Hepatorenal Syndrome/Bile Nephrosis



Hepatorenal Syndrome/Bile Nephrosis


Anthony Chang, MD





Key Facts


Etiology/Pathogenesis



  • Elevated blood bilirubin leads to intratubular bile casts



    • Direct toxicity to tubular epithelial cells by bilirubin and bile salts and subsequent ATN


    • Distal nephron obstruction


  • Circulatory disturbance causing decreased perfusion of kidney


Clinical Issues



  • Presentation



    • Acute renal failure


    • Jaundice


Macroscopic Features



  • Yellow when unfixed; green when fixed


  • Pigment most prominent in renal pyramids


Microscopic Pathology



  • Intratubular yellow-green pigmented casts



    • Hall stain highlights bile casts


  • Dark red and some green-yellowish discoloration of sloughed cells


  • Acute tubular injury


Top Differential Diagnoses



  • Rhabdomyolysis


  • Hemolysis


  • Acute tubular injury/acute tubular necrosis


  • Myeloma cast nephropathy


Diagnostic Checklist



  • Identification of pigment in casts by Hall stain diagnostically helpful







Gross photograph shows the capsular surface of an autopsy kidney from a 32-year-old man with cirrhosis. The green discoloration of the kidney is accentuated after formalin fixation.






Gross photograph of a cross section of renal medulla shows plugging of nephrons by bile casts image and vasa recta congestion image in a patient with end-stage liver disease. (Courtesy C. Abrahams, MD.)


TERMINOLOGY


Abbreviations



  • Hepatorenal syndrome (HRS)


  • Bile nephrosis (BN)


Synonyms



  • Cholemic nephrosis


  • Jaundice-associated acute kidney injury


  • Jaundice-related renal insufficiency


Definitions



  • HRS: Renal failure with cirrhosis



    • Type 1: Rapid reduction of renal function


    • Type 2: Slowly progressive loss of renal function


    • Lack of benefit from volume expansion


    • Absence of other causes


  • BN: Acute renal failure with bile-containing casts in distal nephron


ETIOLOGY/PATHOGENESIS


Hepatorenal Syndrome



  • Circulatory disturbance causing decreased perfusion of kidney



    • Decreased glomerular filtration rate


    • Acute ischemia of tubules


    • Reversible when transplanted into noncirrhotic recipient


Bile Nephrosis



  • Distal nephron casts containing bilirubin and bile salts



    • Direct toxicity to tubular epithelium by bile salts


    • Obstruction in distal nephron



      • Propensity of distal nephron due to admixture of Tamm-Horsfall protein with bile components


CLINICAL ISSUES


Presentation



  • Hepatic failure



    • Total bilirubin levels often > 20 mg/dL


    • Jaundice


  • Acute renal failure



    • Concentrated urine, low Na


  • Chronic renal failure


Treatment



  • Surgical approaches



    • Liver transplantation if cause of liver failure is irreversible


  • Drugs



    • Vasopressors for type 1 HRS


  • Supportive therapy



    • Renal replacement therapy


Prognosis



  • Poor; dependent on reversibility of liver failure


  • Reduction of bilirubin can lead to recovery of renal function


MACROSCOPIC FEATURES


General Features

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Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Hepatorenal Syndrome/Bile Nephrosis

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