Pediatric Cholestatic Disorders

This article addresses select liver diseases that are commonly seen in the pediatric group and pose diagnostic challenges in practice. The key genetic/molecular abnormalities, clinical features, histopathologic findings, diagnostic modalities, differential diagnoses, and possible pitfalls in diagnosis are discussed in detail. Although recent advances in understanding the pathophysiology of bile synthesis and transport along with advances in molecular genetics have allowed a better characterization of many of these liver diseases, significant overlap in the histopathologic features of many of these disorders still leads to diagnostic challenges for the pathologist.