Sphingomyelinase (lysosome) disorder. Type B has only visceral involvement, can survive into adulthood. More common in Ashkenazi Jews.
Typically develop symptoms at around 6 months. Can be prolonged jaundice as baby, else abdominal distension (hepatosplenomegaly), growth failure, hypotonia, failure to meet milestones.
Death usually around 3yrs, recurrent lung infections, interstitial lung disease. Spasticity develops later.
Cherry red spot seen on fundoscopy in macula, possibly not in early stages.