Transaldolase deficiency in a two-year-old boy with cirrhosis

Mol Genet Metab. 2008 Jun;94(2):255-8. doi: 10.1016/j.ymgme.2008.01.011. Epub 2008 Mar 10.

Abstract

Transaldolase (TALDO) deficiency is a rare inborn error of the pentose phosphate pathway. We report the clinical presentation and laboratory findings of a new patient with TALDO deficiency. The two-year-old Arabic boy presented with neonatal onset of anemia and thrombocytopenia, tubulopathy, and rickets and was subsequently found to have cirrhosis and deafness. A comparison with other TALDO deficient patients is given.

Publication types

  • Case Reports
  • Comparative Study

MeSH terms

  • Adolescent
  • Child, Preschool
  • Deafness / etiology
  • Humans
  • Liver Cirrhosis / etiology*
  • Liver Cirrhosis / pathology
  • Male
  • Metabolism, Inborn Errors / complications
  • Metabolism, Inborn Errors / enzymology*
  • Metabolism, Inborn Errors / genetics*
  • Mutation, Missense
  • Pentose Phosphate Pathway*
  • Rickets / etiology
  • Transaldolase / deficiency*
  • Transaldolase / genetics*
  • Transaldolase / metabolism
  • Urine / chemistry

Substances

  • Transaldolase