Hepatorenal syndrome refers to a form of acute kidney injury caused by changes in renal blood flow regulation due to liver pathology 1. Although the syndrome occurs mainly in cirrhotic livers it has been reported in patients with acute fulminant liver failure as well 1.
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Epidemiology
The incidence of hepatorenal syndrome in patients with end-stage liver disease is up to 7.6% 2.
Clinical presentation
The patient usually has chronic liver disease with cirrhosis. Symptoms may include reduced urine output, and there may or not be a precipitant (e.g. spontaneous bacterial peritonitis). The signs and symptoms of underlying chronic liver disease may also be present.
The main laboratory finding of hepatorenal syndrome is an acutely elevated serum creatinine level. However, it is difficult to diagnose hepatorenal syndrome based on these non-specific findings alone, instead it is a diagnosis of exclusion after other causes of acute kidney injury have been ruled out 2.
Pathology
Hepatorenal syndrome is thought to develop from a number of pathological processes occurring in the diseased liver. One of these processes is portal hypertension causing bacterial translocation from the gut into the portal system, as well as causing splanchnic and systemic blood vessel dilatation, and the activation of liver chemoreceptors and baroreceptors. The summative effect of these lead to the activation of neurohumoral pathways (e.g. sympathetic nervous system, renin-angiotensin-aldosterone system) which stimulate renal vasoconstriction and reduce blood flow to the kidneys leading to injury 2,3.
Classification
Two types have been identified, based upon how rapid the decline in renal function is:
- type 1 hepatorenal syndrome: rapidly progressive with a doubling of creatinine in two weeks or less
- type 2: defined as simply less acute than type 1
Radiographic features
Ultrasound
In cases of hepatorenal syndrome where there is no pre-existing intrinsic renal disease the kidneys should show a normal size and no evidence of atrophy 2. Normal appearing kidneys on ultrasound is required for the diagnosis of hepatorenal syndrome according to the Hepatorenal Syndrome-AKI Guidelines produced by the International Club of Ascites 2.
Treatment and prognosis
Management is typically supportive with vasopressor and albumin infusions 1, although management directed at treating a precipitant, if identified, is also important. However, the only definitive treatment for hepatorenal syndrome is liver transplantation 2. Hepatorenal syndrome in patients with liver cirrhosis heralds a poor prognosis in the realm of weeks to months 4.
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