II. Definition

  1. Renal Failure due to Hepatic Cirrhosis
  2. Intrinsic renal disease absent

III. Epidemiology

  1. Less common in Primary Biliary Cirrhosis
  2. Incidence in Cirrhosis with Ascites
    1. One year 18%
    2. Five years: 39%

IV. Pathophysiology

  1. Arterial vasodilation of splanchnic circulation
    1. Results in underfilling of arterial circulation
  2. Renin-Angiotensin System Activation
    1. Results in decreased renal perfusion due to renal vasconstriction

V. Types

  1. Type I Hepatorenal Syndrome
    1. Associated with Spontaneous Bacterial Peritonitis
      1. Occurs in 25% of patients with SBP
    2. Rapid deterioration of Renal Function
      1. Serum Creatinine doubles to >2.5 mg/dl or
      2. Creatinine Clearance <20 ml/minute
    3. Survival
      1. Without treatment: <2 weeks (median)
      2. With Treatment: 10 weeks
  2. Type II Hepatorenal Syndrome
    1. Associated with Diuretic-resistant Ascites
    2. Renal Function declines moderately
      1. Serum Creatinine >1.5 mg/dl
    3. Survival: 3-6 months (median)

VI. Diagnosis

  1. Major Criteria
    1. Liver failure with Portal Hypertension
    2. Decreased Glomerular Filtration Rate (GFR)
      1. Serum Creatinine >1.5 mg/dl or
      2. Creatinine Clearance < 40 ml/min
    3. No alternative causes of Acute Renal Failure
      1. No Nephrotoxins
      2. No shock, Sepsis or hypovolemia due to excessive diuresis
      3. Urine Protein <500 mg/dl
      4. No parenchymal renal disease by renal Ultrasound
      5. No ureteral obstruction by renal Ultrasound
    4. Renal Failure refractory to measures
      1. Diuretics withdrawn
      2. Volume expansion: 1.5 Liters Normal Saline
  2. Minor Criteria
    1. Urine volume <500 ml/day
    2. Urine Sodium <10 meq/Liter
    3. Urine Osmolality increased over plasma osmolality
    4. Urine Red Blood Cells <50 per hpf
    5. Serum Sodium <130 mEq/L

VII. Labs

  1. Pan-culture for Sepsis
    1. Blood Culture
    2. Urine Culture
    3. Ascitic fluid culture (see Paracentesis)
  2. Urinalysis
    1. Evaluate for Hematuria and Proteinuria

VIII. Imaging

  1. Renal Ultrasound
    1. Exclude renovascular disease
    2. Exclude Hydronephrosis

IX. Management

  1. Consider intensive care admission for close monitoring of fluids, electrolytes, hemodynamic status
  2. Avoid Nephrotoxins
  3. Albumin replacement
  4. Vasoconstrictors
    1. Target: Increase Mean Arterial Pressure >15mmHg
    2. Preparations
      1. Norepinephrine OR
      2. Midodrine orally with Octreotide IV
  5. Other management
    1. Consider Hemodialysis
    2. Consult for liver transplant

X. References

  1. Swencki (2015) Crit Dec Emerg Med 29(11):2-10

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Ontology: Hepatorenal Syndrome (C0019212)

Definition (MSHCZE) Zkr. HRS – akutní selhání ledvin vznikající při těžkých chorobách jater. Má funkční charakter, ledviny samy nejsou poškozeny. Podstatou jsou cirkulační změny v ledvinách podmíněné poruchou oběhu vznikající v důsledku jaterní choroby (hyperkinetická cirkulace s vazodilatací a kompenzatorně narůstající vazokonstrikcí v renálním řečišti) často spojené se závažným a na diuretika refrakterním ascitem. Má dva typy – rychle progredující typ I a pozvolně vznikající typ II. Jde o závažný stav s poměrně vysokou smrtností (s ohledem na současnou závažnou jaterní poruchu). (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A syndrome characterized by progressive kidney failure in a patient with cirrhosis or fulminant liver failure.
Definition (MSH) Functional KIDNEY FAILURE in patients with liver disease, usually LIVER CIRRHOSIS or portal hypertension (HYPERTENSION, PORTAL), and in the absence of intrinsic renal disease or kidney abnormality. It is characterized by intense renal vasculature constriction, reduced renal blood flow, OLIGURIA, and sodium retention.
Concepts Disease or Syndrome (T047)
MSH D006530
ICD9 572.4
ICD10 K76.7
SnomedCT 51292008
English HEPATORENAL SYNDROME, Syndrome, Hepatorenal, RENAL FAILURE SECONDARY TO LIVER DISEASE <HEPATORENAL SYNDROME>, hepato-renal syndrome (diagnosis), hepato-renal syndrome, Hepatorenal Syndrome, Failure hepatorenal, Syndrome hepatorenal, RENAL FAILURE SECONDARY TO LIVER DISEASE HEPATORENAL SYNDROME, Hepatorenal Syndrome [Disease/Finding], hepatorenal failure, hepatorenal syndrome, hepatorenal syndrome (diagnosis), Hepatorenal syndrome, Hepatorenal failure, HRF - Hepatorenal failure, Hepatorenal syndrome (disorder), failure; hepatorenal, hepatorenal; failure, hepatorenal; syndrome, syndrome; hepatorenal
Spanish SINDROME HEPATORRENAL, Fallo hepatorrenal, síndrome hepatorrenal (trastorno), síndrome hepatorrenal, Insuficiencia hepatorrenal, Síndrome hepatorrenal, Síndrome Hepatorrenal
German HEPATORENALES SYNDROM, Versagen, hepatorenal, Syndrom, hepatorenal, hepatorenales Syndrom, hepatorenales Versagen, Hepatorenales Syndrom
French Défaillances hépatique et rénale, Défaillance hépatorénale, SYNDROME HEPATO-RENAL, Syndrome hépatorénal, Syndrome hépato-rénal
Italian Insufficienza epatorenale, Sindrome epatorenale
Japanese 肝腎不全, 肝腎症候群, カンジンショウコウグン, カンジンフゼン
Swedish Hepatorenalt syndrom
Czech hepatorenální syndrom, Funkční selhání ledvin, Hepatorenální syndrom, Hepatorenální selhání
Finnish Hepatorenaalinen oireyhtymä
Russian GEPATORENAL'NYI SINDROM, ГЕПАТОРЕНАЛЬНЫЙ СИНДРОМ
Portuguese SINDROME HEPATORENAL, Insuficiência hepatorrenal, Síndrome hepatorrenal, Síndrome Hepatorrenal
Korean 간신 증후군
Croatian HEPATORENALNI SINDROM
Polish Zespół wątrobowo-nerkowy
Hungarian Hepatorenalis elégtelenség, Hepatorenalis syndroma, Hepatorenalis insufficientia
Norwegian Hepatorenalt syndrom
Dutch falen; hepatorenaal, hepatorenaal; falen, hepatorenaal; syndroom, syndroom; hepatorenaal, hepatorenaal falen, hepatorenaal syndroom, Hepatorenaal syndroom, Syndroom, hepatorenaal