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| | __NOTOC__ |
| | {{Hepatorenal syndrome}} |
| | [[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Hepatorenal_syndrome]] |
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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{CMG}} | | {{CMG}}; {{AE}} {{SKA}} |
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| {{Editor Help}}
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| ==Overview==
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| {{Infobox_Disease |
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| Name = Hepatorenal syndrome |
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| Image = |
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| Caption = |
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| DiseasesDB = 5810 |
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| ICD10 = {{ICD10|K|76|7|k|70}} |
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| ICD9 = {{ICD9|572.4}} |
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| ICDO = |
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| OMIM = |
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| MedlinePlus = 000489 |
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| eMedicineSubj = med |
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| eMedicineTopic = 1001 |
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| MeshID = D006530 |
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| }} | |
| {{SI}} | |
| '''Hepatorenal syndrome''' (HRS) refers to [[acute renal failure]] that occurs in the setting of [[cirrhosis]] or [[fulminant]] [[liver failure]] associated with [[portal hypertension]], usually in the absence of other disease of the [[kidney]].<ref name=IAC>Arroyo V, Gines P, Gerbes AL, Dudley FJ, Gentilini P, Laffi G, Reynolds TB, Ring-Larsen H, Scholmerich J. Definition and diagnostic criteria of refractory ascites and hepatorenal syndrome in cirrhosis. International Ascites Club. ''Hepatology.'' 1996 Jan;23(1):164-76. PMID 8550036</ref><ref name=FloWo>Wong F, Blendis L. New challenge of hepatorenal syndrome: prevention and treatment. ''Hepatology'' 2001 Dec;34(6):1242-51. PMID 11732014</ref> The pathology involved in the development of hepatorenal syndrome is thought to be an alteration in blood flow and blood vessel tone in the circulation that supplies the intestines (the ''splanchnic circulation'') and the circulation that supplies the kidney.<ref name=Arroyo1>Arroyo V, Guevara M, Gines P. Hepatorenal syndrome in cirrhosis: pathogenesis and treatment. ''Gastroenterology'' 2002 May;122(6):1658-76. PMID 12016430.</ref> It is usually indicative of an end-stage of perfusion, or [[hemodynamics|blood flow]] to the kidney, due to deteriorating [[liver]] function. Patients with hepatorenal syndrome are very ill, and, if untreated, the condition is usually fatal. Treatment usually involves medical therapy or [[Transjugular intrahepatic portosystemic shunt|TIPS]] as a bridge to [[liver transplantation]].<ref name=FloWo/>
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| ==Definition and diagnosis==
| | {{SK}} Heyd syndrome; HRS. |
| The hepatorenal syndrome is defined as renal failure that occurs in the setting of liver disease as follows <ref name=IAC/>:
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| ===Type I HRS=== | | == [[Hepatorenal syndrome overview|Overview]] == |
| '''Type I HRS''' is characterized by rapidly progressive renal failure with a doubling of serum [[creatinine]] to a level greater than 221 [[Mole (unit)|μmol]]/L (2.5 [[milligram|mg]]/[[decilitre|dL]]) or a halving of the [[creatinine clearance]] to less than 20 mL/min over a period of less than 2 weeks. Type I HRS carries a very poor prognosis, usually of less than 50% over one month.<ref name=Arroyo1/> Patients with type I hepatorenal syndrome are usually ill, may have low blood pressures, and may require therapy with [[inotrope]]s, or intravenous drugs to maintain blood pressure.<ref name=emed>[http://www.emedicine.com/med/topic1001.htm Hepatorenal syndrome] - emedicine.com article</ref>
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| ===Type II HRS=== | | ==[[Hepatorenal syndrome historical perspective|Historical Perspective]]== |
| '''Type II HRS''' is characterized by a slowly progressive:
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| *increase in serum [[creatinine]] level to greater than 133 μmol/L (1.5 mg/dL) or a creatinine clearance of less than 40 mL/min
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| * urine sodium < 10 meq/dl<ref name="pmid3297907">{{cite journal |author=Ginés P, Arroyo V, Quintero E, ''et al'' |title=Comparison of paracentesis and diuretics in the treatment of cirrhotics with tense ascites. Results of a randomized study |journal=Gastroenterology |volume=93 |issue=2 |pages=234-41 |year=1987 |pmid=3297907 |doi=}}</ref>
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| It is typically associated with [[ascites]] that is unresponsive to [[diuretic]] medications, and also carries a poor, if somewhat longer (median survival ~6 months) outlook,<ref>Blendis L, Wong F. The natural history and management of hepatorenal disorders: from pre-ascites to hepatorenal syndrome. ''Clin Med'' 2003 Mar-Apr;3(2):154-9. PMID 12737373</ref> unless the patient undergoes liver transplantation.
| | ==[[Hepatorenal syndrome pathophysiology|Pathophysiology]]== |
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| It can be challenging to distinguish hepatorenal syndrome from other entities that cause renal failure in the setting of advanced liver disease. As a result, additional major and minor criteria have been proposed to assist in the [[diagnosis]] of the hepatorenal syndrome.<ref name=IAC/>
| | ==[[Hepatorenal syndrome causes|Causes]]== |
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| The ''major'' criteria include liver disease in the setting of [[portal hypertension]]; [[renal failure]]; the absence of [[Shock (medical)|shock]], [[infection]], recent treatment with medications that affect the kidney's function ([[Nephrotoxicity|nephrotoxin]]s), and fluid losses; the absence of sustained improvement in renal function despite treatment with 1.5 litres of [[intravenous]] [[Saline (medicine)|normal saline]]; the absence of [[proteinuria]], or [[protein]] in the [[urine]]; and, the absence of renal disease or obstruction of renal outflow as seen on [[ultrasound]].
| | ==[[Hepatorenal syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
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| The ''minor'' criteria are [[laboratory]] in nature, and include a [[oliguria|low urine volume]] (less than 500 cc per day), low [[sodium]] concentration in the urine, a urine [[osmolality]] that is greater than that in the [[blood]], the absence of [[red blood cells]] in the urine, and a serum sodium concentration of less than 130 mmol/L.<ref name=IAC/>
| | ==[[Hepatorenal syndrome risk factors|Risk Factors]]== |
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| ===Similar conditions=== | | ==[[Hepatorenal syndrome screening|Screening]]== |
| Many other diseases of the kidney are associated with liver disease and must be excluded before making a diagnosis of hepatorenal syndrome. They include the following:
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| *[[Renal failure|Pre-renal failure]]: Pre-renal failure usually responds to treatment with intravenous fluids, resulting in reduction in serum [[creatinine]] and the excretion of sodium.<ref name=IAC/>
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| *[[Acute tubular necrosis]] (ATN): This can be difficult to confidently diagnose. It may be an inability to concentrate the urine, if any is being produced. The urine sediment should be bland, microscopy may show [[hyaline cast]]s. ATN may recover with supportive treatment only or progress to [[end-stage renal failure]]. In cirrhosis, urinary sodium is not a reliable guide to the development of ATN, as [[fractional sodium excretion]] may stay below 1 percent, due to the gradual worsening of renal [[ischaemia]].
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| *Other causes may include [[glomerulonephritis|glomerular disease]] secondary to [[Hepatitis B]] or [[Hepatitis C]],<ref>Han SH. Extrahepatic manifestations of chronic hepatitis B. ''Clin Liver Dis.'' 2004 May;8(2):403-18. PMID 15481347</ref> drug toxicity (notably [[gentamicin]]) or [[Radiocontrast|contrast nephropathy]].
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| ==Pathophysiology== | | ==[[Hepatorenal syndrome differential diagnosis|Differentiating Hepatorenal syndrome from other Diseases]]== |
| The [[renal failure]] in hepatorenal syndrome is believed to arise from abnormalities in [[blood vessel]] tone in the splanchnic circulation (which supplies the [[intestines]]).<ref>Gines P, Arroyo V. Hepatorenal syndrome. ''J Am Soc Nephrol'' 1999 Aug;10(8):1833-9. PMID 10446954</ref> It is known that there is an overall decreased [[systemic vascular resistance]] in hepatorenal syndrome, but that the measured [[femoral]] and renal fractions of [[cardiac output]] are respectively increased and reduced, suggesting that splanchnic [[vasodilation]] is implicated in the renal failure.<ref>Fernandez-Seara J, Prieto J, Quiroga J, Zozaya JM, Cobos MA, Rodriguez-Eire JL, Garcia-Plaza A, Leal J. Systemic and regional hemodynamics in patients with liver cirrhosis and ascites with and without functional renal failure. ''Gastroenterology'' 1989 Nov;97(5):1304-12. PMID 2676683</ref>
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| There is activation of the [[renin-angiotensin-aldosterone system]] (RAAS) and the [[sympathetic nervous system]], and profound [[vasoconstriction]] of the kidneys.<ref name=GinesCohort>Gines A, Escorsell A, Gines P, ''et al.'' Incidence, predictive factors, and prognosis of the hepatorenal syndrome in cirrhosis with ascites. ''Gastroenterology'' 1993 Jul;105(1):229-36. PMID 8514039.</ref> Many [[vasocontrictor]] chemicals have been hypothesized as being involved in this pathway, including [[vasopressin]],<ref>Lenz K, Hortnagl H, Druml W, Reither H, Schmid R, Schneeweiss B, Laggner A, Grimm Gm Gerbes AL. Ornipressin in the treatment of functional renal failure in decompensated liver cirrhosis. Effects on renal hemodynamics and atrial natriuretic factor. ''Gastroenterology'' 1991 Oct;101(4):1060-7. PMID 1832407</ref> [[prostacyclin]], [[thromboxane]] A2,<ref>Moore K, Ward PS, Taylor GW, Williams R. Systemic and renal production of thromboxane A2 and prostacyclin in decompensated liver disease and hepatorenal syndrome. ''Gastroenterology'' 1991 Apr;100(4):1069-77. PMID 2001805</ref>, and [[endotoxin]].<ref name=Arroyo1/>
| | ==[[Hepatorenal syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==Epidemiology== | | ==Diagnosis== |
| It is estimated that 39% of patients with [[cirrhosis]] and [[ascites]] will develop hepatorenal syndrome within five years of the onset of their disease.<ref name=GinesCohort/> The prognosis of these patients is grim with untreated patients having an extremely short survival,<ref name=Arroyo1/><ref name=GinesCohort/><ref name=FloWo/> and with the severity of liver disease (as evidenced by the [[Model for End-Stage Liver Disease|MELD]] score) now believed to determine outcome.<ref>Alessandria C, Ozdogan O, Guevara M, Restuccia T, Jimenez W, Arroyo V, Rodes J, Gines P. MELD score and clinical type predict prognosis in hepatorenal syndrome: Relevance to liver transplantation. ''Hepatology'' 2005 Jun;41(6):1282-9. PMID 15834937</ref> Some patients without cirrhosis develop hepatorenal syndrome, with an incidence of about 20% seen in one study of ill patients with [[alcoholic hepatitis]].<ref name=PTX>Akriviadis E, Botla R, Briggs W, Han S, Reynolds T, Shakil O. Pentoxifylline improves short-term survival in severe acute alcoholic hepatitis: a double-blind, placebo-controlled trial. ''Gastroenterology'' 2000 Dec;119(6):1637-48. PMID 11113085.</ref>
| | [[Hepatorenal syndrome history and symptoms|History and Symptoms]] | [[Hepatorenal syndrome physical examination|Physical Examination]] | [[Hepatorenal syndrome laboratory findings|Laboratory Findings]] |
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| ==Treatment== | | ==Treatment== |
| Because of the high [[death|mortality]] associated with hepatorenal syndrome, emphasis is on prevention in patients who are at risk for the condition. Strategies for avoiding hepatorenal syndrome include appropriate and non-aggressive use of diuretics, identification and early treatment of [[infection]] and [[upper gastrointestinal bleeding|hemorrhage]], and avoidance of other toxins that can affect both the liver and kidney.<ref name=Arroyo1/> <ref name=emed/>
| | [[Hepatorenal syndrome medical therapy|Medical Therapy]] | [[Hepatorenal syndrome primary prevention|Prevention]] | [[Hepatorenal syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Hepatorenal syndrome future or investigational therapies|Future or Investigational Therapies]] |
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| The definitive treatment for hepatorenal syndrome is [[liver transplantation]], and all other therapies can best be described as bridges to transplantation.<ref name=FloWo/> These treatment strategies include the following:
| | ==Related Chapters== |
| | [[Cirrhosis]] |
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| ===Albumin===
| | [[Acute liver failure]] |
| All major studies showing improvement in renal function in patients with hepatorenal syndrome have involved expansion of the volume of the [[blood plasma|plasma]] with [[human serum albumin|albumin]] given intravenously <ref name=Guevara/> <ref name=Terli/> One regimen is 1 gm albumin per kg of body weight intravenously on day one followed by followed by 20-40 grams daily.<ref name="pmid15084697">{{cite journal |author=Ginès P, Cárdenas A, Arroyo V, Rodés J |title=Management of cirrhosis and ascites |journal=N. Engl. J. Med. |volume=350 |issue=16 |pages=1646-54 |year=2004 |pmid=15084697 |doi=10.1056/NEJMra035021}}</ref>
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| ===Midodrine and octreotide===
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| Midodrine is an alpha-agonist and octreotide is an analogue of [[somatostatin]]. The medications are respectively systemic vasoconstrictors and inhibitors of vasodilators, and were not found to be useful when used individually in treatment of hepatorenal syndrome.<ref>Pomier-Layrargues G, Paquin SC, Hassoun Z, Lafortune M, Tran A. Octreotide in hepatorenal syndrome: a randomized, double-blind, placebo-controlled, crossover study. ''Hepatology'' 2003 Jul;38(1):238-43.</ref> However, one study of 13 patients with hepatorenal syndrome showed significant improvement when the two were used together (with midodrine given orally, octreotide given [[subcutaneous]]ly and both dosed according to blood pressure), with three patients surviving to discharge.<ref name=Angeli>Angeli P, Volpin R, Gerunda G, Craighero R, Roner P, Merenda R, Amodio P, Sticca A, Caregaro L, Maffei-Faccioli A, Gatta A. Reversal of type 1 hepatorenal syndrome with the administration of midodrine and octreotide. ''Hepatology'' 1999 Jun;29(6):1690-7. PMID 10347109</ref> A nonrandomized, observational study used "100 μg subcutaneously TID, with the goal to increase the dose to 200 μg subcutaneous TID" and "midodrine administration started at 5, 7.5, or 10 mg TID orally, with the goal to increase the dose to 12.5 or 15 mg if necessary" and found that "octreotide/midodrine treatment appears to improve 30-day survival".<ref name="pmid17235705">{{cite journal |author=Esrailian E, Pantangco ER, Kyulo NL, Hu KQ, Runyon BA |title=Octreotide/Midodrine therapy significantly improves renal function and 30-day survival in patients with type 1 hepatorenal syndrome |journal=Dig. Dis. Sci. |volume=52 |issue=3 |pages=742-8 |year=2007 |pmid=17235705 |doi=10.1007/s10620-006-9312-0}}</ref>
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| ===Vasopressin analogues===
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| The [[Vasopressin#Pharmacology|vasopressin analogue]] ornipressin was found in a number of studies to be useful in improvement of renal function in patients with hepatorenal syndrome,<ref name=Guevara>Guevara M, Gines P, Fernandez-Esparrach G, Sort P, Salmeron JM, Jimenez W, Arroyo V, Rodes J. Reversibility of hepatorenal syndrome by prolonged administration of ornipressin and plasma volume expansion. ''Hepatology'' 1998 Jan;27(1):35-41. PMID 9425914 </ref> <ref>Gulberg V, Bilzer M, Gerbes AL. Long-term therapy and retreatment of hepatorenal syndrome type 1 with ornipressin and dopamine. ''Hepatology'' 1999 Oct;30(4):870-5. PMID 10498636 </ref> but has been limited by [[ischemia|ischemic]] complications <ref name=Guevara/>. [[Terlipressin]] is a vasopressin analogue that has been found in one study to be useful for improving renal function in patients with hepatorenal syndrome with a lesser incidence of ischemia.<ref name=Terli>Ortega R, Gines P, Uriz J, Cardenas A, Calahorra B, De Las Heras D, Guevara M, Bataller R, Jimenez W, Arroyo V, Rodes J. Terlipressin therapy with and without albumin for patients with hepatorenal syndrome: results of a prospective, nonrandomized study. ''Hepatology'' 2002 Oct;36 (4 Pt 1):941-8. PMID 12297842</ref> Neither medication is available for use in North America.
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| ===Transjugular intrahepatic portosystemic shunt===
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| [[Transjugular intrahepatic portosystemic shunt]]s (TIPS) involve decompression of the high pressures in the [[portal hypertension|portal]] circulation by placing a small stent between a [[portal vein|portal]] and [[hepatic vein]]. They have also been shown to improve renal function in patients with hepatorenal syndrome.<ref>Wong F, Pantea L, Sniderman K. Midodrine, octreotide, albumin, and TIPS in selected patients with cirrhosis and type 1 hepatorenal syndrome. ''Hepatology''. 2004 Jul;40(1):55-64. PMID 15239086.</ref> <ref>Guevara M, Rodes J. Hepatorenal syndrome. ''Int J Biochem Cell Biol.'' 2005 Jan;37(1):22-6. PMID 15381144.</ref>
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| ===Liver dialysis===
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| [[Liver dialysis]] involves extracorporeal dialysis to remove toxins from the circulation. The [[Liver dialysis#Liver dialysis devices|molecular adsorbents recirculation system]] (MARS) has shown some utility as a bridge to transplantation in patients with hepatorenal syndrome.<ref>Mitzner SR, Stange J, Klammt S, Risler T, Erley CM, Bader BD, Berger ED, Lauchart W, Peszynski P, Freytag J, Hickstein H, Loock J, Lohr JM, Liebe S, Emmrich J, Korten G, Schmidt R. Improvement of hepatorenal syndrome with extracorporeal albumin dialysis MARS: results of a prospective, randomized, controlled clinical trial. ''Liver Transpl.'' 2000 May;6(3):277-86. PMID 10827226.</ref>
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| ===Hemodialysis===
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| [[Renal replacement therapy]] may be required to 'bridge' the patient to liver transplantation, although the condition of the patient may dictate the modality used.<ref>Witzke O, Baumann M, Patschan D, Patschan S, Mitchell A, Treichel U, Gerken G, Philipp T, Kribben A. Which patients benefit from hemodialysis therapy in hepatorenal syndrome? ''J Gastroenterol Hepatol.'' 2004 Dec;19(12):1369-73. PMID 15610310</ref>
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| ===Other medications===
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| Other agents used in treatment include [[pentoxifylline]],<ref name=PTX/> [[acetylcysteine]],<ref>Holt S, Goodier D, Marley R, Patch D, Burroughs A, Fernando B, Harry D, Moore K. Improvement in renal function in hepatorenal syndrome with N-acetylcysteine. ''Lancet''. 1999 Jan 23;353(9149):294-5. PMID 9929029</ref> and [[misoprostol]].<ref>Clewell JD, Walker-Renard P. Prostaglandins for the treatment of hepatorenal syndrome. ''Ann Pharmacother.'' 1994 Jan;28(1):54-5. PMID 8123962</ref>
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| ==History==
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| Historically, the hepatorenal syndrome was first defined as acute renal failure that occurred in the setting of [[Bile duct|biliary surgery]].<ref>Helwig FC, Schutz CB. A liver kidney syndrome. Clinical pathological and experimental studies. ''Surg Gynecol Obstet'' 1932;55:570-580.</ref> The syndrome was soon associated with advanced liver disease.<ref name=FloWo/> It was determined that kidneys transplanted from patients with hepatorenal syndrome were functional,<ref>Koppel MH, Coburn JW, Mims MM, Goldstein H, Boyle JD, Rubini ME. Transplantation of cadaveric kidneys from patients with hepatorenal syndrome. Evidence for the functional nature of renal failure in advanced liver disease. ''N Engl J Med.'' 1969 Jun 19;280(25):1367-71. PMID 4890476</ref> leading to the hypothesis that hepatorenal syndrome was a systemic as opposed to renal disease.
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| ==Prevention==
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| ===Intravenous albumin===
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| A [[randomized controlled trial]] found that intravenous albumin on the day of admission and on hospital day 3 can reduce renal impairment.<ref name="pmid10432325">{{cite journal |author=Sort P, Navasa M, Arroyo V, ''et al'' |title=Effect of intravenous albumin on renal impairment and mortality in patients with cirrhosis and spontaneous bacterial peritonitis |journal=N. Engl. J. Med. |volume=341 |issue=6 |pages=403-9 |year=1999 |pmid=10432325 |doi=}}</ref>
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| ==References==
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| <div class="references-small"><references /></div>
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| {{SIB}}
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| {{Gastroenterology}} | | {{Gastroenterology}} |
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| [[Category:Organ failure]] | | [[Category:Hepatology]] |
| [[Category:Gastroenterology]] | | [[Category:Gastroenterology]] |
| [[Category:Nephrology]] | | [[Category:Nephrology]] |
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| [[de:Hepatorenales Syndrom]]
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