Hepatorenal syndrome refers to a form of acute kidney failure. It manifests in severe liver disease.
What is hepatorenal syndrome?
Hepatorenal syndrome (HRS) is acute progressive kidney failure. It is the result of severe disease of the liver such as cirrhosis. The initial stage of the disease is similar to prerenal renal failure. In the worst cases, the syndrome can end in life-threatening renal failure that cannot be reversed. A link between cirrhosis of the liver and renal failure was first described by the German internist Friedrich Theodor von Frerichs in 1861, and a further description was made by Austin Flint in 1863. In 1956, medicine discovered constriction of the renal vessels as a trigger of renal failure. However, the first description of hepatorenal syndrome occurred in 1937 by Wilhelm Nonnenbruch.
Causes
To this day, the exact origin of hepatorenal syndrome has not been clearly elucidated. In almost every case, there is abdominal dropsy (ascites) when the syndrome occurs. It is possible that a consistent therapy of the water belly, such as ascites puncture or the administration of diuretics, triggers the hepatorenal syndrome. Other risk factors include extensive bleeding or sepsis. No changes are seen in the kidneys on histological examination. Physicians suspect a constriction of the renal vessels as the pathomechanism. This vasoconstriction results in a decrease in glomerular function. In contrast, the tubular system is hardly affected. Due to the hepatorenal syndrome, there is an increase in plasma renin activity. Disorders within the prostaglandin system or angiotensin-aldosterone system are thought to be the originators of renal vasoconstriction. Triggers such as infection, hepatic encephalopathy, hemorrhage, paracentesis in the setting of ascites, sodium intake in dilutional sodemia, or ingestion of nephrotoxic drugs do not always lead directly to hepatorenal syndrome. However, it is possible that they prepare the renal failure. Adverse signs of hepatorenal syndrome include hyponatremia and hypotension in the setting of liver cirrhosis.
Symptoms, complaints, and signs
Hepatorenal syndrome is noticeable by the signs of decompensated cirrhosis. For example, affected individuals suffer from a watery belly, water retention in the tissues (edema), hepatic encephalopathy, and jaundice. In medicine, a distinction is made in hepatorenal syndrome between type 1 and type 2. If type 1 is present, renal function deteriorates rapidly. Doubling of serum creatinine to more than 2.5 mg/dl or a drop in creatinine clearance to values below 20 ml/min are considered important indicators. Common triggers of type 1 include intensive treatment with diuretics, ingestion of certain medications such as nonsteroidal anti-inflammatory drugs, gastrointestinal bleeding, paracentesis without plasma volume expansion, and lactulose overdose. In contrast to type 1, kidney function slowly declines in type 2. Serum creatinine exceeds 1.5 mg/dl. There are no specific triggering factors for this form of hepatorenal syndrome, so it always presents spontaneously. Type 2 is among the most significant causes of therapy-refractory ascites.
Diagnosis and course
Before hepatorenal syndrome can be diagnosed with certainty, all other possible causes of renal failure must be excluded. Thus, establishing the syndrome is ultimately done by diagnosis of exclusion. According to the International Ascites Club, the main criteria are portal hypertension, a creatinine increase to values of more than 1.5 mg/dl or a restriction of creatinine clearance to less than 40 ml/min, and hepatic insufficiency. Other important diagnostic criteria include a urine volume less than 500 ml/day, a serum sodium concentration less than 130 mmol/l, absence of acute bacterial infections, absence of proteinuria greater than 500 mg/day, a urine sodium concentration less than 10 mmol/l, and absence of erythrocyturia greater than 50 cells/face. In addition, urine osmolarity must be greater than serum osmolarity.Narrowing of the renal vessels can be detected by Doppler ultrasound examination of the kidneys. This procedure makes the HRS diagnosis more likely. Thus, hepatorenal syndrome occurs in about 50 percent of all patients with such narrowing as well as cirrhosis of the liver. In most cases, the course of hepatorenal syndrome is negative. Thus, in the case of type 1, survival without medical treatment is less than one month. In type 2, the probability of survival after a period of two years is about 20 percent.
Complications
Kidney failure occurs in this syndrome. This can be fatal for the patient if left untreated and is therefore imperative to be examined and treated by a physician. Usually, there is accumulation of water in the tissues and jaundice. Likewise, a strong water belly develops. There is internal bleeding and thus pain. Due to the malfunction of the kidneys, the patient is usually dependent on dialysis or a donor kidney to continue to survive. The patient’s quality of life is extremely limited 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 alleviated with the help of medication. Discontinuation of some medications may also be necessary after consultation with a physician. In the worst case, a kidney transplant must be performed. Since this syndrome usually does not occur alone, transplantation of the liver is also often necessary so that the patient can continue to survive. In this case, without treatment, the patient will die.
When should you see a doctor?
If symptoms such as jaundice, water retention in the tissues, or a watery belly are noticed, there may be an underlying hepatorenal syndrome. If the symptoms have not subsided after a few days, a visit to the doctor is recommended. If other symptoms develop, medical advice must be sought immediately. For example, 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. Medical help is needed at the latest when these symptoms are noticed. Patients who already suffer from ascites or sepsis are particularly at risk. The use of nephrotoxic drugs can also lead to hepatorenal syndrome. Individuals who belong to these risk groups should see their primary care physician immediately with symptoms mentioned. Other contacts are the nephrologist or a doctor of internal medicine. If symptoms are severe, it is best to go immediately to the nearest hospital or call the emergency physician directly.
Treatment and therapy
To successfully treat hepatorenal syndrome, the kidney circulatory disorder that is causing the condition must be eliminated or positively influenced. This includes discontinuation of medications that are suspected triggers of the syndrome. To improve renal perfusion, the patient often receives vasopressin analogs such as terlipressin. Other treatment options include temporary administration of human albumin or cautious ascites puncture. If a kidney transplant is planned in the foreseeable future, hemodialysis or alternative renal replacement procedures can be performed to keep the patient alive. Although treatment efforts are high, hepatorenal syndrome still has a mortality rate of about 80 percent. In some patients, renal function can be restored with the placement of a transjugular intrahepatic portosystemic shunt. If liver function is restored, this also has a positive effect on kidney function. Therefore, liver transplantation is considered to have the greatest chance of successful treatment.
Outlook and prognosis
Hepatorenal syndrome has a very poor prognosis. Despite intensive treatment, the lethality rate for this disease is 80 percent. Death usually occurs from acute renal failure. Because hepatorenal syndrome is a combined liver-kidney insufficiency, treatment must address both organs. Although the cause of renal failure is not yet fully known. However, hormonal processes that are disturbed by the liver disease are thought to play a major role.Thus, hepatorenal syndrome is always observed in association with abdominal ascites. Ascites represents a symptom of liver cirrhosis. In addition, it has been found that when liver function improves, acute renal failure is reversible. However, despite the usually poor outlook for patients, complete cures are possible. In some patients, drug therapy alone can bring good progress and even contribute to a cure. However, this is the exception. If the drugs do not work, often only a liver transplant can save life. But even a liver transplant is not always possible, especially if the liver disease is too advanced. In very rare cases, a transjugular intrahepatic portosystemic shunt (TIPS) can help the kidneys recover. In TIPS, the hepatic stromal area is bypassed.
Prevention
Preventive measures against hepatorenal syndrome are not known. Thus, it has not yet been possible to elucidate exactly how the disease develops.
Follow-up
In this syndrome, there are usually no direct measures of aftercare available to the affected person. In the first place, a doctor must be consulted at an early stage so that further complications or other complaints do not arise. In the worst case, the affected person will die as a result of 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 must be discontinued in this syndrome. However, a doctor must be consulted first and foremost to ensure that there are no side effects or other interactions. However, if the internal organs have already been severely damaged, only a transplant can completely relieve 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 psychological upsets or depression. Contact with other sufferers of the syndrome can also be very useful. In most cases, the life expectancy of the affected person is significantly reduced by this disease.
This is what you can do yourself
Treatment of hepatorenal syndrome focuses on eliminating the kidney blood flow disorder that is causing the condition. If the syndrome has been diagnosed, the affected person should first take it easy and change his or her diet. The diet should be balanced and healthy. A diet rich in carbohydrates has proved particularly effective in improving 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 take a sick leave for at least four to five weeks. Such a severe procedure can cause discomfort 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 mortality rate is very high despite all possibilities, psychological counseling is also necessary. Not only the patients themselves need support, but often also friends and family members. It is also advisable to participate in a self-help group and to seek conversation with other sufferers.
