Vascular involvement in type 3 neuronopathic Gaucher's disease

Arch Pathol Lab Med. 1985 Jan;109(1):82-4.


A 6 3/4-year-old boy with neuronopathic Gaucher's disease died unexpectedly. Autopsy demonstrated unusual vascular findings with the presence of Gaucher's cells and intimal-medial fibrosis in the ascending aorta and widespread acute hemorrhagic necrosis of the left ventricular myocardium. The combination of eccentric, intimal fibrosis of coronary arteries, left ventricular hypertrophy, pulmonary involvement, and anemia probably accounted for this child's death.

Publication types

  • Case Reports

MeSH terms

  • Aorta / pathology
  • Aortic Diseases / pathology*
  • Child
  • Coronary Disease / pathology*
  • Gaucher Disease / pathology*
  • Humans
  • Male
  • Myocardium / pathology
  • Neurologic Manifestations