SI

sucrase-isomaltase
OMIM: 609845, Gene2Phenotype

4 panels

Panel Reviews Mode of inheritance Details
4 panels
Green SI in Intestinal failure or congenital diarrhoea


Level 2: Gastrohepatology
Version 3.7
Latest signed off version: v3.0 (22 Mar 2023)

review BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
Phenotypes
  • Sucrase-isomaltase deficiency, congenital, OMIM:222900
Green SI in Undiagnosed metabolic disorders

Level 3: Specific metabolic abnormalities
Level 2: Metabolic disorders
Version 1.642

review BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Literature
Phenotypes
  • Disaccharide intolerance 1 (Other carbohydrate disorders)
  • CONGENITAL SUCRASE-ISOMALTASE DEFICIENCY 222900
Tags
  • treatable
Green SI in Likely inborn error of metabolism


Level 2: Metabolic
Version 8.91
Latest signed off version: v8.0 (30 Apr 2025)

Component of the following Super Panels:

  • Childhood onset leukodystrophy
  • Hypotonic infant
  • Paediatric disorders
  • Unexplained death in infancy and sudden unexplained death in childhood
  • review BIALLELIC, autosomal or pseudoautosomal
    Sources
    • London North GLH
    • NHS GMS
    • Expert Review Green
    Phenotypes
    • CONGENITAL SUCRASE-ISOMALTASE DEFICIENCY 222900
    • Disaccharide intolerance 1 (Other carbohydrate disorders)
    Red SI in Childhood onset dystonia, chorea or related movement disorder


    Level 2: Neurology
    Version 7.13
    Latest signed off version: v7.0 (30 Apr 2025)

    review Not set
    Sources
    • Expert Review Red
    • London North GLH