Liver Flashcards
Adenovirus, Influenza, Ebola, Zika, EBV, CMV, HSV, SARS-CoV-2 (COVID) and Parvovirus all have this in common.
ALL can cause significant liver injury and jaundice however 80% of adults have been exposed to EBV and CMV and have immunity in the form of IgG and therefore infections affecting the liver with these viruses would be uncommon UNLESS IMMUNOCOMPROMISED (HIV, chemotherapy, transplant) or YOUNG individuals (EBV - mononucleosis).
Which VIRUS is the most COMMONLY REACTIVATED virus in IMMUNOCOMPROMISED patients that affects the liver?
CMV
What is the MOST COMMON cause of ACUTE HEPATITIS in the USA?
HBV
Which viruses CAN be transmitted from person to person via “CASUAL CONTACT”?
HAV, Adenovirus, Influenza, Ebola, Zika, EBV, CMV, HSV and Parvovirus
Modern-day blood transfusions carry the risk of infection with which VIRUSES that can cause acute hepatitis?
Adenovirus, Influenza, Ebola, Zika, EBV, CMV, HSV and Parvovirus (NOT HAV,HBV, HCV, etc.)
HOMOSEXUAL activity can bring about the transmission of which HEPATITIS VIRUSES?
HAV, HBV (HCV is rare)
HETEROSEXUAL activity can bring about the trnasmission of which HEPATITIS VIRUSES?
HBV (HVA is rare, HCV is very rare)
Which HEPATITIS VIRUSES can be trnasmitted VERTICALLY?
HBV, CMV, HSV (HCV is very rare)
During this PHASE of clinical HEPATITIS a patient has NO SYMPTOMS and is POSITIVE serologically?
INCUBATION PHASE (phase 1) from entry of virus to presence of first symptoms.
What is the LENGTH of the INCUBATION phase for HAV, HBV, HCV and HEV?
HAV: 2-6 WEEKS
HBV: 2-6 MONTHS
HCV: 2-20 WEEKS
HEV: 2-9 WEEKS
Flu-like symptoms (myalgias, arthralgias, fatigue, loss of appetite, nasuea and vomiting, loss of sense of smell and taste) and mild tenderness over the liver for 3-5 days after INFECTION with HAV, HBV or HCV?
PRODROME PHASE
What are the 4 PHASES of ACUTE HEPATITIS INFECTION?
- INCUBATION PHASE (asymptomatic but serologically positive) weeks to months.
- PRODROME PHASE (flu-like illness) 3-5 days.
- ICTERIC PHASE (total bilirubin >2.5 mg/dL and fatigue - <25% pts) several weeks.
- RESOLUTION PHASE (symptoms resolve, LFTs normalize, Ab’s present).
This is a SERIOUS complication that is seen with HAV, HBV, HDV and HEV, (NOT WITH HCV) in older adults and those with CHRONIC LIVER DISEASE, is 50% FATAL and if diagnosed, MUST BE REFERRED to a liver TRANSPLANT CENTER.
ACUTE LIVER FAILURE (ALF)
PROGRESSIVE worsening of JAUNDICE, COAGULOPATHY and MENTAL STATUS CHANGES in a patient with ACUTE HEPATITIS INFECTION?
ACUTE LIVER FAILURE (ALF) - 50% fatal - refer to transplant center ASAP.
PREGNANT WOMEN, ELDERY, those with CHRONIC LIVER DISEASE when contracting HAV, HBV or HEV (preg women) are at an INCREASED RISK of what?
ACUTE LIVER FAILURE (ALF)
What is the central theme driving the EXTRAHEPATIC MANIFESTATIONS of viral hepatitis (even after the virus has resolved)?
CRYOGLOBULINEMIA
Unlike as seen with ACUTE infection, anti-HBVcore IgM can be positive in what other scenario?
Inactive chronic HBV FLARE
WHAT is a prime TRIGGER for transient lower esophageal sphincter relaxation (TLESR), the most frequent mechanism for GERD in a patient who’s symptoms occur following the evening meal, and the pH monitoring demonstrates clustering of reflux episodes around postprandial periods?
Following a meal, PROXIMAL GASTRIC DISTENTION can cause increased TLESR frequency and is an important mechanism for persisting reflux symptoms.
In the post prandial state, gastric acid produced by the stomach layers on top of the ingested meal. This pool of acidic gastric content, termed an ACID POCKET, is in close proximity to the GEJ, and can extend into a hiatus hernia or even the distal most part of the esophagus. Therefore, when an acid pocket exists, what occurs during a transient LES relaxation?
The VOLUME of acidic reflux is INCREASED during a transient LES relaxation (TLESR).
In what way does a HIATUS HERNIA and GASTRIC FUNDIC DISTENTION INFLUENCE the transient LES relaxation (TLESR)?
These can INCREASE TLESR frequency (thereby increasing reflux and related GERD symptoms).
Cryoglobulinemia (HBV, HCV, HEV) has which common positive serologic factor?
Rheumatoid Factor (always positive)
Besides in an acute or resolving infection with HBV, when else can HBV IgM antibodies be positive?
HBV Reactivation
PAS-positive, diastase-resistant globules in periportal hepatocytes represent what?
The hallmark of Z-type α1-AT
Patient with positive HBsAg, HBeAg, positive viral load but asymptomatic and normal LFTs?
Immune Tolerant Phase
Alternative therapy for AIH when pt has reaction to prednisone or azathioprine (pancreatitis)
Mycophenolate Mofetil
Hepatic Caudate Lobe Hypertrophy
Feature associated with Budd Chiari Syndrome
What is used for intrahepatic cholestasis of pregnancy?
Ursodeoxicholic acid
Pt s/p transplant with elevated LFTs & INR in the thousands and Doppler shows no flow through the hepatic artery.
List for re-transplant status 1A (highest priority)
C282Y homozygote
Highest risk genetic genotype to develop severe iron overload in hereditary hemochromocytosis
In vertical transmission risk of HBV, what is the most significant factor?
HBVe AG positivity
Trip to India, otherwise healthy, LFTs in the thousands, pregnant, HAV, HBV, HCV neg?
HEV
Vinyl Chloride causes what disease with what feared complication? How is it treated?
Hepatic Angiosarcoma - local excision/TACE
Best treatment for patient with HepB and hepatocellular carcinoma with one to two lesions, well circumscribed without vascular invasion or mets?
Liver transplant.
What is the BEST predictor of survival in Primary Biliary Cirrhosis (AMA+)?
Total BILIRUBIN level
Recommended DIET for patients with chylous ascites?
HIGH protein, LOW fat and rich in MEDIUM chain triglycerides (long-chain triglycerides are absorbed by the lymphatics)
Maximum recommended dose of CARVEDILOL for patients with esophageal varices?
12.5 mg Daily
What parameter is monitored to determine need for phlebotomy in a patient with hereditary hemochromatosis?
FERRITIN 50-100 ug/L
What SLOWS progression of liver injury in patients with cystic fibrosis?
Ursodeoxycholic acid
What is the best way to treat a pregnant mother who is HepB positive in order to minimize risk of transmission?
Tenofovir (give child immune globulin and vaccine upon birth)
Patient transplanted for HepC cirrhosis has elevated liver enzymes and obviously HepC if not treated, they are on steroids and tacrolimus. What is the next step if US is normal as far as ducts and vascular?
Liver biopsy
What is the indication to start PROPHYLAXIS in a patient with cirrhosis, no SBP and PHG with oozing of blood?
The GI bleed
A patient needs treatment with chemotherapy and steroids for a cancer such as leukemia in the setting of dormant or treated HepB. What is essential to do?
Receive PROPHYLAXIS against HepB reverse seroconversion throughout his chemotherapy and for 12-18 months thereafter.
Patient with alcohol/HCV cirrhosis, well compensated, presents with liver US showing a 1.6 cm mass. What’s the next step?
TRIPHASIC CT of the liver
anti-HBcore IgM can be positive in ACUTE HBV infection and when else?
With REACTIVATION and FLARE of HBV in patients with inactive, chronic HBV
What is the significance of the anti-HAV IgG and anti-HBVsurface antibodies?
These are the ONLY antibodies that actually confer IMMUNITY against future infection
A small portion of people (>50, chronic liver disease) infected with this HEPATITIS virus can develop ACUTE LIVER FAILURE and require LIVER TRANSPLANT. This virus is transmitted casually from person to person mainly via STOOL contamination but can be through saliva and blood contact?
HAV
What can occurr in 3-20% of patients with HAV?
Have a RELAPSE
Asians, Blacks, Vertical Transmission, which HEPATITIS virus?
HBV
What confers RISK of HCC in patients with HBV?
HBV DNA (viral load)
in Immune Tolerant Phase (>1,000,000)
in Chronic Active Phase (>20,000)
in Chronic Inactive Phase (<20,000)
What does E-antigen Negative chronic HBV mean?
Inactive HBV with positive E-antibody
In what TWO scenarios can a patient with HBV have E-antigen NEGATIVE?
Inactive Chronic HBV with E-abtibody POSITIVE
and
When there is a E-antigen PRE-CORE mutation in ACTIVE HBV
Which HBV patients CANNOT develop the INACTIVE HBV phase?
Those with an E-antigen PRE CORE mutation as the cannot develop the E-antibody
Can mothers with CHRONIC HCV (80% of people with HCV) breastfeed?
YES
When HCV and HBV coexist, what can happen when HCV is treated and eradicated as these two viruses compete?
Patient can have an HBV FLARE
This HEPATITIS VIRUS is almost EXCLUSIVELY contracted via IVDA and what is HIGH-RISK about these patients?
HDV, can only occurr with HBV and HBV-HDV infection can cause Acute Liver Failure
What is the RISK when a PREGNANT woman acquires acute HEV infection?
Acute Liver Failure
The presence of anti-HBVsurface (Ab) means what?
Person has been VACCINATED against HBV
Patients at risk for contracting HBV should be given what if they were vaccinated many years ago?
What should they be checked for?
What should be done if after being checked, the result is suboptimal?
HBV BOOSTER (one dose of the HBV vaccine)
Response to mounting an immunity against the BOOSTER
If poor or no response, give HBV IVIG and the remaining two vaccines (1mo, 1 mo, then 6 mo)
Which patients with HBV (phase) should be treated?
ONLY those with ACTIVE HBV (immune tolerant or inactive should be monitored) - this includes CHRONIC ACTIVE and ALL CIRRHOSIS (active or inactive)
HBV DNA >2,000-20,000 IU/mL REGARDLESS of LFTs
ACTIVE HBV
What is the GOAL of HBV treatment?
SEROCONVERSION (absence of the HBE-antigen and formation of the anti-HBE)
How do you treat HBE-antigen NEGATIVE patients (pre-core mutation)?
Suppress HBV DNA long term as they CANNOT seroconvert to inactive HBV
In which patients and why is PEG-INF not recommended?
In patients with CIRRHOSIS, because it can cause a FLARE and acute liver decompensation
Regardless of E-antigen status (positive or negative), which agents are used in treating CHRONIC ACTIVE HBV and HOW do they work?
ORAL antivirals (TENOFOVIR and ENTECAVIR) and these work by suppressing DNA replication resulting in undetectable viral loads.
WHEN CAN the ORAL antivirals (TENOFOVIR and ENTECAVIR) be stopped when treating HBV?
ONLY when SEROCONVERSION occurs (anti-HBE forms and HBE-antigen dissappears), otherwise they must be used long-term
How are patients with BOTH CHRONIC INACTIVE HBV and CIRRHOSIS treated?
ONLY with ORAL antivirals (TENOFOVIR or ENTECAVIR) and CANNOT use PEG-INF (causes flare)
When should HBV treatment be initiated in a PREGNANT patient to avoid VERTICAL trnasmission?
During the THIRD TRIMESTER if HBV DNA viral load is >200,000 IU/mL
In patients with INACTIVE HBV, in order to PREVENT a FLAIR during treatments such as CHEMOTHERAPY, anti-TNF agents (Crohn’s, UC, etc.) and HIGH-DOSE STEROIDS, what must be used as PROPHYLAXIS?
ORAL antiviral agents (TENOFOVIR or ENTECAVIR) at the time of INITIATION of treatment and for 6 MONTHS after.
What does it mean when a patient has anti-HBcore (Ab) and anti-HBsurface (Ab)?
They have RESOLVED HBV infection and are NOT at risk to develop HBV reactivation wehn immunosuppressed.
What constitutes a Sustained Virological Response (SVR) to HCV treatment?
UNDETECTABLE HCV RNA at 12-24 WEEKS and is synnonimous to being CURED
The treatment of HCV requires how MANY agents to be used and why?
At least TWO agents because one has to be either a PROTEASE INHIBITOR (NS3-4) or a POLYMERASE INHIBITOR (NS5B) and one has to be a NS5A INHIBITOR (replication complex)
In patients with HCV genotype 1A, what can occurr when using ORAL antiviral agents and what can be done?
Can develop NS5A resistance and using GRZ-EBV therapy needs to be augmented with RIBAVIRIN (RBV) and extending therapy from 12 weeks to 16 weeks
Why are SOF-LDV and SOF-VEL+/-RBV the ONLY combination of ORAL antiviral therapies possible for patients with DECOPENSATED CIRRHOSIS (CHILD CLASS B)
Because PROTEASE INHIBITORS (GRZ, VOX) do not work in CIRRHOSIS
Which HCV oral antiviral agent CANNOT be used in patients with RENAL FAILURE (GFR <30) and which combination IS USED in these patients?
SOF CANNOT be used (polymerase inhibitors)
Use GRZ-EBV in RENAL patients
BEFORE initiating therapy for HCV, what MUST be tested for first and why?
HBV CO-INFECTION, because it can cause HBV FLAIR and liver decompensation (test for HBsurface antigen, anti-HBcore and anti-HBsurface)
How do you treat the HBV-HCV co-infected patient whether the HBV is ACTIVE or INACTIVE?
MUST treat HBV with oral antiviral agents (TENOFOVIR or ENTECAVIR) up to 6 months after resolution of HCV as well as with HCV antivirals
What is developed when patients acquire HBV infection and what constitutes resolution of HBV?
ALL patients who contract HBV develop HBsurface antigen. When HBV is cleared and cured, HBsurface antigen is lost and if long enough after, so is the anti-HBsurface antibody.
A patient with ISOLATED anti-HBcore means what?
It means that they HAD been infected with HBV in the past and RESOLVED or were NEVER INFECTED
ALL patients with CHRONIC HBV have the pesistence of WHAT?
HBsurface antigen
A PREGNANT woman has HBV and has DNA >200,000 when should she start treatment?
During her THIRD trimester and for ONE MONTH post delivery will virtually ELIMINATE the risk for vertical transmission to her newborn
HOW LONG after SEROCONVERSION (anti-HBE) can oral (DAA) agents be stopped?
After 6 MONTHS
For patients with chronic HBV who are HBE-antigen negative (cannot seroconvert), how long is oral antiviral therapy (DAA)?
LIFE-LONG
Which patients with HCV do not require ANY monitoring for HBV?
Those with anti-HBsurface (these patients RESOLVED their HBV)
ALT >10,000 IU/I, profound jaundice (bilirubin >10 mg/dL), elevated INR, hepatic ENCEPHALOPAHTY?
ACUTE LIVER FAILURE (>80% mortality without transplant)
In a patient with HBV infection (was HBsurface antigen positive, anti-HBcore IgM) what does it mean when they become HBsurface antigen negative but are still anti-HBsurface antibody negative?
They are in the WINDOW period and will become anti-HBsurface antibody positive confering SPONTANEOUS RESOLUTION
PERSISTANCE of HBsurface antigen means?
CHRONIC active HBV infection
anti-HBcore, HBsurface-antigen, HBE-antigen, NORMAL ALT, VERY HIGH HBV DNA, NO INFLAMMATION, minimal fibrosis?
HBV IMMUNE TOLERANT state, NO TREATMENT NEEDED
At what DNA load is the reisk for development of CIRRHOSIS or HCC the greatest?
>20,000 IU/mL
What is the DIFFERENCE with respect to RISK of HCC in patients with HBV and those with HCV?
Only patients with CIRRHOSIS and HCV will develop HCC, whereas in HBV infection, patients with cirrhosis are more likely to develop HCC but those WITHOUT still CAN
How do you treat NEWBORNS to mothers who have HBsurface-antigen?
HBIG + HBV VACCINE within 12 HOURS of BIRTH
What AGENT is used to prevent VERTICAL transmission of HBV if at the THIRD TRIMESTER mother’s HBV DNA is >200,000?
TELBIVUDINE (start third trimester and for ONE MONTH after birth)
Which formulation of TENOFOVIR is best (less toxicity, less drug, better delivery)?
Tenofovir ALAFENAMIDE
When are the TWO occassions in which anti-HBcore IgM is POSITIVE?
ACUTE HBV infection and HBV REACTIVATION (chemotherapy, imunosuppression)
If a patient is found to have HBsurface-antigen, what else MUST you test for?
HBV DNA, HBE-antigen and anti-HBE
PRIOR to treating HCV in a patient, WHAT must you test for?
Presence of HBV (HBsurface-antigen, anti-HBsurface and anti-HBcore)
INH and HALOTHANE can cause Drug Induced Liver Injury (DILI) mostly in whom?
Persons >60 yo
Which patients are at most risk of ALF due to Valproic Acid?
CHILDREN
Anesthetic agents, INH (isonioazid), Acetaminophen, Cocaine, Vitamin A and Methotrexate can all cause an increased risk of DILI in patients that do what?
Consume ALCOHOL regulary
Is the risk for DILI elevated in patients with CIRRHOSIS?
NO
Erythromycin, Ketoconazole, Fluconazole, Cimetadine and Quinidine are significant in hepatic metabolism because?
INHIBIT CYP (cytochrome P450) and REDUCE hepatic metabolism of other drugs
Carbon Tetrachloride and Amanita Mushrooms
Intrinsic Hepatotoxicity (centrolobular necrosis)
What is significant about the METABOLITES of ACETAMINOPHEN and INH?
They are HEPATOTOXIC
Phenytoin, Sulfonamides, Dapsone and Halothane can all cause what type of hepatotoxicity?
Idiosyncratic hypersensitivity (differs in each person) and an ALLERGIC reaction (eosinophillia, fever, rash)
Up to 3X ASYMPTOMATIC elevation in AST/ALT and ALK PHOS is considered what type of DILI?
MILD DILI, no need to stop the medications
What is it called when LFTs are raised WITHOUT JAUNDICE and can stay elevated up to 6 MONTHS after starting a medicaion and only require MONITORING?
ADAPTATION
What can occurr in patients with 5X LFTs and JAUNDICE after starting a MEDICATION?
Acute Liver Failure (ALF)
What is the TYPICAL time frame for a drug to cause DILI from when it is INITIATED?
1-3 MONTHS
Which DRUG CLASS is the most common cause of DILI and which medication in particular?
ANTIBIOTICS, AUGMENTIN
PORTAL HTN and STEATOSIS with elevated LFTs can be caused by these medications?
HAART (all antiretrovirals)
The initiation of which MEDICATION requires a LIVER BIOPSY to check for FIBROSIS and REPEAT biopsy after evey 2g increase or every 2 years? (causes STAGE 1 steatosis, STAGE 2 portal fibrosis, STAGE 3 bridging fibrosis and STAGE 4 cirrhosis)
METHOTREXATE (psoriasis, cancers, etc.)
What is one of the most COMMON adverse effects of CHECK POINT INHIBITORS (CHI)?
HEPATOTOXICITY
What causes the metabolism of ACETAMINOPHEN in the liver to be ramped up thereby causing rapid buildup of the toxic metabolite NAPQI which depletes GLUTATHIONE and causes hepatotoxicity?
Regular ALCOHOL consumption (2-4 drinks per day), PHENYTOIN and RIFAMPIN
Which medications should NOT BE USED in patients with CIRRHOSIS as they can cause bleeding due to exacerbated renal insufficiency and thrombocytopenia?
NSAIDS
What medication should NOT BE USED in treatment of DILI?
STEROIDS (only use N-acetylcysteine)
Is ACETAMINOPHEN safe to use in patients with CIRRHOSIS?
YES, very (less likely to cause hepatotoxicity in these patients due to poor metabolism)
What is a PHASE-I reaction when talking about LIVER METABOLISM of drugs? What is PHASE-II?
The creation of an ACTIVE METABOLITE, phase-II is detoxifocation of the metabolite.
What constitutes 3X elevation of AST, ALT and ALP? 5X?
3X AST - 120
3X ALT - 170
3X ALP - 441
5X AST - 200
5X ALT - 280
5X ALP - 735
What underlying liver issue can commonly cause SLOW ADAPTATION of drugs with elevated liver enzymes such as with STATINS?
NAFLD
What is the COMPONENT of DILI (3X ULN of LFTs to 5X ULN of LFTs) that determines the risk of development of ALF?
Presence of JAUNDICE (bilirubin >2.5)
What is the most COMMON type of HEPATIC INJUY with DILI and what is mostly elevated?
CHOLESTASIS (elevated ALP) and JAUNDICE
Pt took ACETAMINOPHEN, 10-15g and presented >24 hours after with elevated INR and signs of HEPATIC ENCEPHALOPATHY, what do you do?
Immediately give N-acetylcysteine and transfer to LIVER TRANSPLANT CENTER
What are the THREE main IMMUNE LIVER DISEASES?
AutoImmuneHepatitis (AIH) - immune response directed agaisnt HEPATOCYTES
PrimaryBiliaryCholangitis (PBC) - immune against SMALL bile ducts
PrimarySclerosingCholangitis (PSC) - immune against LARGE bile ducts
What are the COMMON serologic markers for AutoimmuneLiverDisease (ALD)?
anti-Nuclear Ab (ANA), anti-Smooth Muscle Ab (ASMA) - AIH
anti-Mitochondrial Ab (AMA) - PBC
anti-Nuclear Cytoplasmic Ab (ANCA) - PSC
What does it mean when a patient has POSITIVE ASMA or AMA, or ANCA but no evidence of any liver disease?
It means that they ae GENETICALLY PREDISPOSED to AIH, or PBC or PSC but have not yet bee TRIGGERED to express the immune disease
What serological marker is ELEVATED in a patient with AUTOIMMUNE LIVER DISEASE and the higher it is the more rapid progreession to CIRRHOSIS?
IgG (levels correlate with histologic severity)
What are the liver injury patterns seen in AIH, PBC and PSC?
AIH - elevated AST and ALT (when severe, ALP can also be elevated) - hepatocellular
PBC - elevated ALP (when severe, AST and ALT can also be elevated) - cholestatic
PSC - elevated AST, ALT and ALP (MIXED)
What is REQUIRED BEFORE initiating THERAPY for all patients with AIH or PBC?
LIVER BIOPSY
Loss of liver function, Coagulopathy and Hepatic Encephalopathy are ALL indicative of what process?
Acute Liver Failure (ALF)
What is the GREATEST RISK of treating ALF with IMMUNOSUPPRESSION?
INFECTION
What are the indications to NOT TREAT ALF with immunosuppression (risk of infection) and proceed DIRECTLY to TRANSPLANT to avoid delay?
HEPATIC ENCEPHALOPATHY and MELD >25
Patient with CHRONIC AIH (normal LFTs, NO INFLAMMATION on liver biopsy) with or without cryptogenic CIRRHOSIS get treated how?
NO TREATMENT necessary without inflammation on liver biopsy.
What MARKERS are positive in TYPE 1 AIH, TYPE 2 and 3?
TYPE 1 - ANA, ASMA - USA
TYPE 2 - anti-LKM (liver, Kidney, Microsomal) - Southern Europe - more agressive
TYPE 3 - NO POSITIVE MARKERS
Portal Tract Infmallation (WITHOUT bile duct injury), Interface Hepatitis, Piecemeal Necrosis, some Eosinophils and Lobular Inflammation are ALL histologic factors associated with what?
AutoImmuneHepatitis (AIH)
What is the MAINSTAY treatment of AIH?
Prednisone + Azathioprine (immunosupression)
At what DOSE of PREDNISONE should this NOT be tapered further until LFTs have NORMALIZED?
At 20 mg (maintained at 5-10 mg for 6 months once LFTs are normal)
What can be used to treat patients with AIH if they do not RESPOND or TOLERATE Prednisone + Azathioprine?
Cyclosporine, Tacrolimus or Mycophenolate Mofetil
What can be used INSTEAD to TREAT AIH if a patient cannot tolerate prednisone?
BUDESONIDE
What is the GREATEST RISK for women who become PREGNANT while CIRRHOTIC?
BLEED from Esophageal VARICES
In pregant women with AIH, do PREDNISONE and Azathioprine need to be stopped?
NO
Asymptomatic or PRURITUS or INTERMITTENT JAUNDICE and associated with HLA-DRW8?
Primary Billiary Cholangitis (PBC)
What is observed with PBC in advanced disease that is similarly seen in Wilson’s Disease?
Elevated SERUM and URINARY copper (causes renal disease)
Xanthelasma caused by HYPERCHOLESTEROLEMIA (without cardiovascular effect) is seen in which liver disease associated with CHOLESTASIS and treatment for the cholesterol is not necessary?
PBC
PRURITUS and Osteoporosis are the presenting complaints of this disease process?
PBC
What is the time at which patients with PBC should be considered for liver transplantation?
When BILIRUBIN is >10 (50% 2-year mortality risk)
What are the TWO available treatments for PBC?
URSODEOXYCHOLIC ACID and OBETICHOLIC ACID
In patients who cannot tolerate URSO for the treatment of PRURITUS in PBC, what else is available that works very well?
RIFAMPIN (Naltrexone and cholestyramine also can help)
Due to CHOLESTASIS, ALL patients with PBC should receive what supplements?
Vitamins A, D E and K as well as CALCIUM + Vit D and ESTROGEN (if post-menopausal) for osteoporosis
Which part of HYPERCHOLESTEROLEMIA is elevated in patients with PBC and thus does NOT require treatment?
HDL
Which is more prevalent in FEMALES and MALES, PBC, PSC?
PBC - FEMALES (95%)
PSC - MALES (75%)
Which LIVER disease process is HIGHLY associated with IBD (Ulcerative Colitis, much less Crohn’s)?
PSC
BEADED BILE DUCT appearance, with multiple strictures with normal intervening segments of bile duct, BEST seen on MRCP (cholangioscopy) and is often POSITIVE for ANCA and NEGATIVE for AMA?
PSC
What test can cause CHOLANGITIS and should NOT be performed DIAGNOSTICALLY for a patient with PSC?
ERCP
What is the SINGLE highest risk factor for CHOLANGIOCARCINOMA (elevated CA 19-9 and CEA)?
PSC
What is the ONLY effective treatment for PSC?
LIVER TRANSPLANTATION
What should be done in elderly patients diagnosed with MILD AIH (low stage elastography, mild biopsy findings)?
OBSERVATION only (takes decades to advance), do NOT treat MILD AIH with DIABETES by giving prednisone and azathioprine is NOT used alone to INITIATE therapy
If URSO does not aleviate symptoms of PBC (pruritus, elevated ALP), what should be ADDED?
OBETICHOLIC ACID
Which AUTOIMMUNE disorders are MOST commonly seen with PBC?
Sjogren’s, RA, Thyroid Disease
Which AUTOIMMUNE disorders are most commonly seen with PSC?
ULCERATIVE COLITIS and Crhon’s
What is the TYPICAL HISTOLOGY noted in AIH?
PIECEMEAL NECROSIS
Why do patients with PBC have HYPERCHOLESTEROLEMIA?
Because BILE is the ONLY exit of cholesterol from the body
Telangiectasias and Palmar Erythema are what findings in PREGNANT women?
NORMAL and not indicative of liver disease
What is increasingly released by the placenta during PREGNANCY causing gradual elevation in this LIVER test serologically?
ALP
When is it safe to treat a PREGNANT mother for HCV? Her positive child?
OLNY AFTER DELIVERY
Once the child is >2 years old
VERY HIGH LFTs but NORMAL to LOW BILIRUBIN in a PREGNANT mother?
HSV (treat with acyclovir)
Hepatic Venous Outflow Obstruction in a PREGNANT patient due to increased estrogens presenting with new onset ASCITES, RUQ pain and HEPATOMEGALY?
Budd-Chiari Syndrome
What PARASITIC disease worsens during PREGNANCY and can cause liver ABSCESS?
Schistosomiasis
What is the RISK to a PREGNANT woman with CIRRHOSIS for which she must be screened for?
Esophageal VARICES
What should be done in PREGNANT women being treated for AIH with prednisone and azathioprine?
CONTINUE treatment during pregnancy (small risk of cleft palate with prednisode)
How is Wilson’s Disease treated during PREGNANCY?
With ZINC or PENICILLAMINE chelation
What should be done with increasing hepatic ADENOMAS, Focal Nodular Hyperplasia (FNH) and HEMANGIOMAS during pregnancy?
Monitored with US (to avoid rupture)
Intrahepatic Cholestasis of Pregnancy (40-60% chance of recurrence with subsequent pregnancies) is treated how?
UCDA (rpruritus) + Vitamin K
Nulliparity, Maternal Age >40, Family Histroy of Pregnancy Induced HTN, CRF and DM are all RISK factors for what condition of pregnancy?
Pre-Eclampsia (HTN, proteinuria, edema, elevated LFTs, thrombocytopenia) - when seizures and coma present, it’s ECLAMPISA
This condition in PREGNANCY occurs BEFORE DELIVERY, manifests with HEMOLYSIS (elevated LDH), elevated LFTs, and LOW PLATELETS?
HELLP Syndrome (treatment is SUPPORTIVE)
Thic COMPLICATION can occur during PRE-ECLAMPSIA, ECLAMPSIA and HELLP SYNDROME and presents with SUDDEN abdominal pain and MARKEDLY elevated LFTs (3,000-6,000) with FEVER and ANEMIA?
Hepatic INFARCTION and RUPTURE (fluids, treat shock, surgery)
This liver condition is associated with PRE-ECLAMPSIA and occurs in the THIRD TRIMESTER, elevated AST/ALT and WBCs, elevated BILIRUBIN, HYPOGLYCEMIA, RENAL INSUFFICIENCY, COAGULOPATHY with nausea and vomiting, abdominal pain?
Acute Fatty Liver of Pregnancy (AFLP) - microvesicular steatosis
HTN, Renal Failure, Edema, Increase in Body Weight, Blurry Vision, N/V, RUQ Abdominal Pain, Shock in THIRD TRIMESTER?
PRE-ECLAMPSIA
In Acute CHOLESTASIS of PREGNANCY, what serologic marker determines WORSE OUTCOMES (fetal morbidity)?
Serum BILE ACIDS >40µmol
Ground Glass Cells are seen inside hepatocystes of patients with what liver disease?
CHRONIC HBV
PATCHY NECROSIS with LITTLE INFLAMATION and VIRAL INCLUSIONS are seen histologically in the liver biopises of which patients?
Those with HSV
Which are the ONLY TWO drugs that can be SAFELY (class B) used to treat HBV in a PREGNANT mother in her THIRD TRIMESTER to avoid vertical transmission?
TENOFOVIR and TELBUVUDINE
Which medication CANNOT be used safely in pregnancy to treat HBV?
ENTECAVIR
PRE-ECLAMPISA is associated with what comlication of pregnancy?
ABRUPTIO PLACENTA (also HELLP, infactrion, rupture)
Nausea, Jaundice, Abdominal Pain, Renal Failure (THIRD TRIMESTER)?
AFLP
Abdominal Pain, HTN, Edema, Proteinuria (THIRD TRIMESTER)?
HELLP
Increased ALP and GGT in PREGNANCY?
Gallstones
Increased ALP but NORMAL GGT in PREGNANCY?
Acute CHOLESTASIS of Pregnancy (UDCA + Vit K)
A significant proprotion of ASIAN people lack this enzyme for processing of ALCOHOL?
ALDEHYDE DEHYDROGENASE
How does ALCOHOL increase the risk of LIVER TOXICITY with use of ACETAMINOPHEN?
It DEPLETES GLUTATHIONE and upregulates CYTP450 (more repid conversion of acetaminophen into toxic compound in PHASE-I)
Macrovesicular Steatosis, NO INFLAMMATION or FIBROSIS, may or may not have ABDOINAL PAIN?
ALCOHOLIC FATTY LIVER
ANOREXIA, FEVER, JAUNDICE, HEPATOMEGALY, MALAISE in alcoholic liver disease?
ALCOHOLIC STEATOHEPATITIS (balooning degeneration, Mallory Bodies, fibrosis, necrosis)
When there is the presence of REGENERATIVE NODULES in the histology of alcoholic liver disease this usually means what?
CIRRHOSIS
What MEDICATIONS can be used to treat moderate to severe ALCOHOLIC STEATOHEPATITIS?
CORTICOSTEROIDS or PENTOXYFILLINE (MADDREY discriminant function) - EXCLUDE infection or active GIB prior to treating
4.6 X [(PT-control) + total bilirubin] if >32, adde corticosteroids
Maddrey Discriminant Function
MACROVESICULAR steatosis, MALLORY bodies, BALOONING degeneration, NECROSIS and FIBROSIS?
NASH
Central OBESITY, DM, HTN and HYPERLIPIDEMIA constitute what important connection to NAFLD?
METABOLIC Syndrome
What are SECONDARY causes of STEATOHEPATITIS aside from ALCOHOL, MEDS and DISEASES?
JEJUNO-ILEAL BYPASS, TPN
The HETEROZYGOUS MZ Phenotype of apha-1 antitrypsin, ANA, ASMA and CRP are all elevated in this disease process of the LIVER?
NASH
What is found on LIVER US in a patient with NASH?
HEPATOMEGALY and HYPERECHOIC liver
What do you HAVE to use to distinguish FATTY LIVER from NASH and from CIRRHOSIS?
Liver BIOPSY, no radiologic study can do this
What are the MEDICATIONS used to treat NASH, ONLY when DM-2 is ALSO PRESENT when DIET and EXERCISE are not sufficient and BARIATRIC SURGERY is not yet recommended?
Thiazolidinediones (TZD) “GLITAZONE” (rosiglitazone, pioglitazone) - this CLASS of meds causes WEIGHT GAIN but treats NASH
If a patient is treated with CORTICOSTEROIDS for elevated MDF (>32) and after several days does not respond with INCREASE in MDF, what do you do?
STOP STEROIDS (adding pentoxyfiline does NOTHING) - pt NOT likely to respond (Lille Score >0.45)
In ALCOHOLIC patient with abdominal PAIN where AST:ALT NOT >2 and AST/ALT >300-400, what else should be considered and tested for?
ACETAMINOPHEN TOXICITY
Amiodarone, Methotrexate, Diltiazem, Corticosteroids, Tamoxifen, HAART can all cause what liver condition?
MACROVESICULAR Fatty Liver Infiltration (NAFLD, NOT NASH)
Valproate, Rye’s Syndrome, Antivirals, HELLP, can all cause what liver condition?
MICROVESICULAR Fatty Liver Infiltration (NAFLD, not NASH)
How MUST alcoholic hepatitis be treated besides CORTICOSTEROIDS (if doesn’t tolerate due to infection or GIB, use PENTOXYFILLINE) depending on MDF?
Nutrition, Adequate Protein, THIAMINE, GLUCOSE, Vit K, Electrolytes
What is considered SIRS in patient with Alcoholic Hepatitis?
HR >100, TEMP <36C or >38C, RR >12, WBC >12
What constitutes TREATMENT of ACUTE ALCOHOLIC HEPATITIS besides MDF?
MELD >20, Hepatic ENCEPHALOPATHY
Elevated ANA and FERRITIN are seen in this fatty liver disease?
NAFLD (weight loss, Vit E)
HFE gene with mutations at the C282Y or H63D are linked to what hereditary disease?
Hereditary Hemochromatosis
Thalassemia, Sideroblastic Anemia, Cirrhosis and Alcoholism can all result in this condition?
Secondary Iron Overload
People of NORTHERN EUROPEAN descent (Spain, etc.) are at HIGH PREVALENCE for this generic liver disease?
Hereditary Hemochromatosis
Abdominal pain, Hepatomegaly, Cirrhosis, Fatigue, Impotence, Diabetes, Cardiomyopathy and Arthralgias are all seen in this disease process of the liver?
Hemochromatosis due to IRON DEPOSITION
What MUST be done once SERUM TESTS suggest Hemochromatosis (TS >45%, Ferritn >200, TIBC)?
LIVER BIOPSY or quantitative PHLEBOTOMY
What are the SCREENING TESTS for hemochromatosis?
Serum Transferrin-Iron Saturation (TS >45%), Ferritin, Unsaturated Iron Binding Capacity
Serum Iron/TIBC = TS (transferrin saturation) if this is >45%, what is the likely diagnosis?
IRON OVERLOAD
Which HOMOZYGOUS or HETEROZYGOUS for the C282Y HFE mutation, will develop Hemochromatosis?
HOMOZYGOUS (heterozygous only if with other liver disease such as HCV, ALD) COMPOUND HETEROZYGOUS for C282Y/H63D also can
What are the two END-DISEASE processes in Hereditary Hemochromatosis which lead to MORTALITY?
CIRRHOSIS and Hepatocellular Carcinoma
How is Hereditary Hemochromatosis and IRON OVERLOAD treated?
PHLEBOTOMY (Weekly and until development of MILD ANEMIA with ferrtin <50)
Which VITAMIN supplement must patients with HEMOCHROMATOSIS/IRON OVERLOAD avoid?
VITAMIN C
In WHICH individuals should there be SCREENING for HEMOCHROMATOSIS?
Family Members of those with HEMOCHROMATOSIS, those with ABNORMAL LFTs and those with end-organ damage (DM, Cardiomyopathy, Pituitary Disfunction and Arthropathy)
This AUTOSOMAL RECESSIVE genetic disease causes progressive CIRRHOSIS in adults and LUNG DAMAGE through EMPHYSEMA?
alpha-1-antitrypsin deficiency (homozygous for the PiZZ allele)
What is POSITIVE on the liver BIOPSY of patients with alpha-1-antitrypsin deficiency?
PAS+ diastase resistent globules within hepatocytes
What does PERSISTENT JAUNDICE in the NEWBORN suggest?
alpha-1-antitrypsin deficiency (homozygous)
What is the ONLY treatment for alpha-1-antitrypsin deficiency?
Orthotopic LIVER TRANSPLANTATION
Autosomal Recessive, Chromosome 13, Billiary Excretion of this compound is affeted, product is accumulated in the LIVER, BRAIN, CORNEA, kidneys, joints, heart and pancreas?
Wilson’s Disease (Kayser-Fleischer Rings)
Pt’s present between the ages of 10 to 22, almost NEVER >40 and with LIVER or NEUROLOGICAL symptoms?
Wilson’s Disease
COOMB’S NEGATIVE HEMOLYTIC ANEMIA, LOW ALP, HIGH BILIRUBIN, progressive RENAL FAILURE, AST>ALT, DEPRESSION, MOOD DISORDERS, PERSONALITY CHANGES, TREMMORS, PARKISON’S-LIKE features?
Wilson’s Disease
How do you diagnose WILSON’S DISEASE?
SLIT-LAMP examination (Kayser-Fleischer Rings and Sunflower Cataracts) and serum CERULOPLASMIN (<20) levels
In which patients is SCREENING for WILSON’S disease appropriate?
ONLY in 1st DEGREE relatives of those affected
What is the TREATMENT for WILSON’S disease?
CHELATION with PENICILLAMINE (with PYRIDOXINE supplementation) or TRIENTINE as well as ZINC and LOW-COPPER diets (avoid shellfish, chocolate, nuts and liver)
What should be used INSTEAD of PENICILLAMINE in patient’s with WILSON’S disease and NEUROPHYCHOLOGICAL symtoms as these may worsen?
TRIENTINE (also a copper chelator)
This compound PREVENTS copper ABSORPTION from the intestine and promotes FECAL EXCRETION?
ZINC
What is considered CURATIVE in WILSON’S disease?
LIVER TRANSPLANTATION
FOCAL BILIARY CIRRHOSIS, FATTY LIVER, PIGMENTED GALLSTONES, Diarrhea, Pancreatitis, Hearing Loss, Short Stature, Chromosones 18, 2 and 7?
CYSTIC FIBROSIS
Which PROTEIN does HEPCIDIN regulate in liver iron storage?
FERROPORTIN
In a patient with AST:ALT >1.5-2, as well as ELEVATED FERRITIN, what would be the diagnosis if they were found to be HFE NEGATIVE?
ALCOHOLIC LIVER DISEASE
Of CIRRHOSIS, ARTHROPATHY, HYPOGONADISM, DM which can be IMPROVED with PHLEBOTOMY in a patient with HEMOCHROMATOSIS or IRON OVERLOAD?
DIABETES MELLITUS only
Which GENOTYPE is associated with MILD alpha-1-antitrypsin defficiency with a level >40 and a serrum ferritin <1,000?
PiMZ (vs PiZZ in which alpha-1-antitrypsin is <40 and ferritin >1,000)
What are the SEROLOGICAL tests to perform in a patient with SUGGESTED WILSON’S disease in an ASYMPTOMATIC patient with a NORMAL physical exam?
24-HOUR URINE for TOTAL COPPER CONTENT (>100 µg/24 hours); TOTAL SERUM COPPER (>80 µg/dL); INCREASED SERUM FREE-COPPER (>200 µg/dL)
Patient with Hemochromatosis is >40 yo, has Thrombocytopenia (<200,000), Ferritin >1,000, elevated LFTs, Hepatomegaly, what should be done NEXT?
LIVER BIOPSY (determine if there is CIRRHOSIS)
Is FERRITIN elevated only in Hemochromatosis and Iron Overload?
NO! It is an ACUTE PHASE REACTANT and is elevated in many scenarios. ONLY biopsy liver if ALT is elevated and so is TS (transferrin saturation)
FANCONI syndrome (GLYCOSuria, AMINOACIDuria, URICOSuria, HYPORURICemia, HYPERCALCIuria, PHOSPHATuria) is associated with this GENETIC LIVER DISEASE?
WILSON’S disease
What tests should be performed on patients with NEW-ONSET ASCITES?
Diagnotic PARACENTESIS and ULTRASOUND with Doppler
What should the NEW-ONSET ASCITES be sent for in a diagnostic PARACENTESIS?
TOTAL PROTEIN (VERY LOW <1 g/dL in cirrhosis and HIGH in heart failure, etc.); ALBUMIN (SAAG >1.1 g/dL is suggestive of ascites due to portal HTN i.e. cirrhosis); CELL COUNT (PMN >250 - SBP), high RBCs may mean malignancy or bleed, leukocyts possible tuberculosis; CULTURE; TRIGYCERIDES (elevated in chylous ascites as in lymphoma); CYTOLOGY (malignancy); AMYLASE (pancreatitis with duct leak) - NO NEED for pH, lactate, LDH or glucose
When do you RESTRICT fluids in ASCITES?
ONLY if there is HYPONATREMIA
If a patient using SPIRONOLACTONE for ascites control and develops GYNECOMASTIA, what diuretic can be used instead?
AMILORIDE
What can be done for REFRACTORY ASCITES (poor response to therapy, adverse reaction to therapy, low GFR, hyponatremia)?
TIPS
Liver Faiure (high MELD >18-20), Elevated Bilirubin (3-5), Elevated INR (>2.5), Hepatic Encephalopathy, CHF, Pulmnary HTN are all CONTRAINDICATIONS for this method of ASCITES management?
TIPS
What can be done for patients with a SHORT LIFE EXPECTANCY (palliative) that are NOT TIPS candidates and have REFRACTORY ASCITES?
Peritoneal Venous shunt (PV shunt)
A patient with CIRRHOSIS and ASCITES presents with abdominal PAIN, FEVER and ENCEPHALOPATHY. What is the likely scenario?
Spontaneous Bacterial Peritonitis (SBP)
ASCITES with very HIGH PMNs, LDH, Alk Phos, CEA, high ANAEROBES suggests what type of SBP?
SECONDARY (PUD, perforated diverticulitis)
What is the COMPLETE treatment of SBP?
5 DAYS of IV CEPHALOSPORIN followed by PO ANTIBIOTICS to complete 7-10 day course followed by INDEFINITE DAILY PROPHYLAXIS (fluoroquinolone or TMP/SMX) if LOW total protein (<1.5), BUN >30 and HYPOnatremia
What is part of the treatment for SBP that PREVENTS HEPATORENAL SYNDROME and should be administered within 6 HOURS of presentation?
IV ALBUMIN
Which CIRRHOTIC patients should NEVER be treated with non-selective ß-BLOCKERS?
Those with HEPATORENAL SYNDROME or HYPOtension
What can OCCUR in patients with CIRRHOSIS and ASCITES if the have DIAPHRAGMATIC BLEBS?
HEPATIC HYDROTHORAX - treat with Pleuro-Venous Shunt (if infected, Spontaneous Bacterial Empyema)
Microscopic examination of URINE in patients with CIRRHOSIS shows CELL DEBRIS and spot urine SODIUM is HIGH?
ATN (Acute Tubular Necrosis) - cannot CONCENTRATE urine
Microscopic examination of URINE in patients with CIRRHOSIS shows CLEAN URINE and spot urine SODIUM is LOW?
HRS with Pre-Renal Azotemia
LOW GFR (<20 mL/min), Cr >2.5, Oliguria, LOW urine SODIUM and no improvement with IVF challenge
HRS
What is the CURE for HRS?
PROMPT LIVER TRANSPLANT
What is the TREATMENT for HRS?
MIDODRINE, OCTREOTIDE, IV ALBUMIN and TERLIPRESSIN (can reverse HRS)
What MEDICATIONS should NEVER be given to patients with CIRRHOSIS and ASCITES?
NSAIDs and AMINOGLYCOSIDES (-mycin)
What is a POOR prognostic marker in a patient with CIRRHOSIS?
HYPOnatremia (Na <130) (exacerbates hepatic encephalopathy, reduce or STOP diuretics in these patients)
What should be done AFTER endoscopy confirms ESOPHAGEAL, GASTRIC VARICES or Portal Hypertensive Gastropathy (PHG)?
Doppler US to look for Portal Vein Thrombosis or Hepatic Vein Thrombosis
When obtaining a Trans-Jugular LIVER BIOPSY, what can be MEASURED and what constitures PORTAL HTN?
Hepatic Venous Pressure Gradient (5-12 mm Hg GRADIENT is positive for PHTN and high rish for VARICES)
How OFTEN should EGD be performed in patients with ACTIVE CIRRHOSIS (Hep C, EtOH, etc.) and in patients with INACTIVE CIRRHOSIS (cured Hep C)?
Every 2 YEARS for ACTIVE and 3 YEARS for INACTIVE; If SMALL VARICES are prenset, then its every 1 YEAR (active) and every 2 YEARS (inactive)
What patients with esophageal VARICES should receive PP (Primary Prophylaxis - treatment BEFORE any bleeding has occurred)?
Those with GRADE >2 esophageal VARICES (non-selective ß-BLOCKER OR variceal BANDING but NEVER BOTH)
Once PP (Primary Prophylaxis) has been initiated with a ß-BLOCKER (HR 50-60 bpm), how often do you repeat EGD for surveillance?
You DON’T, can stop
Once PP (Primary Prophylaxis) has been initiated with Esophageal Varix Band Ligation, how often do you repeat EGD for surveillance?
Every 1-2 WEEKS until varices are GONE
What should be done PRE-TREATMENT of BLEEDING esophageal VARICES?
TRANSFUSION (<7 Hb), ANTIBIOTICS, IV OCTREOTIDE, endotracheal INTUBATION
What can be done for BLEEDING esophageal VARICES if there is FAILURE of BOTH endoscopic and medical managemen and RECURRENT BLEED in spite of treatment, REGARDLESS of presence of hepatic encephalopathy or high MELD?
TIPS
Which are the MOST COMMON types of gastric varices and what is the treatment?
GOV1 (in continuity with esophagus and lesser curvature) - BANDING (GOV2 are same but with greater curvature; IGV1 - rule out spelnic vein thrombosis - are in the fundus, and IGV2 in the antrum, neither in continuity with the esophagus)
What can cause SPLENIC VEIN THROMBOSIS and what GASTRIC VARIX TYPE is associated with this?
PANCREATITIS or Pancreatic CANCER, IGV1 (fundus)
What is the treatment for SPLENIC VEIN THROMBOSIS and what is the TESTING modality?
SPLENECTOMY, CT/MRI
What is the EMERGENCY treatment of CHOICE (if glue with coils not available) for IGV2 or GOV?
TIPS
What are the treatments of choice for BLEEDING from Portal Hypertensive Gastropathy (PHG)?
IV OCTREOTIDE and APC
Which MEDICATIONS can cause Hepatic Encephalopathy in patients with CIRRHOSIS?
benzodiaAZEPINES (-azepam, -zolam)
Hepatic Venous Portal Gradient (HVCG normal is <5 mmHg) is the difference between?
Wedged Hepatic Venous Pressure (WHVP) - Free Hepatic Venous Pressure (FHVP)
- NORMAL WHVP (<5 mmHg) is slightly higher than FHVP (same as IVC and RIGHT atrial pressure)
- HVPG 5-10 mmHg is PORTAL HTN
HVPG >12 mmHg indicates VARICES with risk for BLEED
PRE-SINUSOIDAL (BOTH WHVP & FHVP are low, HVPG is normal)
SINUSOIDAL (WHVP is HIGH, FHVP is normal and HVPG is HIGH)
POST-SINUSOIDAL (BOTH WHVP & FHVP are HIGH and thus HVPG is normal)
In a patient on a non-selevtive ß-BLOCKER with LOW BP and rising CREATININE and FALLING SERUM ALBUMIN, what should be done NEXT?
STOP the ß-BLOCKER (to improve renal perfusion)
The ABSENCE of N/V, HEPATIC ENCEPHALOPATHY, SHOCK and KIDNEY INJURY is suggestive that SBP can be treated how?
On an OUTPATIENT basis
In BOTH RIGHT HEART FAILURE and Budd-Chiari ASCITES is the expected result NOT VARICEAL BLEEDS, why?
They cause POST-Hepatic PORTAL HTN (both the WHVP and FHVP wou;d be elevated and thus HVPG would be normal)
What would the WEDGE pressure (WHVP - wedged hepatic venous pressure) be in a patient with ALCOHOLIC CIRRHOSIS?
ELEVATED (because the damage is HEPATIC, ie at the sinusoidal level)
In what condition causing CHYLOUS ASCITES (high trigltcerides in ascitic fluid can also occurr in alcoholic cirrhosis) is the SAAG LOW and the PROTEIN HIGH?
LYMPHOMA
In a patient with CIRRHOSIS with ACITES and SBP, if the Cr and Albumin begin to approach HRS levels, what should be done to treat FIRST besides LVP?
IV ALBUMIN (BEFORE starting midodrine and octreotide)
What is the URINE OUTPUT goal on DIURETICS for ASCITES and when should DIURETICS be STOPPED?
Urnine putput 500 mL -1 L more per day
If Cr >1.5 or low BP and low HR
DO NOT USE ß-BLOCKERS in COPD patients
What should be AVOIDED when treating SBP in a patient with CIRRHOSIS with ASCITES?
LVP, IV CONTRAST studies and AGRESSIVE DIURESIS
RENAL FAILURE in patients with LIVER FAILURE associated with LOW URINE SODIUM (<20) in the ABSENCE of renal pathology and in spite of VOLUME REPLETION?
HRS
Median SURVIVAL is 2 WEEKS, RAPIDLY progressing RENAL FAILURE, DOUBLING of SERUM Cr (>2.5) in <2 WEEKS?
HRS-I
Median SURVIVAL is 6 MONTHS, SLOW and CHRONIC progressing RENAL FAILURE, and SERUM Cr (>1.5)?
HRS-II
What MAP in HRS is associated with IMPROVED SURVIVAL?
>82 mmHg (Midodrine, Octreotide, Norepinephrine)
In a patient with ACTIVE CIRRHOSIS (continued alcohol, untreated HCV, etc.) and small, grade-1 esophageal VARICES, WHEN do you repeat surveillance?
Every 1-YEAR
WHEN should EGD be performed for BLEEDING esophageal VARICES or GOV-1/2?
WITHIN 12 HOURS
Are PPIs recommended LONG-TERM (beyond the 10-14 days post treatment) for VARICEAL BLEED?
NO
What is the DANGER when SERUM AMMONIA levels are >200?
CEREBRAL EDEMA
Why is the CAUDATE LOBE of the liver enlarged in BUDD-CHIARI syndrome?
Because it surrounds the VENA CAVA and drains DIRECTLY into the IVC via small perforating veins
What agent REGULATES blood flow to the hepatic lobule and adjusts it depending on variations of PORTAL and ARTERIAL BLOOD FLOW?
ADENOSINE (constantly secreted into the liver)
What is indicated for TREATMENT when PVT is ACUTE, SYMPTOMATIC (nausea, RUQ or diffuse abdominal pain from bowel congestion), PROGRESSIVE toward SMV and occurs in the ABSENCE of CIRRHOSIS and NO GIB?
ANTICOAGULATION for 6 MONTHS (do not used in cirrhosis Child classes B and C) if hypercoagulable state, anticoagulation is life-long
What hepatic VASCULAR issue can PANCREATITIS, INFECTION, liver ABSCESS, CHOLANGITIS, and CHOLECYSTITIS cause?
Portal Vein Thrombosis (PVT)
In the ABSENCE of CIRRHOSIS, what are the most COMMON causes of PVT?
MYELOPROLIFERATIVE disorders (JAK-2 mutations), Factor V Leiden, Antiphospholipid Syndrome, Antithrombin III deficiency, Protein C and S deficiency, and Paroxysmal Nocturnal Hemoglobinuria (PNH) - CD 55 and CD 59 flow cytometry
What causes CAVERNOUS TRANSFORMATION (collaterals) of the PORTAL VEIN?
CHRONIC PVT (post-partum infection of the ubilical vein, etc.)
Should patients with PVT due to HCC be anticoagulated?
NO
A syndrome caused by CHEMOTHERAPY (platinic drugs, myeloablative therapy) wherein THROMBI obstruct the CENTRAL HEPATIC VENULES?
Sinusoidal Obstructive Syndrome (SOS)
What is recommended as PROPHYLAXIS to avoid Sinusoidal Obstructive Syndrome (SOS) when undergoing CHEMOTHERAPY?
URSODIOL
This is NOT cirrhosis but may look that way on imaging, it is caused by VASCULAR INJURY (VASCULITIS) and can cause PRE-SINUSOIDAL PORTAL HTN?
Nodular Regenerative Hyperplasia (NRH)
If WITHIN 20 DAYS of CHEMOTHERAPY or BONE MARROW TRANSPLANT, a patient develops total BILIRUBIN >2, HEPATOMEGALY, WEIGHT GAIN, what has occurred?
Sinusoidal Obstructive Syndrome (SOS)
What can be done to treat ACUTE HEPATIC VEIN THROMBOIS causing liver dysfunction and ascites?
ANGIOGRAPHY with BALLOON DILATION, TIPS
What CARDIOPULMONARY conditions can cause CIRRHOSIS with ASCITES?
RIGHT HEART failure (tricuspid regurgitation), PULMONARY HTN
What LIVER injury is required for ASCITES to form?
SINUSOIDAL HTN
What are some examples of Extrahepatic Portal Vein Obsruction (EHPVO - non-cirrhotic, no ascites but positive VARICES)?
Umbilical Vein Thrombosis, HCC, Pancreatitis, Abdominal Trauma, ARSENIC, VINYL CHLORIDE, SCHISTOSOMIASIS
Acute Mesenteric Thrombosis (such as in extention of PVT) is treated how?
SURGERY, cannot use anticoagulants due to HIGH risk of bleed
What are the CONTRAINDICATIONS to TACE (tansarterial chemoembolization used for HCC)?
PVT, CAVERNOUS transformation of the portal vein
SHOCK LIVER (acute hepatic artery hypoperfusion) can recover, however in the setting of what other conditions, can this cause ACUTE LIVER FAILURE?
PVT, CIRRHOSIS (dual blood supply is compromised)
What is the prognosis of SHOCK LIVER if after restoration of blood flow and pressure, the LFTs and INR remain elevated and don’t normalize?
POOR PROGNOSIS, HIGH MORTALITY RISK
What should be done with a COMPLEX CYST in the liver?
RESECT (15% can become malignant from biliary cystadenomas)
What should be suspected if a hemangioma undergoes interval increase in size?
HEMANGIOSARCOMA
What HEPATIC LESION is associated with ORAL CONTRACEPTIVE use?
Hepatic ADENOMA
What should be done for surveillance for those with CIRRHOSIS and those withoiut cirrhosis but with Hep B?
Liver US and AFP every 6-12 months
>2 cm lesion in a CIRRHOTIC LIVER with ARTERIAL ENHAMCEMENT on MRI (derives blood supply ONLY from hepatic artery) and with EARLY WASHOUT (no blood supply for portal vein) is what?
HCC (no biopsy needed unless those characteristics are absent)
A SOLITARY lesion <6.5 cm OR ≤3 NODULES, the largest of which <4.5 cm OR total tumor diameter <8.5 cm WITHOUT vascular invasion
Which patients are candidates for HCC SURGERY?
Child Class A Cirrhosis, NO Portal HTN, No Metastatic disease
Onset of HEPATIC ENCEPHALOPATHY within 8 WEEKS of onset of JAUNDICE in a patient without previously known liver disease?
Acute Liver Failure (ALF)
ACIDOSIS (pH <7.30), severe COAGULOPATHY (PT >100), RENAL FAILURE (Cr >3.4) and HIGH-GRADE HEPATIC ENCEPHALOPATHY indicate the requirement for what?
LIVER TRANSPLANT
A patient with PHTN and HYPOXEMIA (positional dyspnea, cyanosis, clubbing), PaO2 <60 mmHg and NORMAL CXR and SPIROMETRY, CONTRAST-ECHO with BUBBLES in LEFT HEART is confirmatory?
Hepato-Pulmonary Syndrome (HPS)
How is Hepato-Pulmonary Syndrome (HPS) treated?
Supplemental O2 and LIVER TRANSPLANT
Pulmonary HTN with a NORMAL PCWP in a patient with PHTN - ECHO with RIGHT heart enlargement?
Pulmonary-Portal HTN (PPH)
When is ACUTE CELLULAR REJECTION of a TRANSPLANTED liver the greatest?
The FIRST 6 MONTHS post-op (diagnose by BIOPSY - lymphocyte inflammation in portal areas, bile duct injury, central venulitis)
What is the most COMMON cause of POST-TRANSPLANT DEATH in patients post liver transplant (first 30 DAYS)?
Bacterial and Fungal INFECTIONS
If Acute Cellular Rejection of a transplanted liver doens NOT RESPOND to increase in STEROIDS or increased IMMUNOSUPPRESSION, what can be done besides re-transplant for SEVERE acute rejection?
ANTI-THYMOCYTE GLOBULIN
PROGRESSIVE CHOLESTASIS years after liver transplant is due to?
CHRONIC REJECTION
What is the MAJOR cause of RENAL FAILURE (acute or chronic) after liver transplant?
The CALCINEURIN INHIBITORS (cyclosporin, tactrolimus)
How OFTEN should a patient who received a LIVER TRANSPLANT for PSC be screened with a COLONOSCOPY?
YEARLY
What are the most COMMON malignancies that occurr post liver TRANSPLANT?
SKIN cancers, COLON cancer, LYMPHOMA (EBV)
What is given to HBV patients who receive liver transplants to prevent reinfection?
HBIgG and possibly entecavir or tenofovir
In a patient with CIRRHOSIS and ASCITES with HIGH AFP, what is the BEST way to exclude HCC?
Tripple Phase CT (better than MRI especially with ascites present)
In a patient with KNOWN CIRRHOSIS who presents with diuretic-refractory ASCITES, what must still be done?
EVEN if SAAG is >1.1, if the TOTAL PROTEIN of the ascites is high, it is RIGHT HEART FAILURE that caused the ascites not the cirrhosis, and TIPS is contraindicated
In a patient with resolving SHOCK LIVER, bilirubin starts increasing and serum albumin decreasing, what could be happening when all the other LFTs are normalizing?
PVT, do US
When does the BILIRUBIN typically PEAK after the LFTs peak in SHOCK LIVER?
3-5 DAYS after (they lag)
A NODULAR LIVER on imaging in the ABSENCE of CIRRHOSIS, is what and what causes it?
Nodular Regenerative Hyperplasia (usually autoimmune VASCULITIS), check for PHTN (>10 mmHg)
Sudden-onset abd pain and distention in a young patient, with ascites and hepatomegaly and PVT?
Budd-Chiary Syndrome (Rotterdam Class II) - treat with ANTICOAGULATION (if decompensation is present ie elevated LFTs, INR - do TIPS which is less invasive than surgical shunt)
LESION in the LIVER with a CENTRAL SCAR (MRI isointense T1, hyperintense T2)?
Focal Nodular Hyperplasia (FNH) - NO TREATMENT NEEDED
Most common causes of Portal Vein Thrombosis (PVT) are?
Malignancy (liver, cholangio, pancreas), MYELOPROLIFERATIVE disorder (hypercoagulable as well), Cirrhosis, Infection, IBD, Surgery, Pancreatitis
How do you treat CHRONIC PVT (cavernous transformation of the PV?
DO NOT ANTICOAGULATE unless EXTENDS to the SMV
Do you treat PVT secondary to CANCER with anticoagulation?
NO!
Compression of the IVC by the CAUDATE LOBE of the liver (which encircles the IVC) is called what?
Budd-Chiari Syndrome (acute abdominal pain, ascites, GIB, fever, encephalopathy) ASCITES protein just as high as with RIGHT heart falilure
Recurrent EPISTAXIS (MOST COMMON), also GI, LUNGS, BRAIN, 1st DEGREE RELATIVES?
Hereditary Hemorrhagic Telangiectasias (HHT) - Osler-Rendu-Weber (shortness of breath, edema, ascites, PHTN, biliary cysts, cholangitis)
Reorganization of NORMAL hepatocytes into a NODULE caused by vascular injury such as in (VASCULITIS, CHEMO)
Nodular Regenerative Hyperplasia (NRH) - appears as a “mass” on imaging or MANY NODULES and MIMIC CIRRHOSIS (only on imaging and gradient, no fribrosis on biopsy)
Abdominal pain, randomly distributed BLOOD FILLED CAVITIES without endothelium caused by various cancers, infections (BARTONELLA), medications (AZATHIOPRINE, anabolic STEROIDS) and renal disease?
PELIOSIS HEPATITIS
JAK-2 MUTATION (myeloproliferative disorders) is ASSOCIATED with this LIVER issue that affects the CAUDATE LOBE?
Budd-Chiary Syndrome
How do you TREAT Budd-Chiary?
ANTICOAGULATE (PVT) and evaluate for TIPS
In a patient with abdominal pain and ACUTE PVT, without phlebitis and WITHOUT identifiable HYPERCOAGULABLE state, what is the treatment?
ANTICOAGULATION for 3 MONTHS (if hypercoagulable state identified, LIFELONG anticoagulation)
MRI shows (CENTRIPETAL ENHANCEMENT) without high-output heart failure, what is this and how do you treat?
HEMANGIOMA, NO TREATMENT needed unless painful (then surgery)
BENIGN tumor, has NO BILE DUCTS, RIGHT LOBE, associated with oral CONTRACEPTIVES, risk of HEMORRHAGE and malignant degeneration with SIZE, treated by STOPPING OCPs, OBSERVATION and SURGERY as needed?
Hepatic ADENOMA
ASIAN male/female HepB CARRIERS (>40/>50), AFRICAN, North American Blacks with Hep B all need what?
HCC surveillance with US and AFP every 6 MONTHS regardless of Hep B DNA levels
If MRI shows a LIVER lesion >2 cm in a CIRRHOTIC liver, with LATE ARTERIAL ENHANCEMENT and PORTAL VENOUS WASHOUT, what is the diagnosis and what more is needed?
HCC, NO BIOPSY needed unless MRI features are not there
HSV with AST/ALT >1,000 with ALF, what do you do?
Evaluate for LIVER TRANSPLANT
At what MELD score should LIVER TRANSPLANT be considered?
MELD ≥15
Patient with LIVER TRANSPLANT <1 YEAR presents with elevated LFTs, diffuse abdominal pain, FEVER, NAUSEA, DIARRHEA. What is the MOST COMMON CAUSE?
CMV (get PCR)
Skin Cancers, Kaposi Sarcoma, EBV Lymphoproliferative Disease, COLON CANCER (if PSC with UC), Oropharyngeal Cancers are all seen in these patients?
Post LIVER TRANSPLANT (due to meds)
ANNUAL COLONOSCOPY in liver transplant for pts with PSC/UC
When a DISCREPANCY exists between ELASTOGRAPHY and FIBROSURE (serum) regarding fibrosis and whether cirrhosis exists or not, what should be done next?
LIVER BIOPSY
It a patient is either HBsurface-antigen POSITIVE or anti-HBcore POSITIVE, what can occurr when treating HCV with DAAs?
REACTIVATION of HBV
In a patient who FAILED treatment for HCV GENOTYPE 1a, what must be tested for and how must they be treated?
Test for NS5A if previously treated with elbasvir/grazoprevir or any other NS5A INHIBITOR
If a patient is HCV positive and will be undergoing DAA therapy, and is on a TRANSPLANT LIST for an organ, what is to their ADVANTAGE?
If they are CONSENTING to receiving a HCV positive ORGAN donation (much faster!)
What is the SINGLE, most frequently identified NSAID associated with DRUG-INDUCED liver injury with AUTOIMMUNE HEPATITIS PHENOTYPE pattern (positive ANA)?
DICLOFENAC
What MUST be done for a patient who DEVELOPS elevated LFTs while on AIH therapy with AZATHIOPRINE?
STOP azathioprine and check for TOXIC metabolites THIOGUANINE and 6-MMP
Pt s/p >1 year LIVER TRANSPLANT who develops PRURITUS with elevated ALP, no symptoms, negative serologies and normal US?
ANASTOMOTIC BILIARY STRICTURE, do ERCP with stent
HEPATO-PULMONARY SYNDROME (HPS) with a ROOM AIR PO2 >60 mmHg; PORTO-PULMONARY HTN (PPH) with a mean PULMONARY ARTERY PRESSURE >35 mmHg affect LIVER TRANSPLANT how?
CONTRAINDICATED
ALT:AST >2.2 and ALP:T.Bili <4?
100% SPECIFIC for WILSON’s disease
Besides acute ALCOHOLIC HEPATITIS, where else is the AST:ALT >2? What other ratio makes it 100% SPECIFIC?
WILSON’s disease (ALP:T.Bili <4)
What LIVER LESION can OCPs cause or need to be stopped for as these are HORMONALLY responsive?
Hepatic ADENOMAS
Past history of PSC with UC, received liver TRANSPLANT <1 YEAR ago, presents with BLOODY DIARRHEA, FEVER and ABDOMINAL PAIN, what shoud you do NEXT?
Check CMV PCR first, BEFORE considering invasive colonoscopy
In an ASYMPTOMATIC patient with GENETIC HEMOCHROMATOSIS with serum FERRITIN <1,000 what should be done NEXT?
NO liver biopsy necessary at this point, should just do THERAPEUTIC PHLEBOTOMY (serum ferritin levels dictate this)
What is the GOAL serum FERRITIN LEVEL that GUIDES PHLEBOTOMY in a patient with genetic HEMOCHROMATOSIS or IRON OVERLOAD?
SERUM FERRITIN of 50-100 µg/L
If a patient presents with CIRRHOSIS and also has positive markers for other disease such as AIH (ANA, ASMA, etc.), what MUST be done PRIOR to starting treatment (prednisone + azathioprine)?
US of LIVER to check for HCC
WHEN do you start TREATMENT in a patient with AIH?
- INCAPACITATING symptoms (arthralgia and fatigue)
- AST/ALT >10 X normal values
- BRIDGING or NECROSIS on liver BIOPSY
Patients with INACTIVE CIRRHOSIS do NOT require CORTICOSTEROIDS
What can occur in a patient being treated with LAMIVUDINE for Hep B and what do you do if suspected (has not seroconverted, ie anti-HBe negative)?
50% develop RESISTANCE (after years) and should be tested for this
Can ADD TENOFOVIR or ENTECAVIR or adefovir
What should be TESTED for if suspecting ACUTE Hep C infection (IVDA), with AST/ALT in the THOUSANDS (can be >2,000)?
HCV RNA (because antibodies take a while to form)
WHEN should PO CHARCOAL be included in the treatment of an OVERDOSE (tylenol, etc.)?
ONLY if <4 HOURS since ingestion
What is the most COMMON cause of DEATH in a patient with NAFLD (not NASH)?
CARDIOVASCULAR disease
What is recommended for patients with PSC who undergo ERCPs to relieve dominant strictures causing pruritus?
Prophylactic ANTIBIOTICS
In a patient with CRRHOSIS and with NORMAL AMMONIA levels, what is NOT causing mental stateus changes?
HEPATIC ENCEPHALOPATHY
How should a patient with CRYOGLOBULINEMIC VASCUITIS with RENAL FAILURE be treated?
DAA
Pt with HCV, FATIGUE, JOINT PAIN, WEAKNESS, mild ANEMIA, RENAL INSUFFIEICENCY, LOW GFR, how do you TREAT?
DAA
Pt with chronic CHOLESTASIS, CROHN’s disease, mild PRURITUS, NEGATIVE MRCP, what could this be and what should be done?
SMALL DUCT PSC (more common in PSC with CROHN’s as 10% have negative MRCP), BIOPSY
Which PSC patients have the WORST PROGNOSIS?
Those with LARGE DUCT PSC - CHOLANGIOCARCINOMA (10% with small duct progress to large duct)
Drugs such as NITROFURANTOIN can cause HEPATOTOXICITY that mimics what?
AIH (elevated ANA)
In patients who developed RESISTANCE to LAMIVUDINE, what medication CANNOT be used to treat HBV and what medication should be used?
Cannot use ENTECAVIR (will also be resistant), must use TENOFOVIR
In a patient with acute ALCOHOLIC HEPATITIS with an ACTIVE INFECTION (PNA, etc.) would you start STEROIDS if MDF was >32?
NO
In a patient with VARICEAL BLEED, besides INDEFINITE non-selective ß-Blockers, after EGDs no longer show varices, how OFTEN do you repeat them after the FIRST NEGATIVE EGD?
Repeat in 1-3 MONTHS, if negative again, repeat every 6 MONTHS
What would INCRESING DIURETICS cause in a patient with WORSENING ASCITES with HYPOnatremia?
It would further WORSEN the HYPOnatremia
What is the BEST treatment for REFRACTORY ASCITES when pt is NOT a TIPS candidate (HE, T.Bili >3, high MELD score)?
Serial PARACENTESIS with ALBUMIN replacement
What should be done for a patient presenting with CHRONIC INDETERMINATE CHOLESTATIC LIVER DISEASE?
LIVER BIOPSY (AMA-neg livefr disease or infiltrative process such as sarcoid, etc.)
What antibodies are a feature of TYPE-II AIH (hepatocellular not cholestatic)?
LKM1 autoantibodies
For NAFLD, what is a way to monitor for improvement?
Weight loss with SERIAL monitoring of LFTs
FEVER, SOMNOLENT, VERY HIGH AST/ALT (thousands) and imaging showing TEMPORAL LOBE CHANGES (encephalitis)?
HERPES HEPATITIS - acyclovir
What is RECOMMENDED after the initiation of STATINS when LFTs rise after starting this therapy?
OBSERVATION with serial LFT monitoring
Liver biopsy demonstrating LYMPHOCYTIC INFILTRATES, LYMPHOCYTIC CHOLANGITIS, STEATOSIS, AMA-negative in a patient with steady and chronic LFT elevation and hepatomegaly?
AMA-negative PBC (can have positive ANA and SMA)- treat with URSODIOL
What needs to be excluded in a YOUNG patient with CIRRHOSIS (thrombocytopenia, elevated INR, low albumin, coarse liver echotexture, splenomegaly, lymphoplasmacytic portal inflammation, septal FIBROSIS and NODULAR REGENERATION)?
Must exclude WILSON’s disease (hepatic COPPER quantitation)
In a patient with minimally elevated LFTs and a normal Ŷ-globulin level, would AUTOIMMUNE hepatitis be likely?
NO
STEATOSIS in a patient WITHOUT obesity or DM is suggestive of what?
WILSON’s, HEP C, DRUGS or CELIAC DISEASE
Patient presents with FEVER, elevated ALK PHOS and liver biopsy shows NON-CASEATING GRANULOMAS AFTER receiving intra-VESICAL BCG for BLADDER CANCER?
MYCOBACTERIUM BOVIS (non-TB mycobacterial infection)
In a YOUNG patient with Hep B, POSITIVE HBeAg and VERY HIGH HBV DNA level but with NO ELEVATION of LFTs, what NEEDS to be DONE?
Repeat LFTs in 6 months (INACTIVE CARRIER STATE)
WHEN is the monitoring of the liver with US and AFP NEEDED?
When the patient has CIRRHOSIS (OR asian men >40, asian women >50, blacks >20 and pts with family history of HCC)
In WHICH PATIENTS with Hep B and WITHOUT CIRRHOSIS is an US every 6 MONTHS and an AFP level NEEDED?
BLACKS >20, ASIAN MEN >40, ASIAN WOMEN >50 and patients with a FAMILY HISTORY of HCC
Do Hep B patients with INACTIVE CARRIER STATE require LIVER BIOPSY?
NO
What is RECOMMENDED for ALL PATIENTS with Hep B and DECOMPENSATED CIRRHOSIS with ANY DETECTABLE HBV DNA?
TREATMENT (tenofovir or entecavir)
Can you TREAT a patient with DECOMPENSATED CIRRHOSIS with PEGINTERFERON?
NO (precipitates a FLARE of hepatitis)
In a patient with Hep C who develops CRYOGLOBULINEMIA WITH SYSTEMIC EFFECTS (RENAL FAILURE), how do you treat?
RITUXIMAB FIRST, then HCV THERAPY (plasmapharesis and corticosteroids as well as needed)
What is special about HEPATITIS C IL28B genotype CC?
HIGH chance for SPONTANEOUS CLEARANCE in ACUTELY INFECTED PATIOENT (OBSERVE for 3 months and only treat if still positive)
In a patient with CHRONIC HBV and CIRRHOSIS, MRI shows a new mass with ARTERIAL ENHANCEMENT and PORTAL VENOUS WASHOUT?
HEPATOCELLULAR CARCINOMA
In an OTHERWISE healthy patient with Hep B CIRRHOSIS and a LOCALIZED LIVER MASS, what is the best treatment?
ORTHOPIC LIVER TRANSPLANT (RFA ablation or ethanol only if comorbidities preclude surgery)
How do you TREAT ADVANCED STAGE INTRA-HEPATIC CHOLANGIOCARCINOMA?
GEMCITABINE + CISPLATIN
LIVER MASS with RAPID HOMOGENEOUS ENHANCEMENT in the ARTERIAL PHASE and return to ISOINTENSITY in the PORTAL VENOUS PHASE with a CENTRAL SCAR and CONTRAST RETENTION in the DELAYED HEPATOBILIARY PHASE?
FOCAL NODULAR HYPERPLASIA (bening, no treatment needed)
What is the BEST way to ABLATE an intra-hepatic SEROUS CYST after drainage?
ALCOHOL INSTILLATION (fenestration if alcohol fails)
What is the RECOMMENDATION for ₿-catenin-activated inflammatory hepatocellular adenoma?
SURGICAL RESECTION (risks malignant transformation)
What does the presence of ₿-catenin signify when a heaptic lesion stains POSITIVE?
HIGH RISK for MALIGNANT TRANSFORMATION
A MADDREY DISCRIMINANT FUNCTION SCORE of what would SUGGEST use of PRENDISOLONE OR PENTOXIFYLLINE (not both)?
>32
What is the KEY to INTERVENTION in ALCOHOLIC CIRRHOSIS?
ABSTINENCE and MANAGEMENT of ALCOHOLISM
SHOULD patients with CHRONIC HEPATITIS of ANY KIND (Hep C, etc.) consume ALCOHOL?
NO (synnergistic effect and causes more damage)
What can occur in a patient who drinks ALCOHOL and takes normal amounts of ACETAMINOPHEN?
DILI ith elevated AST adn ALT
What causes MACROVESICULAR STEATOSIS?
ALCOHOL
Where do you see BALLOONING DEGENERATION and MALLORY BODIES?
ALCOHOLIC HEPATITIS
Where do you see INTERFACE HEPATITIS with PLASMA CELLS?
AUTOIMMUNE HEPATITIS
Can you treat PORTAL VEIN THROMBOSIS or TUMOR THROMBOSIS of any kind with anticoagulation or anti-thrombotic therapy?
NO, no role for that
What is the BEST RECOMMENDATION for an ELDERLY patient with CHRONIC Hep B with HCC and tumor-related PVT (arterial enhancement of the clot)?
POOR PROGNOSIS (2-3 months) - HOSPICE
What do ISOLATED gasric FUNDUS VARICES suggest in the ABSENCE of liver disease?
SPLENIC VEIN THROMBOSIS (pancreatitis, etc.)
What should be done for a patient with SPLENIC VEIN THROMBOSIS and FUNDUS VARICES with bleed?
SPLENECTOMY
ASCITES due to BUDD-CHIARI syndrome (hepatic veins are not well visualized on doppler US) is treated how?
DIURESIS and ANTICOAGULATION
When is THROMBOTIC THERAPY appropriate for HEPATIC VEIN THROMBOSIS?
When it is ACUTE (catheter-directed therapy, NOT SYSTEMIC)
How is ACUTE PVT due to INFLAMMATION (diverticulitis) TREATED?
ANTICOAGULATION (WARFARIN for 6 MONTHS)
Post LIVER TRANSPLANT, pt develops FEVER, ABDOMINAL PAIN, N/V, RAPID DETERIORATION, HYPOTENSIVE, TOXIC, TACHYCARDIA, RESPIRATORY DISTRESS, VERY HIGH AST/ALT, ACUTE KIDNEY INJURY, HIGH INR, US shows PNEUMOBILIA, HEPATIC ARTERY NOT VISUALIZED?
ACUTE HEPATIC ARTERY THROMBOSIS (usually within 7 DAYS)- severe ischemic necrosis - LIST FOR RETRANSPLANT
IN a patient with BLEEDING gastric FUNDUS VARICES due to LIVER CIRRHOSIS (not pancreatitis), what is the RECOMMENDED TREATMNET?
TIPS (NOT band ligation)
In a patient with UGIB with CIRRHOSIS and ANY SIZE ESOPHAGEAL VARICES, WHERE should the bleeding be suspected to have originated even if NO STIGMATA are noted on the esophageal varices?
The ESOPHAGEAL VARICES - so BAND THEM
In a patient with DIFFUSE GAVE, NOT AMENABLE to ENDOSCOPIC THERAPY, what is the RECOMMENDED TREATMENT?
Estrogen therapy
In a patient with CHRONIC BLEEDING from PORTAL HYPERTENSIVE GASTROPATHY which is NOT amenable to endoscopic therapy (most cases), what is the recommended TREATMENT?
TIPS, B-blockers and Iron supplementation
What should be done for a patent on B-blocker (nadolol) as primary variceal prophylaxis if they develop side effects (fatigue, sexual dysfunction, orthostatic hypotension)?
STOP the NADOLOL and perform EGD with variceal BANDING as primary prophylaxis
What is the BEST antibiotic CHOICE for a patient with ASCITES and GIB?
IV CEFOTAXIME (gm-negative bacilli coverage)
What would a patient’s TRANSFERRIN SATURATION be in HEMOCHROMATOSIS?
HIGHLY ELEVATED (>50%)
OBESITY, HTN & IMPAIRED FASTING GLUCOSE are all components of what LIVER CONDITION?
NAFLD
WHEN should phlebotomy be started in a patient with HEMOCHROMATOSIS (2 abnormal copies of the C282Y mutation)?
ONLY when there is evidence of IRON OVERLOAD (elevated serum FERRITIN >300)
Patients with CIRRHOTIC-STAGE HEMOCHROMATOSIS are at HIGH RISK for HCC, and if they develop PVT, what MUST BE DONE?
MUST EXCLUDE thrombus due to tumor, DO NOT start anticoagulation (TRIPPLE PHASE CT of the ABDOMEN or MRI)
ALL patients with CIRRHOSIS presenting with PVT, what must be ruled out FIRST before starting anticoagulation?
HCC (CT or MRI)
In ONE parent with HEMOCHROMATOSIS, HOW MANY MUTANT HFE GENE copies can that parent’s chilren INHERIT?
JUST ONE (which is why you MUST TEST THE WIFE to know whether the children will also have HEMOCHROMATOSIS or just be CARRIERS)
In a patient with POSTIVE AMA (PSC) and being treated with URSODIOL, what is the BEST NEXT TEST to use to gauge respone to therapy?
ALK PHOS (ALP)
In an ADVANCED STATE of LIVER DISEASE (CIRRHOSIS), what complication commonly occurs METABOLICALLY?
FAT-SOLUBLE VITAMIN DEFICIENCIES (DEKA)
In a NEWLY-DIAGNOSED patient with PSC, what is RECOMMENDED to be done diagnostically when BILIARY STRICTURES are noted?
ERCP with BILIARY BRUSHING (NOT LIVER BIOPSY)
What is it called when a patient meets criteria for BOTH AIH and PBC (positive AMA and biopsy showing LYMPHOCYTIC CHOLANGITIS and PLASMA CELLS, positive SMA and ANA)?
OVERLAP SYNDROME
Patients with this TYPE of PSC DO NOT have positive AMA and have normal Ŷ-globulin results?
SMALL-DUCT PSC
NON-CASEATING GRANULOMAS involving the PROTAL TRACTS are found in the liver of patients with what?
SARCOIDOSIS and MYCOBATERIA (TB or BOVIS)
WHICH LIVER disease is UCDA used for?
PRIMARY BILIARY CIRRHOSIS (not PSC)
Is biliary duct DILATION and BEADING on MRCP sufficient for a diagnosis of PSC?
YES
What MUST be done for patients with BOTH UC and PSC?
ANNUAL SURVEILLANCE COLONOSCOPY (increased risk of COLON CANCER over UC alone)
PAINLESS JAUNDICE, elevated BILIRUBIN, NODULAR PANCREAS and LONG TAPERING EXTRAHEPATIC CBD STRICTURE?
AUTOIMMUNE PANCREATITITS (IgG4)
Most COMMON MEDICATION causing IDIOSYNCRATIC DILI?
AUGMENTIN (AMOXICILLIN-CLAVULANATE)
What medication is HELPFUL with ACUTE LIVER FAILURE (jaundice, pruritus, confusion, elevated INR and LFTs) whether caused by acetaminophen or not?
N-acetylcysteine
In a patient who is a HUNTER and eats RAW PORK or DEER MEAT, what are they at RISK for (elevated LFTs, nausea, vomiting, malaise and abdominal pain)?
Hepatitis E (check IgM)
CHRONIC ALCOHOL abusers who take ACETAMINOPHEN are at INCREASED RISK of acetaminophen TOXICITY by what MECHANISM?
ALCOHOL INDUCTION of CYP2E1 resulting in INCREASED HEPATOTOXIC METABOLITE N-acetyl-p-benzoquinone imine
CHRONIC ALCOHOL INGESTION and MULTIPLE SUPRATHERAPEUTIC DOSES of ACETAMINEOPHEN?
HIGHEST RISK for ACETAMINOPHEN-RELATED HEPATOTOXICITY (CYP2E1 induction with production of more toxic N-acetyl-p-benzoquinone imine)
What is the TREATMENT of CHOICE for VALPROATE HEPATOTOXICITY with features of HEPATIC ENCEPHALOPAHTY?
CARNITINE
MIDDLE-AGED WOMAN with FATIGUE and PRURITUS, XANTHELASMAS (from HIGH-LIPIDS) is HIGHLY-SUSPICIOUS for what condition and how should you test?
PRIMARY BILIARY CIRRHOSIS (PBC) - check AMA
PERIDUCTAL ONION SKIN FIBROSIS with edema and mild INFLAMMATORY INFILTRATE in a patient with UC and abnormal LFTS including ALP, AST and ALT?
PSC
What is the ONLY MEDCIATION shown to DELAY time to LIVER TRANSPLANT in a patient with (hypercholesterolemia, elevated ALP, AST ALT, GGT, BILIRUBIN, INR, ANA, AMAand IgG)?
URSODIOL at 13-15 mg/kg DAILY (PBC)
POSITIVE ANA, SMA very HIGH IgG, with FATIGUE, SOB, RUQ pain with elevated ALP, ALT, AST, BILIRUBIN and FERRITIN (acute phase reactant)?
AIH (treat with STEROIDS +/- azathioprine) - 90% BIOCHEMICAL improvement in 2 weeks and 60% HISTOLOGIC improvement in 18 MONTHS and 85% in 3 YEARS (in the 10% who don’t respond, consider OVERLAP SYNDROME or WILSON’s disease)
WHAT HISTOLOGIC FEATURE can distinguish NASH (Non-Alcoholic Steato-Hepatitis) from ASH (Alcoholic Steato-Hepatitis)?
NONE (diagnosis is made by HISTORY and CLINICAL EXAM)
Approximately what PERCENTAGE of patients with ALCOHOLIC FATTY LIVER will PROGRESS to more ADVANCED LIVER DISEASE with either ABSTINENCE or CONTINUED EXCESS DRINKING?
With ABSTINENCE - 0%
With continued EXCESS DRINKING - 25%
FATTY LIVER DISEASE from ALCOHOL is REVERSIBLE
Which are the IMPORTANT CLINICAL and LABORATORY features of ALCOHOLIC HEPATITIS which predict SURVIVAL?
Those used in calculating the MELD SCORE and MADDREY DISCRIMINANT FUNCTION (bilirubin, PT, INR, Na, Cr, hepatic encephalopathy) - NOT albumin
ALCOHOLIC patient WALKS into clinic and complains of PAIN and is found to have ALT/AST in the THOUSANDS?
ACETAMINOPHEN TOXICITY most likely diagnosis
What is the FIRST LINE of therapy for FATTY LIVER (ANA and FERRITIN are usually elevated)?
WEIGHT LOSS (at least 10% of EXCESS body weight)
In the EARLY SECOND TRIMESTER (weeks 13-24) and having a CHILD in DAY CARE, is a RISK FACTOR for what LIVER DISEASE?
ACUTE Hep A (AST/ALT in the thousands)
ASCITES, HEPATOMEGALY, VENOUS COLLATERALS in the POST-PARTUM period?
ACUTE BUDD-CHIARI SYNDROME (history of thromboembolism, etc.)
This CONDITION develops in the SECOND or THIRD TRIMESTER, with ELEVATED AST/ALT and ALP with BILIRUBIN and SEVERE PRURITUS, HOW is it TREATED?
INTRAHEPATIC CHOLESTASIS of PREGNANCY (ICP) - UDCA
WHEN is PREGNANCY SAFEST after LIVER TRANSPLANT?
2-YEARS POST TRANSPLANT
Is PREGNANCY ASSOCIATED with TRANSPLANT REJECTION?
YES, only ACUTE CELLULAR REJECTION, NOT CHRONIC
Is MYCOPHENOLATE MOFETIL SAFE in PREGNANCY?
NO! ABSOLUTELY NOT! (known TERATOGEN)
THIRD TRIMESTER, with ACUTE LIVER FAILURE represented by ANOREXIA, MALAISE, N/V, DISORIENTED, TREMMOR, JAUNDICE, HYPERTENSION, LE EDEMA, LEUKOCYTOSIS, ELEVATED AST/ALT, ALP, BILIRUBIN, INR and Cr?
ACUTE FATTY LIVER of PREGNANCY
THIRD TRIMESTER, with ACUTE LIVER FAILURE represented by ANOREXIA, MALAISE, N/V, NO JAUNDICE, HYPERTENSION, LOW PLATELETS, LE EDEMA, LEUKOCYTOSIS, ELEVATED AST/ALT, ALP, BILIRUBIN, INR and Cr?
H(hemolysis)EL(elevated LFTs)LP(low platelets) SYNDROME
WHEN is HEPATECTOMY and LIVER TRANSPLANTATION CONTRAINDICATED in a CIRRHOTIC with LOCALIZED HCC?
When there is PORTAL HYPERTENSION
What is the MILAN CRITERIA for LIVER TRANSPLANTATION in HCC?
ONE LESION ≤5 cm
or
UP TO 3 LESIONS that are EACH ≤3 cm with NO MACROVASCULAR INVASION or EXTRAHEPATIC SPREAD
In a HIGHLY-SYMPTOMATIC patient with PSC and a LOW MELD (<28) HOW could they QUALIFY for a LIVER TRANSPLANT?
LIVING DONOR TRANSPLANT
Pt post RECENT LIVER TRNSPLANT presents with PRURITUS, JAUNDICE without fever or pain and ELEVATED ALP and BILIRUBIN with MILD AST/ALT ELEVATIONS, NON-DILATED INTRAHEPATICS and NORMAL ARTERIAL and VENOUS FLOW?
ANASTOMOTIC BILIARY STRICTURE
FEVER, malaise, N/V, abdominal pain and PROFUSE DIARRHEA 3-12 MONTHS post LIVER TRANSPLANT?
CMV INFECTION
FEVER, malaise, N/V, abdominal pain and PROFUSE DIARRHEA 3-12 MONTHS post LIVER TRANSPLANT?
CMV INFECTION
Are patients with METASTATIC DISEASE to the LIVER (as in colon metastatic to liver) CANDIDATES for LIVER TRANSPLANT?
NO
What is the MOST COMMON viral hepatitis etiology especially in IMMUNOCOMPROMISED and YOUNG individuals?
EBV
Which HEP virus has shown the greatest INCREASE in incidence over the past 10 years?
HAV (narcotic abuse, homelessness)
Where is the HDV DNA located?
Inside the HBsAg
Acute FULMINANT hepatic failure is associated with what infection?
HBV + HDV CO-INFECTION
Patients with HBV + HDV superinfection have ~100% risk of what?
CHRONICITY (HDV is found in 40% of patients with HBV)
For either MALEs or FEMALEs, acute hepatitis infection negative for HBV, HCV, HAV, and especially from EGYPT, what is the likely acute viral infection?
HEV (highest prevalence in 60-80 yo, meat eaters, immigrants, hunters, fishermen)
Do Hepatitis C or D develop PROTECTIVE Ab’s?
NO
Does HAV develop CHRONIC hepatitis?
NO
Does HEV develop CHRONIC hepatitis?
YES, ONLY in IMMUNOCOMPROMISED
What is the % of individuals in which HBV develops CHRONICITY? HCV?
HBV - 15%
HCV - 85%
An ALT OF >10,000, jaundice with bilirubin of >10 is an idication of what?
Acute Liver Failure (prolonged INR, hepatic encephalopathy)
What are the 3 types of HAV infection presentation?
- Acute (4 weeks, T.bili <10, NL alk phos)
- Cholestatic (up to 12 weeks, T.bili >10, Elev alk phos)
- Relapsing (up to 1 year, every 4-15 wks, IgM stays POSITIVE)
When do you see HBeAg-NEGATIVE active hepatisis B?
When there is an HBeAg MUTATION in the e-GENE
What is the TARGET of the immune response to INACTIVATE HBV?
The eAg (thus, when this is mutated, an eAb cannot be formed and this cannot inactivte HBV (“e-nagative, active hepatitis - chronic)
Cirrhosis and HCC are directly related to what feature of HBV infection?
Amount of HBV DNA present (risk starts increasing significantly at 2-20,000 IU of HBV DNA ie 10x4 - 10x5 copies) so, TREAT at >20,000
What pts with HBV do you screen for HCC?
US (+/- AFP) EVERY 6 months in those with ACTIVE (20%) (HBeAG +/-) and those with INACTIVE (80% of HBV ps) (HBeAG -)
essentially, there are so many more inactive HBV patients than active (80%/20%) that HCC is higher collectively in inactive patients
What is the difference in development of HCC in patients with HBV vs HCV?
In HBV, both pts with and without CIRRHOSIS will develop HCC whereas in HCV ONLY CIRRHOTIC patients will develop HCC
What hepatitis VACCINE is recommended in ALL children?
HBV
For whom is the HAV vaccine recommended?
ALL those traveling to endemic areas (Caribbean, Mexico, Egypt, etc. and individuals at RISK) - all household or intimate contacts of an HAV patient must receive IVIGg
Do you need to get re-boosted every time you have a needle-stick exposure once you got your HBV vaccine or booster 10 years later?
NO (even if Ab levels wane and decreased over 6-12 months)
CHRONIC HBeAg POSITIVE patients are treated with what and how long?
TENOFOVIR or ENTECAVIR (6 MONTHS after HBeAb forms - seroconversion (20%), LFT normalization and DNA <2,000 IU)
Which HBV Direct Antiviral Agent (DAA) is the most potent?
TENOFOVIR ALAFENAMIDE (less drug, less toxicity)
When treated with PEG-INTERFERON, which GENOTYPE of HVB will experience a 50% SEROCONVERSION (HBeAb)?
Genotype A
Can PEG-INTERFERON be used in HBeAg NEGATIVE pts?
NO (only tenofovir or entecavir)
What does HBV SEROCONVERSION (HBeAg to HBeAb) mean?
INACTIVE STATE
When can DAA be stopped in HBeAg NEGATIVE pts?
NEVER
In a patient with UC needing treatment, who has normal LFTs BUT is HBsAg positive (immune tolerant phase) and HCV-Ab positive, how do you treat?
Start treatment for UC AND HBV AND HCV
Which CIRRHOTIC patients CANNOT use PROTEASE INHIBITORS (NS3-4A) to treat HCV?
CTP (Child-Turcotte-Pugh) classes B or C
A patient with LIMITED life expectancy and mild fibrosis; ADVANCED stage 3-4 HCC; CPT class C with HIGH MELD who are transplant candidates; ACTIVE drug users ALL have this in common?
NON-HCV therapy candidates
BEFORE starting a patient on immunotherapy, antibiologics, HCV treatment, chemotherapy, ALWAYS screen for?
HBV (HBsAg, HBsAb, HBcAb)
While treating an HCV pt who had inactive HBV, WHEN after curing HCV do you STOP HBVprophylaxis?
SEVERAL MONTHS later
In treating a patient with HCV and ONLY HBcAb (NO HBsAG or HBsAb) what do you do for the HBV exposure?
MONITOR ONLY
In treating a patient for HCV with HBsAb (seroconverted), what is required for HBV?
NOTHING (no monitoring nor prophylaxis)
IF a pt is tested for HBsAg and is POSITIVE, what do you test for NEXT?
HBV DNA, HBeAg and HBeAb
LYMPHOMA, DM and ESLD are all significantly REDUCED when treating what?
HCV
ALL HBV pts and ALL CIRRHOTIC pts who have been successfully treated for HCV must be SCREENED for?
HCC every 6 months with US +/- AFP
60% of ACUTE LIVER FAILURE cases are caused by?
DILI (drug induced liver injury)
Does CHRONIC LIVER DISEASE or CIRRHOSIS increase the risk of DILI?
NO (but if does occur, it increases the severity and chance of developing ALF)
Elevated AST/ALT sometimes AlkPhos as well, up to 3xNL then adapts?
DILI (when no adaptation occurs, may have underlying NAFLD)
LFT elevations of 3-5 x ULN after taking MEDS with JAUNDICE and >5x ULN may result in what?
10% - 50% risk of ACUTE LIVER FAILURE
When someone is taking a med that results in elevated LFTs (AST, ALT and sometimes AlkPhos) what change besides elevation in LFTs (3-5x) signals risk for ACULE LIVER FAILURE)?
JAUNDICE (STOP med immediately)
Pt is on a new MED, LFTs 3-5X ULN, then plateus. WHEN must you STOP the drug?
If JAUNDICE develops
When AlkPhos is mainly ELEVATED more so than AST/ALT, mostly with associated itching, what is the pattern of this liver INJURY?
CHOLESTATIC (when jaundice is seen in this situation, it is LESS OMINOUS than with hepatocellular pattern) - in JAUNDICE, AlkPhos & T.bili are up while AST/ALT are low high or normal
What type of ISOLATED liver injury results from METHOTREXATE or Vitamin A?
FIBROSIS
SAAG (serum Alb - ascites Alb) >1.1 gm/dL?
Portal HTN (CIRRHOSIS, hepatic failure, veno-occlusive, Budd-Chiari, CHF, malignancy, malnutrition)
SAAG (serum Alb - ascites Alb) <1.1 gm/dL?
Mesothelioma, peritoneal carcinomatosis, TB, sarcoidosis, SLE, Whipple Disease, Endometriosis
HVPG (hepatic vein pressure gradient) = Wedged HVP - Free HVP. What is normal and elevated?
HVPG WNL 1-5
HVPG ≥10 Portal HTN
HVPG ≥12 Varices
What can cause stiff liver sinusoids, non-cirrhotic portal HTN, ascites, varices with normal liver on imaging and biopsy?
CHEMOTHERAPY
Pt receives CHEMOTHERAPY and develops a NODULAR liver, non-cirrhotic portal HTN, liver synthetic function normal, HVPG normal, normal biopsy?
Nodular Regenerative Hyperplasia (NRH)
Pt receives CHEMOTHERAPY with another DRUG and AST, ALT and AlkPhos are high with 5% developing JAUNDICE in about 60 DAYS after initiation, what did they get?
CHECKPOINT INHIBITOR (pembrolizumab, etc.) treat with steroids+mycophenolate mofetil or just stop the drug, does recurr with restart (17-28%)
ACETAMINOPHEN TOXICITY is INCREASED by what?
ALCOHOL (not liver disease or cirrhosis)
Acetaminophen toxicity >24 hours, elevated INR or Hepatic Encephalopahty?
Immediate transfer to Liver Transplant Center
What are the serologic markers usually positive in AIH I, II?
AIH I - ANA, ASMA
AIH II - anti-LKM or anti-LC1 (European, more severe)
What should be done when suspecting AIH, what is found?
Liver Biopsy - PIECEMEAL & LOBULAR NECROSIS
What is the TREATEMENT for AIH when its CRYPTOGENIC CIRRHOSIS (stable or decompensated cirrhosis, normal LFTs, no inflammation on biopsy, positive serologic markers for AIH)?
NO TREATMENT
In patients with AIH with liver decompensation, what should be considered?
Liver Transplant
