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Background: Cystic fibrosis transmembrane conductance regulator (CFTR) modulators improve nutritional status and are of importance in achieving normal growth among younger children with CF. The study was designed to examine CFTR modulator-associated changes in nutrition status, including bile acids and fatty acids after lumacaftor/ivacaftor therapy for 24 weeks., Methods: Children 2 to 5.9 years were recruited from US and Canadian CF Centers. Eligible children were lumacaftor/ivacaftor naïve and approved to initiate therapy. Anthropometrics, diet, energy expenditure, nutrition biomarkers, pancreatic status, serum and fecal calprotectin, serum bile acids and plasma fatty acids were measured. Changes from baseline at 12 and 24 weeks were examined using mixed effects linear regression modeling., Results: Weight-for-age z-score (WAZ) increased at 12 (0.15 ± 0.1, p = 0.01) and 24 weeks (0.23 ± 0.1, p = 0.001) from baseline following modulator therapy. Head circumference-for-age (HCZ) increased at 12 weeks compared to baseline (0.22 ± 0.1, p = 0.03) and subscapular Z score increased from baseline at 24 weeks following therapy (0.33 ± 0.1, p = 0.02). There were no changes in energy expenditure. Serum total bile acids (6.7 ± 2.0, p = 0.001), chenodeoxycholic acid (CDCA) (2.4 ± 1.1, p = 0.001), and cholic acid (CA) (3.5 ± 0.8, p < 0.0001) increased at 24 weeks compared to baseline. Fecal calprotectin decreased at 12 and 24 weeks compared to baseline (-463 ± 310, p = 0.03 and 566 ± 347, p = 0.047). A number of plasma fatty acids changed over the course of 24 weeks of therapy. Noteably, alpha-linolenic acid (ALA) decreased at 12 and 24 weeks (-24 ± 10,p = 0.03 and -18 ± 10, p = 0.02, respectively)., Conclusions: Overall, young children experienced favorable changes in nutritional and growth, with the exception of plasma ALA status in the first 24 weeks of lumacaftor/ivacaftor therapy., Competing Interests: Declaration of competing interest All authors disclose they have no conflicts of interest related to this project and manuscript. The funding sources had no role in study design, analysis or drafting of the manuscript., (Copyright © 2024. Published by Elsevier B.V.)

Patients with cystic fibrosis (CF) are at increased risk of malnutrition and growth failure due to multiple factors as a result of suboptimal or absent function of the CFTR chloride channel protein. Dysfunctional CFTR contributes to increased energy expenditure, exocrine pancreatic insufficiency causing impaired dietary macronutrient digestion and absorption, intestinal dysbiosis, and impaired bile acid homeostasis. Poor nutritional status as a result of these mechanisms is associated with decreased lung function, worse clinical outcomes, and ultimately, increased mortality. Nutritional interventions addressing these mechanisms, such as pancreatic enzyme-replacement therapy and enteral caloric supplementation, have improved nutritional status and, by association, clinical outcomes. In the last decade, the advent of medications targeting defective CFTR proteins has revolutionized the care of patients with CF by reducing the overall impact of CFTR dysfunction. Below, we summarize the effects of highly effective CFTR modulators on nutritional status overall as well as specific factors including bile acid metabolism, pancreatic function, energy expenditure, and intestinal dysbiosis. The future of CF nutrition care will require a paradigm shift away from focusing on methods addressing CFTR dysfunction such as excess calorie provision and toward an individualized, holistic approach in the context of specific mutations and CFTR-directed therapy.

Optimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes., Competing Interests: Dr. Brownell reports grants from Chiesi USA, Inc., grants from AbbVie, Inc., grants from NASPGHAN Foundation, outside the submitted work., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Background: Choline depletion is seen in cystic fibrosis (CF) and pancreatic insufficiency in spite of enzyme treatment and may result in liver, fatty acid, and muscle abnormalities. This study evaluated the efficacy and safety of an easily absorbed choline-rich structured lipid (LYM-X-SORB™ [LXS]) to improve choline status., Methods: Children with CF and pancreatic insufficiency were randomized to LXS or placebo in a 12-month double blind trial. Dietary choline intake, plasma cholines, plasma and fecal phospholipids, coefficient of fat absorption, pulmonary function, growth status, body composition, and safety measures were assessed. Magnetic resonance spectroscopy for calf muscle choline and liver fat were assessed in a subgroup and compared with a healthy comparison group matched for age, sex, and body size., Results: A total of 110 subjects were enrolled (age 10.4 ± 3.0 years). Baseline dietary choline, 88% recommended, increased 3-fold in the LXS group. Plasma choline, betaine, and dimethylglycine increased in the LXS but not placebo (P = 0.007). Plasma lysophosphatidylcholine and phosphatidylcholine increased, and fecal phosphatidylcholine/phosphatidylethanolamine ratio decreased (P ≤ 0.05) in LXS only, accompanied by a 6% coefficient of fat absorption increase (P = 0.001). Children with CF had higher liver fat than healthy children and depleted calf muscle choline at baseline. Muscle choline concentration increased in LXS and was associated with improvement in plasma choline status. No relevant changes in safety measures were evident., Conclusions: LXS had improved choline intake, plasma choline status, and muscle choline stores compared with placebo group. The choline-rich supplement was safe, accepted by participants, and improved choline status in children with CF.

Objectives: Unexpectedly high serum B12 concentrations were noted in most study subjects with cystic fibrosis (CF) and pancreatic insufficiency (PI) participating in a nutrition intervention at the baseline evaluation. The objectives of this study were to determine dietary, supplement-based, and enzyme-based B12 intake, serum B12 concentrations, and predictors of vitamin B12 status in children with CF and PI., Study Design: Serum B12 status was assessed in subjects (5-18 years) and categorized as elevated (serum B12 above reference range for age and sex [Hi-B12]) or within reference range (serum B12 within reference range for age and sex) for age and sex. Serum homocysteine, plasma B6, red blood cell folate, height, weight, and body mass index z scores, pulmonary function, energy, and dietary and supplement-based vitamin intake were assessed., Results: A total of 106 subjects, mean age 10.4 ± 3.0 years, participated in the study. Median serum B12 was 1083 pg/mL, with 56% in the Hi-B12 group. Dietary and supplement-based B12 intakes were both high representing 376% and 667% recommended dietary allowance (RDA), respectively. The Hi-B12 group had significantly greater supplement-based B12 intake than the serum B12 within reference range for age and sex group (1000% vs 583% RDA, P < 0.001). Multiple logistic regression analysis showed that high supplement-based B12 intake and age >12 years increased the risk of Hi-B12, whereas higher forced expiratory volume at 1 second (FEV1) decreased the risk (pseudo-R = 0.18, P < 0.001)., Conclusions: Serum B12 was elevated in the majority of children with CF and PI. Supplement-based B12 intake was 6 to 10 times the RDA, and strongly predicted elevated serum B12 status. The health consequences of lifelong high supplement-based B12 intake and high serum B12 are unknown and require further study, as does the inversed correlation between serum B12 and forced expiratory volume at 1 second.

Background: Suboptimal vitamin A status is prevalent in children with type SS sickle cell disease (SCD-SS) and is associated with hospitalizations and poor growth and hematologic status. The supplemental vitamin A dose that optimizes suboptimal vitamin A status in this population is unknown., Objective: The efficacy of Recommended Dietary Allowance (RDA) doses (based on age and sex) of vitamin A (300, 400, or 600 μg retinyl palmitate/d) or vitamin A + zinc (10 or 20 mg zinc sulfate/d) compared with placebo to optimize vitamin A status was assessed in children aged 2.0-12.9 y with SCD-SS and a suboptimal baseline serum retinol concentration (<30 μg/dL)., Design: In this randomized, double-blind, placebo-controlled trial, vitamin A status (serum retinol, prealbumin, retinol-binding protein, and relative-dose-response test) and disease-related illness events were assessed., Results: Twelve months of vitamin A supplementation at the doses recommended for healthy US children (based on age and sex) failed to improve serum retinol values in either group (vitamin A: n = 23; vitamin A + zinc: n = 18) compared with placebo (n = 21). By 12 mo, the increase (±SD) in serum retinol (3.6 ± 2.8 μg/dL) in those taking 600 μg vitamin A/d was significantly different from the decrease (±SD; -2.8 ± 2.4 μg/dL) in those taking 300 μg/d, which possibly suggests a dose-response relation (P < 0.05) with RDA doses., Conclusions: Compared with placebo, 12 mo of vitamin A supplementation at the RDA for healthy children did not improve serum retinol values in children with SCD-SS, which possibly suggests that higher doses are needed. However, the existence of alternative conclusions emphasizes the need for future research.

Background: For children and adolescents with cystic fibrosis (CF) and pancreatic insufficiency, the efficacy of routine vitamin K supplementation to normalize vitamin K status remains unclear., Objective: This study examined and determined predictors of vitamin K status in subjects aged 8-25 y with CF and pancreatic insufficiency taking various vitamin K supplements., Design: In 97 subjects, serum 25-hydroxyvitamin D [25(OH)D], dietary intake, vitamin K supplement intake, and vitamin K statusmdashdetermined on the basis of the percentage of serum undercarboxylated osteocalcin (%ucOC; sufficient: lt 20%) and plasma proteins induced by vitamin K absence-factor II (PIVKA-II; n = 60; sufficient: le 2 microg/L)mdashwere assessed. The vitamin K supplementation groups were as follows: lt 150 microg/d (low; multivitamins or no supplement), 150-999 microg/d (middle; CF-specific vitamins), and ge 1000 microg/d (high; mephyton). %ucOC values were compared with 140 healthy subjects aged 6-21 y., Results: In subjects with CF, the median (range) %ucOC was 35% (3%, 76%) and the median (range) for PIVKA-II was 2 (0, 42) micro g/L. Subjects with CF had a higher %ucOC with low [45% (10%, 76%)] and medium [41% (3%, 66%)] supplement intakes but not with a high supplement intake [16% (4%, 72%)] compared with healthy subjects [23% (0%, 43%); both P lt 0.05]. Supplementation group for males and females and 25(OH)D and age for males were significant predictors of vitamin K status., Conclusions: Vitamin K status was often suboptimal despite routine supplementation. Only subjects taking high-dose vitamin K achieved a status similar to healthy subjects, and only the vitamin K supplementation dose predicted vitamin K status for males and females. These data suggest that higher doses of vitamin K are required.

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.

The Cystic Fibrosis Foundation established a process of systematic review of evidence to inform the development of clinical care guidelines and encourage evidence-based practice. The Subcommittee on Growth and Nutrition reviewed the evidence in two areas: energy intake and dosing for pancreatic enzyme replacement therapy. Evidence-based recommendations are presented here. Also, an ad hoc working group conducted a review of the literature and performed new analyses using the Cystic Fibrosis Foundation Patient Registry to update the recommendations for growth and weight-status monitoring. These Registry data-based recommendations are presented.

Previous studies of children with sickle cell disease (SCD) reported poor growth and delayed maturation. However, the prevalence, magnitude, and correlates of suboptimal growth remain poorly understood. A prospective longitudinal study was undertaken to determine the effects of disease severity and nutritional status on growth, an indicator of childhood well-being. Children, birth to 18 y of age, with SCD-SS were evaluated annually for 4 y. Growth, nutritional status, skeletal and sexual maturation, disease severity, dietary intake, and maternal education were assessed. In this sample of 148 children (78 females), growth in height, weight, or body mass index declined in 84% of subjects; 38% fell below the 5th percentile in one or more measures. Puberty was delayed 1 to 2 y, and median age at menarche was 13.2 y. Skeletal age was delayed by 0.7 +/- 1.4 y overall and by 1.3 +/- 1.5 y in children 10 to 15 y old. Height status declined over time and was positively associated with advancing puberty and hematological measures in girls, and nutritional status in girls and boys. Growth failure and maturational delay remain significant chronic problems in children with SCD-SS and are related to potentially modifiable factors such as nutritional status.

Purpose: The aim of this study was to describe vitamin D status in children with intractable epilepsy prescribed newer antiepileptic drugs (AEDs) before initiation of and during 15-month treatment with the ketogenic diet (KD)., Methods: Serum vitamin D (25-OHD and 1,25-OHD) and parathyroid hormone (PTH) were assessed in prepubertal children with intractable epilepsy before initiation of and during KD therapy. Three-day weighed dietary records including KD and vitamin and mineral supplementation were obtained at baseline and at 1 month., Results: Forty-five children (aged 5.1 +/- 2.7 years) were enrolled. Before KD therapy, 4% had deficient and 51% had insufficient serum 25-OHD levels. Vitamin D intake was less than recommended in 47%. Adequate vitamin D intake, fewer AEDs, and generalized seizures were associated with higher serum 25-OHD levels (p < 0.01). After 3 months on the KD, 25-OHD levels increased (p < 0.001), and PTH declined (p < 0.001). Over the next 12-month period, 25-OHD levels steadily declined (p < 0.001), and PTH did not significantly change., Conclusions: Children with intractable epilepsy treated with newer AEDs had poor vitamin D status. Their status improved over the first 3 months of KD therapy with vitamin D supplementation and slowly declined thereafter.

Background and Aims: Poor nutrition and growth status are common in people with cystic fibrosis (CF), and females often have a worse clinical course. Relationships between sexual maturity, nutrition, resting energy expenditure (REE), and pulmonary status in females with CF and pancreatic insufficiency (PI) were evaluated., Methods: Pre- and post-menarcheal females with CF and PI (8-29 yr) were compared to healthy females. Z-scores for growth and body composition, anthropometry and DEXA were assessed. REE was measured in all subjects and pulmonary function in CF., Results: Compared to healthy females (n=28, 14.6+/-4.1 yr), females with CF (n=16, 14.7+/-4.4 yr) had lower height Z (-0.1+/-0.9 versus -0.9+/-0.9, P=0.009) and muscle area Z (0.8+/-1.3 versus -0.4+/-1.2, P=0.007), and higher REE (100+/-10 versus 110+/-11% predicted, P=0.008). Difference in REE was more pronounced for post-menarcheal girls. REE adjusted for fat and fat-free mass was significantly higher with CF (+110 calories/day), and declined with menarcheal age in all subjects. FEV1 was positively associated with BMI Z score, and negatively associated with age at menarche., Conclusions: Height and muscle stores were reduced and REE elevated in subjects with CF compared to healthy controls. Poorer growth and nutritional status and delayed menarche were associated with poorer pulmonary function in CF and were likely related to the cumulative effect of energy imbalance on growth and body composition.

Objectives: To evaluate fecal elastase 1 (FE) levels in young children with cystic fibrosis and pancreatic insufficiency and to explore the relationship between FE and growth, nutrition, pulmonary status and fat absorption over a 24-month period., Methods: FE, indicating pancreatic lipase activity, was assessed in children (6.0 to 8.9 years of age) with cystic fibrosis and pancreatic insufficiency. FE >15 microg/g stool was defined as residual pancreatic activity, suggesting some pancreatic function, and FE

Objective: To qualitatively evaluate parent perspectives of eating problems, nutritional status, and the potential for nutritional intervention in children with sickle cell disease (SCD)., Methods: Twenty parents of children with SCD participated in one of three focus groups to discuss questions related to the study's objectives. Three coders rated transcripts to identify common perceptions and experiences (themes) among participants., Results: Poor appetite and its impact on nutritional and general health were particular concerns for parents. Parents addressed eating challenges with dietary supplementation, limit setting, and compromising at meals, often without consultation from health professionals. An intervention program should include facilitators, such as flexible scheduling and incentives to counter barriers, such as scheduling and child care conflicts., Conclusions: Parents of children with SCD reported a range of eating behavior challenges. Parents presented ideas on how nutritional intervention could be culturally sensitive and on how to promote participation in such programs.

Objectives: To describe nutritional status in a population-based sample of children with moderate or severe cerebral palsy (CP) and to explore the relationships between nutritional status and health and functional outcomes., Study Design: A population-based strategy was used to enroll children with CP at 6 geographic sites. Research assistants performed anthropometric assessment, determined severity of motor impairment, and interviewed caregivers with the Child Health Questionnaire and a questionnaire designed specifically for this study. Anthropometric measures were converted to Z scores and the relationship between health and nutritional status was assessed using regression models., Results: Among the 235 participants, indicators of malnutrition were common. Poor nutritional status correlated with increased health care utilization (hospitalizations, doctor visits) and decreased participation in usual activities by the child and parent., Conclusions: Malnutrition is common in children with moderate or severe CP and associated with poorer health status and limitations in societal participation. Further studies are needed to determine the nature of these associations and how to manage nutrition in children with CP to optimize growth and health outcomes.

Purpose: In vitro, vitamin B(6) has antisickling properties, but the effect of vitamin B status on the health of children with sickle cell disease-SS (SCD-SS) is not well described. The purpose of this study was to assess vitamin B(6) status of children with SCD-SS ages 3 to 20 years and determine its relationship to growth, dietary intake, and disease severity., Patients and Methods: Vitamin B(6) status was assessed by serum pyridoxal 5-phosphate (PLP) concentration in subjects with SCD-SS and by urinary 4-pyridoxic acid (4-PA) concentration in other subjects with SCD-SS and healthy control children. Concentration of PLP was compared with anthropometric measures of growth and nutritional status, dietary intake, hematologic indices, and frequency of SCD-related illness., Results: The PLP concentration of subjects with SCD-SS was 15.6 +/- 15.2 nmol/L. Seventy-seven percent had a PLP concentration below the deficiency criterion (20 nmol/L) suggested by the Dietary Reference Intakes (1998). Controlling for alkaline phosphatase, age, and gender, PLP concentration was associated positively with weight, body mass index, and arm circumference -scores and negatively with reticulocyte count. Urinary 4-PA was lower in children with SCD-SS versus controls, although 4-PA/creatinine values did not differ between groups., Conclusions: Children with SCD-SS had apparently low serum PLP concentrations in the absence of excess vitamin B(6) excretion, suggesting low vitamin B(6) status. Low serum PLP concentration was associated with indicators of poor nutritional status and may be related to increased hemolysis in children with SCD-SS.

Objective: To determine prospectively the relationship among growth, nutritional status, and pulmonary function over a 4-year period in a large cohort of children with cystic fibrosis (CF)., Study Design: CF Foundation National CF Patient Registry data collected from 1991 to 1995 for 968 children (507 male) aged 5 to 8 years with pancreatic insufficiency and forced expiratory volume in 1 second within 60% to 140% of predicted values (FEV(1)%) were analyzed longitudinally. Variables hypothesized to affect FEV(1)% included age, sex, z scores for height, weight, percent of height-appropriate body weight, and annual number of days hospitalized., Results: The significant decline in FEV(1)% was curvilinear and dependent on baseline FEV(1)%; children with initial FEV(1)% > or = 90 declined 2.6 U/y more than those with initial FEV(1)% <90. Boys gained but girls declined in z scores for height. Girls decreased in z scores for weight at a greater rate than boys. The z scores for weight and percent of height-appropriate body weight were significantly associated with longitudinal changes in FEV(1)%, after adjustment was done for hospitalizations., Conclusions: Growth, nutritional status, and pulmonary function are not stable in prepubertal children with CF and pancreatic insufficiency. Important sex-related differences in growth occur before puberty. Growth and nutritional status are associated with changes in FEV(1)%, suggesting that nutritional intervention may slow the decline in pulmonary function in children with CF.

Background: Several cross-sectional studies have shown improvement in the growth of children with cystic fibrosis (CF) because of increased awareness of and more comprehensive care of their special nutritional needs. However, longitudinal data on the nutritional status of these children are rare., Objective: The objective was to compare changes in growth, body composition, and nutritional status between children with and without CF., Design: This was a prospective 3-y cohort study of 25 children aged 5-10 y with CF, mild pulmonary disease, and pancreatic insufficiency and of 26 healthy control children. Three methods were used to assess body composition: measurements of skinfold thickness, total body water by deuterium oxide, and total-body electrical conductivity. Growth and body-composition changes over time were analyzed by a longitudinal mixed-effects model., Results: Over the 3 y of the study, the statural growth of the boys with CF was slower than that of the control subjects (P = 0.004). The same divergence over time between the boys with and without CF was observed for fat-free mass assessed by skinfold-thickness measurements and total body water (P = 0.008 and 0.02, respectively) and for fat mass assessed by skinfold-thickness measurements and total-body electrical conductivity (P = 0.009 and 0.001, respectively). The differences in the pattern of changes in growth and body composition were less striking for girls., Conclusions: Despite comprehensive care, the growth of boys with CF was impaired on the basis of height, fat-free mass, and fat mass, when observed longitudinally. Caution should be used when interpreting cross-sectional measurements because they often do not detect suboptimal growth.

Objective: To examine growth, body composition, and nutritional status in a large sample of children, adolescents, and young adults with Crohn's disease (CD)., Methods: One hundred thirty-two subjects (48 females) with CD, aged 5 to 25 years, and 66 healthy control subjects (37 females) of similar age. Growth, nutritional status, and body composition were measured by anthropometry and dual-energy x-ray absorptiometry. Genetic potential of linear growth was predicted using the adjusted heights of Himes et al. Pubertal status and skeletal age were assessed. Average Pediatric Crohn's Disease Activity Index (PCDAI) and lifetime steroid exposure (in milligrams per day) were obtained from medical charts. The variables were examined in relation to gender and measures of disease activity., Results: Males and females with CD did not differ by age, disease duration, or PCDAI. Males with CD had significantly lower values for growth and nutritional status than control subjects (z-scores for weight: -0.66 +/- 1.18 vs. 0.26 +/- 0.95, P = 0.00002; height -0.81 +/- 1.14 vs. 0.28 +/- 0.93, P = 0.00001; adjusted height -1.05 +/- 1.03 vs. 0.40 +/- 1.03, P = 0.00001) and delayed skeletal age of 0.9 +/- 1.6 years. Impaired linear growth in the males was present regardless of pubertal stage. Associations between disease severity indicators and growth parameters were more consistent for females., Conclusions: Crohn's disease is associated with impaired growth. Despite similarities in disease duration, activity, and lifetime steroid exposure, growth in males was more impaired. Gender may confer risk for impaired growth in CD.

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.

Growth failure and malnutrition are common clinical features in cystic fibrosis (CF), but the relationships among resting energy expenditure (REE), pulmonary function, and nutritional status, are poorly understood. To better understand these relationships, REE, growth, nutritional status, and pulmonary function were measured prospectively in 25 prepubertal children with CF and 26 prepubertal control subjects of similar age and gender over a 3-y period. All subjects with CF had pancreatic insufficiency and mild pulmonary disease. REE was elevated for the CF children compared with control subjects throughout the study. This increased REE was not associated with declining pulmonary function. Longitudinal analyses revealed different patterns of change over time in boys and girls, such that REE significantly increased in the girls with CF and pulmonary function decreased in the boys. Boys with CF experienced a decline in weight Z score and percent ideal body weight, whereas the girls with CF experienced a decline in height Z score. Pulmonary function was not associated with REE, but nutritional status (percent ideal body weight) and genotype (delta F508 homozygotes versus others) were predictive of changes in pulmonary function over time. Fat free mass and height were found to be the best predictors of REE, and after accounting for these important body size and composition variables, differences in REE between boys and girls and CF and control groups increased over time. These findings identify the importance of investigating gender differences in the course of disease and considering REE as an early indicator of disease severity independent of pulmonary function.

Objective: To determine the pattern of body composition and nutritional status in a group of prepubertal children with spastic quadriplegic cerebral palsy (SQCP) compared with healthy control children., Study Design: Subjects were enrolled for this cross-sectional study from two tertiary care settings. One hundred thirty-six subjects with SQCP, 2 to 12 years of age, were evaluated by anthropometric measures, or by anthropometric and total body water (TBW) measures (n = 28), with 39 control subjects., Results: Body composition and nutritional status indicators were significantly reduced in children with SQCP. Accretion of fat-free mass with age was smaller for children with SQCP. Calculation of body fat from two skin folds correlated best with measures of fat mass from TBW., Conclusion: Malnutrition is common in children with SQCP. Clinically available, serial anthropometric measures enable the clinician to identify malnourished children with SQCP.

The growth failure and nutritional status were assessed of 154 children with diplegic or hemiplegic cerebral palsy, aged between two and 17.4 years. Linear growth was significantly reduced compared with norms for healthy children. About 30 per cent of the sample were undernourished, indicated by bodyweight or depleted subcutaneous fat stores at the triceps skinfold site. 8 per cent were overly fat by triceps skinfold and 14 per cent overweight by bodyweight. 23 per cent of the children had stunted growth. Children in the youngest age-group were most at risk for poor nutritional status and delayed growth. These findings suggest that children with diplegic or hemiplegic cerebral palsy are at risk for a variety of abnormalities of growth and nutritional status, and that a growth and nutritional assessment should be conducted periodically so that under- or overnutrition can be corrected when identified.