Hypercalcemia in Children Using the Ketogenic Diet: A Multicenter Study.

CONTEXT: The ketogenic diet is associated with progressive skeletal demineralization, hypercalciuria, and nephrolithiasis. Acute hypercalcemia has been described as a newly recognized complication of this treatment. OBJECTIVE: To describe the clinical characteristics of acute hypercalcemia in children on the ketogenic diet through analysis of the presentation, response to treatment, and natural history in a large cohort of patients. DESIGN: A multicenter case series was performed including children who developed acute hypercalcemia while treated with the ketogenic diet. Information on clinical presentation, treatment, and course of this complication was collated centrally. RESULTS: There were 14 patients (median (range) age 6.3 (0.9 to 18) years) who developed hypercalcemia 2.1 (range, 0.2-12) years after starting the ketogenic diet. All had low levels of parathyroid hormone and levels of 1,25-dihydroxyvitamin D were low in all except one. Seven (50%) had impaired renal function at presentation. All except the 2 oldest had low alkaline phosphatase levels for age. Once normocalcemia was achieved, hypercalcemia recurred in only 2 of these patients over observation of up to 9.8 years. One patient discontinued the ketogenic diet prior to achieving normocalcemia while 4 more stopped the diet during follow-up after resolution of hypercalcemia. CONCLUSIONS: Ketotic hypercalcemia can occur years after starting the ketogenic diet, especially in the setting of renal impairment. The mechanism is unknown but appears to be due to reduced osteoblast activity and impaired bone formation. We recommend close attention to optimizing bone health in these children, and screening for the development of ketotic hypercalcemia.

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.

An update on diets in clinical practice.

Ketogenic diet therapies involve a collaborative healthcare team and therefore are typically offered in tertiary care centers. Centers that utilize these therapies with frequency gain valuable experience and become skilled in their practice. This chapter is a summary from the presentations of 5 practitioners including a nurse, pharmacist, and 3 dietitians who shared their expertise during the clinical session of the 2012 International Symposium.