ABSTRACT
B-scan echo-amplitude measurements of the liver were recorded in a prospective clinical trial involving 56 persons--28 normal volunteers and 28 patients with Gaucher disease. The nonspecular liver parenchymal echoes were found to be abnormally elevated in Gaucher patients despite relatively normal liver biochemical values and absence of symptomatic liver disease. Echo amplitude corresponded directly to the amount and histologic distribution of abnormal glycolipid accumulation within the liver. The highest abnormal values were recorded when there was clumping of the glycolipid cells, producing discontinuities within the range of ultrasound resolution. B-scan echo-amplitude measurements accurately represent the degree of histologic abnormality in Gaucher patients, suggesting that this technique should be useful for monitoring enzyme replacement therapy.
