358.3 🏥 內科專科考前版

358.3.1 Mechanistic Deep Dive

358.3.1.1 Portal Hypertension Mechanism

  • ↑ Intrahepatic resistance (mechanical + functional)
  • ↑ Splanchnic blood flow (vasodilation)
  • ↑ Cardiac output
  • ↓ Systemic vascular resistance

358.3.1.2 Hyperdynamic Circulation

  • Splanchnic + systemic vasodilation
  • Hyperdynamic state
  • Effective hypovolemia → RAAS, AVP, SNS activation

358.3.1.3 Ammonia + HE

  • Astrocyte glutamine accumulation
  • GABA-glutamate imbalance
  • Manganese deposition (chronic)
  • Inflammation contributory

358.3.2 Recent Trials & Updates

358.3.2.1 CONFIRM (2021) — Terlipressin HRS

  • FDA approval 2022
  • ↓ HRS reversal

358.3.2.2 REVERSE Trial — Terlipressin

  • Phase 3 in HRS-1
  • Approved

358.3.2.3 Early TIPS (Garcia-Pagan 2010)

  • Pre-emptive TIPS for high-risk
  • ↓ Rebleeding + mortality
  • Practice-changing

358.3.2.4 TRIGGER (Restrictive Transfusion)

  • Hgb 7 vs 9 target
  • Restrictive better outcomes
  • Applies to UGIB

358.3.2.5 Beta-Blockers in End-Stage

  • Caution in advanced (refractory ascites, hypotension)
  • May worsen prognosis

358.3.2.6 Rifaximin in Cirrhosis

  • Reduces HE recurrence (PROFIT)
  • Reduces SBP recurrence (some)
  • Now incorporated

358.3.3 High-Yield Specialist Points

358.3.3.1 MELD Exception Points

  • HCC within Milan criteria
  • Hepatopulmonary syndrome
  • Portopulmonary hypertension
  • Familial amyloidotic polyneuropathy
  • Cholangiocarcinoma (Mayo protocol)
  • Cystic fibrosis

358.3.3.2 TIPS Indications

  • Refractory ascites
  • Refractory variceal bleeding
  • Hepatorenal syndrome (selected, with vasoconstrictor)
  • Budd-Chiari syndrome
  • Hepatic hydrothorax

358.3.3.3 TIPS Contraindications

  • Heart failure (right-sided)
  • Severe encephalopathy
  • Polycystic liver
  • Severe pulmonary hypertension
  • Active infection
  • Coagulopathy uncorrected

358.3.3.4 TIPS Complications

  • Encephalopathy (worsens)
  • Stent occlusion
  • Cardiac decompensation
  • Hepatic injury

358.3.3.5 Hepatopulmonary Syndrome (HPS)

  • Triad: liver disease + intrapulmonary vasodilation + hypoxemia
  • Orthodeoxia + platypnea
  • Treatment: O2 + liver transplant

358.3.3.6 Portopulmonary Hypertension (POPH)

  • PAH in portal hypertension
  • PAH-specific therapy
  • Liver transplant in select

358.3.3.7 Hepatic Hydrothorax

  • Ascites communicating with pleural space
  • Usually right-sided
  • Treatment similar to ascites; TIPS

358.3.3.8 Variceal Bleeding Mortality

  • 15-20% at 6 weeks
  • Active management critical

358.3.3.9 Sarcopenia + Cirrhosis

  • Common
  • Prognostic
  • Nutrition + exercise
  • BCAA (branched-chain amino acids) supplementation

358.3.3.10 Cirrhotic Cardiomyopathy

  • Subclinical
  • Stressed in surgery / pregnancy / fluid loading
  • May contribute to HRS

358.3.3.11 Coagulation in Cirrhosis

  • “Rebalanced” hemostasis
  • INR not reliable for bleeding
  • TEG / ROTEM for selected

358.3.3.12 Liver Transplant + Cirrhosis

  • See Ch358
  • MELD-based priority
  • Many indications

358.3.3.13 Acute-on-Chronic Liver Failure (ACLF)

  • Cirrhosis + acute precipitant
  • Multi-organ failure
  • High mortality
  • ICU + liver transplant evaluation
  • Different staging (CLIF-C)

358.3.4 Pearls

  • Cirrhosis main causes: HBV, HCV, ALD, MASLD
  • MELD-Na for transplant priority
  • HVPG > 12 mmHg: variceal bleeding risk
  • SAAG ≥ 1.1: portal HTN
  • SBP: PMN > 250; ceftriaxone + albumin (1.5 + 1 g/kg)
  • Variceal bleeding: octreotide + ceftriaxone + banding + TIPS
  • HE: lactulose + rifaximin
  • HRS: terlipressin + albumin (FDA 2022)
  • HCC surveillance: US ± AFP every 6 mo
  • Early TIPS for high-risk variceal bleeding