Clinical audit results in earlier nutritional intervention in malnourished children with cystic fibrosis with improved outcome

Ledder, Oren; Oliver, Mark; Heine, Ralf G.; Graham, Joanne C.; Volders, Evelyn; Robinson, Philip

doi:10.1111/jpc.12888

Cited by 13 publications

(14 citation statements)

References 21 publications

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“…Given the rarity of A-T, extrapolation from other conditions is inevitable. In children with CF, clinical audit27 and a structured nutritional intervention approach28 (with early referral for PEG in those with severe malnutrition), improved outcomes in terms of growth parameters, lung function and the 2-year survival post PEG insertion improved from 70% to 100%. The long-term nutritional benefit of PEG tube placement is critically dependent on presurgical pulmonary function with better growth if PEGs are placed early when the child has better pulmonary function 29…”

Section: Discussionmentioning

confidence: 99%

Growth and nutrition in children with ataxia telangiectasia

Stewart¹,

Prayle

Tooke³

et al. 2016

Arch Dis Child

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BACKGROUND: Ataxia telangiectasia (A-T) is a rare multisystem disease with high early mortality from lung disease and cancer. Nutritional failure adversely impacts outcomes in many respiratory diseases. Several factors influence nutrition in children with A-T. We hypothesised that children with A-T have progressive growth failure and that early gastrostomy tube feeding (percutaneous endoscopic gastrostomy, PEG) is a favourable management option with good nutritional outcomes. METHODS: Data were collected prospectively on weight, height and body mass index (BMI) at the national paediatric A-T clinic. Adequacy and safety of oral intake was assessed. Nutritional advice was given at each multidisciplinary review. RESULTS: 101 children (51 girls) had 222 measurements (32 once, 32 twice, 24 thrice) between 2009 and 2016. Median (IQR) age was 9.3 (6.4 to 13.1) years. Mean (SD) weight, height and BMI Z-scores were respectively -1 (1.6), -1.2 (1.2) and -0.4 (1.4). 35/101 children had weight Z-scores below -2 on at least one occasion. Weight, height and BMI Z-scores declined over time. Decline was most obvious after 8 years of age. 14/101 (14%) children had a PEG, with longitudinal data available for 12. In a nested case control study, there was a trend for improvement in weight in those with a PEG (p=0.10). CONCLUSIONS: Patients with A-T decline in growth over time. There is an urgent need for new strategies, including an understanding of why growth falters. We suggest early proactive consideration of PEG from age 8 years onwards to prevent progressive growth failure

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Section: Discussionmentioning

confidence: 99%

Growth and nutrition in children with ataxia telangiectasia

Stewart¹,

Prayle

Tooke³

et al. 2016

Arch Dis Child

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“…(46) According to a Cochrane systematic review, no eligible randomized controlled trial evaluating the efficacy of ENT in CF was identified. (47) However, nonrandomized studies showed several beneficial effects of ENT in CF patients, such as increased energy supply, (48) weight gain (49)(50)(51)(52)(53)(54)(55)(56)(57) and BMI. (53)(54)(55) It has also been observed to increase growth rate -especially in children (48,49,53) increase body fat (49,58) and muscle mass, as well as improve biochemical markers.…”

Section: Nutritional Intervention (Oral Supplementation Enteral and P...mentioning

confidence: 99%

“…(53)(54)(55) It has also been observed to increase growth rate -especially in children (48,49,53) increase body fat (49,58) and muscle mass, as well as improve biochemical markers. (57,59) In addition, ENT has contributed to the stabilization (48,49,51,52,54,55) or even improvement of lung function (50,51,56,57) (level of evidence 4).…”

Section: Nutritional Intervention (Oral Supplementation Enteral and P...mentioning

confidence: 99%

Brazilian Guidelines for Nutrition in Cystic Fibrosis

Neri¹,

Simon²,

Ambrósio³

et al. 2022

Einstein (São Paulo)

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Objective: To develop a scientific consensus on nutrition in cystic fibrosis. Methods: Sixteen coordinators elaborated relevant questions on nutritional therapy in cystic fibrosis, which were divided into six sections: nutritional assessment, nutritional recommendations, nutritional intervention, dietary counseling, special situations and enzyme replacement, and gastrointestinal manifestations. Two to three specialists in the field were responsible for each section and obtaining answers formulated based on standardized bibliographic searches. The available literature was searched in the PubMed ® /MEDLINE database, after training and standardization of search strategies, to write the best level of evidence for the questions elaborated. Issues related to disagreement were discussed until a consensus was reached among specialists, based on the current scientific literature. Results: Forty-two questions were prepared and objectively answered, resulting in a consensus of nutritional therapy in cystic fibrosis. Conclusion: This work enabled establishing a scientific consensus for nutritional treatment of cystic fibrosis patients.

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“…Following the exclusion of alternate causes, supplemental enteral feeding should be considered for those failing non‐invasive strategies or earlier in those clearly at nutritional compromise or with recognised additional nutritional challenges such as emerging portal hypertension, feeding disorders and intestinal inflammation. Gastrostomy feeding should be initiated prior to the onset of significant lung disease as aggressive enteral nutrition support is increasingly more challenging as lung function deteriorates and is not usually successful in end‐stage lung disease. Gastrostomy feeds are usually introduced gradually as tolerated and administered as a continuous infusion overnight via a pump to preserve appetite and oral intake during the day.…”

Section: Nutritionmentioning

confidence: 99%

Practical approach to the gastrointestinal manifestations of cystic fibrosis

Bolia

Ooi

Lewindon

et al. 2018

J Paediatrics Child Health

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Cystic fibrosis (CF) is the most common, life-shortening, genetic illness affecting children in Australia and New Zealand. The genetic abnormality results in abnormal anion transport across the apical membrane of epithelial cells in a number of organs, including the lungs, gastrointestinal tract, liver and genito-urinary tract. Thus, CF is a multi-system disorder that requires a multi-disciplinary approach. Respiratory disease is the predominant cause of both morbidity and mortality in patients with CF. However, there are significant and clinically relevant gastrointestinal, liver, pancreatic and nutritional manifestations that must be detected and managed in a timely and structured manner. The aim of this review is to provide evidence-based information and clinical algorithms to guide the nutritional and gastrointestinal management of patients with CF.

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Clinical audit results in earlier nutritional intervention in malnourished children with cystic fibrosis with improved outcome

Abstract: Earlier referral of patients in the recent cohort resulted in sustained improvements in weight-for-age and lung function. Survival at 2 years post-procedure was significantly improved. This study confirms the value of clinical audits and subsequent re-evaluation of clinical services.

Cited by 13 publications

References 21 publications

Growth and nutrition in children with ataxia telangiectasia

Growth and nutrition in children with ataxia telangiectasia

Brazilian Guidelines for Nutrition in Cystic Fibrosis

Practical approach to the gastrointestinal manifestations of cystic fibrosis

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