RADIOGRAPHIC FINDINGS IN TYPE 3B GAUCHER DISEASE

The purpose of this paper is to describe the radiographic findings in type 3b Gaucher disease, a chronic neuronopathic form of the illness w ith severe systemic manifestations. Between 1980 and 1985 17 consecuti ve patients were evaluated with radiography of the chest, long bones a nd spine, CT of the head and chest, abdominal sonography, and MRI of t he head, abdomen and spine. Clinical manifestations were severe, and l ed to death from hepatic, pulmonary or cardiac failure in nine patient s. Type 3b Gaucher disease shares the same spectrum of radiographic fi ndings observed in type 1 disease, but the systemic manifestations are more severe. Pulmonary infiltrates, thoracic lymph node enlargement, vertebral compression fractures and osteonecrosis of the long bones oc cur much more frequently in patients with type 3b disease.