title: Alternative nighttime nutrition regimens in glycogen storage disease type I: a controlled crossover study.
abstract: BACKGROUND
  Traditional approaches for nighttime glycemic control in glycogen storage disease type I (GSDI) include continuous tube feeding, or ingestion of uncooked corn starch (CS) at bedtime. A modified corn starch (MCS) has been shown to prolong euglycemia in some patients. The aim of this study was to evaluate whether stable nighttime glucose control can be achieved with other types of slowly digested carbohydrates in adult GSDI patients.
  
  
  METHODS
  In this cross-over study, nocturnal glucose control and fasting times were assessed with three different nocturnal nutrition regimens in five patients, using continuous glucose monitoring (CGMS) in an outpatient everyday life setting. For each patient, continuous glucose profiles were measured after ingestion of (1) CS, (2) MCS or (3) a pasta meal at bedtime, during 5 to 6 consecutive nights for each regimen.
  
  
  RESULTS
  Stable nocturnal glucose control was achieved for all patients with a pasta meal, with a mean duration of glycemia >3.5 mmol/l of 7.6 h (range 5.7-10.8), and >4 mmol/l of 7 h (5.2-9.2), similar to CS and MCS. Fasting glucose before breakfast on workdays (after 7.1 ± 0.8 h) was not significantly different between the three interventions (CS 4.1 ± 0.5 mmol/l, MCS 4.6 ± 0.7 mmol/l, pasta 4.3 ± 0.9 mmol/l). During prolonged fasting on weekends, longer duration of normoglycemia was achieved with CS or MCS than with pasta.
  
  
  CONCLUSIONS
  Consumption of cooked pasta is a suitable and more palatable alternative to uncooked corn starch to achieve nighttime glucose control in adult patients with GSDI.
authors.affiliation: Division of Endocrinology, Diabetes, and Clinical Nutrition, University Hospital Zurich, Rämistrasse 100, CH-8091, Zurich, Switzerland. michel.hochuli@usz.ch.|Radiz - Rare Disease Initiative Zurich, Clinical Research Priority Program for Rare Diseases, University of Zurich, Zurich, Switzerland. michel.hochuli@usz.ch.
authors.forename: Michel
authors.initials: M
authors.lastname: Hochuli
authors.affiliation: Division of Endocrinology, Diabetology and Clinical Nutrition, University Hospital of Bern, Inselspital, Bern, Switzerland.
authors.forename: Emanuel
authors.initials: E
authors.lastname: Christ
authors.affiliation: Endocrinology, Diabetes, and Metabolism, University Hospital Basel, Basel, Switzerland.
authors.forename: Fabian
authors.initials: F
authors.lastname: Meienberg
authors.affiliation: Division of Endocrinology, Diabetes, and Clinical Nutrition, University Hospital Zurich, Rämistrasse 100, CH-8091, Zurich, Switzerland.
authors.forename: Roger
authors.initials: R
authors.lastname: Lehmann
authors.affiliation: Division of Endocrinology, Diabetes, and Clinical Nutrition, University Hospital Zurich, Rämistrasse 100, CH-8091, Zurich, Switzerland.
authors.forename: Jan
authors.initials: J
authors.lastname: Krützfeldt
authors.affiliation: Division of Metabolism and Children's Research Center, University Children's Hospital, Zurich, Switzerland.|Radiz - Rare Disease Initiative Zurich, Clinical Research Priority Program for Rare Diseases, University of Zurich, Zurich, Switzerland.
authors.forename: Matthias R
authors.initials: MR
authors.lastname: Baumgartner
mesh_terms: D000328:Adult; D001786:Blood Glucose / Q000378:metabolism*; D018592:Cross-Over Studies; D004040:Dietary Carbohydrates / Q000008:administration & dosage*; D005215:Fasting; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000178:diet therapy*; D006801:Humans; D007003:Hypoglycemia / Q000517:prevention & control*; D007328:Insulin / Q000097:blood*; D016014:Linear Models; D008297:Male; D013213:Starch / Q000008:administration & dosage*; D055815:Young Adult
chemical_list: D001786:Blood Glucose; D004040:Dietary Carbohydrates; D007328:Insulin; D013213:Starch