DSA: Approach to Hepatobiliary Patient - Chronic Liver Disease (McGowan) Flashcards
What are two associations with HBV and HCV infection, and what are two non-invasive ways to identify presence/absence of fibrosis in Chronic Hepatitis?
HBV: polyarteritis nodosa
HCV: mixed cryoglobulinemia
ID: serum FibroSure and US elastography
Chronic HBV vs Chronic HVC
HBV: endemic in Asia/sub-Saharan Africa
- 90% of infants with maternal transmission
- inc. risk in males, high chronicity with younger age
- asymptomatic healthy carrier state (75%)
HCV: cirrhosis inc. in males, heavily EtOH, tobacco, infection after 40 yrs
- coffee slows progression; HCC has high risk
- can have normal AST/ALT
- HCV Ab + HCV RNA needed for chronic diagnosis
Autoimmune Hepatitis
What is the difference between Type I and Type II, how does it clinically manifest, and what are diagnostic findings for each type?
How can it be treated?
Type I: classic, anti-SM, or antinuclear Ab (ANA)
- MOST COMMON
Type II: anti-liver/kidney microsomal Ab (anti-LKM)
CM: progressive jaundice, epistaxis, amenorrhea (healthy young female with stigmata of cirrhosis)
D: serum aminotransferase lvls may be > 1000, total bilirubin usually inc; Type I hypergammaglobulinemia/SM Ab (SMA), ANA; Type II anti-LMK Ab
treat with glucocorticoids
Alcohol Liver Disease
What is it, what does it cause, and how does it present?
- excessive alcohol causing fatty liver, alcoholic hepatitis, cirrhosis (exceeds 80g/day in males and 30-40 g/day in females x 10 yrs)
- causes Fatty Liver (Steatosis) = asymptomatic hepatomegaly and mild elevations in liver tests
P: anorexia, nausea, vomiting, fever, jaundice, tender hepatomegaly, RUQ pain
Alcohol Liver Disease
What labs are associated with it (4), what are 3 imaging options, and what is seen on liver biopsy?
L: AST 2x > ALT, bilirubin inc. (>10 mg/dL), anemia (usually macrocytic –> folic acid deficiency), marked prolongation of PROTHROMBIN time
I: US, CT w/intravenous contrast/MRI, US elastography
LBx: Mallory-Dank bodies (alcoholic hyaline)
Alcohol Liver Disease
How is it treated (non-severe vs severe) and what are 2 complications it can lead to (WE/KS)?
Tx: abstinence from alcohol, thiamine 100 mg, folic acid 1 mg, zinc
- give thiamine with or before glucose
- glucose can precipitate Wernicke-Korsakoff
- for severe (DF > 32, MELD > 21, GAW > 9)
- steroid and pentoxifylline
- liver transplant (abstain from EtOH for 6 months)
- can cause Wernicke Encephalopathy, Korsakoff Syndrome (permanent)
What are Wernicke Encephalopathy and Korsakoff Syndrome?
What 4 things is severe alcoholic hepatitis characterized by? (B/P/H/A)
WE: confusion, ataxia, abnormal eye movements
- treat with Thiamine
KS: severe memory issues, confabulation
SAH: total bilirubin > 8-10 mg/dL, PTT > 6 sec, hypoalbuminemia, azotemia
- critically ill = 30 day mortality of > 50%
What is the threshold for alcohol consumption in female and male patients with Non-alcoholic Fatty Liver Disease (NAFLD)?
Female: < 20 g EtOH/day
Male: < 30 g EtOH/day
Non-Alcoholic Fatty Liver Disease
What is it, what causes it, what labs are associated with it and how can it be viewed?
How is it treated?
- MOST COMMON cause of chronic liver disease in US
- caused by Metabolic Syndrome (obesity, DM, hypertriglyceridemia = inc. risk of disease)
L: mild AST/ALT elevation (can be normal in up to 80%)
I: Ultrasound elastography (assess fibrosis) and Liver Biopsy (diagnostic) –> NASH
T: lifestyle modifications, liver transplant (if fibrosis)
- physical activity and coffee protect against it
Alpha-1 Antitrypsin Deficiency
What is it, what two findings are diagnostic for it (A/P), how is it treated, and what are two complications of disease?
- AR defective a1-antitrypsin that accumulates in hepatocytes and causes liver damage (protease activity NOT inhibited)
MC inherited hepatic disorder in children/infants
D: low a1-antitrypsin, check phenotype, PiZZ genes
T: no smoking, liver transplant
C: emphysema at young age, micronodular cirrhosis (risk of HCC)
Primary Biliary Cholangitis
What is it, what are 4 common risk factors of disease (U/S/HR/HD), and how does it clinically present?
What molecule has isolated increase?
- chronic liver disease; autoimmune destruction of small intrahepatic bile ducts and cholestasis (F > 50 yo)
- asymptomatic isolated inc. in ALP
RF: UTI, smoking, hormone replacement, hair dye
C: pruritus, fatigue, progressive jaundice, XANTHELASMA; associated with other autoimmune disease
Primary Biliary Cholangitis
What are 3 diagnostic findings of disease (A/A/M), how is it treated (U), and what complication does it lead to?
D: AMA Abs (90-95%), inc. ALP, inc. IgM lvls (get liver biopsy if AMA (-))
T: ursodeoxycholic acid
C: cirrhosis –> liver failure
Hemochromatosis
What is it, what is the classic tetrad it presents with (C/SP/DM/HF), what are 4 diagnostic findings, and how can it be visualized (3)?
- AR HFE gene mutation leading to inc. iron absorption in the duodenum (hemosiderin in liver, pancreas, heart, testes, kidneys of MALES)
T: cirrhosis/hepatomegaly, skin pigment (bronze), diabetes mellitus, heart failure (cardiac dysfunction)
D: HFE gene mutation, inc. plasma iron w/> 45% transferrin, inc. serum ferritin, mildly abnormal liver test
V: MRI, CT, liver biopsy (is cirrhosis present)
Hemochromatosis
Who should get screening, how is disease treated/managed, and what 3 organisms are pts at inc. risk of infection from (VV/LM/YE)?
- screen all first-degree family members w/iron studies and HFE testing (evidence of iron overload)
T: phlebotomy therapy (deplete iron stores), avoid iron-rich food, monitor, PPIs, possible liver transplant
- if anemia/thalassemia = DEFEROXAMINE
- inc. risk of Vibrio vulnificus, Listeria monocytogenes, Yersinia enterocolitica
Wilsons Disease
What is it, how does it present, what are diagnostics of disease, and how can it be treated (OP/LT)?
- AR disorder of Chromosome 13 (ATP7B) in persons < 40 yo causing impaired copper excretion into bile and failure to incorporate in ceruloplasmin
- liver, brain, eye accumulations (also Hemo Anemia)
- excessive absorption and dec. excretion of copper
P: kids w/hepatitis, Coombs (-) hemolytic anemia, portal hypertension, hypersplenism, psychiatric abnormalities
D: Kayser-Fleischer rings, inc. urine copper, low serum ceruloplasmin, inc. hepatic copper, inc. copper in brain
T: oral penicillamine and liver transplant
How does Heart Failure affect the liver?
What molecular marker is elevated?
What is the hallmark finding if ischemia is involved?
- pts w/right heart failure cause passive congestion of the liver = “Nutmeg” liver –> ischemic hepatitis
- hepatojugular reflux is present and tricuspid regurgitation can cause pulsatile liver (jaundice in worse outcomes)
- marked elevated serum N-terminal-proBNP or BNP
Hallmark: “Shock Liver” - elevated serum aminotransferases > 5000 units/L and early rapid rise in LDH levels (ALP/bilirubin elevation is mild)
What is the peak time of incidence for Cirrhosis and what are the 3 most common causes?
What does cirrhosis due to the liver and what can help protect against development?
- peaks around ages 40-60
CC: Hepatitis C, alcoholic liver disease, NAFLD
- causes fibrosis that replaces normal liver and can lead to formation of regenerative nodules (destroys livers vascular and lobular architecture)
P: higher coffee and tea consumption
Liver Cirrhosis
What is Dupuytren’s Contracture and Muehrcke/Terry Nails?
DC: pts. ring and pinky finger are contracted on hand
Muehrcke lines: double white lines at base of nail
Terry nails: dark line at tip of nail
- both are problems associated with low albumin
- commonly seen in pts with cirrhosis
What is Abdominal Paracentesis used for?
What findings would lead you to find Portal Hypertension vs SBP?
- diagnostic and therapeutic procedure done on all pts with new onset ascites to determine cause (want to rule out Spontaneous Bacterial Peritonitis)
- check fluid lvls for albumin, WBC w/diff, culture and gram staining (due blood culture of fluid)
PH: SAAG > 1.1
SBP: > 250 PMNs/mL
What are two non-invasive predictive indices for hepatic fibrosis? (UE/F)
- Ultrasound Elastography
- Fibrosure (more specific serum diagnostics)
- non-invasive blood test for markers of fibrosis
- high = advanced fibrosis/low = no advanced fibrosis
What are 3 complications that liver cirrhosis can lead to?
Hepatocellular carcinoma, HIV co-infection, decompensated cirrhosis
How much acetaminophen should a patient with liver disease take to help manage cirrhosis?
What should be checked and how often to help screen for Hepatocellular Carcinoma in a pt. with cirrhosis?
- max 2 grams every 24 hours
- check Alpha fetoprotein (AFP) and ultrasound every 6 months for HCC screening
What would you use to treat Spontaneous Bacterial Peritonitis?
Ceftriaxone or cefotaxime
- consider IV albumin to help with renal perfusion pressure
MELD/MELD-Na (B/C/S/I) and Child-Turcotte-Pugh (B/A/P) Scoring for Cirrhosis
What do you order for each (4/3) and what scores are associated with worse prognosis?
M: order bilirubin, creatinine, sodium (check CMP), order INR, check for recent dialysis
- > 10 = worsening condition
- > 14 = needs to be on transplant list
CTP: order bilirubin, albumin (check CMP/hepatic function panel), order PT/INR, check for ascites/encephalopathy
- reported usually A, B, C w/C being the most severe
- > 7 indicates higher severity if numerical
Ascites
What is the most common cause, what are two physical exam findings seen in patients with it, and what laboratory testing should be done?
- MCC is PORTAL HYPERTENSION secondary to chronic liver disease (80%)
PE: asterixis secondary to encephalopathy may be present, “shifting dullness” - change in percussion based on position (need 1500 mL of fluid)
L: Abdominal US w/Doppler (vascular evaluation) and Abdominal Paracentesis
- check cell count (< 500 leukocytes, < 250 PMN)
- check albumin/total protein
- culture and Gram stain
What is a SAAG Score and what can it tell us?
SAAG = serum albumin - ascites albumin
- used to determine cause of ascites in pt.
SAAG > 1.1 g/dL
- portal hypertension, nonperitoneal cause of ascites
SAAG < 1.1 g/dL
- ascites caused by peritoneal cause
What are 5 conditions related to SAAG score of < 1.1 g/dL (BL/NS/P/PC/TB) and what are 6 conditions related to SAAG score of > 1.1 g/dL(C/BC/LM/C/IO/SO)?
SAAG < 1.1 g/dL
- biliary leak, nephrotic syndrome, pancreatitis
- peritoneal carcinomatosis, tuberculosis
SAAG > 1.1 g/dL
- cirrhosis, Budd-Chiari, massive liver metastasis
- CHF, IVC obstruction, sinusoidal obstruction
What is Hepatic Encephalopathy and what is it caused by?
- alteration in mental status in the presence of liver failure (ammonia lvls typically elevated, but do not correlate with severity of liver disease)
PE: asterixis (flapping tremor), confusion, slurred speech, being sleeping/difficult arousal
mild confusion –> drowsy –> stupor –> COMA
Hepatocellular Carcinoma
Who is most at risk, how is it screened, how is it diagnosed, and how can it be treated (S/A/TACE)?
RF: 50-60 yo M w/cirrhosis from Asia/Africa (AFLATOXIN EXPOSURE)
S: AFP/US every 6 months, Hep B vaccine
D: pt. w/liver disease with abnormality on ultrasound or rising alpha fetoprotein (AFP)
- also abnormal liver function tests/enzymes
T: surgical resection/transplant, radiofrequency ablation, transcatheter arterial embolization (TACE)
Liver Transplant
When should it be considered, what are considerations for pts. with alcoholism, and what is needed after transplant?
- considered in pts. with irreversible, progressive CLD, ACLF, and certain metabolic diseases
- check MELD/MELD-Na score
- > 14 should be on transplant listing
- pts. with alcoholism should abstain for 6 months
- need immunosuppression after transplant
