Biliary atresia (BA) is a rare disease of unknown etiology which leads to cirrhosis and death if left untreated. The standard of care is an early hepatoportoenterostomy (HPE). Long-term follow-up is mandatory, during which most patients will require a liver transplant.
View Article and Find Full Text PDFAutoimmune pancreatitis type 2 is a relatively novel entity with some still controversial issues. The current diagnostic algorithm relies on imaging studies and histology. Therapy includes corticosteroids with consequently low risk of relapse in the following year.
View Article and Find Full Text PDFCrigler-Najjar syndrome (CNS) is an exceedingly rare autosomal recessive disease with an estimated incidence of 1 in a million live births. CNS type 1 (CNS1) is the most severe form, characterized by severe unconjugated hyperbilirubinemia since birth due to the absence of hepatic uridine 5'-diphosphate glucuronyltransferase (UGT1A1) activity. Daily phototherapy (PT) and liver transplant (LT) are the mainstays of therapy.
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