An interventional study of Tyr sphere in Tyrosinemia and AKU, sponsored by Vitaflo International, Ltd. Completed at 1 site in France. Open to participants aged 3 Years and older. Per ClinicalTrials.gov, last updated 2025-11-28.
Sponsored by Vitaflo International, Ltd (part of Nestlé S.A.) · Not applicable, Interventional, and Supportive care
10 participants aged 3 years and over with tyrosinaemia or alkaptonuria will be prescribed Tyr sphere following an assessment of their individual needs by their dietitian.
All participants will enter a 4-week evaluation period, assessing adherence and gastrointestinal tolerance. Evaluations of Tyr sphere's palatability are made at the end of the evaluation period. Dried blood spots are taken on days 1 and 28 and once per week in between.
Participants who continue to take the product at the end of their evaluation period will enter a follow-up period during which metabolic control, anthropometric and nutritional status data will be collected during the yearly standard of care routine visits.
10 participants with tyrosinaemia or alkaptonuria will be invited to participate in this study. Following consent they will be assessed by their dietitian and a recommended amount of Tyr sphere will be prescribed based on their individual needs. Tyr sphere is a food for special medical purposes as defined by Regulation (EU) No 609/2013. It is designed to be prescribed based on its protein content, not its energy content.
Visit 1, baseline clinic visit activities: consent, demographics, anthropometric measurements, gastrointestinal history, dietary assessment, metabolic control assessment including collection of dried blood spots for phenylalanine and tyrosine levels, record routinely taken urine succinylacetone, serum albumin and pre-albumin levels, new dietary prescription.
Weeks 1 to 4 (patient at home): dried blood spots, adverse events reporting/assessment, daily Tyr sphere adherence diary, daily gastrointestinal symptoms diary (weeks 1 and 4 only), daily intake diary for Nitisinone which is routinely prescribed. There will be a telephone call
Visit 2, end of acceptability phase clinic visit activities: anthropometric measurements, review of diaries, review of adverse events, metabolic control assessment including collection of dried blood spots for phenylalanine and tyrosine levels, investigator's assessment of Tyr sphere for the participant and decision on continuing with the prescription.
Three-year routine follow-up: for the participants that do continue with the Tyr sphere prescription (in consultation with the investigator), they will enter a follow-up period of three years, during which only routine, standard of care visits and procedures will take place. Data will be collected on anthropometrics, phenylalanine and tyrosine levels from dried blood spots, adverse events, record routinely taken urine succinylacetone, serum albumin and pre-albumin levels.
20 studies on the registry are indexed under Tyrosinemias; 3 are open to participants now.
This study's enrollment of 14 is below the median of 20 across 11 interventional studies indexed under Tyrosinemias.
Browse Tyrosinemias studies →Vitaflo International, Ltd is the lead sponsor of 23 studies on the registry; none are open to participants now.
First submitted after Vitaflo International, Ltd became part of Nestlé S.A..
Counted across the registry records on this site, refreshed daily.
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
Exclusion Criteria:
N.B.: Women who become pregnant during the study will no longer be able to participate and will be withdrawn.
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
A drug or device being studied in a clinical trial.
It may be new, or already approved and being studied for another use, dose or form. Criteria often leave out people who took part in another study of one within a recent period, commonly 30 days or five half-lives.
Informed consent
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Investigational drug or device
A drug or device being studied in a clinical trial.
It may be new, or already approved and being studied for another use, dose or form. Criteria often leave out people who took part in another study of one within a recent period, commonly 30 days or five half-lives.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
All patients to receive Tyr sphere as part of their dietary management for tyrosinaemia or alkaptonuria (AKU).
Dietary Supplement: Tyr sphere
Tyr sphere is a powdered, low phenylalanine and tyrosine protein substitute, containing a balanced mix of casein glycomacropeptide (cGMP) isolate, essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals and the long chain polyunsaturated fatty acid (LCP) and docosahexaenoic acid (DHA). It contains sugars and sweetener. The product is designed to be prescribed based on its protein content, not its energy content.
Change in adherence to recommended amounts of Tyr sphere
Patient reported data on actual compared to prescribed intakes of Tyr sphere, assessed by HCPs periodically as per routine practice.
Time frame: Recorded daily on days 1-28
Change in gastrointestinal tolerance
Patient reported data on any gastrointestinal symptoms experienced, assessed by HCPs periodically as per routine practice.
Time frame: Recorded daily on days 1-28
Patient evaluation of Tyr sphere's palatability
Patient-reported assessment of Tyr sphere's palatability on a Likert scale: 1 (really didn't like it) to 5 (loved it).
Time frame: Day 28, end of acceptability phase
Change in metabolic control: tyrosine levels
Tyrosine levels obtained from dried blood spots
Time frame: Day 1, week 1, week 2, week 3, week 4, day 28
Change in metabolic control: phenylalanine levels
Phenylalanine levels obtained from dried blood spots
Time frame: Day 1, week 1, week 2, week 3, week 4, day 28
Long term change in growth
Recorded progress in growth from standard of care follow-up visits for three years
Time frame: 3-year follow-up period
Long term change in urine succinylacetone level
Recorded changes in urine succinylacetone levels from standard of care follow-up visits for three years
Time frame: 3-year follow-up period
Long term change in pre-albumin level
Recorded changes in pre-albumin levels from standard of care follow-up visits for three years
Time frame: 3-year follow-up period
Long term change in serum albumin level
Recorded changes in serum albumin levels from standard of care follow-up visits for three years
Time frame: 3-year follow-up period
Plan to share: No
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This study is completed, as verified in Nov 2025. You cannot join it, but the record below documents what was studied.
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