Gaucher's disease of the lung causing severe pulmonary hypertension with associated acute recurrent pericarditis.

GAUCHER'S DISEASE is a familial disorder characterized by accumulation in reticuloendothelial cells of glucocerebrosides, compounds containing sphingosine, fatty acid, and glucose in equimolar amounts. The storage cells, called Gaucher cells, have a characteristic appearance, and their increasing numbers in the liver, spleen, lymph nodes, and bone marrow are responsible for most of the clinical manifestations of the disorder.' Although nearly all patients have hepatosplenomegaly, Gaucher cells in marrow aspirates, and an abnormally high concentration of non-tartrateinhibitable serum acid phosphatase, at least two forms of Gaucher's disease exist clinically. They probably represent expression of different mutations. In the infantile or acute form, the children have a characteristic stereotyped appearance (strabismus, opisthotonus, retracted lips and often spastic and flexed extremities), and death generally occurs before 2 years of age. The adult or chronic form comprises the majority of cases of Gaucher's disease. It may be detected at any age, usually because of hepatosplenomegaly. Neurological signs rarely if ever occur, osseous lesions are