title: Glycogen storage disease type III: modified Atkins diet improves myopathy.
abstract: BACKGROUND
  Frequent feeds with carbohydrate-rich meals or continuous enteral feeding has been the therapy of choice in glycogen storage disease (Glycogenosis) type III. Recent guidelines on diagnosis and management recommend frequent feedings with high complex carbohydrates or cornstarch avoiding fasting in children, while in adults a low-carb-high-protein-diet is recommended. While this regimen can prevent hypoglycaemia in children it does not improve skeletal and heart muscle function, which are compromised in patients with glycogenosis IIIa. Administration of carbohydrates may elicit reactive hyperinsulinism, resulting in suppression of lipolysis, ketogenesis, gluconeogenesis, and activation of glycogen synthesis. Thus, heart and skeletal muscle are depleted of energy substrates. Modified Atkins diet leads to increased blood levels of ketone bodies and fatty acids. We hypothesize that this health care intervention improves the energetic balance of muscles.
  
  
  METHODS
  We treated 2 boys with glycogenosis IIIa aged 9 and 11 years with a modified Atkins diet (10 g carbohydrate per day, protein and fatty acids ad libitum) over a period of 32 and 26 months, respectively.
  
  
  RESULTS
  In both patients, creatine kinase levels in blood dropped in response to Atkins diet. When diet was withdrawn in one of the patients he complained of chest pain, reduced physical strength and creatine kinase levels rapidly increased. This was reversed when Atkins diet was reintroduced. One patient suffered from severe cardiomyopathy which significantly improved under diet. Patients with glycogenosis IIIa benefit from an improved energetic state of heart and skeletal muscle by introduction of Atkins diet both on a biochemical and clinical level. Apart from transient hypoglycaemia no serious adverse effects were observed.
authors.affiliation: Clinic for Paediatric Kidney-, Liver and Metabolic Diseases, Carl-Neuberg-Str.1, D-30625, Hannover, Germany. sebene.mayorandan@ukmuenster.de.|Present address: Department of Paediatrics, University Hospital Münster, Albert-Schweitzer-Campus 1, D-48161, Münster, Germany. sebene.mayorandan@ukmuenster.de.
authors.forename: Sebene
authors.initials: S
authors.lastname: Mayorandan
authors.affiliation: Clinic for Paediatric Kidney-, Liver and Metabolic Diseases, Carl-Neuberg-Str.1, D-30625, Hannover, Germany. meyer.uta@mh-hannover.de.
authors.forename: Uta
authors.initials: U
authors.lastname: Meyer
authors.affiliation: Clinic for Paediatric Kidney-, Liver and Metabolic Diseases, Carl-Neuberg-Str.1, D-30625, Hannover, Germany. hartmann.hans@mh-hannover.de.
authors.forename: Hans
authors.initials: H
authors.lastname: Hartmann
authors.affiliation: Clinic for Paediatric Kidney-, Liver and Metabolic Diseases, Carl-Neuberg-Str.1, D-30625, Hannover, Germany. das.anibh@mh-hannover.de.
authors.forename: Anibh Martin
authors.initials: AM
authors.lastname: Das
mesh_terms: D001786:Blood Glucose / Q000378:metabolism; D002648:Child; D050528:Diet, Carbohydrate-Restricted / Q000379:methods*; D004044:Dietary Proteins / Q000008:administration & dosage; D006010:Glycogen Storage Disease Type III / Q000097:blood / Q000175:diagnosis* / Q000178:diet therapy*; D006801:Humans; D008297:Male; D009135:Muscular Diseases / Q000097:blood / Q000175:diagnosis* / Q000178:diet therapy*
chemical_list: D001786:Blood Glucose; D004044:Dietary Proteins