Abstract
A case of Letterer-Siwe disease in a 1-year-1-month-old girl with an enlarged liver, markedly swollen gums and seborrhoic eruptions is reported.
She died from pyopneumothorax in spite of treatment with prednisolone and vinblastine.
Lipids of the patient's liver were analyzed. It was found that there was remarkable increase in concentration of free fatty acid, which is not present in normal human liver, and cholesterol ester was also accumlated in the liver. The concentration of phospholipids and glycerides, however, was low and lysophospholipid was found in her liver.
It was assumed that an accelerated lipolysis had undergone in the proliferating reticuloendothelial cells.
