An interventional study of MDR diet assessment and 24 hour recall in Urea Cycle Disorder, Propionic Aciduria and Maple Syrup Urine Disease, sponsored by Oregon Health and Science University. Not yet recruiting. Open to participants aged 1 Year to 80 Years. Per ClinicalTrials.gov, last updated 2024-10-30.
Sponsored by Oregon Health and Science University · Not applicable, Interventional, and Supportive care
The investigators propose to develop and validate MyRareDiet® (MRD) to address an unmet need in the inborn errors of metabolism (IEM) population to assist with dietary management designed to increase adherence and compliance to treatment guidelines, while facilitating the collection of dietary data from individuals with IEM for research purposes.
Inborn errors of metabolism (IEM) are a group of rare genetic disorders that cause a block in a metabolic pathway leading to clinically significant consequences. Most of the disorders are caused by single gene defects that result in a deficiency of a particular enzyme that metabolizes dietary nutrients, eventually causing an accumulation of abnormal metabolites, lack of endogenous production of substances, and/or production of toxic substances that have catastrophic effects on the brain and organs. IEM can lead to developmental and intellectual disabilities, seizures, and, if untreated, coma and death. The majority of the disorders are managed by manipulation and modification of diet alone to reduce toxic metabolites, or with a combination of dietary modification and medications. The complex dietary therapies used in IEM challenge routine dietary recommendations and render diet apps designed for the general public of little utility in these conditions. Additionally, no diet app is developed specifically for the IEM community and meets the rigor required for research, particularly the need to measure specific and multiple amino acids, fats and carbohydrates in the diet to facilitate more study on impact on brain and organs. In IEM, paper diet diaries are often utilized to track food intake and inform patients and their dietitians whether their diets are consistent with the therapy recommendations. These diaries are frequently inadequate in capturing what is actually being consumed due to recall errors and other factors. Novel methods of assessing dietary intake are required to reduce the diet tracking burden, improve accuracy in dietary surveys, and improve diet adherence.
The investigators propose to develop and validate MyRareDiet® (MRD) to address an unmet need in the IEM population to assist with dietary tracking while facilitating the collection of dietary data from individuals with IEM for research purposes. MRD includes a Patient Portal, a Clinical Dietician Portal, and a Researcher Portal. The Patient Portal is to help IEM patients track and monitor their own diet so they can achieve their strict diet targets. The main features include the integration of a major food database for regular foods with a third database for medical foods, the recording of food intake, and daily dietary reports for food monitoring. The Clinical Dietician Portal helps clinical dieticians manage diet modifications for individuals with IEM. It allows a dietitian to individualize MRD for a specific patient based on findings from their nutrition assessment, and set their nutrient goals and restrictions. The Researcher Portal will facilitate the conduct of diet-based studies and the analysis of study results. It offers typical clinical trial management features such as site and staff management, protocol specification, recruitment status, and study data exports.
66 studies on the registry are indexed under Urea Cycle Disorders, Inborn; 14 are open to participants now.
This study's planned enrollment of 60 is above the median of 17 across 39 interventional studies indexed under Urea Cycle Disorders, Inborn.
Browse Urea Cycle Disorders, Inborn studies →Oregon Health and Science University is the lead sponsor of 696 studies on the registry; 131 are open to participants now.
Of its 52 completed or terminated interventional studies of FDA-regulated products, 39 (75%) have results posted.
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Exclusion Criteria:
MyRareDiet (MRD) entries: Participants will enter their dietary intake into the MyRareDiet app on a series of three random nonconsecutive days. Participants will receive reminders from both OHSU bionutritionist (via email, phone, or text) and from the MRD system to record their dietary intake on the MRD app for particular randomized days.
Other: MDR diet assessment
24-hour diet recalls: A series of three randomized nonconsecutive days of 24-hour food recall interviews by telephone will be completed by trained OHSU Bionutrition personnel to determine nutrient intake. Dietary intake will be evaluated with on Nutrition Data System for Research (NDSR) and Metabolic Pro nutrient databases.
Other: 24 hour recall
Participants will record their dietary intake on the MyRareDiet (MRD) app and nutrient content will be assessed to validate MRD compared to a traditional interviewer assessed 24 hour recall.
A trained nutritionist will conduct a telephone multi-pass 24 hour diet recall on 3 randomized nonconsecutive days. The dietary intake will be analyzed using NDSR and metabolic pro and nutrient intake will be compared to the nutrient intake assessed with the MRD app.
Energy
Total reported energy intake
Time frame: 24 hour
Protein
Total percent of calories from protein
Time frame: 24 hour
Carbohydrate
total percent of calories from carbohydrate
Time frame: 24 hour
Fat
Total percent of calories from Fat
Time frame: 24 hour
No study locations are listed for this record.
Plan to share: No — The confidentiality of subjects' data will be maintained by use of de-identification procedures. Informed consent forms that include the subject's name will be stored separately in locked file cabinets. Electronic records will be handled according to HIPAA rules where individually identifiable data, such as names, any identification numbers and certain demographic information, are stored separately and password-protected so that they can be accessed only by the project Investigators. A unique subject identifier (ID) will be generated for each participant and used by the subject to access the developed prototype system and for researchers to retrieve information related to a specific subject. At the end of this study, all patient identifiable data in all formats will be completely destroyed.
No publications or documents are linked to this record.
Start is estimated for Nov 2024; primary completion for Sep 2025, 10 months later.
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This study is not yet recruiting, as verified in Oct 2024. You cannot join it, but the record below documents what was studied.
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