Novel biochemical abnormalities and genotype in Farber disease

Indian Pediatr. 2012 Apr;49(4):320-2.

Abstract

Farber disease caused by acid ceramidase deficiency is characterised by a triad of painful and swollen joints, subcutaneous nodules, and laryngeal involvement. A one year old female with overlapping features of the classical and type 5 variants is reported. Sialuria and elevated plasma chitotriosidase were unusual findings. A novel mutation of the ASAH 1 gene was detected from DNA extracted from the umbilical stump.

Publication types

  • Case Reports

MeSH terms

  • Acid Ceramidase / genetics
  • Farber Lipogranulomatosis / blood
  • Farber Lipogranulomatosis / diagnosis
  • Farber Lipogranulomatosis / genetics*
  • Fatal Outcome
  • Female
  • Genotype
  • Humans
  • Infant

Substances

  • ASAH1 protein, human
  • Acid Ceramidase