Assessment of the liver and spleen in children with Gaucher disease type I with diffusion-weighted MR imaging.

Purpose: To assess hepatic and splenic apparent diffusion coefficient (ADC) in children with Gaucher disease type I with diffusion-weighted MR imaging and to correlate hepatic and splenic ADC with parameters of disease severity. Subjects and

Methods: Prospective study was conducted upon 25 children (11 treated and 14 untreated) with Gaucher disease and 12 age and sex matched control children. They underwent diffusion-weighted MR imaging of abdomen. Hepatic and splenic ADC and volume were calculated.

Results: There was statistically difference in hepatic and splenic apparent diffusion coefficient (P=0.001) between patients and controls. The cutoff ADC of liver and spleen used to differentiate patients from controls were 0.47 and 0.39×10-3mm2 with area and curve of 0.947 and 0.886 respectively. There was significant difference in hepatic and splenic ADC between untreated and treated patients (P=0.003 and 0.001). Hepatic ADC correlated with splenic volume (r=-0.721), hepatic volume (r=-0.555) and chitotriosidase (r=-0.413). Splenic ADC correlated with splenic volume (r=-0.652), hepatic volume (r=-0.544) and chitotriosidase (r=-0.355).

Conclusion: Hepatic and splenic ADC can detect hepatic and splenic infiltration in Gaucher disease and correlated with some parameters of disease severity.