Pediatric Hepatorenal Syndrome Clinical Presentation
- Author: Rajendra Bhimma, MB, MD, ChB, PhD, DCH (SA), FCP(Paeds)(SA), MMed(Natal); Chief Editor: Timothy E Corden, MD more...
Patients with hepatorenal syndrome (HRS) present with symptoms of renal failure in the setting of acute or chronic liver failure with portal hypertension complicated by ascites, jaundice, hepatic encephalopathy, and coagulopathy.
Urine volume is typically less than 500 mL/d and does not show sustained improvement after diuretic withdrawal and plasma volume expansion.
The 1996 International Ascites Club (IAC) defined 6 major criteria necessary for the diagnosis of hepatorenal syndrome and 5 additional criteria, which are findings usually associated with hepatorenal syndrome but are not required for diagnosis.
The 6 required major criteria are as follows:
Chronic or acute hepatic disease with advanced liver failure and portal hypertension
Low glomerular filtration rate (GFR), defined as a serum creatinine level of more than 1.5 mg/dL or 24-hour clearance of less than 40 mL/min
Absence of shock, ongoing bacterial infection, fluid loss, or current or recent treatment with nephrotoxic medications
No sustained improvement in renal function (serum creatinine level ≤1.5 mg/dL or clearance >40 mL/min) after the withdrawal of diuretics and after plasma volume expansion with 1.5 L of isotonic sodium chloride solution
Proteinuria of less than 500 mg/d
Absence of any evidence of obstructive uropathy or parenchymal disease, as demonstrated on ultrasonography
The 5 additional criteria are as follows:
Urine volume of less than 500 mL/d
Urine sodium concentration of less than 10 mEq/L
Urine osmolality greater than plasma osmolality
Urine RBC count of less than 50 per high-power field
Serum sodium concentration of less than 130 mEq/L
The new IAC criteria for HRS are as follows :
Cirrhosis with ascites
Serum creatinine greater than 1.5 mg/dL
No improvement of serum creatinine (a decrease in serum creatinine < 1.5 mg/dL) after 2 days of diuretics and volume expansion with albumin (1 g/kg body weight up to a maximum of 100 g/day)
Absence of shock
No current or recent treatment with nephrotoxic drugs
Absence of signs of parenchymal renal disease, as suggested by proteinuria (>500 mg/day) or hematuria (>50 red blood cells per high-power field) and/or abnormal renal ultrasound
The essential differences between the two sets of diagnostic criteria are as follows:
Creatinine clearance no longer incorporated in the diagnostic criteria
Ongoing bacterial infection does not exclude the diagnosis of HRS, provided septic shock is not present
Albumin is preferred to saline for plasma volume expansion
Nonessential minor diagnostic criteria, including low fractional sodium excretion of sodium and oliguria, have been omitted
Ascites is almost always present in patients with advanced hepatic failure and cirrhosis. The mechanism of ascites formation in these patients is likely a combination of increased sinusoidal pressure and hepatic insufficiency, as well as decreased renal function.
Jaundice, hepatic encephalopathy, and clinically significant coagulopathy may all be present to some degree as part of the hepatic failure, especially in patients with type 1 hepatorenal syndrome.
Resting low arterial blood pressure is a frequent finding in patients with hepatorenal syndrome despite increased plasma volume, high cardiac output, the presence of potent vasoconstrictors ((e.g., renin, angiotensin II, vasopressin), and an activated sympathetic nervous system. The systemic hypotension is attributed to a marked splanchnic vasodilation secondary to portal hypertension and local hepatic vasodilators.
Hepatorenal syndrome may complicate any form of severe liver disease. Many liver transplantations are performed in children with chronic liver disease secondary to biliary atresia. In children, 50% of all cases of acute hepatic failure are secondary to acute viral hepatitis. Other etiologies include acetaminophen toxicity, Wilson disease, liver malignancy, and autoimmune hepatitis.
Several findings are often seen in patients who develop hepatorenal syndrome and include the following:
Previous episodes of ascites
Absence of hepatomegaly
Poor nutritional status
Moderately increased BUN and creatinine levels secondary to the lack of endogenous production and low muscle mass
Severely decreased GFR
Low serum sodium value (ie, dilutional hyponatremia)
Moderately elevated serum potassium levels
Low urinary sodium excretion
High plasma renin activity
Low arterial blood pressure
Increased plasma norepinephrine level
Clinically significant esophageal varices
Among adults with cirrhosis and portal hypertension, several precipitating factors have been implicated in the progression to hepatorenal syndrome. These factors include spontaneous bacterial peritonitis, large volume paracentesis without simultaneous or subsequent plasma expansion, and GI or variceal bleeding.
Patients with hepatic failure and ascites are prone to develop spontaneous bacterial peritonitis. In approximately 20% of adults with advanced liver disease and spontaneous bacterial peritonitis, their condition progresses to hepatorenal syndrome.
Large-volume paracentesis without plasma volume replacement is associated with the development of hepatorenal syndrome in as many as 15% of patients with ascites. Renal failure is attributed to a circulatory dysfunction after paracentesis and is preventable by administering an isotonic solution, or preferably intravenous (IV) albumin, during or soon after the procedure.
Hepatorenal syndrome develops in as many as 10% of adults after a clinically significant upper or lower GI bleed. Care must be taken to rule out acute tubular necrosis from volume depletion as the etiology of the renal failure after a GI bleed because hepatorenal syndrome is a diagnosis of exclusion.
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