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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease gigantism
Comorbidity C0008370|cholestasis
Sentences 3
PubMedID- 23430923 We describe a patient who presented in the immediate postnatal period with cholestasis without hepatomegaly associated with hepatocellular giant-cell transformation on liver biopsy, thrombocytopenia, and failure to thrive.
PubMedID- 22937766 (a) canalicular cholestasis (arrow) with scattered hepatic giant cells containing eight to ten nuclei (asterisks), and (b) a representative portal tract containing two small dilated veins, a small artery, and no recognizable bile ducts.
PubMedID- 21255461 Pathologic findings in this disease include intralobular cholestasis with giant cell transformation, prevalence of necrotic hepatocytes including giant cell forms, and hepatic injury confined to the portal limiting plate where the smallest bile ductules may be injured, resulting in neocholangiolar proliferation.

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