Overnight Feeding Study in Glycogen Storage Disease Type 1

University of Zurich (UZH)

Status: Completed

Conditions: Glycogen Storage Disease Type 1 (GSD 1)

Treatments: Other: overnight nutrition regime

Study type: Interventional

Funder types: Other

Identifiers

NCT01961076

KEK-ZH-Nr. 2013-0161

Details and patient eligibility

About

In this clinical cross-over study, we will compare the efficacy of different oral nutrition regimens for night-time glucose control in adult GSD 1 patients. Three different over-night nutrition regimens (=interventions) will be compared in each patient sequentially, (1) uncooked corn starch (UCSS, "Maizena"), (2) modified corn-starch, (3) other carbohydrate (starch) containing meal. During each intervention, glucose profiles will be continuously monitored by continuous glucose monitoring (CGMS). The duration of each intervention is 3d (mimimum) to 6d (maximum), depending on the quality of night-time glucose control and the technical quality of glucose sensor readings. Between the interventions, the patients follow their normal prescribed diet.

Enrollment: 5 patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Glycogen storage disease type 1 (type 1a and 1b)
• Stable nighttime glucose control without frequent or severe hypoglycaemia under current dietary treatment, according to capillary glucose measurements.

Exclusion criteria

• Unstable night-time glucose control during the last 8 weeks with frequent (> 4x weekly) hypoglycaemia during night-time, or a hospitalisation due to unstable glucose control during the last 4 weeks, or any episode of severe hypoglycaemia (requiring assistance or loss of consciousness) during the last 4 weeks.
• Pregnancy or breast feeding
• Drug or alcohol abuse
• Acute gastrointestinal problems (e.g. acute gastroenteritis)
• Known malignancy (e.g. hepatocellular carcinoma)

Trial design

Primary purpose: Treatment

Allocation: Non-Randomized

Interventional model: Crossover Assignment

Masking: None (Open label)

---

5 participants in 3 patient groups

uncooked corn starch

Experimental group

Description:

Patients receive uncooked corn starch before bed-time

Treatment:

Other: overnight nutrition regime

modified corn starch

Experimental group

Description:

Patients receive modified corn starch before bed-time

Treatment:

Other: overnight nutrition regime

other carbohydrate (starch) containing meal

Experimental group

Description:

Patients receive a carbohydrate (starch) containing meal before bed-time

Treatment:

Other: overnight nutrition regime