What is the Hepatorenal Syndrome?

General Description 

Hepatorenal Syndrome (HRS) is a characteristic form of renal failure that happens in patients with hepatic cirrhosis, of a functional nature that appears as a consequence of an intense vasoconstriction of the secondary renal circulation to the systematic circulatory dysfunction.

It is potentially reversible that happens in patients with cirrhosis, ascites and hepatic failure and it is characterized by a renal functions alteration, marked alterations of the cardiovascular function and activation of the sympathetic nervous system and renin-angiotensin-aldosterone. An intense renal vasoconstriction leads to a reduction of the glomerular filtration. The HRS can appear spontaneously or after a precipitant factor. 

It is an entity that appear in advanced phases of cirrhosis and it is associated with a bad forecast.

Causes

Hepatorenal Syndrome happens when the kidneys stop their functions in people with serious hepatic problems. Less urine from the body is eliminated, so the disposal products that contain nitrogen are accumulated in the bloodstream (azotemia).

The disorder happens up to 1 in 10 hospitalized people because hepatic failure and it leads to renal failure in people with:

• Acute hepatic failure.

• Alcoholic hepatitis.

• Cirrhosis

• Infected abdominal liquid.

Risk Factors 

• Blood pressure that decreases when a person stands up o changes of position suddenly (orthostatic hypotension).

• The use of diuretic medicines.

• Gastrointestinal Bleeding.

• Infection.

• Recent extraction of abdominal liquid (paracentesis).

Symptoms

These includes: (ascites, a symptom of a liver disease).

• Mental confusion.

• Muscle spams.

• Turbid urine.

• Decrease of the urinary output.

• Nauseas and vomiting.

Increase of weight.

• Yellow skin (jaundice, a symptom of a liver disease).

What is the clinic presentation of the HRS?

HRS is a form of renal lesion, because of that patients present an increase of serum creatine and an urine´s low production.

Clinically, there are two types.

1. The acute HRS or type 1 is characterized for a renal failure rapidly progressive in a cirrhotic patient with ascites. Generally, it is developed after a precipitant event, but it can happen spontaneously.

The patients are usually very sick, with serious jaundice, coagulopathy and hepatic failure. The HRS type 1 is now renamed as AKI-HRS.

2. The chronic HRS or type 2 is characterized for a moderate renal failure, with a serum creatine between 1,5 and 2,5 mg/dl. It evolves slowly during weeks to months in patients with ascites, refractory to diuretic. The patients with HRS type 2 are usually less sick than the ones who have HRS type 1, with a slighter degree of jaundice and coagulopathy.

Diagnosis

Usually the diagnosis is done in a hospital institution after a good physical exam of the patient and the realization of laboratory tests. 

A physical exam, usually shows signs of chronic liver disease such as:

• Ascites (excess of liquid in the abdomen).

• Confusion.

• Jaundice. 

• Testicles with less size.

• Increase of breast tissue.

• Abnormal reflexes.

• Skin ulcers.

When to contact a professional?

This illness is usually diagnosed at hospital, during the treatment of a liver disorder.

Tests and exams

The diagnosis of this condition after discard other causes of renal failure.

A physical exam will not reveals directly the renal failure. However, the exam usually shows signs of chronic liver disease, such as:

• Confusion (usually because to a hepatic encephalopathy).

• Excess of liquid in the abdomen (ascites).

• Jaundice.

• Other signs of hepatic failure.

Other signs embrace: 

• Abnormal reflexes.

• Smaller testicles.

• Deaf sound in the abdomen when percussion with fingertips is done.

• Increase of breast tissue (gynecomastia).

• Ulcers (lesions) in the skin.

The following are signs of renal failure:

• Absence or decrease in urine´s production.

• Retention of liquid in the abdomen or the limps.

• Increase in the levels of BUN (Blood urea nitrogen) and Creatine.

• Increase of the urine´s specific density and its osmolarity.

• Low serum sodium.

• Very low concentration of sodium in the urine.

The following can be sings of hepatic failure:

• Time of abnormal prothrombin.

• Increase in levels of blood ammoniac.

• Low bloodly albumin.

• Paracentesis that shows ascites.

• Sings of hepatic encephalopathy (an EGG can be done).

Complications

The complications can include:

• Bleeding.

• Injury and insufficiency of multiple organ systems.

• Terminal renal disease.

• Fluid overload with cardiac insufficiency.

• Coma caused by hepatic failure. 

• Secondary infections.

• Hepatic coma.

Treatment

The objective of the treatment is help to the liver to work better and guarantee that the heart can pump enough blood to the body.

The treatment is usually the same as for a renal failure for any cause and includes: 

• Suspend all the unnecessary medicines, specially the ibuprofen and others NSAIDs, some antibiotics and diuretics.

• Dialysis for improve the symptoms. 

• Take medications for improve the blood pressure and help to the kidneys work better, the albumin infusion can be very useful.

• Place a derivation (known as TIPS) for relieve the symptoms of ascites (it also can help to the renal function, but the process can be dangerous).

• Surgery for place a derivation from the abdominal space to the jugular vein for relieve some symptoms of renal failure (this process is dangerous and it is done rarely).

How is the liver transplant used in the treatment of HRS?

The liver transplant is the definitive treatment for both AKI-HRS and chronic HRS type 2. It corrects the dysfunction of liver and eliminates the portal hypertension, both fundamental pathogenic mechanisms for the development of HRS. However, just between the 50% and the 75% of patients with AKI-HRS before the transplant will get normal renal function after the transplant, and this throwback to the normal renal function is independent to pre-transplant pharmacotherapy and dialysis. 

Frequently dialysis is started for treat the electrolytic anomalies and volume overload before the liver transplant. Many studies have proved that the longer a patient is in dialysis before transplant, is less probable that it throws back the renal dysfunction. Most of guides suggest that patients must be considerate for a joint liver surgery and renal transplant if they have been in dialysis pre-transplant during a prolonged period (8 weeks). For patients with AKI-HRS, the general survival is significantly better for those who recover the post-transplant kidney´s function; thus, it is imperative offer to those patients an opportune liver transplant.

The use of liver transplants from an alive donor seems provide similar results to the donation of cadaverous liver transplant.

What is its forecast?

AKI-HRS is associated with a bad forecast. The studies show a mortality at two weeks up to 80% in AKI-HRS not treated. In chronic HRS type 2, the decrease of normal renal function is more gradual, but it is also associated with a bad forecast, with a median survival of 3 to 6 months.

Conclusions

1. Hepatorenal syndrome (HRS) is a form of acute kidney injury (AKI), which happens in patients with terminal liver cirrhosis and circulatory dysfunction.

2. The circulatory dysfunction of terminal cirrhosis leads to a reduction in the volume of effective arterial blood, which stimulates the renal vasoconstriction through various systematic vasoconstrictors systems, predisposing to patients to renal failure.

3. Any condition that causes a bigger reduction of effective arterial bloody volume can potentially precipitate AKI-HRS. Those includes sepsis, over-diuresis, a paracentesis of great volume (5 liters) without replacement of intravascular volume and gastrointestinal bleeding.

4. In hospitalized patients with cirrhosis, the prerenal azotemia is the most common causes of renal lesion. HRS can be considered a form of prerenal azotemia, which does not respond to volume and it is exclusively seen in patients with severe hepatic dysfunction.

5. HRS type 1 has been renamed as AKI-HRS.

6. The pharmacologic treatment with: (midodrine and octreotide, terlipressin, norepinephrine) increase survival, but the only definitive treatment is the hepatic transplant.