Disease Description (Causes and Symptoms)
- Definition: Hepatorenal syndrome (HRS) is a condition in which acute/progressive deterioration of kidney function occurs without structural damage to the kidneys themselves in patients with cirrhosis or acute liver failure.
- Pathophysiology: In severe liver disease, dilation of the portal (splanchnic) blood vessels reduces the effective circulating blood volume. Compensatory systemic and renal vasoconstriction then occurs, reducing renal blood flow and worsening kidney function. It may be aggravated by inflammation·infection, bleeding, or large-volume paracentesis.
- Triggers: Spontaneous bacterial peritonitis (SBP), excessive fluid removal (large-volume paracentesis), upper gastrointestinal bleeding, diuretic overuse, nephrotoxic drugs (NSAIDs, aminoglycosides), shock, etc.
- Main symptoms: Decreased urine output (oliguria), jaundice, ascites·generalized edema, dark urine, itchy skin, confusion·hepatic encephalopathy, etc. In general, kidney-related tests show increased blood creatinine·blood urea nitrogen and hyponatremia.
- Diagnostic points (summary): Background of cirrhosis·ascites, rapid deterioration of kidney function (increased creatinine), no improvement even after volume expansion (albumin), and exclusion of shock·nephrotoxic drugs·structural kidney disease (proteinuria·marked hematuria·abnormal renal ultrasound findings).
Treatment Methods (Standard Treatment·Medications·Procedures/Surgery·Lifestyle Management and Precautions)
- Emergency treatment (in hospital):
- Correcting the trigger: Rapid administration of antibiotics for infection (especially SBP), hemostasis·fluid replacement for upper gastrointestinal bleeding, discontinuation of diuretics, etc.
- Combination therapy with a vasoconstrictor + albumin (current standard treatment):
- Terlipressin + intravenous albumin: First-line treatment for HRS-AKI when available. The method and dosage of terlipressin administration vary by institution, so follow the specialist’s instructions. Albumin is often used at an initial dose (e.g., 1 g/kg, up to 100 g), followed by a maintenance dose (20–40 g/day).
- Alternative (in the intensive care unit): Continuous infusion of norepinephrine (used with a blood pressure target).
- In outpatient settings or when terlipressin is unavailable: A combination of oral midodrine + subcutaneous or intravenous octreotide + albumin may also be used.
- Fluid management: Attempt to restore the effective circulating volume by discontinuing diuretics and providing appropriate fluids·albumin replacement.
- Renal replacement therapy (RRT): Temporary dialysis (as a bridge) may be considered if uremia·electrolyte abnormalities·fluid overload develop. Dialysis itself does not improve long-term prognosis.
- Definitive treatment:
- Liver transplantation: This is the curative treatment for HRS, and kidney function may recover after transplantation. Combined liver-kidney transplantation may be considered in cases of long-term dialysis dependence or changes indicating pre-existing kidney disease.
- Lifestyle management and precautions (self-care):
- Avoid drugs that may worsen kidney function, such as NSAIDs and ACE inhibitors/ARBs (consult a doctor).
- Abstain from alcohol, follow a low-sodium diet, and seek medical attention early in the event of infection.
- Report changes in weight·urine output·edema immediately.
- Risk of complications:
- Shock, multiple organ failure, pulmonary edema, worsening infection, end-stage kidney disease (requiring dialysis), and high mortality. Prognosis is poor if treatment is delayed.
- Clinical precautions:
- Vasoconstrictors such as terlipressin may cause cardiovascular adverse effects (including ischemic symptoms)—use caution in patients with a cardiovascular history.
- Be cautious of fluid overload with excessive albumin administration (such as in patients with heart failure).
- Treatment decisions (including indications for liver transplantation and the need for combined transplantation) require multidisciplinary consultation (gastroenterology·transplant surgery·nephrology, etc.).
Checklist Before an Outpatient Visit (Including Items to Prepare · Things to Check Before the Visit · Example Questions)
- Items to prepare (recommended to bring):
- List of currently used medications (including prescriptions·OTC medications·herbal medicines) and times of administration
- Recent test results (blood tests: creatinine, BUN, electrolytes, liver function, INR, albumin, serum bilirubin, etc.)
- Recent weight records (if possible, every morning under the same conditions), and records of daily urine volume·urination frequency
- Whether and how much paracentesis was recently performed, and history of hospitalization·surgery·infection (especially SBP)
- Recent alcohol use and amount
- Symptoms to check before the outpatient visit:
- Changes in urine output (whether it has decreased), decreased energy, confusion·disorientation, loss of appetite, abdominal distension·rapidly increasing edema, shortness of breath (suspected fluid overload·pulmonary edema)
- Materials to show the doctor at the outpatient visit:
- Most recent blood test results (if possible, from within the past 1–2 weeks), abdominal ultrasound·renal ultrasound reports, and ascites test findings
- Example questions (may be used during the consultation):
- “How much has my creatinine increased in the recent tests, and how quickly is it progressing?”
- “Are there any of the medications I am currently taking that I should stop?”
- “Is my condition one that requires hospitalization, and what treatments are possible as an outpatient?”
General Example Questions to Ask the Doctor
- “What are the expected effects and possible side effects of this medication (e.g., terlipressin, albumin)?”
- “Is my kidney damage reversible? How likely is recovery with treatment?”
- “Do I meet the indications for liver transplantation, and do I need to be placed on the waiting list?”
- “If dialysis becomes necessary, how will that decision be made? What does dialysis mean in the long term?”
- “Are there any medications·foods·activities that I must avoid in daily life?”
Examples of Disease-Specific Questions
- “Based on my current kidney function (using my recent creatinine level), how likely is improvement with a vasoconstrictor (such as terlipressin)?”
- “If I become a candidate for liver transplantation, is there a possibility that I would also need a kidney transplant (whether I meet the indications for combined liver-kidney transplantation)?”
(Note: Specific treatment doses·indications·decisions vary depending on each patient’s condition and must be determined through consultation with the treating specialist·transplant team.)