Objective To determine whether oral protein energy supplements, used long term in children with cystic fibrosis who are moderately malnourished, improve nutritional and other outcomes. Design Multicentre randomised controlled trial. Setting Seven specialist paediatric cystic fibrosis centres and their associated shared care clinics and seven smaller paediatric cystic fibrosis clinics. Participants 102 children with cystic fibrosis, aged between 2 and 15 years, who were moderately malnourished. Interventions Oral protein energy supplements in addition to usual dietary advice compared with dietary advice alone, for 12 months. Main outcome measure Change in body mass index centile over one year. Results Use of supplements was not associated with a change in body mass index centile (mean difference 2.99 centile points, 95% confidence interval − 2.70 to 8.68) or other nutritional and spirometric outcomes in this group of children. Conclusions Long term use of oral protein energy supplements did not result in an improvement in nutritional status or other clinical outcomes in children with cystic fibrosis who were moderately malnourished. Oral protein energy supplements should not be regarded as an essential part of the management of this group of children. Trial registration ISRCTN: 95744468.

Aims-To assess the reliability of percentage ideal weight for height (%WFH) as a measure in children. The expression of body weight as a percentage of ideal weight for height, gender, and age (%WFH) has been suggested by the American Cystic Fibrosis Foundation as a useful measure of nutritional status in children with cystic fibrosis (CF). Methods-Forty1 It is also used regularly as an outcome measure in CF research and as a measure of nutritional status in other areas of paediatrics including liver disease and oncology. [2][3][4][5] %WFH has the advantage of integrating both weight and height in the same measure and can easily be calculated. However, we have noticed variability between individuals in the calculation of %WFH. A comprehensive literature review has failed to identify any work on the reliability of this measure. Therefore, the aim of this study was to investigate the reliability of using %WFH as a measure of nutritional status. MethodsFifty two members of UK CF Dietitians Interest Group were sent a questionnaire examining the type of measures which they used to assess nutritional status. If they used %WFH they were asked how frequently they did so and by which method.Each dietitian was asked to complete %WFH calculations based on the following details of six CF patients: date of birth, sex, height, weight, and the date on which the height and weight were taken. Growth charts were provided, as were the US CF Foundation instructions for calculating %WFH:1. Plot the height on the growth chart and determine height centile. 2. Determine the weight that corresponds to the same point on the centile chart as the height. 3. Divide actual weight by ideal weight, then multiply by 100. Eleven of 44 dietitians were contacted a month after receiving the first %WFH calculation sheet, and asked to repeat the calculations based on the same data without referring back to their previous calculations.Means, standard deviations, and ranges of the results obtained for each patient and for each dietitian were calculated. ResultsForty four of the 52 dietitians completed the questionnaire. The majority (35/44) used %WFH as a measure of nutritional status. Of these, 14 assessed %WFH annually and nine at each clinic visit. Height and weight centile measurements were recorded at each clinic by 41/44. The US CF Foundation method for calculating %WFH was used by 22/35 dietitians and the Cole Slide Rule was used by 9/35. %WFH calculations were completed by 42/52. The range of estimates for each of the six patients varied by 16.5 to 40 percentage points (mean 27.8; fig 1). On average, 25% of the %WFH calculations were more than two percentage points either side of the mean, and 8.5% were more than five percentage points either side of the mean.Eleven of the dietitians completed the second set of calculations. The range of estimates for each of the six patients varied by between 13 and 24 percentage points (mean 16.8). For each of the 11 dietitians the average diVerence between the two sets of results was calculated and th...

Background Poor growth and nutritional status are common in children with chronic diseases. Oral protein calorie supplements are used to improve nutritional status in these children. These expensive products may be associated with some adverse effects, e.g. the development of inappropriate eating behaviour patterns. This is a new update of a Cochrane review last updated in 2009. Objectives To examine evidence that in children with chronic disease, oral protein calorie supplements alter daily nutrient intake, nutritional indices, survival and quality of life and are associated with adverse effects, e.g. diarrhoea, vomiting, reduced appetite, glucose intolerance, bloating and eating behaviour problems. Search methods Trials of oral protein calorie supplements in children with chronic diseases were identified through comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. Companies marketing these products were also contacted. Most recent search of the Group's Trials Register: 24 February 2015. Selection criteria Randomised or quasi‐randomised controlled trials comparing oral protein calorie supplements for at least one month to increase calorie intake with existing conventional therapy (including advice on improving nutritional intake from food or no specific intervention) in children with chronic disease. Data collection and analysis We independently assessed the outcomes: indices of nutrition and growth; anthropometric measures of body composition; calorie and nutrient intake (total from oral protein calorie supplements and food); eating behaviour; compliance; quality of life; specific adverse effects; disease severity scores; and mortality; we also assessed the risk of bias in the included trials. Main results Four studies (187 children) met the inclusion criteria. Three studies were carried out in children with cystic fibrosis and one study included children with paediatric malignant disease. Overall there was a low risk of bias for blinding and incomplete outcome data.Two studies had a high risk of bias for allocation concealment. Few statistical differences were found in the outcomes we assessed between treatment and control groups, except change in total energy intake at six and 12 months, mean difference 304.86 kcal per day (95% confidence interval 5.62 to 604.10) and mean difference 265.70 kcal per day (95% confidence interval 42.94 to 485.46), respectively. However, these were based on the analysis of just 58 children in only one study. Only two chronic diseases were included in these analyses, cystic fibrosis and paediatric malignant disease. No other studies were identified which assessed the effectiveness of oral protein calorie supplements in children with other chronic diseases. Authors' conclusions Oral protein calor...

Risk of type 1 diabetes at 3 years is high for initially multiple and single Ab+ IT and multiple Ab+ NT. Genetic predisposition, age, and male sex are significant risk factors for development of Ab+ in twins.

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