Elles van der Louw1, Dorine van den Hurk2, Elizabeth Neal3, Bärbel Leiendecker4, Georgiana Fitzsimmon5, Laura Dority6, Lindsey Thompson7, Maddelena Marchió8, Magdalena Dudzińska9, Anastasia Dressler10, Joerg Klepper11, Stéphane Auvin12, J Helen Cross13. 1. Erasmus Medical Center Sophia Children's Hospital, Wytemaweg 80, 3015 CN, Rotterdam, The Netherlands. Electronic address: e.vanderlouw@erasmusmc.nl. 2. Wilhelmina Children's Hospital, University Medical Center Utrecht, The Netherlands. Electronic address: t.vandenhurk@umcutrecht.nl. 3. Matthews Friends Clinics, Lingfield, UK. Electronic address: e.neal@mfclinics.com. 4. University of Essen, Children's Hospital, Germany. Electronic address: baerbel.Leiendecker@uk-essen.de. 5. Great Ormond Street Hospital for Children, London, UK. Electronic address: Georgiana.Fitzsimmons@gosh.nhs.uk. 6. Medical University of South Carolina, USA. Electronic address: kenla@musc.edu. 7. Children's Mercy Hospital and Clinics, Overland Park, KS, USA. Electronic address: lmthompson@cmh.edu. 8. University of Modena and Reggio Emilia, Italy. Electronic address: maddalena.marchio@unimore.it. 9. Center of Pediatrics and Oncology, Chorzow, Poland. Electronic address: mdudzinskapl@yahoo.com. 10. Medical University Vienna, Austria. Electronic address: anastasia.dressler@meduniwien.ac.at. 11. Klinikum Aschaffenburg-Alzenau, Germany. Electronic address: Jorg.Klepper@klinikum-ab-alz.de. 12. Pediatric Epilepsy & Child Neurology Paris-Diderot University, France. Electronic address: stephane.auvin@aphp.fr. 13. UCL Institute of Child Health, Great Ormond Street Hospital for Children NHS Trust, London, UK. Electronic address: h.cross@ucl.ac.uk.
Abstract
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.
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.
Authors: M Caprio; M Infante; E Moriconi; A Armani; A Fabbri; G Mantovani; S Mariani; C Lubrano; E Poggiogalle; S Migliaccio; L M Donini; S Basciani; A Cignarelli; E Conte; G Ceccarini; F Bogazzi; L Cimino; R A Condorelli; S La Vignera; A E Calogero; A Gambineri; L Vignozzi; F Prodam; G Aimaretti; G Linsalata; S Buralli; F Monzani; A Aversa; R Vettor; F Santini; P Vitti; L Gnessi; U Pagotto; F Giorgino; A Colao; A Lenzi Journal: J Endocrinol Invest Date: 2019-05-20 Impact factor: 4.256
Authors: Colin P Hawkes; Sani M Roy; Bassem Dekelbab; Britney Frazier; Monica Grover; Jaime Haidet; James Listman; Sarianne Madsen; Marian Roan; Celia Rodd; Aviva Sopher; Peter Tebben; Michael A Levine Journal: J Clin Endocrinol Metab Date: 2021-01-23 Impact factor: 5.958
Authors: Eric H Kossoff; Beth A Zupec-Kania; Stéphane Auvin; Karen R Ballaban-Gil; A G Christina Bergqvist; Robyn Blackford; Jeffrey R Buchhalter; Roberto H Caraballo; J Helen Cross; Maria G Dahlin; Elizabeth J Donner; Orkide Guzel; Rana S Jehle; Joerg Klepper; Hoon-Chul Kang; Danielle A Lambrechts; Y M Christiana Liu; Janak K Nathan; Douglas R Nordli; Heidi H Pfeifer; Jong M Rho; Ingrid E Scheffer; Suvasini Sharma; Carl E Stafstrom; Elizabeth A Thiele; Zahava Turner; Maria M Vaccarezza; Elles J T M van der Louw; Pierangelo Veggiotti; James W Wheless; Elaine C Wirrell Journal: Epilepsia Open Date: 2018-05-21