Hepatorenal syndrome is a form of acute kidney failure. It shows up in severe liver diseases.
What is hepatorenal syndrome?
According to Abbreviationfinder, hepatorenal syndrome ( HRS ) is acute, progressive renal failure. It is the result of a serious liver disease such as cirrhosis. The initial stage of the disease resembles prerenal renal failure.
In the worst case, the syndrome can end in life-threatening renal failure that cannot be reversed. The German internist Friedrich Theodor von Frerichs first described a connection between liver cirrhosis and kidney failure in 1861.
A further description took place in 1863 by Austin Flint. In 1956, medicine discovered narrowing of the kidney vessels as a trigger for kidney failure. However, the first description of the hepatorenal syndrome was made in 1937 by Wilhelm Nonnenbruch.
To this day, the exact origin of the hepatorenal syndrome has not been clearly clarified. In almost every case there is abdominal dropsy (ascites) when the syndrome occurs. It is possible that a consistent therapy of the abdominal water such as an ascites puncture or the administration of diuretics triggers the hepatorenal syndrome.
Other risk factors include extensive bleeding or blood poisoning (sepsis). A histological examination did not reveal any changes in the kidneys. Doctors suspect a narrowing of the renal vessels as the pathomechanism.
This vasoconstriction results in a decrease in glomerular function. The tubular system, on the other hand, is hardly affected. The hepatorenal syndrome leads to an increase in plasma renin activity. Disorders within the prostaglandin system or angiotensin-aldosterone system are considered to be the cause of renal vasoconstriction.
Triggers such as infections, hepatic encephalopathy, bleeding, paracentesis in the context of ascites, administration of sodium in the case of dilutional natraemia, or the use of nephrotoxic drugs do not always lead directly to hepatorenal syndrome.
But it is possible that they prepare the kidney failure. Hyponatraemia and hypotension associated with liver cirrhosis are considered unfavorable signs of hepatorenal syndrome.
Symptoms, Ailments & Signs
A hepatorenal syndrome becomes noticeable through the signs of decompensated cirrhosis of the liver. The people affected suffer from a water belly, accumulation of water in the tissue ( oedema ), hepatic encephalopathy and jaundice. In medicine, a distinction is made between type 1 and type 2 in the hepatorenal syndrome.
If type 1 is present, kidney function deteriorates rapidly. Important indications are a doubling of the serum creatinine to more than 2.5 mg/dl or a drop in the creatinine clearance to values below 20 ml/min. Common triggers of type 1 are intensive treatment with diuretics, use of certain medications such as nonsteroidal anti- inflammatory drugs, gastrointestinal bleeding, paracentesis without plasma volume expansion, and lactulose overdose.
In contrast to type 1, in type 2 the function of the kidneys slowly decreases. The serum creatinine is more than 1.5 mg/dl. There are no specific triggering factors for the form of hepatorenal syndrome, so it always shows up spontaneously. Type 2 is one of the most important causes of therapy-refractory ascites.
Diagnosis & History
Before hepatorenal syndrome can be diagnosed with certainty, all other possible causes of renal failure must be ruled out. The syndrome is ultimately diagnosed by exclusion. According to the International Ascites Club, the main criteria are portal hypertension, an increase in creatinine to values of more than 1.5 mg/dl or a reduction in creatinine clearance to less than 40 ml/min, and hepatic insufficiency.
Other important diagnostic criteria are a urine volume of less than 500 ml/day, a serum sodium concentration below 130 mmol/l, the absence of acute bacterial infections, the absence of proteinuria of more than 500 mg/day, a urinary sodium concentration of less than 10 mmol/l and the absence of erythrocyturia greater than 50 cells/visual field. In addition, the urine osmolarity must be higher than the serum osmolarity.
The narrowing of the renal vessels can be detected by a Doppler ultrasound examination of the kidneys. This procedure makes the HRS diagnosis more likely. Around 50 percent of all patients with such constriction and liver cirrhosis develop hepatorenal syndrome.
In most cases, the course of hepatorenal syndrome is negative. In the case of type 1, the survival time without medical treatment is less than a month. In type 2, the probability of survival after a period of two years is approximately 20 percent.
Kidney failure occurs in this syndrome. Without treatment, this can be fatal for the patient and must therefore be examined and treated by a doctor. As a rule, there is accumulation of water in the tissues and jaundice. A strong water belly also develops. Internal bleeding occurs, causing pain.
Due to the malfunction of the kidneys, the patient is usually dependent on dialysis or a donor kidney in order to continue to survive. The quality of life of the patient is extremely restricted and reduced by this syndrome. Treatment usually depends on the severity of the disease. If the kidneys have not yet been completely damaged, the symptoms can be relieved with the help of medication.
It may also be necessary to discontinue some medications after consulting a doctor. In the worst case, a kidney transplant must be performed. Since this syndrome usually does not occur alone, a liver transplant is often necessary so that the patient can continue to survive. If left untreated, the patient will die.
When should you go to the doctor?
If symptoms such as jaundice, fluid retention, or abdominal water are noticed, hepatorenal syndrome may be the underlying cause. If the symptoms do not subside after a few days, a doctor’s visit is recommended.
If other symptoms develop, medical advice must be sought immediately. Pain in the upper abdomen, severe skin itching and bleeding should be clarified immediately. If acute kidney failure is not treated, it can lead to internal bleeding, chronic pain and, in the worst case, complete organ failure.
At the latest when these symptoms are noticed, medical help is needed. Patients who are already suffering from ascites or blood poisoning are particularly at risk. The use of nephrotoxic drugs can also lead to hepatorenal syndrome. People who belong to these risk groups should go to their family doctor immediately with the symptoms mentioned. Other contacts are the nephrologist or a doctor specializing in internal medicine. In the event of severe symptoms, it is best to go to the nearest hospital immediately or call the emergency doctor directly.
Treatment & Therapy
In order to successfully treat the hepatorenal syndrome, the triggering renal circulatory disorder must be eliminated or positively influenced. This includes stopping medication that may be the cause of the syndrome. To improve renal blood flow, the patient is often given vasopressin analogues such as terlipressin.
Other treatment options include temporary administration of human albumin or gentle ascites puncture. If a kidney transplant is planned in the foreseeable future, hemodialysis or alternative kidney replacement methods can be used to keep the patient alive. Although therapeutic efforts are high, hepatorenal syndrome still has a mortality rate of around 80 percent.
In some patients, renal function can be restored with the creation of a transjugar intrahepatic portosystemic shunt. If liver function is restored, this also has a positive effect on kidney function. The greatest chance of success of the treatment is therefore attributed to liver transplantation.
Outlook & Forecast
Hepatorenal syndrome has a very poor prognosis. Despite intensive treatment, the mortality rate for this disease is 80 percent. Death usually occurs from acute kidney failure. Since the hepatorenal syndrome is a combined liver-kidney insufficiency, the treatment must refer to both organs. The cause of kidney failure is not yet fully known. However, it is believed that hormonal processes disturbed by liver disease play a major role.
The hepatorenal syndrome is always observed in connection with abdominal dropsy (ascites). Ascites is a symptom of cirrhosis of the liver. It has also been found that acute renal failure is reversible when liver function improves. Despite the usually poor prospects for the patients, complete cures are possible.
In some patients, drug therapy can already bring good progress and even contribute to healing. However, this is the exception. If the drugs don’t work, often only a liver transplant can save life. But even a liver transplant is not always possible, especially when the liver disease is too advanced. In very rare cases, the installation of a transjugular intrahepatic portosystemic shunt (TIPS) can lead to the kidneys recovering. In the context of TIPS, the liver stream area is bypassed.
Preventive measures against the hepatorenal syndrome are not known. So the origin of the disease could not be explained exactly.
In the case of this syndrome, the affected person usually has no direct follow-up measures available. First and foremost, a doctor must be consulted at an early stage so that further complications or other symptoms do not arise. In the worst case, the person affected dies from this syndrome if no treatment is given.
Therefore, a doctor should be consulted at the first symptoms and signs of the disease. In most cases, certain medications have to be stopped with this syndrome. However, a doctor must first and foremost be consulted so that side effects or other interactions do not occur. However, if the internal organs have already been severely damaged, only a transplant can completely alleviate the symptoms.
With this disease, most of those affected are heavily dependent on the help and support of their own family and friends. Above all, psychological support is very useful in order to prevent mental upsets or depression. Contact with other people affected by the syndrome can also be very useful. In most cases, the life expectancy of those affected is significantly reduced by this disease.
You can do that yourself
Treatment of hepatorenal syndrome focuses on eliminating the causative renal circulatory disorder. Once the syndrome has been diagnosed, the person concerned should take it easy and change their diet. The diet should be balanced and healthy. Bland diets have proved their worth, which in combination with carbohydrate-rich foods can bring about an improvement in the symptoms. People with kidney disease should not take any stimulants such as alcohol, coffee or nicotine.
Further treatment focuses on reducing the pain. Drug therapy can be supported by some natural remedies such as valerian or the homeopathic remedy belladonna. After a liver transplant, the patient should be on sick leave for at least four to five weeks. Such a serious procedure can cause symptoms such as pain and feelings of pressure, which must be treated by a doctor. In general, extensive medical monitoring is indicated after liver transplantation.
Since the death rate is very high despite all possibilities, psychological counseling is also necessary. Not only do those affected need support themselves, but often also friends and family members. It is also advisable to take part in a self-help group and to seek dialogue with other people affected.