Management of primary sclerosing cholangitis.

Minerva Gastroenterol Dietol. 2009 Jun;55(2):163-72

Authors: Björnsson E

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with fibrosis surrounding the intrahepatic and/or the extrahepatic bile ducts. PSC is characterized by progressive periductal obliterating fibrosis and bile duct strictures. In individual cases, PSC can have favorable prognosis but in most cases it is a progressive disorder which leads to liver related morbidity, mortality and the need for liver transplantation. In previous early cohorts median survival free of transplantation was 12 years whereas more recent studies indicated a median transplantation free survival of 18 years. More recently, patients with small-duct PSC, who have biochemical and histological features similar to other PSC patients but with a normal cholangiography have been shown to have a better prognosis than classic large-duct PSC. PSC is complicated by cholangiocarcinoma (CCA) which develops in 10-30% of PSC patients depending on the length of follow-up. The diagnosis of an early CCA in the setting of PSC is a major challenge and no consensus on screening strategies exists. No curative therapy for PSC is available at the current time except liver transplantation. The etiopathogenesis of PSC is unknown but the underlying pathophysiology of PSC is beyond the scope of this paper.

PMID: 19305376 [PubMed - indexed for MEDLINE]

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The management of hepatorenal syndrome.

Minerva Gastroenterol Dietol. 2009 Jun;55(2):207-26

Authors: Carl DE, Sanyal A

The hepatorenal syndrome (HRS) is a common complication in advanced liver cirrhosis, and often occurs in patients with ascites and severe circulatory dysfunction. HRS is a functional renal failure which was believed to be the end result of progressive splanchnic vasodilatation. However, recent data have implicated a role of reduced cardiac output as well as endothelial dysfunction in the etiology of HRS. Type 1 HRS is associated with a poor prognosis and often occurs in conjunction with microcirculatory dysfunction in other organs, including the heart, brain and liver. The treatment of type 1 HRS has centered around vasoconstrictors and intravenous hydration, traditionally midodrine and albumin. However, new vasoconstrictors (specifically vasopressin analogues), transjugular intrahepatic portacaval shunts, and albumin dialysis have been introduced as potential therapies. This review will discuss new advances in the diagnosis and pathogenesis of HRS, with an emphasis on the management.

PMID: 19305378 [PubMed - indexed for MEDLINE]

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