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.
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.
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.
metaX Institut fuer Diatetik GmbH
Lead sponsor
Birmingham Women's and Children's NHS Foundation Trust
Collaborator
Great Ormond Street Hospital for Children NHS Foundation Trust
Collaborator