Clinical DSA 5: Hepatobiliary - Chronic Liver Disease and Cirrhosis Flashcards
Primary complication of chronic hepatitis
Cirrhosis => ascites, variceal bleeding, encephalopathy, coagulopathy, hypersplenism (thrombocytopenia)
Complications of chronic HBV infection
Cirrhosis
HCC
Polyarteritis nodosa
Who is at the highest risk of developing chronic HBV?
Babies who are born to HBsAG+ mothers have 90% risk of chronic HBV
*perinatal transmission
Complications of chronic HCV infection
Cirrhosis
HCC
Mixed cryoglobulinemia
HCV prevention
If HCV+ do not share razors or toothbrush and practice safe sex
*no HCV vaccine
Diagnosis?
Chronic, progressive liver disease
*Appears healthy with stigmata of cirrhosis
Association with other autoimmune disease
*ASMA+ or ANA+
Hypergammaglobulinemia
AST/ALT > 1000, hyperbili
*Female 30s-50s
Extrahepatic manifestations (rash, arthralgias, keratoconjuctivitis, thyroiditism hemolytic anemia, nephritis, UC)
AIH Type 1
Diagnosis?
Chronic, progressive liver disease
*Appears healthy with stigmata of cirrhosis
Association with other autoimmune disease
*Anti-liver/kidney microsomal Abs (anti-LKM)
AST/ALT > 1000, hyperbili
*Children
Extrahepatic manifestations (rash, arthralgias, keratoconjuctivitis, thyroiditism hemolytic anemia, nephritis, UC)
AIH Type 2
Treatment for AIH
Glucocorticoids
Azathioprine
Monitor LFTs
Possible liver transplant
Continuum of alcohol induced liver disease
Steaosis => Steatohepatitis => Cirrhosis
Diagnosis?
Asymptomatic hepatomegaly and mild elevations in ALP and bili
AST>ALT 2:1
CBC shows leukocytosis or leukopenia
Macrocytic anemia
Signs of liver failure (dec albumin, coagulopathy)
History of alcohol abuse
Fatty liver (steatosis) phase of alcohol induced liver disease
Diagnosis?
Asymptomatic progresses to signs of liver failure
AST>ALT 2:1
CBC shows leukocytosis or leukopenia
Macrocytic anemia
Signs of liver failure (dec albumin, coagulopathy)
History of alcohol abuse
Alcoholic hepatitis (steatohepatitis) phase of alcohol induced liver disease
Diagnostic imaging for alcohol induced liver disease
US to rule out gallstones
CT with IV contrast or MRI
US elastography (look for fibrosis)
Liver biopsy finding in alcoholic hepatits and non-alcoholic steatohepatitis (NASH)
Mallory-Denk bodies (alcoholic hyaline)
Alcohol induced liver disease treatment and management
Abstinance from alcohol Daily multivitimin Thiamine 100 mg Folic acid 1 mg Zinc
Special consideration of hypoclycemia management in alcohol induced liver disease
Administer glucose and thiamine at same time
otherwise may precipitate Wernicke-Korsakoff syndrome
Maddrey’s discriminant function (DF) is a prognostic indicator for _______________
calculated using __ and _______
Values >___ associated with poor prognosis and may benefit from steroids
Maddrey’s discriminant function (DF) is a prognostic indicator for alcohol induced liver disease
calculated using PT* and bilirubin*
Values >32* associated with poor prognosis
Glasgow Alcoholic Hepatitis score is a prognostic indicator for _______________
calculated using age, bili, BUN, PT, WBC count
Values >___ associated with poor prognosis and may benefit from glucocorticoids
Glasgow Alcoholic Hepatitis score is a prognostic indicator for alcohol induced liver disease
calculated using age, bili, BUN, PT, WBC count
Values >9* associated with poor prognosis
Model for End Stage Liver Disease (MELD/MELD-Na) score >___ is associated with significant mortality in alcoholic hepatitis
Model for End Stage Liver Disease (MELD/MELD-Na) score >21 is associated with significant mortality in alcoholic hepatitis
Treatment for severe alcoholic hepatitis (DF ≥ 32 or MELD >21 or GAW ≥ 9)
Steroids and pentoxifylline
Liver transplant requirement in alcohol induced liver disease
must abstain from alcohol for 6 months to be considered
Diagnosis?
Patient with alcoholic liver disease
Mental status changes
Ataxia
Involuntary eye movements
Wernicke encephalopathy
Tx with thiamine
Diagnosis?
Patient with alcoholic liver disease
Severe memory issues
Confabulations/make-up stories
Korsakoff syndrome (permanent)
Diagnosis?
Asymptomatic or mild RUQ discomfort
Hepatomegaly
Metabolic syndrome***
Insignificant alcohol history
NAFLD
Prevention of NAFLD
Regular physical activity and coffee consumption
Treatment for NAFLD
Lifestyle modification (diet, exercise, vit E, gastric bypass)
Liver transplant in advanced cirrhosis
Diagnosis of NAFLD
Liver biopsy is diagnostic
Predominantly shows macrovesicular steatosis, no inflammation or fibrosis, Mallory-Denk bodies
*NASH and alcoholic steatohepatitis indistinguishable histologically
Diagnosis?
Liver disease and panacinar emphysema
Most commonly presents in children
alpha 1 antitrypsin deficiency
Alpha 1 antitrypsin deficiency pathogenesis
Defective a1-AT gene => misfolded protein
- Misfolded protein accumulates in liver leading to liver damage
- Decreased functional a1-AT leads to disinhibition of proteases leading to pulmonary emphysema
a1-AT is a protein that inactivates proteases
Adult smoker presentation of a1-AT deficiency
Liver disease
COPD/emphysema of upper lobes
*tx is smoking cessation
Child presentation of a1-AT deficiency
Liver disease
Panacinar emphysema of lower lobes
*tx is liver transplant in severe cases (most common cause of child liver transplant)
a1-AT phenotypes
PiZ or PiZZ have severe deficiencies
PiS have mild deficiency
Pim is phenotypically normal
Pi gene on c14
Complications of a1-AT deficiency
Emphysema at young age
Micronodular cirrhosis => HCC risk
Diagnosis?
Cirrhosis with hepatomegaly Bronze skin Diabetes mellitus Cardiac dysfunction >45% transferrin saturation
HFE gene mutation
Increased iron absorption in duodenum
Hemosiderin (iron) deposits in organs
Hemochromatosis
Progression of hemochromatosis
Early: nonspecific symptoms (fatigue, arthralgia)
Later: Cirrhosis with hepatomegaly, Bronze skin, Diabetes mellitus, Cardiac dysfunction, arthropathy
Hemochromatosis treatments
Phlebotomy
In hemochromatosis due to transfusions use Deferoxamine (chelating agent)
Hemochromotosis complications
Cirrhosis, diabetes mellitus, heart failure
Infection risk for siderophilic organisms (Y. enterocolitica, V. vulnificus, L. monocytogenes)
Hemochromotosis screening
Test for HFE mutation in any patient with iron overload
Iron studies and HFE testing in all first degree family members
Diagnosis?
Child or young adult (<40) presents with neurologic and psychiatric problems
Kayser-Fleischer rings
Low serum ceruloplasmin
Coombs - hemolytic anemia
Copper accumulation in liver, brain, eyes
ATP7B mutation
Wilson disease
AR disorder, impaired copper excretion into bile and failure to incorporate copper into ceruloplasmin
Wilson disease treatment
Oral penicillamine (increases urinary excretion of chelated copper)
Liver transplant in severe case (cirrhosis)
Diagnosis?
Elevated AST/ALT
Elevated BNP
Hepatojugular reflux is present
Nutmeg liver
Right heart failure
Diagnosis?
Rapid and striking elevation in ALT/AST over 5000
Rapid rise in LDH
Ischemic hepatitis (shock liver)
*caused by acute MI, arrhythmia, septic/hemorrhagic shock
Initial imaging study for cirrhosis
RUQ US + Doppler of hepatic/portal veins
Complications of cirrhosis
Risk of HCC
Portal HTN
Survival prognostic scores for cirrhosis
Child-Turcotte-Pugh score (A,B,C with C being most severe)
MELD/MELD-Na score (>10 is worsening, >14 for transplant list)
Tests to order for MELD/MELD-Na score
Bili Creatinine Na INR Dialysis recently?
Tests to order for Child-Turcotte-Pugh score
Bili
Albumin
PT/INR
PE for encephalopathy and ascites
Screening for HCC in cirrhosis pts
alpha fetoprotein and US every 6 months
Diagnosis?
Abdominal distension in chronic liver disease, portal HTN
Abdominal US shows fluid in peritoneum
Shifting dullness on percussion (requires 1500 mL of fluid)
Ascites
SAAG score
serum albumin - ascites fluid albumin
> 1.1 indicates ascites due to portal HTN
Who gets paracentesis?
Pt with new onset ascites
Pt admitted to hospital with known cirrhosis and ascites
Pt with known cirrhosis and ascites deteriorates quickly
How to diagnose spontaneous bacterial peritonitis
Paracentesis of ascites fluid
WBC with diff of fluid
PMN (total neutrophils) >250 = neutrocytic ascites
highly sus
Treatment and prevention of spontaneous bacterial peritonitis (SBP)
Abx (prophylactic or treatment)
Prevention of esophageal varicieal bleeding/rebleeding
beta blockers
band ligation (30% chance of rebleed)
Treatment for bleeding esophageal varicies
Resuscitation in ICU
Emergent EGD with banding
IVF +/- blood products
Correct coagulopathy
Abx, octreotide, TIPS, liver transplant
Grades of hepatic encephalopathy
- mild confusion
- drosiness
- stupor
- coma
Diagnosis?
Alteration of mental status in the presence of liver failure
Elevated ammonia (no correlation of amount to severity of mental status change)
GI bleeding
Constipation
Hepatic encephalopathy
Treatment of hepatic encephalopathy
Lactulose (produce 2-3 soft stools per day)
acidifies colon and traps ammonium for excretion
Diagnosis?
Cirrhosis
Cachexia, abdominal pain, fever, jaundice
Abnormal LFTs
Abnormal US or rising alpha fetoprotein
HCC