DSA: Approach to the Hepatobiliary Patient (McGowan) Flashcards
What are 4 molecules that are used to assess TRUE LIVER FUNCTION? (P/A/C/A)
PT/INR, albumin, cholesterol, and ammonia
What two findings are typically elevated with hepatocellular injury and cholestatic injury?
H: injury to hepatocytes
- alkaline and aspartate aminotransferase (ALT, AST)
- ALT is MORE specific than AST
C: injury to the bile ducts
- alkaline phosphatase and bilirubin elevation
- jaundice and pruritus
What do the Liver Function Tests for Coagulation Factors, Albumin, and Ammonia tell us about liver status?
CF: SINGLE BEST measure of hepatic synth function
- all clotting factors except factor 8 synthed here
- replace Vit. K w/no change = hepatic disease
Albumin: hypoalbuminemia correlates w/severity of liver dysfunction
Ammonia: elevation does NOT correlate w/hepatic function or degree of acute encephalopathy
What does a positive Murphy Sign indicate?
Acute Cholecystitis
Cholelithiasis
What are the two types, when does pain present and where does it radiate, and what labs/imaging is associated with it?
Two types of GALLSTONES:
- Cholesterol (80%): >50% cholesterol monohydrate
- Pigment (20%): primarily made of Ca bilirubinate
C: most silent; can have steady RUQ/epigastric pain 30-90 min after eating and can radiate to RIGHT SCAPULA (also nausea/vomiting)
L/I: normal labs, some bilirubin elevation; ULTRASOUND is best diagnostic (see stones and “acoustic shadowing”)
What are the 6 ‘F’s’ of Gallstones?
What are specific protective measures for women and men?
Female, Fair (Caucasian), Fat, Fertile, Forty, and Family History
W: consumption of caffeinated coffee
M: high intake of Mg/mono and polyunsaturated fats
Acute Cholecystitis
What are the two types, what are common symptoms, what are 3 common lab findings, and what are two complications it can cause? (GG/EC)
Two types of Gallbladder INFLAMMATION:
- Calculous: gallstones (90%); cystic duct impaction
- Acalculous: no stones; many causes
S: (+) Murphy Sign, sometimes Jaundice, acholic stool or tea-colored urine
L: leukocytosis, bilirubinemia, inc. ALP/GGT lvls
- RUQ ultrasound (thick wall, pericholecystic fluid)
C: gallbladder gangrene, emphysematous cholecystitis
Choledocholithiasis
What is it, what can it lead to, how does it present, and how can it be treated (2)?
- stones in the COMMON BILE DUCT
- can lead to Ascending Cholangitis (infection)
S: biliary pain, +/- jaundice, nausea, vomiting
- inc. AST/ALT, DIRECT hyperbilirubinemia
- ALP/GGT rise slowly
T: ERCP with sphincterotomy/stone extraction and cholecystectomy
Ascending Cholangitis
What is it, what are its two classical presentations (CT/RP), what 3 organisms are seen on blood cultures (E/K/E), and how is it treated (2)?
- infection of choledocholithiasis (LEUKOCYTOSIS)
P: Charcot Triad (RUQ pain, fever, jaundice) and Reynold Pentad (Triad, altered mental status, hypotension)
BC: E. coli, Klebsiella, Enterococcus (GRAM -)
T: ECRP with sphincterotomy/stone extraction and cholecystectomy
What is Endoscopic Retrograde Cholangiopancreatography (ERCP)?
- invasive but diagnostic/therapeutic
- measure INR prior to procedure and get pregnancy test
Complications: ACUTE PANCREATITIS
How are the following treated:
- Cholelithiasis
- Acute Cholecystitis
- Choledocholithiasis
- Cholangitis
- Primary Sclerosing Cholangitis
- monitor, elective cholecystectomy
- NPO, IV fluids, Abx, Surgery (urgent cholecystectomy)
- ERCP, laparoscopic cholecystectomy
- Urgent ERCP (stones removed)
- no satisfactory therapy, treat cholangitis as outlined above; possible liver transplant w/END-STAGE CIRRHOSIS
Biliary Dyskinesia
What is it, how does it present, and how can HIDA scans be used?
- symptomatic functional disorder of the gallbladder (unknown etiology)
C: RUQ pain (like biliary colic) with normal ultrasound (use ROME III criteria), normal liver enzymes/conjugated bilirubin/amylase/lipase
HIDA: radionucleotide ion - nuclear medicine Technetium Tc 99m
- Abnormal: no gallbladder seen (stone)
- cholecystokinin ejection fraction < 35-38%
- get cholecystectomy
What is an imaging finding of Chronic Cholecystitis, what is at an increased risk of developing, and when should surgery be used?
Porcelain Gallbladder: seen on plain X-Ray
- calcified lesions of gallbadder
- inc. risk of developing gallbladder cancer (POOR PROGNOSIS)
- use surgery if: symptomatic or if porcelain gallbladder is seen on imaging
Primary Sclerosing Cholangitis (PSC)
Who is it commonly seen in, how does it present clinically (P/J/O), what are diagnostic findings, and how is it treated?
- fibrosis of BILE DUCTS = “beads on a string”, usually Males with IBD (ulcerative colitis); dec. risk with smoking and coffee consumption
C: pruritis, jaundice, osteoporosis
D: ALP/bilirubin elevated, use ERCP/MRCP
T: no proven therapy; use symptomatic treatment or potential liver transplant
What are two findings upon ERCP and Liver biopsy of a patient with Primary Sclerosing Cholangitis?
What is a patient with PSC at an increased risk of developing?
ERCP: “beads on a string” due to fibrosis
Liver Bx: “onion skinning”
- pts are at inc. risk for CHOLANGIOCARCINOMA and inc. risk of colon cancer in patients with Ulcerative Colitis
What bilirubin levels are clinical jaundice seen at?
What are the normal lvls for conjugated and unconjugated?
- seen at bilirubin > 3 mg/dL (norm: 0.2-1.2 mg/dL)
Direct (cong): 0.1-0.3 mg/dL
Indirect (uncong): 0.2-0.9 mg/dL
What are common diseases associated with elevated Unconjugated (2) and Conjugated (4) bilirubin? (JAUNDICE)
Unconjugated
- Hemolytic Syndrome, Gilbert Syndrome
- also Crigler-Najjar Syndrome/Viral Hepatitis
Conjugated
- Dubin-Johnson Syndrome, Rotor Syndrome
- Drug Rxn and Pregnancy
Hemolytic Syndrome
What bilirubin is increased and what should you check if you believe your pt. has a hemolytic syndrome (H/R/L)
- inc. unconjugated hyperbilirubinemia (PREHEPATIC)
- order CBC to look for AMENIA/THROMBOCYTOPENIA
- also: haptoglobin, reticulocyte count, LDH
- check peripheral smear: shistocytes/sickle cell
Gilbert Syndrome
What bilirubin is increased, what activity is reduced, and how does fasting affect lab values?
- inc. unconjugated hyperbilirubinemia (PREHEPATIC)
- hereditary, benign, asymptomatic
- dec. activity of uridine diphosphate glucuronyl transferase
- 24-36 hour fast inc. hyperbilirubinemia
Dubin-Johnson Syndrome vs Rotor Syndrome
What bilirubin is increased?
DJS: dec. excretory function of hepatocytes
- benign hereditary jaundice, dark black liver
- gallbladder NOT seen on oral cholecystography
RS: dec. hepatic reuptake of bilirubin conjugates
- benign, hereditary jaundice, no liver pigmentation
- gallbladder SEEN on oral cholecystography
-inc. conjugated bilirubin
Intrahepatic Cholestasis of Pregnancy
What is it, what does it look like clinically, and what bilirubin is increased?
- caused by cholestasis
- benign jaundice occurring during THIRD trimester of pregnancy, with itching and GI symptoms
- inc. conjugated bilirubin
Acute (Fulminant) Liver Failure
What is it most commonly caused by, what are two major causes of death (CE/S), how does it present (4), and how can it be treated?
- massive hepatic necrosis with impaired consciousness (8 weeks of the onset of illness) usually due to ACETAMINOPHEN poisoning (2nd: idiosyncratic drugs)
CoD: cerebral edema and sepsis (80% w/deep comas)
P: encephalopathy –> deep coma; rapidly inc. bilirubin, markedly prolonged PT time (AST/ALT can be > 5000 in acetaminophen poisoning)
T: support pt (fluid, circulation, breathing), early transplant transfer, N-Acetylcysteine (NAC) for OD
Hepatitis A (HAV)
What is it, how is it transmitted, what labs does it have, and how is it treated?
- ssRNA that can cause aversion to smoking; usually ACUTE lasting up to 6 weeks (self-limited)
T: fecal-orally, usually during international travel
L: Markedly elevated AST/ALT, elevated bilirubin and ALP (cholestasis), detection of IgM anti-HAV is EXCELLENT TEST (IgG indicates previous exposure)
Treatment: HAV vaccine
Hepatitis B (HBV)
What is it, what is is commonly associated with (PN), how is it transmitted, and what labs does it have?
- partially dsDNA that can be associated with POLYARTERITIS NODOSA, glomerulonephritis
T: parenteral, sexual, perinatal, percutaneous (needle)
- Endemic in Africa/Southeast Asia
L: Markedly elevated AST/ALT (higher than HAV)
Treatment: HBV Vaccine
Hepatitis B (HBV)
What is the difference between Acute/Chronic and what are two complications of infection?
Acute: subsides in 2-3 weeks w/90% recovery
Chronic: 10% of acute progress
Risk: inc. risk of cirrhosis (especially with HDV superinfection) and hepatocellular carcinoma
What is the Window Period of HBV infection?
- time between HBsAg disappearance and HBsAb appearance (can be several weeks)
- patient still considered to have ACUTE HBV and infection is ONLY detectable with HBcAb IgM
important when screening blood donations
Hepatitis D (HDV)
What is it, when does it infect, what labs are associated with it, and how is it treated?
- defective RNA virus requiring HBV for replication (either coinfects or superinfects chronic HBV carrier)
L: HDV Ag and HDV RNA (PCR)
T: vaccinate against HBV (hepatitis B vaccine)
Hepatitis C (HCV)
What is it, how is it transmitted, what labs are associated with it, and how can it be treated?
- ssRNA virus that is most commonly CHRONIC that is transmitted by transfusion, IV drug use, and bloody fisticuffs
L: fluctuating AST/ALT levels, most sensitive indicator of infection is HCV RNA (also HCV Ab in serum)
- dec. in serum cholesterol
T: no vaccine; chronic disease should be vaccinated against HAV/HBV
Hepatitis C (HCV)
What are 4 complications of infection (C/HCC/HIV/MC) and how can it be screened (2)?
C: cirrhosis, hepatocellular carcinoma, HIV co-infection, mixed cryoglobulinemia
P: birth cohort screening for persons born between 1945-1965 (Baby Boomers) and new recs over 18 should have once in a lifetime HCV screening
Hepatitis E (HEV)
What is it, who is it commonly seen in (2), how is it transmitted, and what is its mortality?
- ssRNA herpevirus that is self-limited and is ACUTE with NO carrier state
- commonly seen in IMMUNOCOMPROMISED pts and in endemic Asia, Middle East, Africa, India, Central America (transmitted fecal-oral OR by swine)
- HIGH mortality rate in pregnant women specifically
What is a major dose-dependent hepatotoxin and what are two common idiosyncratic drugs indicated in Drug-induced Liver Injury?
HT: ACETAMINOPHEN, mushroom poisoning (48 hr onset)
I: isoniazid and sulfonamides (variable dose/time of onset)
Treatment: supportive, liver transplant if necessary, NAC for acetaminophen
What is Rumack-Matthew Nomogram?
- used for Tylenol/Acetaminophen overdose (treat with N-acetylcysteine or NAC)
- therapy should begin within 8 hours of ingestion; important to get a 4 hour acetaminophen level
- can help you determine how much toxicity the liver may experience based on the timing and amount of acetaminophen ingested
Budd-Chiari Syndrome
What is it, what are two common presentations of it, what is seen on imaging, and what complication can it lead to?
- occlusion of flow to hepatic vein or IVC causing caval webs, right-sided heart failure (NUTMEG LIVER) due to hypercoagulable states
I: occlusion/absence of flow in hepatic veins/inferior vena cava with PROMINENT CAUDATE LIVER LOBE
- use Contrast Enhanced color/pulsed Ultrasonography (CEUS)
- liver biopsy = centrilobular congestion (NUTMEG)
Comp: bleeding varices, hepatocellular carcinoma
What is the most common cause of jaundice in pregnancy?
VIRAL HEPATITIS
Hepatic Disease of Pregnancy
Pre-eclampsia (MH/P/PE/C)/eclampsia, Cholestasis of pregnancy, and Acute Fatty Liver of pregnancy
P/E: maternal hypertension, proteinuria, peripheral edema, coagulation abnormalities
- Eclampsia = w/hyperreflexia and convulsions
- life-threatening!
- also HELLP syndrome
CP: pruritus in the 3rd trimester, darkening of urine, light stools, jaundice
AFL: spectrum of disorders, can cause hepatic failure, coma, death in severe cases
