From the Guidelines
Hepatorenal syndrome is a serious kidney condition that occurs in people with advanced liver disease, particularly cirrhosis, and is characterized by progressive kidney failure due to reduced blood flow to the kidneys. The diagnosis of hepatorenal syndrome involves several major criteria, including cirrhosis with ascites, serum creatinine ≥1.5 mg/dL, no improvement of serum creatinine after at least 2 days with diuretic withdrawal and volume expansion with albumin, absence of shock, no current or recent treatment with nephrotoxic drugs, and absence of parenchymal kidney disease as indicated by proteinuria, microhematuria, and/or abnormal renal ultrasonography 1.
Key Characteristics
- Hepatorenal syndrome is classified into two types: Type 1 is rapidly progressive with kidney function deteriorating within two weeks, while Type 2 has a more gradual onset 1.
- The pathogenesis of hepatorenal syndrome involves splanchnic and systemic vasodilatation, which causes effective arterial underfilling, leading to compensatory vasoconstrictor system activation and resulting in kidney vasoconstriction 1.
- Treatment focuses on improving liver function and circulation, often using vasoconstrictors like terlipressin or norepinephrine combined with albumin 1.
- Liver transplantation is the definitive treatment when possible, and early identification and management of underlying liver disease are crucial for preventing this serious complication 1.
Treatment Options
- Vasoconstrictors, such as terlipressin (0.5-2mg IV every 4-6 hours) or norepinephrine (0.5-3 mg/hour), combined with albumin (20-40g/day) are commonly used to restore blood flow to the kidneys 1.
- Albumin has a dose-dependent effect on both survival and complications in patients with cirrhosis with acute renal failure, and the optimal dose is not established 1.
- Liver transplantation is the optimal treatment for hepatorenal syndrome, as it addresses the underlying liver disease and can reverse the kidney dysfunction 1.
From the Research
Definition and Characteristics of Hepatorenal Syndrome
- Hepatorenal syndrome (HRS) is a form of kidney dysfunction that occurs in liver cirrhosis, characterized by a marked impairment of kidney function in response to circulatory and hemodynamic alterations 2.
- It is associated with advanced stages of liver cirrhosis, aggravated by systemic inflammation and bacterial translocation 2.
- HRS has two forms: the acute form, referred to as acute kidney injury (HRS-AKI), and the chronic form, referred to as chronic kidney disease 2.
Types of Hepatorenal Syndrome
- Type 1 HRS is characterized by an acute progressive decrease in kidney function and very short survival without treatment 3.
- Type 2 HRS features stable, less severe kidney failure and longer survival compared with type 1 3.
- HRS-AKI is one of the most severe forms of AKI in patients with cirrhosis, consisting of an abrupt impairment of kidney function, frequently triggered by an infection 2.
Diagnosis and Prognosis
- Differential diagnosis with other causes of AKI is crucial because HRS-AKI requires a specific treatment 2.
- Differential diagnosis with AKI-acute tubular necrosis may be challenging, and kidney biomarkers may be useful in this setting 2.
- Prognosis of HRS-AKI is poor, and the ideal definitive treatment consists of liver transplantation or simultaneous liver-kidney transplantation 2.
- Increased serum creatinine and urinary sodium at the initiation of therapy are prognostic factors for mortality 4.
Treatment
- Treatment of HRS-AKI is based on the administration of vasoconstrictor drugs in combination with volume expansion with albumin 2.
- A standardized approach with albumin, midodrine, and octreotide may improve treatment response rates and reduce the need for renal replacement therapy and liver transplantation 5.
- Transjugular intrahepatic portosystemic stent shunt (TIPS) is an effective treatment for type 1 HRS in suitable patients with cirrhosis and ascites, following improvement of renal function with combination therapy of midodrine, octreotide, and albumin 6.
- Liver transplant is the preferred treatment for HRS, and pharmacologic treatment with vasoconstrictors, together with albumin, is effective in 40%-50% of patients with type 1 HRS and improves survival 3.