Figure 2 –

Natural history of telomere-mediated noncirrhotic portal hypertension. A-C, Sequential abdominal CT scan images show progressive signs of portal hypertension and right-lobe atrophy that progressed with the clinical course. The age at the time of imaging is shown in the left upper corner. Images are from case 6 (Table 1) and are representative. A, In early stages, splenomegaly and caudate lobe hypertrophy (arterial phase) are seen. B, Subsequently, the liver edge becomes more nodular and the right lobe appears atrophic. C, In decompensated portal hypertension, there is severe liver nodularity, worsening caudate lobe hypertrophy, and ascites. D, Schema summarizes the natural clinical, radiographic, and hallmarks of progression of telomere-mediated noncirrhotic portal hypertension. E, Schema showing typical age of onset and natural history of bone marrow failure, liver disease, and pulmonary fibrosis and emphysema in the telomere syndromes.