Article

Growth failure in children with cystic fibrosis

Department of Pediatrics, University of Chieti, Italy.
Journal of pediatric endocrinology & metabolism: JPEM (Impact Factor: 0.71). 06/2012; 25(5-6):393-405. DOI: 10.1515/jpem-2012-0012
Source: PubMed

ABSTRACT Poor linear growth and inadequate weight gain are very common problems in cystic fibrosis (CF) children. The most important factors involved in growth failure are undernutrition or malnutrition, chronic inflammation, lung disease, and corticosteroid treatment. Nutritional support and pharmacological therapy with recombinant human growth hormone are essential for a good management of children with CF, although these children are shorter and lighter than healthy children, and despite the catch-up growth observed after diagnosis, deficit in length/height and weight continues to be seen until adulthood. Early diagnosis is essential to ensure better nutritional status and growth, potentially associated with better respiratory function and prognosis. The aims of this review are try to explain etiology and pathogenetic mechanisms of growth failure in CF children and clarify their role in the disease morbidity and in clinical outcome, especially in relation to progressive decline of pulmonary function.

0 Followers
 · 
116 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Sleeping Metabolic Rate (SMR) is used as a proxy for Basal Metabolic Rate in infants, when measurement while awake is not practical. Measuring SMR via indirect calorimetry (IC) can be useful for assessing feeding adequacy especially in compromised neonates. Standard IC equipment, including the hood placed over the head, is not designed for the smallest of patients. Our aim was to determine whether a smaller, non-standard hood measures SMR in neonates similarly compared to a standard large hood. SMR was measured in healthy neonates (controls) and those born with single ventricle congenital heart disease (cases). Two measurements were performed: SMR using a standard large hood (LH) and SMR using a smaller hood (SH). Time to steady state, minute ventilation (VE), and expired carbon dioxide (FECO2) - an indicator of data quality - were also measured. Primary outcome was SMR using both hoods. Results are stated as Median (IQR). Spearman correlations measured association between SH and LH. We studied nine controls and seven cases. SMR in controls was not different between SH and LH [35.7 (15.14) vs. 37.8 (7.41) kcals/kg/day, respectively]. In cases, SH was significantly greater than LH [45.5 (4.63) vs. 34.2 (8) kcals/kg/day, p<0.02]. FECO2 was significantly higher in SH versus LH in both groups, and VE was significantly lower in SH versus LH in controls only. SMR values for SH and LH were significantly correlated in the control group (r = 0.80, p < 0.01). Time to steady state was similar in both groups regardless of hood size. SMR measured with a small hood yields results similar to those measured with a standard large hood in healthy neonates without affecting testing time or other aspects of the IC procedure. Furthermore,resultsin compromised infants suggest that a smaller hood may facilitate SMR testing in this population.
    11/2013; 59(7). DOI:10.4187/respcare.02636
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Previous studies reported a wide range of estimated malnutrition prevalence (6-30%) in paediatric inpatients based on various anthropometric criteria. We performed anthropometry in hospitalised children and assessed the relationship between malnutrition and length of hospital stay (LOS) and complication rates. In a prospective multi-centre European study, 2567 patients aged 1 month to 18 years were assessed in 14 centres in 12 countries by standardised anthropometry within the first 24 h after admission. Body mass index (BMI) and height/length <-2 standard deviation scores (SDS, WHO reference) were related to LOS (primary outcome), frequency of gastrointestinal (diarrhoea and vomiting) and infectious complications (antibiotic use), weight change during stay (secondary outcomes) and quality of life. A BMI <-2 SDS was present in 7.0% of the patients at hospital admission (range 4.0-9.3% across countries) with a higher prevalence in infants (10.8%) and toddlers aged 1-2 years (8.3%). A BMI <-2 to ≥-3 SDS (moderate malnutrition) and a BMI <-3 SDS (severe malnutrition) was associated with a 1.3 (CI95: 1.01, 1.55) and 1.6 (CI95: 1.27, 2.10) days longer LOS, respectively (p = 0.04 and p < 0.001). Reduced BMI <-2 SDS was also associated to lower quality of life, and more frequent occurrence of diarrhoea (22% vs 12%, p < 0.001) and vomiting (26% vs 14%, p < 0.001). Disease associated malnutrition in hospitalised children in Europe is common and is associated with significantly prolonged LOS and increased complications, with possible major cost implications, and reduced quality of life. This study was registered at clinicaltrials.gov as NCT01132742.
    Clinical nutrition (Edinburgh, Scotland) 01/2014; 34(1). DOI:10.1016/j.clnu.2014.01.003 · 3.94 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Human immunodeficiency virus (HIV) infected youth are healthier due to effective antiretroviral therapies (ARVs). We compared anthropometric measurements and prevalence of overweight and obesity between perinatally-HIV-infected youth, a local HIV-uninfected comparison group and 2007-2010 National Health and Nutrition Examination Survey (NHANES) data. In addition, we compared only African American HIV-infected to NHANES African American. Height, weight, body mass index (BMI) and waist circumference (WC) of HIV-infected youth, age 10-19 years, were compared among groups. BMI percentiles were categorized as underweight (≤5%), normal (5-<85%), overweight (85-<95%), and obese (≥95%). Clinical correlates were modeled as predictors of BMI and WC. 134 HIV-infected (including 103 African Americans) (mean age 16.5y), 75 HIV-uninfected (mean age 14.2y), and 3216 NHANES (including 771 NHANES African Americans) (mean age 15.0y) youth were included in the analysis. Height and weight z-scores of HIV-infected were lower than HIV-uninfected and NHANES (P ≤ 0.056). BMI, WC, and BMI category were not statistically different between groups. In the HIV-infected African American group, BMI z-score was lower (0.49 vs 0.76; P = 0.04) compared to NHANES African American. There were no significant predictors of BMI or WC for the HIV-infected group. HIV-infected children have similar BMIs and WCs as uninfected children both locally and nationally and show similar high rates of obesity and overweight. However, when compared to a more racially similar African American national sample, HIV-infected children have a lower BMI, suggesting that there may be persistent anthropometric differences in HIV.
    Journal of pediatric gastroenterology and nutrition 04/2014; 59(4). DOI:10.1097/MPG.0000000000000394 · 2.87 Impact Factor