Rare Medical Problems

Transjugular intrahepatic portosystemic shunts Only a few studies have reported on the effects of transjugular intrahepatic portosystemic shunts (TIPS) in patients with type 1 HRS. This procedure consists of insertion of an intrahepatic stent between the portal and hepatic veins by a transjugular approach. The main effect is to lower portal pressure. In type 1 HRS, TIPS improve circulatory function and reduce the activity of vasoconstrictor system.These effects are associated with a slow, moderate to strong increase in renal perfusion and GFR and a fall in serum creatinine concentrations in about 60% of patients. Median survival after TIPS in type 1 HRS is between 2 months and 4 months.As with vasoconstrictor drugs, the improved renal function probably, but not definitely, results in longer survival. Information currently available on the use of TIPS in type 1 HRS has been obtained in a very selected population of patients and may not be appli...

Liver transplantation The treatment of choice for patients with cirrhosis and type 1 HRS who are suitable for the procedure is liver transplantation, because it allows both the liver disease and the associated renal failure to be cured. The most common contraindications for transplantation in HRS are advanced age, active alcoholism, and infection. The main problem in the use of liver transplantation for type 1 HRS is that many patients die before transplantation is possible because of the short survival expectancy and long waiting times in most transplant centres. The issue can be solved by assigning these patients a high priority for transplantation from a cadaveric donor. This approach was used with the former method of organ allocation used by the United Network for Organ Sharing in the USA, which classified patients with HRS in the 2a status, with a median waiting time of about 7 days. Now this system has been changed and livers are allocated on the basis of the MELD (...

Hepatorenal syndrome The hepatorenal syndrome refers to renal failure of unknown etiology that occurs in a fully hydrated patient with severe, often progressive, liver disease. Urine biochemistry is characteristic and renal histopathology unremarkable. The pathogenesis of the hepatorenal syndrome appears to involve intense intrarenal vasoconstriction and alteration in renal cortical blood flow, possibly due to an imbalance of prostaglandins and thromboxane. Early clinical experience suggested that only orthotopic liver transplantation was able to reverse the hepatorenal syndrome, although more recent evidence has reported up to 50 per cent survival in hepatorenal failure following paracetamol (acetaminophen) overdose. Renal failure is reversed after liver transplantation, suggesting that it is due to circulating or systemic factors. Excessive use of diuretics, sepsis, abdominal paracentesis, and gastrointestinal hemorrhage are all associated with an increased risk...