Acceptability/Tolerance of Protein Substitutes in Tablet Form for the Dietary Management of Rare Aminoacidopathies
The purpose of this prospective, observational study is to evaluate the tolerability and acceptability of Zero minis, a range of protein substitute tablets for use in the dietary management of children with either TYROSINAEMIA Type I, II, III or ALKAPTONURIA, HOMOCYSTINURIA, or MAPLE SYRUP URINE DISEASE (MSUD) over the age of 7 years.
- EligibilityCriteria: Inclusion Criteria:  Diagnosis of Tyrosinaemia type I, II III or Alkaptonuria requiring a tyrosine- and phenylalanine-free protein substitute. Diagnosis of Homocystinuria requiring a methionine-free, cystine-enriched protein substitute. Diagnosis of MSUD requiring a valine-, leucine- and isoleucine-free protein substitute. Subjects who are already taking a protein substitute for one of the specified rare metabolic disorders and are willing to try the study product for 7 days. Children aged 7 years and over. Written informed consent obtained from parental caregiver.  Exclusion Criteria:  Presence of serious concurrent illness Lead Dietitian's uncertainty about the willingness or ability of the patient to comply with the protocol requirements Participation in any other studies involving investigational or marketed products concomitantly or within two weeks prior to entry into the study. Any children having taken antibiotics over the previous 2 weeks leading up to the study. Children less than 7 years of age. - HealthyVolunteers: No - Gender: All - MinimumAge: 7 Years - MaximumAge: 18 Years - StdAgeList: Child, Adult - StudyPopulation: 15 children out of a patient pool with proven tyrosinaemia type I, II, III or alkaptonuria, homocystinuria, or MSUD will be recruited from two specialist metabolic centres. - SamplingMethod: Non-Probability Sample