Fig. 4.

CXR of patient B.

B-1: Lung involvement found on the image with no respiratory symptoms after 30 months of ERT (60 IU/kg/2 weeks). ERT dosage was then increased to 100 IU/kg/2 weeks. Reticular infiltration over bilateral lung fields, with increased perihilar infiltration and suspicious consolidative patchy over bilateral lower lung fields.

B-2: 11 months after treatment with higher dose ERT (100 IU/kg/2 weeks). Patient B still presented with no respiratory symptoms and normal pulmonary function tests, radiologic findings were mildly improved. Increased interstitial infiltration noted over the bilateral lung field, with prominent pulmonary vasculature.