The development of renal dysfunction in patients with severe liver disease (acute or chronic) in the absence of any other identifiable causes of renal pathology.
Symptoms: hepatorenal syndrome has no specific signs. features include decreased urine production, dark coloured urine, skin jaundiced (yellow-orange colour), scleral icterus, fetor hepaticus, weight gain, palmar erythema, leuconychia, asterixis, hepatosplenomegalia, abdominal swelling, gynecomastia, change in mental status (dementia, delirium, confusion), coarse muscle movements, jerking, loss of pubic hair/secondary sexual characteristics in men, atrophic testes, nausea, vomiting, blood in vomit, blood in stools etc.
Two types of hepatorenal syndrome have been described. Type I mainly is associated with acute liver failure or alcoholic cirrhosis, but can develop in any other form of liver failure. It is characterized by rapid deterioration of renal function, with marked increase in serum creatinine and blood urea nitrogen. Hyponatremia and hyperkalemia are usual findings. Type I hepatorenal syndrome is characterized by rapid and progressive renal impairment and most commonly is precipitated by spontaneous bacterial peritonitis. Type 1 hepatorenal syndrome occurs in approximately 25% of patients with spontaneous bacterial peritonitis, despite rapid resolution of the infection with antibiotics. Without treatment, median survival of patients with type I hepatorenal syndrome is less than 2 weeks, and virtually all patients die within 10 weeks after the onset of renal failure
Type II is a more stable form. It's characterized by a moderate and stable reduction in glomerular filtration rate and commonly occurs in patients with relatively preserved hepatic function. Median survival is 3-6 months. Although this is markedly longer than type I hepatorenal syndrome, it is still shorter than patients with cirrhosis and ascites without renal failure.
The syndrome occurs in about 4 out of 10 000 people. Data from adult studies suggest that 20% of patients with hepatic failure, ascites, and an initial glomerular filtration rate greater than 50 mL/min develop hepatorenal syndrome within the first year. Incidence increases to 40% by 5-year follow-up visit. Data on children are very scarce, but the suggestion is that hepatorenal syndrome occurs more rarely in children.
This description was submitted by Zoran Bojanic, MD, PhD, Serbia.
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