BACKGROUND: We present nine @entity1 with progressive @entity991 after @entity326 . @entity1 : All nine @entity1 had previously required long term treatment in an intensive care unit for @entity326 : two @entity1 with @entity1230 , five with @entity130 , and two with extensive surgery. They were admitted to our hospital because of @entity255 . Endoscopic retrograde cholangiography revealed severe intrahepatic stenoses in all @entity1 and liver biopsies showed typical signs of @entity991 . No @entity1 had pre-existing @entity409 . RESULTS: Mean follow up time was 35 months. In @entity1 with major bile duct stenoses (3/9), 12 endoscopic dilations were performed in total. In one @entity1 , concrements were extracted and intermittent stenting was necessary. To date, 4/9 @entity1 have rapidly developed @entity1575 . During follow up, 5/9 @entity1 died: two after fulminant @entity255 , one after @entity1578 , one due to liver transplantation associated problems, and one after @entity1779 . One @entity1 has been registered for transplantation and the remaining three @entity1 show no acute signs of @entity1578 . CONCLUSIONS: @entity1 with @entity991 , following @entity326 , represent a new variant of @entity1157 . In such @entity1 @entity409 rapidly progresses to @entity82 . Endoscopic treatment may only transiently improve the course of the disease. Orthotopic liver transplantation is indicated in @entity454 .

Progressive @entity991 after XXXX : a new variant of @entity1157 .

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients', 'patient', 'PATIENTS'] @entity82 :: ('MESH:D005355', 'Disease') :: ['cirrhosis'] @entity326 :: ('MESH:D012772', 'Disease') :: ['septic shock'] @entity1157 :: ('MESH:D001649', 'Disease') :: ['vanishing bile duct disorders'] @entity991 :: ('MESH:D015209', 'Disease') :: ['sclerosing cholangitis'] @entity409 :: ('MESH:D008107', 'Disease') :: ['liver disease'] @entity1578 :: ('MESH:D017093', 'Disease') :: ['liver failure'] @entity1230 :: ('MESH:D009104', 'Disease') :: ['polytrauma'] @entity1779 :: ('MESH:D002543', 'Disease') :: ['cerebral ischaemia'] @entity1575 :: ('MESH:D008103', 'Disease') :: ['liver cirrhosis'] @entity255 :: ('MESH:D002761', 'Disease') :: ['cholangitis'] @entity130 :: ('MESH:D014947', 'Disease') :: ['burn injury'] @entity454 :: ('MESH:D007676', 'Disease') :: ['end stage disease']
@entity326 :: (MESH:D012772,Disease) :: ['septic shock']
