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CT of the nasal sinuses showing findings compatible with acute inflammatory changes, with histology of the nasal mucosa inconclusive. Thus, this case shows an exuberant and potentially fatal form of diffuse alveolar hemorrhage that culminated in the initial diagnosis of granulomatous vasculitis in a young adult. The truncating mutations in tight junction protein 2 (TJP2) cause progressive cholestasis, liver failure, and hepatocyte carcinogenesis. Due to the lack of effective model systems, there are no targeted medications for the live
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