What is Hepatorenal Syndrome?
Hepatorenal syndrome (HRS) is a condition that affects both the liver and kidneys. It’s a reason why people might suddenly experience kidney damage, and it often happens in people with existing liver disease, whether that disease is new or long-term. The link between kidney failure and liver disease has been known since the late 1800s. More detailed research was carried out in the mid to late 1900s where it was discovered that kidney failure in people with liver disease wasn’t due to physical damage to the kidneys. This finding was proven when patients with hepatorenal syndrome had normal kidney structures and didn’t show excess protein in their urine, which can be signs of kidney damage. To clinch this, kidneys from patients with HRS were successfully transplanted into people with chronic kidney disease. Also, when people with liver disease received a liver transplant, their kidney function improved. More studies on kidney function discovered that there is a significant link between narrow or constricted blood vessels in the kidney and HRS.
What Causes Hepatorenal Syndrome?
Viral hepatitis, an infection causing inflammation of the liver, is the leading cause of liver failure and related kidney issues in developing countries. This often comes from Hepatitis B or less often, Hepatitis C. In wealthier countries, the main causes of liver failure are usually drugs or medications. The most common are acetaminophen (a common pain reliever), chronic alcohol abuse, or any drugs that activate a specific protein in our body, cytochrome p450. Non-alcoholic fatty liver disease (NASH), a disease resulting from fat built-up in the liver, is also a possible cause.
Less common causes of liver failure include other types of viruses like CMV, HHV6, and Parvovirus B19. Certain blood clot conditions like hepatic/portal vein thrombosis (clots forming inside the liver) and metabolic issues such as non-alcoholic fatty liver disease can also lead to liver failure.
Risk Factors and Frequency for Hepatorenal Syndrome
Hepatorenal syndrome, a condition that affects patients with severe liver disease, is not very common, with a rate of about 4%. The majority of these patients often have high blood pressure in the liver’s blood vessels, resulting from conditions like excessive alcohol-induced liver inflammation, liver cirrhosis, or cancers that have spread. The risk of developing this syndrome within a year is 18% and increases to 39% over a five-year period for individuals with severe liver disease. The most at-risk individuals are those with low sodium levels in their blood and high kidney hormone activity. Additionally, about one in three patients with spontaneous bacterial peritonitis, a type of abdominal infection, can develop hepatorenal syndrome.
- Hepatorenal syndrome affects about 4% of patients with severe liver disease.
- Most of these patients have high blood pressure from conditions like alcoholic hepatitis, cirrhosis, or metastatic cancers.
- There is an 18% chance of getting hepatorenal syndrome in one year and a 39% chance over five years for these patients.
- The people most at risk have low sodium levels and high kidney hormone levels.
- One in three patients with spontaneous bacterial peritonitis can also develop hepatorenal syndrome.
Signs and Symptoms of Hepatorenal Syndrome
Hepatorenal syndrome is a severe condition which develops due to liver failure. The symptoms of this condition can start off with tiredness and lack of energy, which could be due to different reasons. This can also be accompanied by a decreased appetite because of constant nausea. As the disease progresses, patients start looking more yellow, a symptom called jaundice, which begins from the whites of the eyes then covering the whole body. The liver starts losing its ability to perform its usual functions, leading to an increased risk of bleeding. In addition to all these issues, fluid slowly collects in the belly due to the loss of albumin, a protein, from the bloodstream. The last and most dangerous stage of the liver disease results in confusion due to a condition called hepatic encephalopathy. This happens because the liver fails to remove harmful chemicals from the body. An important symptom these patients notice is that they pee less frequently and the volume keeps decreasing.
- Tiredness and lack of energy
- Decreased appetite
- Constant nausea
- Jaundice, starting from the whites of the eyes and then covering the whole body
- Increased risk of bleeding
- Fluid collection in the belly
- Confusion due to hepatic encephalopathy
- Decreased frequency and volume of urination
Testing for Hepatorenal Syndrome
Hepatorenal syndrome is a condition that has two types and it’s usually diagnosed after other diseases have been ruled out. Type 1 is marked by a quick and significant drop in kidney function. This is identified when the level of creatinine (a waste product) in your blood doubles to a point higher than 2.5 or when the ability of your kidneys to clear creatinine decreases by half or more over a span of two weeks. Usually, if you have Type 1 hepatorenal syndrome, you may also produce less than 500mL of urine per day, your urine may not contain any troubling substances, and you may pass a small amount of sodium when you urinate.
On the other hand, Type 2 hepatorenal syndrome is often associated with a lesser degree of kidney injury and patients tend to get it along with diuretic-resistant ascites (a buildup of fluid in the abdomen that can’t be treated with water pills). Before diagnosing hepatorenal syndrome, doctors have to rule out other possible causes of sudden kidney injury such as dehydration, harmful drugs, a blockage in the urinary tract, and diseases of the kidney tissue itself.
Treatment Options for Hepatorenal Syndrome
Firstly, treating Hepatorenal Syndrome (HRS), a type of kidney failure that happens to people with severe liver problems, involves keeping the body’s fluid levels balanced and stopping the use of any medications that might harm the kidneys. The primary goal of treatment is to increase blood flow and pressure in the body. By doing this, the body decreases the production of chemicals that can constrict the kidneys’ blood vessels, leading to an improvement in blood flow to the kidneys. Multiple different combinations of medications can be used to achieve this and they vary depending on location. For example, a mix of terlipressin and albumin is used in Europe, while in North America, drugs like noradrenaline or midodrine and octreotide are used.
When medications are not effective, a surgical procedure can help. This involves inserting a tube (or shunt) from the portal vein (a large vein that carries blood to the liver) to the hepatic vein (a vein that carries blood away from the liver). This shunt allows blood to bypass the liver and flow directly into the body’s general circulation, reducing pressure in the liver and increasing the flow of blood back to the heart. Ultimately, this helps reduce the overstimulation of the body’s hormones and nervous system, which is often a problem in HRS.
The most effective long-term solution for HRS is the replacement of the unhealthy liver with a healthy one via liver transplantation.
There are some other experimental treatments, like a modified form of dialysis or kidney replacement therapy, but these have not been proven to extend life. They may, however, be used as a temporary measure while waiting for a liver transplant.
Patients with Spontaneous Bacterial Peritonitis, an infection of the fluid in the abdomen, should be treated immediately and continue with long-term antibiotic therapy to prevent the development of HRS. Likewise, patients with infections from other parts of the body should be treated promptly to reduce the release of chemicals that can lead to HRS.
What else can Hepatorenal Syndrome be?
Here are different types of glomerulonephritis, a type of kidney disease, you might come across:
- Acute glomerulonephritis
- Acute tubular necrosis
- Chronic glomerulonephritis
- Crescentic glomerulonephritis
- Diffused proliferative glomerulonephritis
- Membranoproliferative glomerulonephritis
- Membranous glomerulonephritis
- Poststreptococcal glomerulonephritis
- Rapidly progressive glomerulonephritis
What to expect with Hepatorenal Syndrome
The outlook for Type 1 Hepatorenal Syndrome (HRS), which is a type of kidney failure related to liver disease, can be quite challenging, with typical survival around two weeks. On the other hand, survival in Type 2 HRS depends on the seriousness of the liver disease. ‘Child-Pugh class’ refers to a grading system used to assess the severity of liver disease. However, Type 2 is generally more of a long-term condition with a median survival time of 6 to 12 months without any treatment.
Possible Complications When Diagnosed with Hepatorenal Syndrome
Kidney failure and death can occur as a severe consequence.
Preventing Hepatorenal Syndrome
New, innovative treatments for Type 1 Hepatorenal Syndrome (HRS Type 1, a kidney disorder in people with severe liver disease) have shown to increase the lifespan of patients. However, the effectiveness of these treatments for Type 2 Hepatorenal Syndrome (HRS Type 2, another form of kidney disorder in people with liver disease) isn’t as clearly understood yet.
For people with HRS Type 1, having a procedure known as paracentesis (where a thin needle or tube is used to remove excess fluid from the abdomen) combined with replacing albumin (a type of protein that the liver makes) resulted in an average lifespan of just over a year. Also, in patients undergoing a procedure called TIPS (Transjugular Intrahepatic Portosystemic Shunt, a procedure that creates a new connection between two blood vessels in your liver), the average lifespan was seen to extend to almost 20 months.
Looking at the bigger picture, within two years of receiving paracentesis, the survival rate was between 30% to 32% in patients. For those that had the TIPS procedure, the two-year survival rate ranged from 30% to 58%.
