Hepatorenal syndrome

The Hepatorenal syndrome (HRS ) is a functional, progressive and irreversible decline in renal function ( glomerular filtration rate ) with the consequence of oliguric renal failure in patients with liver disease ( cirrhosis or fulminant hepatitis ) in the absence of evidence of other causes of renal failure ( diagnosis of exclusion ). Through the release of vasoactive ( vasodilatory / narrowing ) substances, this results in a deterioration of renal blood flow.

Pathogenesis

The combination of portal hypertension and arterial vasodilation in the splanchnic alters the intestinal capillary pressure with an increase in vascular permeability. Thus, there is a leakage of fluid into the abdominal cavity and to the emergence of ascites. With progression of the disease, this results in a relative intravascular volume depletion in the systemic circulation with decrease in renal excretion of free water and renovascular vasoconstriction to maintain the perfusion pressure ( underfill theory). Reactive and the renin-angiotensin -aldosterone system (RAAS ) is stimulated, which results in a retention of sodium and water. However, this in healthy people perfectly sensible mechanism results in patients with portal hypertension in a further deterioration of the disease.

Symptoms

The clinic is manifested by signs of decompensated liver cirrhosis. These include ascites, edema, jaundice and hepatic encephalopathy.

There are two types of HRS.

In type 1, there is a rapidly progressive deterioration of renal function. Criteria are a doubling of serum creatinine above 2.5 mg / dl, or a decline in creatinine clearance by half to less than 20 ml / min, respectively, within two weeks. It can be in this type of call often triggering factors, including gastrointestinal bleeding, forced diuretics, nephrotoxic drugs (eg, nonsteroidal anti-inflammatory drugs ), Laktuloseüberdosierung, or paracentesis without plasma volume expansion.

In type 2, there is a slowly progressive deterioration of renal function with a serum creatinine above 1.5 mg / dl. In contrast to type 1, this form often occurs spontaneously, without triggering factor and represents an important cause of refractory ascites

Definition and diagnosis

When hepatorenal syndrome is a diagnosis of exclusion. According to the definition of the International Ascites Club following main criteria must be present:

  • Creatinine above 1.5 mg / dl or creatinine clearance restriction to less than 40 mg / min.
  • Portal hypertension
  • No circulatory depression
  • Pronounced hepatic insufficiency
  • No bacterial infections
  • No application of nephrotoxic drugs, which can explain the kidney failure
  • No improvement in renal function after expansion of the plasma volume
  • No proteinuria about 500 mg / day
  • Urine volume less than 500 ml / day
  • Urine sodium concentration of less than 10 mmol / l
  • A urine osmolarity is higher than the osmolarity
  • No erythrocyturia of more than 50 cells / high power field
  • Serum sodium concentration below 130 mmol / l

A vasoconstriction of the renal vessels can be detected by Doppler ultrasound examination of the kidneys. It makes the diagnosis of HRS likely. Approximately half of the patients with such a narrowing and cirrhosis develop HRS.

The creatinine may be in the presence of liver cirrhosis despite renal failure in the normal range. Creatinine is released from the muscles. In liver cirrhosis significantly less creatinine than in the liver healthy person is discharged.

Therapy

Therapeutically, the triggering factors must be eliminated. In addition, the acid -base balance is corrected anemia by transfusion balanced, administered intravenously albumin and shunned nephrotoxic substances. Under certain circumstances, the use of renal replacement therapy comes into question, in severe tissue damage a transplant. Liver transplantation is the only definitive treatment option. If the above mentioned options are not feasible or sufficient to use as additional options TIPS ( transjugular intrahepatic portosystemic shunt) and - in patients with poor performance status or with contraindication for a TIPS - pharmacological treatment with vasopressin analogues ( terlipressin ) or alphaadrenerger substances such as norepinephrine and Milodril ( Vasokonstrikion splanchnic ) in combination with albumin in question.

Cure views

The prognosis of HRS is bad. The survival time in type 1 is without therapy usually less than one month, in Type 2 the survival probability is about 20 % after two years. Approximately half of the patients with rapidly progressive type I can survive through adequate therapy with a vasopressin analogue, and albumin about three months.

History of Medicine

The relationship between renal failure and liver cirrhosis was described in 1861 by free Rich and 1863 by Flint for the first time. In 1956, the narrowing of the renal vessels was identified as the triggering cause. Wilhelm Nonnenbruch has the hepatorenal syndrome described in 1937. Source: German medical weekly, Volume 63, 1937, pages 7 to 10: " About the inflammatory edema of the kidney and the hepatorenal syndrome."

Oliguria Acute kidney injury Renin–angiotensin system Decompensation Nephrotoxicity
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