Natural history and management of liver dysfunction in lysosomal storage disorders

Figure 1 Pathogenesis of Gaucher disease. GD: Gaucher disease.

Figure 2 Histology of Gaucher disease. Red arrow shows Gaucher cells.

Figure 3 Pathogenesis of Niemann-Pick disease types A, B, and C. NPD: Niemann-Pick disease.

Figure 4 Fundus examination showing a cherry red spot (red arrow) with a background of white retina.

Figure 5 Histology in Niemann-Pick disease type B. Red arrow shows a foamy vacuolated histiocyte.

Figure 6 Infant with Niemann-Pick disease type C presenting as cholestasis, dilated abdominal veins, and massive hepatosplenomegaly.

Figure 7 Pathogenesis of lysosomal acid lipase deficiency. CESD: Cholesteryl ester storage disease; VLDL: Very low-density lipoproteins.

Figure 8 Computed tomography of the abdomen in an infant with Wolman disease showing bilateral adrenal calcifications.