Hepatorenal Syndrome
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Contents |
Introduction
- Severe renal hypoperfusion in patients with terminal hepatic cirrhosis. It may also develop with toxic agents that damage both the liver & kidney.
Etiology
- pre-renal hypoperfusion
- vasculitis
- acute Wilson's disease
Epidemiology
- occurs in 40-50% of patients with terminal cirrhosis
- generally develops in a hospital setting
- some degree of functional renal impairment occurs in 1/2 of patients with cirrhosis & ascites [7]
Pathology
- liver failure resulting in renal hypoperfusion
- endothelin levels are 10 X normal
- leukotriene abnormalities suggested
- activated sympathetic nervous system
- activated renin- angiotensin axis
- severe vasoconstriction
- shunting of renal plasma flow from cortical to medullary segments
- transition to acute tubular necrosis is possible
- recovery occurs in about 10% of patients
- histology does NOT suggest primary renal disease
Clinical-manifestations
- ascites
- portal hypertension
- jaundice
- progressive azotemia & oliguria
- hypotension/ low blood pressure
- esophageal varices may be present
Diagnostic-criteria
- major criteria*
- acute or chronic liver disease with advanced hepatic failure & portal hypertension
- low glomerular filtration rate
- serum creatinine > 1.5 mg/dL
- creatinine clearance < 40 mL/min
- absence of
- treatment with nephrotoxic agents
- shock
- infection
- significant recent fluid loss
- no sustained improvement of renal function after discontinuation of diuretics & administration of 1.5 L of normal saline
- proteinuria < 500 mg/dL & no ultrasonographic evidence of obstruction or renal parenchymal disease
- minor criteria**
- urine volume < 500 mL/day
- urine Na+ < 10 meq/L
- urine osmolality > plasma osmolality
- urine RBC < 50/ hpf
- serum [Na+] < 130 meq/L
- * must be present for diagnosis ** based on criteria of low GFR & avid Na+ retention
Laboratory
- (in addition to above)
- hyponatremia
- hypoalbuminemia
- fractional excretion of Na+ ( FENA) < 1%
- high urine Na+ in the absence of diuretics suggests another diagnosis
- high plasma renin activity
- low plasma osmolality, high urine osmolality
- high serum potassium
- increased serum urea (>30 mg/dL)
- increased serum creatinine (>1.5 mg/dL)
- diminished GFR (<50 mL/min)
Management
- supportive measures
- discontinue potentially offending agents
- optimized central & renal hemodynamics
- volume expansion with albumin to assess component of prerenal azotemia
- low dose (renal) dopamine
- high doses of spironolactone ( Aldactone)
- norepinephrine may delay mortality [5]
- combination of octreotide + midodrine is often used
- terlipressin is used in Europe (not FDA-approved)
- LaVeen shunt
- liver transplantation is treatment of choice
- prognosis: 3-6 month survival is ~ 20-40%
More General Terms
Additional Terms
References
- Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 596
- Medical Knowledge Self Assessment Program (MKSAP) 11, 15, American College of Physicians, Philadelphia 1998, 2009
- Medical Guidelines for Determining Prognosis in non-Cancer Diseases, 2nd edition, Stuart et al (eds), National Hospice Organization, Arlington, VA, 1996
- Journal Watch 22(18):145, 2002 Duvoux C et al, Hepatology 36:374, 2002 Gines P & Guevara M, Hepatology 36:504, 2002
- eMedicine: Hepatorenal Syndrome [1]
- Montoliu S et al. Incidence and prognosis of different types of functional renal failure in cirrhotic patients with ascites. Clin Gastroenterol Hepatol 2010 Jul; 8:616. PMID: [2]
