Michel Hochuli1,2, Emanuel Christ3, Fabian Meienberg4, Roger Lehmann5, Jan Krützfeldt5, Matthias R Baumgartner6,7. 1. Division of Endocrinology, Diabetes, and Clinical Nutrition, University Hospital Zurich, Rämistrasse 100, CH-8091, Zurich, Switzerland. michel.hochuli@usz.ch. 2. Radiz - Rare Disease Initiative Zurich, Clinical Research Priority Program for Rare Diseases, University of Zurich, Zurich, Switzerland. michel.hochuli@usz.ch. 3. Division of Endocrinology, Diabetology and Clinical Nutrition, University Hospital of Bern, Inselspital, Bern, Switzerland. 4. Endocrinology, Diabetes, and Metabolism, University Hospital Basel, Basel, Switzerland. 5. Division of Endocrinology, Diabetes, and Clinical Nutrition, University Hospital Zurich, Rämistrasse 100, CH-8091, Zurich, Switzerland. 6. Division of Metabolism and Children's Research Center, University Children's Hospital, Zurich, Switzerland. 7. Radiz - Rare Disease Initiative Zurich, Clinical Research Priority Program for Rare Diseases, University of Zurich, Zurich, Switzerland.
Abstract
BACKGROUND: Traditional approaches for nighttime glycemic control in glycogen storage disease typeI (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. CONCLUSION: 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.
RCT Entities:
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. CONCLUSION: 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: Jan Peter Rake; Gepke Visser; Philippe Labrune; James V Leonard; Kurt Ullrich; G Peter A Smit Journal: Eur J Pediatr Date: 2002-08-24 Impact factor: 3.183
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Authors: Priya S Kishnani; Stephanie L Austin; Jose E Abdenur; Pamela Arn; Deeksha S Bali; Anne Boney; Wendy K Chung; Aditi I Dagli; David Dale; Dwight Koeberl; Michael J Somers; Stephanie Burns Wechsler; David A Weinstein; Joseph I Wolfsdorf; Michael S Watson Journal: Genet Med Date: 2014-11 Impact factor: 8.822