Clinical Commander

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hep.acute-liver-failure.core.v1

Acute Liver Failure

hepatologyacuteadultacuteinpatient

Fresh hep.* prefix dossier; manifest is batch23 scaffold (sourceWorkupId acute_liver_failure already registered). NAC continuous Prescott protocol for ALL ALF per AASLD + Lee 2009 (PMID 19524577; transplant-free survival benefit non-APAP coma I-II). Kings College + Bernal lactate criteria drive UNOS Status 1A listing; no calculator-registry entry yet (formulas embedded in rationale). Cerebral edema bundle (Bernal Wendon 2013 NEJM PMID 24369077): hypertonic saline target Na 145-150 + mannitol rescue + HOB 30deg + minimize stimulation. Etiology-specific Step 2: prednisolone (AIH); entecavir/TAF (HBV); penicillamine/zinc NOT useful in Wilson ALF (transplant-only); emergent delivery (AFLP/HELLP). Sibling differentiation vs cirrhosis (ACLF) + alcoholic hepatitis explicit.

Entry points (5)

  • lab_abnormality
    INR >=1.5 in patient without known chronic liver disease (AASLD ALF)
    inr_ge_1_5_no_chronic_lvr
  • symptom
    New-onset hepatic encephalopathy / altered mental status (AASLD ALF)
    new_encephalopathy
  • symptom
    Rapid-onset jaundice + coagulopathy in previously healthy patient (Stravitz Lee 2019)
    jaundice_rapid
  • history
    Known or suspected acetaminophen overdose (AASLD ALF)
    acetaminophen_overdose
  • lab_abnormality
    AST/ALT >1000 U/L with INR derangement (Stravitz Lee 2019)
    transaminitis_severe

Required inputs (20)

  • agerequired
    demographic • used at CONTEXT
    Age <11 or >40 = adverse Kings College non-APAP criterion (OGrady 1989)
  • sbprequired
    vital • used at CONTEXT
    Distributive shock + cerebral perfusion pressure; MAP >=75 target for cerebral edema
  • temperaturerequired
    vital • used at CONTEXT
    Hypothermia 35-36C neuroprotective; hyperthermia worsens ICP
  • spo2required
    vital • used at RED_FLAGS
    ARDS / aspiration risk in HE grade 3-4; intubation trigger
  • inrrequired
    lab • used at INITIAL_WORKUP
    INR >=1.5 = ALF diagnostic criterion; Kings College component (AASLD ALF)
  • total_bilirubinrequired
    lab • used at INITIAL_WORKUP
    Kings College non-APAP component; severity marker (OGrady 1989)
  • creatininerequired
    lab • used at INITIAL_WORKUP
    Kings College APAP component (Cr >3.4 mg/dL); HRS risk (OGrady 1989)
  • arterial_phrequired
    lab • used at INITIAL_WORKUP
    Kings College APAP component (pH <7.30 after resuscitation) (OGrady 1989)
  • arterial_lactaterequired
    lab • used at INITIAL_WORKUP
    Kings College APAP component (lactate >3.5 early or >3.0 post-resus) (Bernal 2002)
  • ast_altrequired
    lab • used at INITIAL_WORKUP
    Pattern recognition: AST/ALT >5000 = ischemia; >1000 with ALT>AST = APAP/viral (Stravitz Lee 2019)
  • ammonia
    lab • used at INITIAL_WORKUP
    Arterial NH3 >150 micromol/L predicts cerebral edema and intracranial HTN (Bernal Wendon 2013)
  • glucoserequired
    lab • used at RED_FLAGS
    Hypoglycemia from hepatic gluconeogenesis failure; D10 infusion (Stravitz Lee 2019)
  • phosphorus
    lab • used at INITIAL_WORKUP
    Low phosphorus = regeneration; high phosphorus = poor prognosis (Schmidt 2002)
  • acetaminophen_levelrequired
    lab • used at INITIAL_WORKUP
    Identifies treatable APAP toxicity; Rumack-Matthew nomogram
  • hbv_hav_hcv_hev_serologyrequired
    lab • used at BRANCHING_WORKUP
    Viral ALF workup (anti-HAV IgM, HBsAg + anti-HBc IgM, HCV RNA, HEV IgM) (AASLD ALF)
  • ceruloplasmin
    lab • used at BRANCHING_WORKUP
    Wilson disease workup in <40yo with hemolytic anemia + ALP/bilirubin ratio <4 (Korman 2008)
  • doppler_abdominal_usrequired
    imaging • used at INITIAL_WORKUP
    Rule out Budd-Chiari, portal vein thrombosis, biliary obstruction (Stravitz Lee 2019)
  • head_ct_for_he
    imaging • used at BRANCHING_WORKUP
    Rule out structural brain lesion + assess cerebral edema in HE grade 3-4 (AASLD ALF)
  • medication_supplement_reviewrequired
    history • used at CONTEXT
    DILI/HDS most common non-APAP cause; LiverTox database (Stravitz Lee 2019)
  • pregnancy_statusrequired
    history • used at CONTEXT
    AFLP, HELLP, eclamptic ALF differential in women of reproductive age (AASLD ALF)

12-phase flow (12)

  1. 1FRAME
    Confirm ALF scope: INR >=1.5 + encephalopathy + no chronic liver disease + <26 wk duration (AASLD ALF)
    inputs: inr, age
    advance: ALF criteria met
  2. 2ENTRY
    Recognize coagulopathy + new encephalopathy or known APAP overdose with rising INR (AASLD ALF)
    advance: one entry trigger present
  3. 3CONTEXT
    Vitals, full medication/supplement review (DILI/HDS LiverTox), pregnancy status, alcohol/substance use, exposure history (mushroom, herbal) (Stravitz Lee 2019)
    inputs: age, sbp, temperature, medication_supplement_review, pregnancy_status
    advance: context captured
  4. 4RED_FLAGS
    HE grade 3-4 with airway compromise, cerebral edema (papilledema, posturing, Cushing reflex), hypoglycemia, hyperammonemia >150, refractory acidosis (Bernal Wendon 2013)
    inputs: spo2, glucose, ammonia
    actions: acute_liver_failure
    advance: red flags identified and addressed
  5. 5INITIAL_WORKUP
    INR/PT, full LFTs, BMP, ABG with lactate, ammonia (arterial), CBC, type and screen, acetaminophen level, viral hepatitis serologies (anti-HAV IgM, HBsAg, anti-HBc IgM, HCV RNA, HEV IgM in endemic), pregnancy test, Doppler abdominal US (AASLD ALF)
    inputs: inr, total_bilirubin, creatinine, arterial_ph, arterial_lactate, ast_alt, acetaminophen_level, doppler_abdominal_us
    actions: panel.lft, panel.cbc, panel.renal, panel.coag, panel.abg
    advance: core labs and US obtained
  6. 6BRANCHING_WORKUP
    Etiology-specific: ceruloplasmin + slit-lamp for Wilson <40yo; ANA/SMA/IgG for AIH; HEV IgM if travel/transplant; head CT if HE grade 3-4 or focal deficit; ICP transducer for selected HE 4 (Stravitz Lee 2019)
    inputs: hbv_hav_hcv_hev_serology, ceruloplasmin, head_ct_for_he
    actions: acute_liver_failure
    advance: etiology assigned or indeterminate documented
  7. 7DIFFERENTIAL
    Categorize: acetaminophen (50% US), DILI/HDS (11%), indeterminate (11%), viral (HAV/HBV/HEV) (12%), autoimmune (5-7%), Wilson, Budd-Chiari, ischemic, AFLP/HELLP, mushroom (Amanita), malignant infiltration (Stravitz Lee 2019)
    inputs: ast_alt, medication_supplement_review
    advance: etiology category assigned
  8. 8RISK_STRATIFICATION
    Kings College criteria for transplant listing: APAP - pH <7.30 OR (Cr >3.4 + INR >6.5 + grade 3-4 HE); non-APAP - INR >6.5 OR any 3 of (age <11 or >40, non-A/non-B/DILI etiology, jaundice-to-encephalopathy >7 days, INR >3.5, bilirubin >17.5 mg/dL); MELD >=33 also predicts mortality; arterial lactate >3.5 early postresuscitation (Bernal lactate criteria) (OGrady 1989; Bernal 2002)
    inputs: inr, total_bilirubin, creatinine, arterial_ph, arterial_lactate
    advance: transplant listing decision made
  9. 9TREATMENT
    NAC for ALL ALF (Lee 2009 non-APAP benefit; standard for APAP); etiology-specific (penicillamine + Wilson; corticosteroids + AIH; entecavir/TAF + HBV; delivery + AFLP/HELLP); cerebral edema bundle (head of bed 30deg, hyperventilation to PaCO2 30-35, 3% saline or mannitol, target Na 145-150); broad-spectrum antibiotics for sepsis; PPI; renal replacement therapy for hyperammonemia or AKI (AASLD ALF; Stravitz Lee 2019)
    inputs: inr, creatinine, ammonia
    advance: NAC infusion started + etiology-specific therapy + transplant center notification
  10. 10DISPOSITION
    ICU at transplant center; UNOS Status 1A listing if Kings criteria met; transfer if not at transplant center; intubation for HE grade 3-4 (AASLD ALF)
    inputs: sbp
    advance: destination + transplant evaluation locked
  11. 11MONITORING
    q1h neuro checks (HE grading), continuous arterial BP, hourly glucose initially, q4h NH3 / lactate / Cr / INR, MELD trend; ICP monitor for selected; serial transcranial Doppler for cerebral edema (Bernal Wendon 2013)
    inputs: inr, ammonia, glucose
    advance: response or progression documented
  12. 12FOLLOWUP
    Survivors: hepatology follow-up; counseling on hepatotoxin avoidance (APAP <2 g/day if at-risk; alcohol abstinence); LiverTox reporting for DILI; vaccinations (HAV/HBV/influenza/pneumococcal); transplant recipients per transplant center protocol (Stravitz Lee 2019)
    advance: follow-up scheduled