TN 8 (11-12)
DI 23022.957 Hepatorenal Syndrome
COMPASSIONATE ALLOWANCE INFORMATION
Hepatorenal Syndrome Type I/II; Hepato Renal Syndrome
Hepatorenal Syndrome (HRS) is a condition in which there is progressive kidney failure in a person with cirrhosis of the liver, along with portal hypertension and ascites. It is a serious and often life-threatening complication of cirrhosis. HRS occurs when there is a decrease in kidney function in a person with a severe liver disorder. It occurs when there is a decrease in the amount of urine that is removed from the body due to severe liver dysfunction resulting in an increase of nitrogen-containing waste products in the bloodstream (azotemia). Symptoms of HRS may include orthostatic hypotension (a fall in blood pressure occurring when a person sits up or stands up suddenly), change in mental status, muscle spasms/jerks, dark-colored urine, decreased urine production, nausea/vomiting, unexplained weight gain, and yellow skin (jaundice).
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnostic testing: HRS is diagnosed when other causes of kidney failure are clinically ruled out. Laboratory studies may include BUN and serum creatinine levels, urinalysis showing increased urine specific gravity, and tests for low serum sodium and very low urine sodium concentration. Liver tests will show increased prothrombin time, low serum albumin, and sometimes increased serum ammonia levels. Imaging studies may include abdominal ultrasound. Signs of hepatic encephalopathy may also be present.
Physical findings: Edema and reduced urine output (oliguria). The clinical examination will also show signs of chronic liver failure.
ONSET AND PROGRESSION
A diagnosis of HRS occurs in up to 1 in 10 people who are in the hospital due to liver failure and is diagnosed when other causes of kidney failure are ruled out. Complications of HRS may include bleeding, damage to and failure of multi-organ systems, end stage kidney disease, fluid overload with congestive heart failure or pulmonary edema, hepatic coma and secondary infections. The prognosis for people with HRS is poor. Mortality is usually associated with secondary infection or severe bleeding (hemorrhage).
Liver transplantation is the only definitive treatment for HRS. Alternative treatments are supportive and symptomatic. Dialysis, nonsurgical shunt (TIPS) and surgical shunts (Levine) relieve the symptoms of kidney failure.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for evaluation:
Clinical history and physical examination that describes the diagnostic features of the impairment.
Laboratory studies documenting serum creatinine elevation of at least 2 mg/dL; or oliguria with 24-hour urine output < 500 mL; or sodium retention with urine sodium < 10 mEq per liter.
Suggested Listings for Evaluation:
The requirements of 5.05D are met with documentation of chronic liver disease and any one of the three laboratory findings on one evaluation.
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.