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Phenotype profile

maple syrup urine disease

SaudiVarKB evidence summary derived from retained literature mentions.

15Phenotype mentions
15Publications
4Associated gene records
0Associated variant records

Associated gene records

Co-mentioned in the same publications
GeneIdentifier / contextArticlesMentions
BCKDHAHGNC:98622
BCKDHBHGNC:98722
DBTHGNC:269822
DLDHGNC:289811

Associated variant records

Co-mentioned in the same publications

No retained variant associations.

Associated population records

Co-mentioned in the same publications
PopulationIdentifier / contextArticlesMentions
Saudi Arabia11
Population record11
Population recordCohort 1011
Population recordCohort 2211
Population recordCohort 4,03811
Population recordCohort 3311
Saudi ArabiaCohort 13511
Saudi Arabia · RiyadhCohort 56,63211
Saudi ArabiaCohort 5211

Supporting publications

15 records
  1. 2026Case Report: Graves' disease in a toddler with maple syrup urine disease: therapeutic challenges and surgical resolution.Frontiers in pediatrics1 mentions
  2. 2025Unlocking hope: domino liver transplantation for maple syrup syndrome, a single center experience work carried out at the King Fahad Specialist Hospital.Frontiers in immunology1 mentions
  3. 2025Insights from Metabolomics Profiling of MSUD in Pediatrics Toward Disease Progression.Metabolites1 mentions
  4. 2025Cerebral edema in maple syrup urine disease: spectrum of clinical presentation and treatment outcomes.Orphanet journal of rare diseases1 mentions
  5. 2025Pediatric liver transplant for maple syrup urine disease a single center experience.Frontiers in pediatrics1 mentions
  6. 2024Successful adult domino living donor liver transplantation in methylmalonic acidemia: case report.Translational gastroenterology and hepatology1 mentions
  7. 2024Exploratory Untargeted Metabolomics of Dried Blood Spot Samples from Newborns with Maple Syrup Urine Disease.International journal of molecular sciences1 mentions
  8. 2023A Smart Monitoring System for Self-Nutrition Management in Pediatric Patients with Inherited Metabolic Disorders: Maple Syrup Urine Disease (MSUD).Healthcare (Basel, Switzerland)1 mentions
  9. 2023Knowledge-Based Dietary Intake Recommendations of Nutrients for Pediatric Patients with Maple Syrup Urine Disease.Healthcare (Basel, Switzerland)1 mentions
  10. 2020Incidence of newborn screening disorders among 56632 infants in Central Saudi Arabia. A 6-year study.Saudi medical journal1 mentions
  11. 2020Genotype-phenotype correlation of 33 patients with maple syrup urine disease.American journal of medical genetics. Part A1 mentions
  12. 2017Twenty novel mutations in BCKDHA, BCKDHB and DBT genes in a cohort of 52 Saudi Arabian patients with maple syrup urine disease.Molecular genetics and metabolism reports1 mentions
  13. 2011New and known mutations associated with inborn errors of metabolism in a heterogeneous Middle Eastern population.Saudi medical journal1 mentions
  14. 2005Diffusion-weighted MR imaging in leukodystrophies.European radiology1 mentions
  15. 1996Saudi aminoacidemias: a six-year study.Indian journal of pediatrics1 mentions