Optimal clinical management of children receiving ketogenic parenteral nutrition: a clinical practice guide.

AIM: To give evidence-based recommendations on the application of ketogenic diet parenteral nutrition (KD-PN) in emergency situations. METHOD: An international group of experts (n=14) researched the literature and distributed a survey among 150 expert centers. International accepted guidelines (European Society for Clinical Nutrition and Metabolism/European Society for Paediatric Gastroenterology Hepatology and Nutrition and the American Society for Parenteral and Enteral Nutrition) and handbooks for parenteral nutrition were considered general standards of care. RESULTS: In the literature, we identified 35 reports of patients treated by KD-PN. International guidelines and handbooks provided some conflicting information. Twenty-four expert teams from nine countries responded to the survey, reflecting the limited clinical experience. INTERPRETATION: This paper highlights 23 consensus-based recommendations for safe and effective KD-PN (e.g. diet initiation, calculation, application, monitoring, and evaluation) based on the best evidence available and expert opinions. WHAT THIS PAPER ADDS: In acute settings, ketogenic diet therapy (KDT) can be administered parenterally. Parenteral administration of KDT should be started only at the intensive care unit. Initiate ketogenic parenteral nutrition stepwise to the highest ratio possible with the lowest level of complications. Evaluate the risk-benefit ratio of parenteral administration continuously. Restart enteral feeding as soon as appropriate.

MANEJO CLÍNICO ÓPTIMO DE LOS NIÑOS QUE RECIBEN NUTRICIÓN PARENTERAL CETOGÉNICA: UNA GUÍA DE PRÁCTICA CLÍNICA: OBJETIVO: Dar recomendaciones basadas en evidencia sobre la aplicación de dieta cetogénica en la nutrición parenteral (DC-NP) en situaciones de emergencia. MÉTODO: Un grupo de expertos (n=14) investigó la literatura y distribuyó una encuesta en 150 centros especializados. Considerando como estándares de manejo las guías aceptadas internacionalmente (Sociedad Europea para la Nutrición Clínica y Metabolismo/Sociedad Europea de Gastroenterología Pediátrica, Hepatología y Nutrición, y la Sociedad Americana para Nutrición Enteral y Parenteral) y los manuales para la nutrición parenteral. RESULTADOS: En la literatura se identificaron 35 informes de pacientes tratados por DC-NP. Las directrices y manuales internacionales proporcionaron alguna información contradictoria. Veinticuatro equipos de expertos de nueve países respondieron a la encuesta, reflejando la limitada experiencia clínica. INTERPRETACIÓN: Este documento destaca 23 recomendaciones basadas en consensos para una DC-NP segura y eficaz (por ejemplo; iniciación de la dieta, cálculo, aplicación, monitoreo y evaluación) basada en la mejor evidencia disponible y las opiniones de expertos.

MANEJO CLÍNICO ÓTIMO DE CRIANÇAS RECEBENDO NUTRIÇÃO CETOGÊNICA PARENTERAL: UM GUIA PARA A PRÁTICA CLÍNICA: OBJETIVO: Oferecer recomendações baseadas em evidências da aplicação de dieta cetogênica por nutrição parenteral (DC-NP) em situações de emergência. MÉTODO: Um grupo internacional de especialistas (n=14) pesquisou a literatura e distribuiu um questionário em 150 centros especializados. Diretrizes internacioansi aceitas (Sociedade Européia de Nutrição Clínica e Metabolismo/ Sociedade Européia de Gastroenterologia, Hepatologia e Nutrição Pediátrica, e a Sociedade Americana de Nutrição Enteral e Parenteral) e livros sobre nutrição parenteral foram considerados padrão geral de atenção. RESULTADOS: Na literatura, identificamos 35 relados de pacientes tratados por DC-NP. As diretrizes internacionais e os livros forneceram informações conflitantes. Vinte e quatro equips de especialistas de nove países responderam ao questionário, refletindo a experiência clínica limitada. INTERPRETAÇÃO: Este artigo destaca 23 recomendações baseadas em consenso para DC-NP segura e efetiva (ex: início da dieta, cálculo, aplicação, monitoramento e avaliação) com base na melhor evidência disponível e opiniões de especialistas.

Proposal for an individualized dietary strategy in patients with very long-chain acyl-CoA dehydrogenase deficiency.

BACKGROUND: Patients with very long chain acyl-CoA dehydrogenase deficiency (VLCADD), a long chain fatty acid oxidation disorder, are traditionally treated with a long chain triglyceride (LCT) restricted and medium chain triglyceride (MCT) supplemented diet. Introduction of VLCADD in newborn screening (NBS) programs has led to the identification of asymptomatic newborns with VLCADD, who may have a more attenuated phenotype and may not need dietary adjustments. OBJECTIVE: To define dietary strategies for individuals with VLCADD based on the predicted phenotype. METHOD: We evaluated long-term dietary histories of a cohort of individuals diagnosed with VLCADD identified before the introduction of VLCADD in NBS and their beta-oxidation (LC-FAO) flux score (rate of oleate oxidation) in cultured skin fibroblasts in relation to the clinical outcome. Based on these results a dietary strategy is proposed. RESULTS: Sixteen individuals with VLCADD were included. One had an LC-FAO flux score >90%, was not on a restricted diet and is asymptomatic to date. Four patients had an LC-FAO flux score <10%, and significant VLCADD related symptoms despite the use of strict diets including LCT restriction, MCT supplementation and nocturnal gastric drip feeding. Patients with an LC-FAO flux score between 10 and 90% (n = 11) showed a more heterogeneous phenotype. CONCLUSIONS: This study shows that a strict diet cannot prevent poor clinical outcome in severely affected patients and that the LC-FAO flux is a good predictor of clinical outcome in individuals with VLCADD identified before its introduction in NBS. Hereby, we propose an individualized dietary strategy based on the LC-FAO flux score.

Supplementation with a powdered blend of PUFAs normalizes DHA and AA levels in patients with PKU.

UNLABELLED: Patients with phenylketonuria (PKU) have a poor LC-PUFA status and require supplementation. The objective of this study was to evaluate the LC-PUFA status of PKU patients supplemented with fish oil or the fatty acid supplement KeyOmega. Plasma and erythrocyte docosahexaenoic acid (DHA) and arachidonic acid (AA) levels were determined in 54 patients (1-18.5years of age) with confirmed PKU. The influence of supplementation with fish oil versus KeyOmega, a powdered blend of DHA and AA, on LC-PUFA status was investigated and compared to the status in samples obtained from unsupplemented patients. Differential effects on LC-PUFA status were observed upon suppletion with fish oil versus KeyOmega. Whereas supplementation with fish oil increased the level of DHA, the AA concentration did not increase to normal values in these patients. In contrast, both DHA and AA levels increased and reached reference values upon supplementation with KeyOmega. IN CONCLUSION: these results indicate that KeyOmega offers additional benefit over fish oil since both AA and DHA status are normalized in PKU patients supplemented with KeyOmega.

Whole body composition analysis by the BodPod air-displacement plethysmography method in children with phenylketonuria shows a higher body fat percentage.

BACKGROUND: Phenylketonuria (PKU) causes irreversible central nervous system damage unless a phenylalanine (PHE) restricted diet with amino acid supplementation is maintained. To prevent growth retardation, a protein/amino acid intake beyond the recommended dietary protein allowance is mandatory. However, data regarding disease and/or diet related changes in body composition are inconclusive and retarded growth and/or adiposity is still reported. The BodPod whole body air-displacement plethysmography method is a fast, safe and accurate technique to measure body composition. AIM: To gain more insight into the body composition of children with PKU. METHODS: Patients diagnosed with PKU born between 1991 and 2001 were included. Patients were identified by neonatal screening and treated in our centre. Body composition was measured using the BodPod system (Life Measurement Incorporation©). Blood PHE values determined every 1-3 months in the year preceding BodPod analysis were collected. Patients were matched for gender and age with data of healthy control subjects. Independent samples t tests, Mann-Whitney and linear regression were used for statistical analysis. RESULTS: The mean body fat percentage in patients with PKU (n = 20) was significantly higher compared to healthy controls (n = 20) (25.2% vs 18.4%; p = 0.002), especially in girls above 11 years of age (30.1% vs 21.5%; p = 0.027). Body fat percentage increased with rising body weight in patients with PKU only (R = 0.693, p = 0.001), but did not correlate with mean blood PHE level (R = 0.079, p = 0.740). CONCLUSION: Our data show a higher body fat percentage in patients with PKU, especially in girls above 11 years of age.

Ketogenic diet guidelines for infants with refractory epilepsy.

BACKGROUND: The ketogenic diet (KD) is an established, effective non-pharmacologic treatment for drug resistant childhood epilepsy. For a long time, the KD was not recommended for use in infancy (under the age of 2 years) because this is such a crucial period in development and the perceived high risk of nutritional inadequacies. Indeed, infants are a vulnerable population with specific nutritional requirements. But current research shows that the KD is highly effective and well tolerated in infants with epilepsy. Seizure freedom is often achieved and maintained in this specific patient group. There is a need for standardised protocols and management recommendations for clinical use. METHOD: In April 2015, a project group of 5 experts was established in order to create a consensus statement regarding the clinical management of the KD in infants. The manuscript was reviewed and amended by a larger group of 10 international experts in the KD field. Consensus was reached with regard to guidance on how the diet should be administered and in whom. RESULTS: The resulting recommendations include patient selection, pre-KD counseling and evaluation, specific nutritional requirements, preferred initiation, monitoring of adverse effects at initiation and follow-up, evaluation and KD discontinuation. CONCLUSION: This paper highlights recommendations based on best evidence, combined with expert opinions and gives directions for future research.