hospital staff. RESULTS: Females with CF had a higher REE than controls (111.6+/-12.8% of predicted from controls P<0.001). There was a significant effect of menarche on REE with a decrease in the postmenarche -470 kJ/24 h compared with premenarche after adjustment for fat-free mass, fat mass and group (control or CF). There was no difference in REE between the follicular and luteal phases for either CF or controls. CONCLUSIONS: Females with CF had raised REE that appeared to be independent of menarche. This study implies all females with CF and pancreatic insufficiency may need more intensive dietary management, owing to raised REE, to maintain growth and nutritional status, and possibly improve survival.

Vitamin D, calcium, and bone status in children with developmental delay in relation to anticonvulsant use and ambulatory status.

Reports of abnormalities in vitamin D, calcium, and bone status associated with anticonvulsant use are inconsistent and difficult to interpret because of widely varying study designs, particularly for ambulatory status. We examined the relative effects of anticonvulsant use and ambulatory status on vitamin D, calcium, and bone status in a large group (n = 338) of children who had either normal motor function (ambulatory) or were nonambulatory and either receiving anticonvulsants or not; all had developmental delays. Data included diet records, serum analyses (calcium and calcidiol), and hand-wrist radiographs evaluated for bone maturation and quality. Data were analyzed by using a general linear models (GLM) procedure. Dietary and biochemical data were compared with those of a group of 34 normal children. There were no differences in calcium or vitamin D intakes among the four study groups; however, a high percentage of intakes was below the recommended dietary allowances for calcium (56%) and vitamin D (70%). Vitamin D intakes were positively associated with serum calcium (P < 0.005) and calcidiol (P < 0.01) concentrations. Analysis of covariance indicated that ambulatory status but neither anticonvulsant use nor their interaction contributed significantly to the prediction of serum calcium (P < 0.009) and calcidiol (P < 0.0001), the Z scores for number of ossified centers (P < 0.008), bone age (P < 0.0001), and bone area (P < 0.003). A strong interaction between anticonvulsant use and ambulatory status was seen for percentage cortical area (P < 0.0008), which was entirely due to anticonvulsant use in nonambulatory children (effect size = 0.98). Results suggest that ambulatory status is more important than was recognized previously in relation to abnormalities in vitamin D, calcium, and bone statuses; that all nonambulatory children may be at risk for low serum calcidiol and osteopenia; and that routine monitoring of risk and consideration of prophylactic vitamin D supplementation are warranted.

Anticonvulsant medication use and circulating levels of total thyroxine, retinol binding protein, and vitamin A in children with delayed cognitive development.

Circulating thyroxine (T4), retinol binding protein (RBP), and vitamin A were measured in conjunction with nutritional status assessment of 707 cognitively delayed children, ages 3.0-9.0 y. Twenty percent were receiving anticonvulsant (AC) medication. T4 was lower and RBP and vitamin A were higher (p less than 0.0001) among AC than non-AC subjects. Molar ratios of vitamin A:RBP did not differ between the two groups nor did intakes of protein or vitamin A. Lower T4 and higher RBP were found among children who received diphenylhydantoin (DPH), phenobarbital, or AC combinations, but vitamin A was higher only among those who received DPH. RBP and vitamin A were lower (p less than 0.05) among children with infections and vitamin A was lower (p less than 0.05) among those with serum zinc less than 70 micrograms/dL (less than 10.7 mumol/L); differences between AC and non-AC subjects remained when other variables were considered.

The obese child.

Forty-five percent of adult obesity cases begin in infancy. Obese children can be identified early; eating and activity patterns are characteristic. It is not known whether obesity is genetically or environmentally determined, but it clearly runs in families; the child of obese parents is at risk. After endogenous obesity has been ruled out, management requires caloric restriction, increased physical activity, and careful record keeping.