title: A pilot longitudinal study of the use of waxy maize heat modified starch in the treatment of adults with glycogen storage disease type I: a randomized double-blind cross-over study.
abstract: BACKGROUND
  Uncooked corn-starch (UCCS) has been the mainstay of therapy for the hepatic glycogen storage diseases (GSD) but is not always effective. A new starch (WMHMS) has demonstrated a more favourable short-term metabolic profile.
  
  
  OBJECTIVE
  To determine efficacy and safety of a new uncooked starch (WMHMS) compared to UCCS over 16 weeks treatment with each.
  
  
  METHODS
  A double-blind cross-over study of 10 adults (aged 16 - 38 years, six male) with GSD Ia and Ib. After an individualised fast, subjects were randomised to take a 50 g starch-load of either WMHMS or UCCS. Starch-loads terminated when blood glucose was < 3.0 mmol/L or the subject felt subjectively hypoglycaemic. Anonymous biochemical profiles were assessed by 2 investigators and a starch administration schedule recommended. Each starch was delivered in coded sachets and intake was monitored for the following 16 weeks. After a washout period, the protocol was repeated with the alternative product.
  
  
  RESULTS
  4 subjects failed to establish therapy on the cross-over limb. Data from 7 paired starch load showed: longer median fasting duration with WMHMS (7.5 versus 5 hours; p = 0.023), slower decrease in the glucose curve (0.357 versus 0.632 mmol/hr p = 0.028) and less area under insulin curves for the first 4 hours (p = 0.03). Two of six subjects took 50% or less WMHMS compared to UCCS and one took more. Plasma triglycerides, cholesterol and uric acid were unchanged after each study phase.
  
  
  CONCLUSIONS
  WMHMS leads to significant reduction in insulin release and reduced starch use in some GSD patients.
authors.affiliation: Charles Dent Metabolic Unit, National Hospital for Neurology and Neurosurgery, WC1N 3BG, London, UK. Kaustuv.Bhattacharya@health.nsw.gov.au.|Discipline of Paediatrics and Child Health, The Children's Hospital at Westmead Clinical School, University of Sydney, Sydney, Australia. Kaustuv.Bhattacharya@health.nsw.gov.au.
authors.forename: Kaustuv
authors.initials: K
authors.lastname: Bhattacharya
authors.affiliation: Evelina London Children's Hospital; St Thomas's Hospital, SE1 7EH, London, UK. Helen.Mundy@gstt.nhs.uk.
authors.forename: Helen
authors.initials: H
authors.lastname: Mundy
authors.affiliation: Charles Dent Metabolic Unit, National Hospital for Neurology and Neurosurgery, WC1N 3BG, London, UK. Maggie.lilburn@btinternet.com.
authors.forename: Maggie F
authors.initials: MF
authors.lastname: Lilburn
authors.affiliation: Evelina London Children's Hospital; St Thomas's Hospital, SE1 7EH, London, UK. Michael.Champion@gstt.nhs.uk.
authors.forename: Michael P
authors.initials: MP
authors.lastname: Champion
authors.affiliation: Department of Epidemiology & Biostatistics, Imperial College, SW7 2AZ, London, UK. d.w.morley@gmail.com.
authors.forename: David W
authors.initials: DW
authors.lastname: Morley
authors.affiliation: CHRU de Tours, 37044, Tours, Cedex, France. francois.maillot@univ-tours.fr.
authors.forename: François
authors.initials: F
authors.lastname: Maillot
mesh_terms: D000293:Adolescent; D000328:Adult; D001786:Blood Glucose / Q000187:drug effects; D018592:Cross-Over Studies; D004311:Double-Blind Method; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000178:diet therapy*; D006801:Humans; D008297:Male; D010865:Pilot Projects; D013213:Starch / Q000737:chemistry* / Q000494:pharmacology*; D055815:Young Adult
chemical_list: D001786:Blood Glucose; D013213:Starch