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Hepatorenal Syndrome: Current Concepts Related to Diagnosis and Management

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Hepatorenal Syndrome: Current Concepts Related to Diagnosis and Management 474 Zambam de Mattos A, et al. , 2016; 15 (4): 474-481 CONCISE REVIEW July-August, Vol. 15 No. 4, 2016: 474-481 The Official Journal of the Mexican Association of Hepatology, the Latin-American Association for Study of the Liver and the Canadian Association for the Study of the Liver Hepatorenal syndrome: Current concepts related to diagnosis and management Ângelo Zambam de Mattos,*,**,*** Angelo Alves de Mattos,*,** Nahum Méndez-Sánchez**** * Post-Graduation Course of Hepatology of Federal University of Health Sciences of Porto Alegre, Brazil. ** Irmandade Santa Casa de Misericórdia de Porto Alegre Hospital, Brazil. *** Pontifical Catholic University of Rio Grande do Sul, Brazil. **** Liver Research Unit, Medica Sur Clinic & Foundation, Mexico City, Mexico. ABSTRACT Renal failure in cirrhotic patients is a very severe condition. Hepatorenal syndrome has the worst prognosis among all causes of kid- ney failure in such patients. Hepatorenal syndrome is diagnosed especially in cirrhotic patients with ascites who develop loss renal function, despite diuretic suspension and volume expansion with albumin and for whom other causes of kidney injury have been ex- cluded. Patients with hepatorenal syndrome should be treated with a vasoconstrictor in combination with albumin as a bridge to re- ceiving a liver transplant. The vasoconstrictor of choice is terlipressin or noradrenaline. In spite of higher drug-related costs associated to terlipressin, initial evidence demonstrates that, considering all direct medical costs involved, the treatment strategy us- ing terlipressin is probably more economical than that using noradrenaline. Key words. Cirrhosis. Acute kidney injury. Terlipressin. Noradrenaline. Albumin. CIRRHOSIS AND RENAL FAILURE cause their creatinine level can be affected by renal dys- function as well as changes in its production and distribu- Cirrhosis is the final stage of chronic liver disease and tion volume.6 is detected in 4.5 to 9.5% of all necropsies. In 2001, it was The RIFLE classification (RIFLE: risk, injury, failure, the fourteenth most frequent cause of death worldwide loss of kidney function, and end-stage kidney disease), for and was considered responsible for 771,000 deaths. It is ex- instance, has been studied in critically ill cirrhotic pa- pected that cirrhosis will be the twelfth most frequent tients. It was demonstrated that patients without renal im- cause of death by 2020.1 Most cirrhosis-related deaths are pairment had an in-hospital mortality of 32.1%, while related to decompensation. The complications of cirrho- mortality rates were 68.8%, 71.4% and 94.8% for patients sis have been used to describe its natural history in four classified as RIFLE-R, RIFLE-I and RIFLE-F respective- stages2 and more recently as five or six stages, the last of ly (P < 0.001).6 which includes sepsis and/or renal failure.3,4 The acute kidney injury network (AKIN) criteria (Ta- Renal failure has a poor prognosis in cirrhotic patients.4 ble 1)7 have been applied in cirrhotic patients in a much Its impact on prognosis is so dramatic that it is the only wider context.8-12 In the outpatient environment, cirrhotic single-organ failure used to determine the diagnosis of patients diagnosed with kidney injury using the AKIN cri- acute-on-chronic liver failure.5 The diagnosis and classifi- teria have worse survival compared with controls, regard- cation of renal failure in cirrhotic patients have been stud- less of whether they recover normal renal function.9 ied thoroughly. It is believed that the classical criterion Among hospitalized cirrhotic patients, mortality was based solely on a creatinine level > 1.3-1.5 mg/dL is not an greater for patients who lost renal function during hospi- ideal marker of kidney injury in these patients. This is be- talization than for those who were already admitted with Manuscript received: May 18, 2016. Manuscript accepted: May 18, 2016. Hepatorenal syndrome. , 2016; 15 (4): 474-481 475 kidney injury (36% and 21% respectively, p = 0.01).10 kidney injury could lead to detrimental clinical deci- Moreover, the progression of renal failure according to sions.14 AKIN classification was independently associated with a greater mortality. On the other hand, in this study, the HEPATORENAL SYNDROME mortality of patients who did not progress beyond AKIN stage 1 was much lower than that of patients who reached Among the causes of renal failure in cirrhosis, hepato- AKIN stages 2 or 3 (2%, 15% and 44% respectively).10 Such renal syndrome (HRS) has the worst prognosis. In a a low mortality among patients who did not progress be- prospective study of cirrhotic patients with loss of renal yond AKIN stage 1 is noteworthy because it implies the function, 3-month survival for patients with parenchymal need to weigh the advantages of using more-sensitive cri- nephropathy, hypovolemia-associated renal failure, renal teria for the diagnosis of renal failure in cirrhotic patients failure associated with infection, or HRS were 73%, 46%, with the disadvantages of using less-specific criteria for as- 31%, and 15%, respectively (P < 0.0005).15 sessing the prognosis of these patients. HRS is a severe, yet potentially reversible, complica- 11 An Italian study also called attention to the same tion in patients with cirrhosis and ascites, severe acute liv- matter, when authors evaluated the role of AKIN criteria er failure, or severe alcoholic hepatitis.16 Among cirrhotic on defining the prognosis of in-hospital cirrhotic pa- patients with ascites, the incidence of HRS development tients with ascites and compared it to that of the conven- is 18% at 1 year and 39% at 5 years.17 Known since the 19th tional diagnostic criterion for renal failure (≥ 50% Century, HRS is characterized by mainly functional renal increase in creatinine concentration to > 1.5 mg/dL). failure that is not usually related to significant injuries in Acute kidney injury was diagnosed in 26% of patients ac- kidney anatomy or histology. The etiopathogenic substrate cording to AKIN criteria and in only 12% according to the conventional criterion. Normal renal function was of HRS consists of an important vasoconstriction of renal 16,18 recovered by 50.8% of the former patients and only by arterial system. 35.7% of the latter patients. Besides, a cutoff creatinine level of 1.5 mg/dL was associated to progressive renal Pathophysiology of HRS failure, which was strongly associated to mortality. In this study, there was no difference between mortality The process begins with severe liver dysfunction, a rates for patients without renal dysfunction compared restriction to blood flow to the liver, a reduction in the with patients with AKIN stage 1 kidney injury, and the production of vasodilators by the liver and an increase conventional criterion was a better predictor of mortali- in the contractility of stellate cells. The resultant portal ty than the AKIN criteria.11 A Spanish study that evaluat- hypertension leads to increased tension of the walls of ed the use of these criteria in in-hospital cirrhotic splanchnic vessels and, therefore, to increased produc- patients reported similar results. This study confirmed tion of vasodilators, such as nitric oxide, causing that patients diagnosed with AKIN stage 1 with a creati- splanchnic vasodilation. With the progression of portal nine level ≤ 1.5 mg/dL had a similar survival as that of pa- hypertension and the development of portosystemic tients with a normal renal function (84% and 88%, collaterals, blood flow and vasodilators are redirected respectively; P = 0.52). These survival rates were higher to systemic circulation, leading to systemic vasodila- than that of patients diagnosed with AKIN stage 1 with a tion, effective arterial hypovolemia and a hyperdynamic 12 creatinine level > 1.5 mg/dL (68%; P < 0.05). circulation. In order to compensate for effective arterial Another study evaluated 337 hospitalized cirrhotic pa- hypovolemia, vasoconstrictor systems are activated, tients with infection. Thirty-day mortality was higher for such as renin-angiotensin-aldosterone system, as well as the 166 patients who developed renal dysfunction diag- sympathetic nervous system, leading to a reduction of nosed using the AKIN criteria than for those who did not renal perfusion and to a decrease of glomerular filtra- develop kidney injury (34% and 7%, respectively; P < tion rate, once kidneys in cirrhotic patients are unable 0.001).13 In a reanalysis of the data, the authors confirmed to produce proper quantity of vasodilators, such as pros- that 30-day survival was lower in patients who developed acute kidney injury using the AKIN criteria and had a cre- taglandins and kallikrein. Renal hypoperfusion also in- atinine level ≤ 1.5 mg/dL than in those who did not lose creases the production of renal vasoconstrictors, such renal function (81% and 93%, respectively; P = 0.038) and as angiotensin II and endothelin. Finally, cirrhotic pa- was similar to that of patients with a creatinine level > 1.5 tients have a limited cardiac reserve, and the increased mg/dL (81% and 63%, respectively; P = 0.09). The authors cardiac output associated to a hyperdynamic circulation concluded that using the conventional criterion of serum is not sufficient to compensate for an excessively low creatinine concentration > 1.5 mg/dL to diagnose acute systemic vascular resistance. This leads to systemic arte- 476 Zambam de Mattos A, et al. , 2016; 15 (4): 474-481 rial hypotension and undermine even more renal per- Classification and diagnosis of HRS fusion.8 Figure 1 shows the pathophysiology of HRS with therapeutic targets. HRS is classified as types 1 and 2. Type 1 HRS is rapid- ly progressive renal failure, in which the creatinine level more than doubles and may reach > 2.5 mg/dL in < 2 Table 1. Acute kidney injury network (AKIN) criteria for staging weeks. It is usually associated with a precipitating factor, acute kidney injury.
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