Hepatitis C
Hepatitis B
Metabolic Liver Diseases
Vascular Diseases of the Liver
POO Pourri
100
A 54-year-old man presents with new onset of rash with palpable purpura, visible nonblanching raised areas of the skin.
He has a history of obesity, hypertension, and untreated chronic hepatitis C virus infection genotype 1b.
He notes the rash is painful and has not responded to topical steroid therapy.
He has also noted mild pitting lower extremity edema.
Laboratory results are below in Figure 1.
What is the next best step for therapeutic intervention?
A: Glecaprevir / pibrentasvir
B. IV steroids
C: IVIG
D: HD
A: Glecaprevir/Pibrenasvir (Mavyret)
This patient is presenting with essential mixed cryoglobulinemia.
The skin involvement is classic.
In the setting of elevated cryoglobulins, low complement levels, and renal disease, the diagnosis can be made clinically.
The next best course of action is to treat underlying hepatitis C virus infection with a regimen approved for patients with renal dysfunction.
Of note, all current DAA that we use are okay in CKD
Harvoni (sofosbuvir/ ledipasvir) (genotypes 1,4,5,6)
Epclusa (sofosbuvir / velpatasvir)
Zepatier (elbasvir / grazoprevir) (genotypes 1,4)
Vosevi (sofosbuvir / velpatasvir / voxilaprevir )
100
A 53-year-old woman, with a history of chronic hepatitis B virus (HBV) receiving entecavir and type 2 diabetes mellitus taking insulin, is referred for evaluation of chronic HBV and elevated liver test results.
She has no symptoms at present, and her examination is unremarkable.
Liver ultrasound does not show any liver masses.
Laboratory results are shown below in Figure 1. 
What is your next step in managing this patient?
A: Elastography with CAP measurement
B: Assessment for viral resistance
C: Add Tenofovir
D: Multiphase CT liver
E: MRI
In patients with HBV and low HBV DNA levels but elevated liver test results, other etiologies of liver disease should be entertained.
In this patient with risk factors for nonalcoholic fatty liver disease, nonalcoholic steatohepatitis could be a possible etiology for liver test result elevations.
100
Which one of the following is most consistent with a diagnosis of wilsons disease?
A. High ALP, ALT/AST ratio of 2:1, high urinary copper > 100
B. Low ALP, AST/ALT ratio of 2:1, high urinary copper > 100
C. High ALP, AST/ALT ratio of 2:1, high urinary copper > 100
D. Low ALP, AST/ALT ratio of 2:1, Low urinary copper < 100
B.2. Low ALP, AST/ALT ratio of 2:1, high urinary copper > 100
Lab anomalies seen with wilsons can sometimes mimick AH, with the exception of low ALP. Ceruloplasmin alone is a good screening test, but traditional lab cutoff of < 20 can be seen in a variety of liver diseases. A Chinese cohort study found that a ceruloplasmin level below 13 mg/dL had a sensitivity of 97.0% and a specificity of 96.1% for diagnosing WD.
100
After TIPS, what is the goal HVPG in mmHg to eliminate the need for continuation of NSBB long term?
12 mmHg or 50% decrease from baseline. If this level is not reached, may still need to continue NSBB long term
100
A 50-year-old woman presents with fatigue, nausea, and dark urine.
Three months before, she had been diagnosed with acute hepatitis A and recovered completely.
On examination, she has mild scleral icterus, and her abdomen is nontender and nondistended without fluid wave.
Her laboratory results are below in Figure 1. 
Which test will help identify this patient's most likely cause of elevated LAE's?
ASMA
Reasoning: Hepatitis A virus can trigger autoimmune hepatitis, and it is important to recognize this association.
Autoantibodies for autoimmune hepatitis can be elevated and should be checked, after which a liver biopsy should be obtained.
200
A 22-year-old man presents to the emergency department with jaundice and abdominal pain.
Clinical examination reveals no stigmata of chronic liver disease.
The patient notes he works as an emergency medical technician and had a needle stick injury approximately 5 weeks ago for which he did not file a report or seek medical attention.
He drinks 3 beers daily and more on the weekends.
His history is notable for vaccination to hepatitis A virus and hepatitis B virus.
Laboratory results are shown below in Figure 1.
Which one of the following tests should you order next?
1. ETOH level
2. Hep A IgG
3. Hep C PCR
4. ANA
5. Iron Panel
This patient's clinical presentation with elevated aminotransferases greater than 10 to 20 times the upper limit of normal in the setting of a possible high-risk exposure make acute hepatitis C viral infection high in the differential diagnosis.
He is vaccinated for hepatitis A and B viruses, and the serology are consistent with this.
Antibodies to hepatitis C virus are not typically detectable until 8 weeks from exposure, and an evaluation of viral load is essential to establish the diagnosis
200
A 42-year-old Chinese woman presents for follow-up in clinic after recently completing treatment for non-Hodgkin lymphoma, a regimen that included rituximab.
She has a history of immune-tolerant chronic hepatitis B virus (HBV) and was not receiving treatment before starting chemotherapy.
She began HBV prophylaxis with tenofovir 2 weeks before initiation of chemotherapy.
Prechemotherapy laboratory results are shown below in Figure 1.
What do you recommend regarding her HBV prophylaxis?
A Continue indefinitely
B Continue for 12 months after finihsing chemo
C Continue for 6 months after finishing chemo
D Measure DNA at 3 months after completion of chemo. If DNA elevated, tx indefinitely
E Stop Now
For patients who begin HBV prophylaxis forvthe reactivation of HBV in the setting of immunosuppression, HBV prophylaxis should be continued for 12 months after cessation of immunosuppressive agent(s).
200
A 57-year-old man presents to the emergency department with a complaint of right upper quadrant (RUQ) pain, fever, chills, and jaundice for 5 days.
He reports no dysphagia or odynophagia, no diarrhea or constipation. His vital signs are as follows: heart rate, 100 bpm and regular; blood pressure, 145/70 mmHg; respiratory rate, 14 breaths per minute; oxygen saturation 98% on room air.
His examination is remarkable for moderately tender hepatomegaly.
Laboratory tests reveal the following shown below in Figure 1.
RUQ ultrasound revealed a normal gallbladder without gallstones, pericholecystic fluid, and gallbladder wall thickening.
His common bile duct was 3 mm, the liver was enlarged, and no liver masses were seen.
The spleen was also mildly enlarged. Doppler study showed normal flow in the hepatic and portal veins.
Upon questioning, his family reports that he drinks 1 to 2 pints of hard liquor daily. 
Which of the following treatments is the best option for this patient?
A. Steroids
B. Pentoxyfylline
C. NAC
D. Transplant
In a patient with a history of heavy alcohol use, the diagnosis of acute alcohol-associated hepatitis (AH) is suggested by the onset or sudden worsening of jaundice with or without other signs of hepatic dysfunction.
Physical examination in AH may reveal stigmata of liver cirrhosis, as well as systemic inflammatory response syndrome criteria, due to the significant inflammatory response in the liver.
Laboratory findings include an elevated bilirubin, and an AST/ALT ratio greater than 1.5 with a total AST or ALT less than 400 IU/L. Biopsy is not typically performed, but will show macrovesicular steatosis, a neutrophilic lobulitis, the presence of Mallory-Denk bodies, and pericellular fibrosis.
Unfortunately, these findings are also seen in nonalcoholic steatohepatitis, so an alcohol history is a crucial part of the workup. Current guidelines define “Definite AH” by the biopsy findings but allow a diagnosis of “Probable AH” if the following criteria are met: 1) Heavy alcohol use for greater than 5 years; 2) active alcohol use until 4 weeks before presentation; 3) sudden onset or worsening of jaundice; 4) AST/ALT greater than 1.5 with levels less than 400 IU/L; and 5) absence of other causes of liver disease. In patients with AH, prognosis can be estimated using the Model for End-Stage Liver Disease (MELD) score or the Maddrey Discriminant Function (DF); a DF greater than 32 predicts a 20-50 percent 30-day mortality and patients meeting this threshold should be treated as by definition they have severe AH.
Current US guidelines call for the use of corticosteroids (specifically prednisolone) in severe AH.
Response to steroid therapy is assessed at day 4 or 7 using the Lille score, with therapy continued for a total of 28 days in initial responders. Pentoxifylline had shown possible benefit in earlier studies, but more recent data show no benefit, and this is no longer recommended in the most recent US guidelines.
There is limited data to support the addition of N-acetylcysteine to corticosteroids in AH, though there is no role for this agent as a monotherapy. Liver transplantation for AH should be considered, but only in patients refractory to medical therapy.
200
According to the Baveno VII guidelines, what is the recommended management approach for a patient with non-malignant partially occlusive portal vein thrombosis (PVT) in the setting of cirrhosis, with compensated liver function and no clinical signs of decompensation?
A. Immediate liver transplantation
B. Observation without anticoagulation
C. Initiation of anticoagulation therapy
D. Surgical thrombectomy
Correct Answer: B. Observation without anticoagulation
Rationale: The Baveno VII guidelines suggest that for patients with non-malignant PVT in the setting of compensated cirrhosis and no clinical signs of decompensation, observation without anticoagulation is often recommended, as PVT may be asymptomatic and anticoagulation therapy can increase the risk of bleeding in cirrhotic patients. Anticoagulation is typically reserved for those with progressive thrombosis, symptoms, or decompensation
It is important to classify based upon
1. acuity
2. extent of thrombosis (partially, non or fully occlusive)
3. response to treatment.
These criteria will help you to determine need to start anticoagulation
200
A 67-year-old woman, with history of a renal transplant 1 year before, had routine labs after she returned from a trip to China.
She was feeling tired and complained of mild nausea but no vomiting.
Initial laboratory studies reveal the following results shown below in Figure 1.
Given her history of recent travel and renal transplant, hepatitis E virus (HEV) IgM was obtained and returned positive.
Six months later, HEV IgM and HEV polymerase chain reaction (PCR) are both positive.
Patient does not have any symptoms.
What do you recommend to this patient?
A Start Ribavirin
B Start IFN-A
C Reduce immunosuppresion
D Reassure
E Recheck Hep E PCR in 3-6mo
The initial management of chronic HEV infection in solid organ transplant recipients is reduction of immunosuppression.
If, after reduction of immunosuppression, there is still detectable HEV, ribavirin is first-line treatment.
300
A 32-year-old woman with chronic hepatitis C virus (HCV) infection genotype 4 presents to the clinic to discuss treatment.
She has a history of HIV and is receiving therapy.
She exercises regularly and does not drink alcohol. She takes a statin for dyslipidemia.
Liver elastography indicates F0 fibrosis.
Her HCV viral load is 432,000 IU/mL. What factor increases her risk of fibrosis progression?
A Dyslipidemia
B Genotype 4
C HIV
D High viral load
Several risk factors exist for progression of fibrosis including coinfection with HIV or hepatitis B virus.
Viral load does not correlate with disease progression.
Other risk factors include presence of fibrosis, older age, previous transplantation, alcohol consumption, obesity, and insulin resistance.
Patients with HIV and HCV coinfection are at higher risk of fibrosis progression and should be targeted for screening and treatment
300
What is the overall viral resistance rate for patient's receiving tenofovir or entacavir?
A. 1%
B. 5%
C. 10%
D. 20%
2% entacavir
Very rare tenofovirIf patients on these medications have rising DNA Hep B levels, first question their compliance. Then consider viral resistance if they swear on compliance.
300
A 51-year-old man with an 8-year history of alpha-1 antitrypsin (A1AT) deficiency is found to have the following laboratory results shown below in Figure 1.
Liver biopsy shows established cirrhosis and periodic acid-Schiff (PAS)-positive, diastase-resistant globules in the hepatocytes.
What is the mechanism of liver injury in this disorder? A. Deficient A1AT leads to hepatocyte damage by enzymes that would normally be deactivated by this enzyme
B.Deficient A1AT leads to neutrophilic infiltration of the liver and hepatocyte necrosis
C.Excess misfolded A1AT accumulates in the liver, leading to lymphocytic infiltration of the liver and subsequent fibrosis
D. Excess misfolded A1AT accumulates in the liver, leading to oxidative damage to hepatocytes
A1AT deficiency is a common metabolic cause of liver disease in children and adults, affecting approximately 1 in 3500 live births.
Production of a misfolded A1AT protein leads to low A1AT levels in the serum (<15% of normal), which leads to lung damage via the unopposed action of pulmonary neutrophil elastase. In the liver, however, damage is caused by accumulation of the abnormal protein in the endoplasmic reticulum of hepatocytes, causing oxidative damage and eventual fibrosis and cirrhosis.
On biopsy, the misfolded protein appears as PAS-positive globules within hepatocyte that are resistant to digestion with diastase. A1AT should be suspected in any patient with unexplained cirrhosis or unexplained mild elevations of AST and ALT.
A clinical clue to A1AT deficiency is a personal or family history of early chronic obstructive pulmonary disease (before age 50).
Although a low A1AT level in the serum can suggest the diagnosis, this enzyme is an acute phase reactant and the A1AT level may be falsely elevated in the setting of inflammation.
The gold standard of diagnosis is A1AT phenotyping using isoelectric focusing. This testing allows identification of the abnormally folded protein (the S or Z forms) instead of the normal protein (the M form).
Patients with the P*ZZ phenotype express 10 to 20 percent of the normal A1AT and tend to have the most severe disease.
300
A 43-year-old man undergoes liver transplantation for cirrhosis due to primary sclerosing cholangitis.
His donor was positive for cytomegalovirus.
His surgery is uncomplicated, with peak laboratory test results following reperfusion noted in the table below (Figure 1).
He has conventional arterial anatomy with a choledochocholedochostomy (duct-to-duct) biliary anastomosis.
On day 1 after his operation, his liver enzymes rise dramatically to peak, as noted in the table shown (Figure 1).
A liver ultrasound with dopplers reveals no flow through his hepatic artery. He returns to the operating room for surgical thrombectomy and hepatic artery reconstruction.
Which of the following is most likely to be longterm sequelae from this ischemic injury?
A. Biliary anastomotic stricture
B. Biliary non-anastomotic stricture
C. Portal Hypertension
D. Hepatic fibrosis
The liver receives 70% of its blood supply from the portal vein and 30% from the hepatic artery, though bile ducts are disproportionately dependent upon arterial blood supply and therefore particularly susceptible to arterial ischemic injury.
Ischemic cholangiopathy is a rare condition of bile duct injury due to impaired blood supply.
It is most commonly associated with liver transplantation and with the administration of hepatic intraarterial chemotherapy, but it is also seen in AIDS cholangiopathy, polyarteritis nodosa, and hereditary hemorrhagic telangiectasia.
Clinical features can range from asymptomatic liver enzyme abnormalities (mainly alkaline phosphatase and gammaglutamyltransferase) to progressive cholestasis with jaundice. Ischemic stenosis of the common bile duct can also present as acute obstructive jaundice or cholangitis, though more commonly, this global arterial ischemia results in the finding of diffuse biliary strictures.
Although anastomotic strictures can occur, these are rarely in isolation if there has been a significant arterial ischemia event.
Despite significant rise in transaminases, acute hepatic necrosis is rare in hepatic artery thrombosis and in general, ischemic injury to the liver, whether from thrombosis or low flow states, leads to a rapid rise and fall of transaminases with no chronic hepatocyte injury consequent to the transient ischemia.
300
A 23-year-old woman presents to you for evaluation of elevated liver enzymes.
She is originally from East Africa and has a family history of liver disease and heart failure of unclear etiology.
Her laboratory results include the following shown below in Figure 1.
HFE gene mutation testing is negative, but liver biopsy confirms significant iron deposition in the hepatocytes (hepatic iron index, 2.7).
Which subtype of hereditary hemochromatosis (HH) is most likely present in this patient?
A. Type 1
B. Type 2
C. Type 3
D. Type 4
Hemochromatosis is subdivided into 4 types based on genetic mutations present.
Type 1 (hereditary) represents disease due to HFE gene mutations and comprises 95% of all HH cases. Non-HFE gene mutation types of hemochromatosis include type 2 to 4 and occur in less than 5% of cases.
All gene mutations impact the hepcidin-ferroportin axis.
Hepcidin is produced by the liver and acts on enterocytes and macrophages to provide negative feedback to block ferroportin excretion of iron into the blood stream. Type 1, 2 (juvenile), and 3 (TFRrelated) hemochromatosis all involve genetic defects that impair synthesis or function of hepcidin resulting in low hepcidin levels and loss of negative feedback regulation of ferroportin excretion of iron into the circulation.
Type 4 (ferroportin disease) (AKA FerroFOURtin disease) hemochromatosis involves mutations in ferroportin itself, making it no longer susceptible to the negative feedback signal of hepcidin, and therefore, results in a high level of hepcidin but with excess iron excreted into the circulation. Notably, type 4 hemochromatosis is autosomal dominant and though rare, it is prevalent in African countries.
Mutations in the SLC40A gene for ferroportin impact disease presentation; however, in instances where tissue iron overload is confirmed, as in this case, phlebotomy is indicated.
400
A 65-year-old man underwent liver transplantation with a liver from a deceased, hepatitis C virus (HCV) viremic donor 6 weeks before presentation to the clinic.
The patient has had a complicated postoperative course with severe organ rejection.
He received thymoglobulin for induction.
He presents to the clinic with complaint of jaundice but imaging results showing a normal liver.
Laboratory results are listed below in Figure 1.
What should you do next?
1. IV solumedrol
2. Liver Biopsy
3. increase tacrolimus to goal trouh 10-12
4. MRCP
5. ERCP
This patient has fibrosing cholestatic HCV, which should be diagnosed by a combination of liver histology, clinical assessment, and HCV viral load assessment, which is typically high.
This complication can occur in the setting of chronic HCV and is most closely associated with immunosuppression. It is typically associated with rapidly progressive cholestatic hepatitis and high HCV viral load.
Diagnosis is made with histology, and treatment with direct-acting antiviral therapy can improve outcomes. Prompt recognition and therapy is essential.
When suspicion exists, particularly in patients who have undergone liver transplantation, biopsy should always be obtained before starting empiric therapy for acute cellular rejection.
400
A 22-year-old man with history of injection drug use and a diagnosis of chronic hepatitis B virus (HBV) infection receiving tenofovir for 3 years presents to clinic for routine follow-up.
He has laboratory testing after his visit.
Laboratory results are shown below in Figure 1. 
What should you do next?
1. Fibroscan
2. RUQUS
3. Hep C tx
4. Hep A Vaccination
5. Multiphase CT
Patients with HBV/hepatitis D virus coinfection should receive surveillance for hepatocellular carcinoma.
Patient is already immune to hepatitis A virus.
Hepatitis C viremia should be confirmed with hepatitis C virus polymerase chain reaction. Multiphase computed tomography is not indicated in this patient.
400
A 32-year-old Black woman presents to you for evaluation of asymptomatic elevations in her liver-associated enzymes.
Her total bilirubin level is normal.
She has no other past medical history and does not drink alcohol or smoke tobacco.
She complains of fatigue and a chronic nonproductive cough.
Abdominal ultrasound reveals an enlarged and heterogeneous-appearing liver with patent vasculature as well as splenomegaly.
Chest radiography reveals bilateral hilar adenopathy and subtle interstitial changes. Her laboratory results demonstrate the table shown below in Figure 1 
Which of the following would most likely be the next step to establish this patient's diagnosis?
A. ACE level
B. MRCP
C. Liver Biopsy
D. Response to steroids
This patient has hepatic sarcoid. With the liver test abnormalities, liver biopsy may be recommended to establish the presence of noncaseating granulomas.
As clinically important liver injury is rare, treatment with corticosteroids in the absence of symptoms (50-80% of patients are asymptomatic) often does not impact long-term outcomes as it is unclear whether or not steroids prevent, halt, or reverse disease progression.
Moreover, there is emerging evidence to suggest weight-based ursodiol (10-15 mg/kg daily) as first-line therapy for 3 months before the initiation of corticosteroids.
Hepatic sarcoid alone does not require initiation of treatment with corticosteroids.
Although serum angiotensin converting enzyme level may be elevated in patients with hepatic sarcoid, this is not specific to this condition in that it may be elevated in other diseases.
Magnetic resonance cholangiopancreatography would be less helpful with no mention of bile duct dilation on the abdominal ultrasound.
400
A 31-year-old previously healthy woman presents to the emergency department with 3 days of abdominal pain and abdominal distention.
She denies fevers, nausea, vomiting, melena, or hematochezia.
She denies any alcohol or drug use.
She takes no medications or supplements.
On examination, she is alert and oriented. Her heart rate is 110 bpm, blood pressure is 110/70 mmHg, and temperature is 37.2°C. Her abdominal examination shows shifting dullness, fullness at the flanks, and hepatomegaly.
Laboratory testing reveals the following results shown below in Figure 1.
Abdominal ultrasound with Doppler shows caudate lobe hypertrophy, no blood flow in the middle hepatic vein, and partial thrombosis of the main portal vein.
(Figure 1) Which of the following is the best test to establish this patient's diagnosis?
A. Factor V leiden mutation
B. JAK2 V617F mutation
C. Protein C deficiency
D. Protein S defiency
E. Prothrombin 20201A mutation
This patient has Budd-Chiari syndrome (BCS) in the setting of a polycythemia and thrombocytosis, each of which are hypercoagulable states. BCS is a rare disease and can be primary or secondary with compression or invasion by a lesion originating outside of the veins from a mass, hepatic abscess or liver cyst.
Concomitant PVT is common in patients with BCS. Primary BCS typically occurs in the setting of an underlying risk factor.
Myeloproliferative disorders can be found in as many as 50% of patients with BCS. In the absence of a major provoking factor such as in this patient, the following testing should be considered in collaboration with a hematologist: JAK2 V617F mutation, antiphospholipid antibodies, and flow cytometry to rule out paroxysmal nocturnal hemoglobinuria. If JAK2 V617F mutation is negative, testing for the CALR mutation can be considered.
Testing for heritable thrombophilia, such as Factor V Leiden mutation, Protein C or S deficiency or prothrombin gene mutation is not routinely recommended as the results generally do not influence management and Protein C and S can be low in the context of acute thrombosis such as in this patient.
400
A 23-year-old woman presents to her local emergency department with confusion.
On examination she is noted to be febrile with altered mental status and asterixis.
Her past medical history includes a recent diagnosis of lymphoma, and she is currently undergoing treatment with chemotherapy.
Her laboratory results are shown in the table below in Figure 1.
Imaging with ultrasound demonstrates a normal appearing liver without vascular or biliary abnormalities. 
Which of the following therapies should you order?
A Acyclovir
B Mannitol
C Oseltamivir
D Prednisone
E Rifaximin
Herpes simplex virus can cause severe hepatitis and acute liver failure that is highly fatal.
Prompt recognition and early therapy with acyclovir is essential.
Diagnosis is challenging as the disease is rapidly progressive. Immunocompromised patients and women in the third trimester of pregnancy are most affected; however, immunocompetent individuals may also be affected.
Herpes simplex virus skin lesions are not always present, and characteristic features on laboratory tests include significantly elevated transaminases and typically low bilirubin level
500
A 65-year-old Black man with decompensated nonalcoholic steatohepatitis cirrhosis is listed for liver transplantation with a MELD (Model for End-Stage Liver Disease) score of 35.
The patient is transplanted with a graft from a hepatitis C virus (HCV) viremic donor.
His course was complicated by atrial fibrillation requiring amiodarone.
A viral load and genotype are obtained at week 4 after transplantation revealing HCV genotype 1a.
What is the best management plan after liver transplantation for the patient's HCV infection in this setting?
A.Glecaprevir/pibrentasvir (MAVYRET) for 12 weeks
B. Repeat viral load at 6 months to assess for spontaneous clearance C. Sofosbuvir/velpatasvir(EPCLUSA) for 12 weeks
D. Sofosbuvir/ledipasvir (Harvoni) for 24 weeks
E. Treatment is contraindicated after liver transplantation in this setting
Due to organ shortages and the advent of direct-acting antiviral therapy, centers are increasingly using HCV viremic donors for transplantation into HCV-negative recipients.
Experience thus far indicates excellent outcomes; however, informed consent and counseling is essential.
Early therapy within the first month is recommended when the patient is stable.
Particular attention to drug interactions after transplantation is imperative as interactions, amiodarone in this case, require selection of specific drug secondary to interactions.
Sofosbuvir and amiodarone should not be coadministered
500
A 32-year-old woman presents to clinic for evaluation of chronic hepatitis B virus (HBV) and is currently 14 weeks pregnant with her first child.
She is referred for advice on management of HBV during her pregnancy.
Laboratory results are shown in the table below (Figure 1).
What is the best management plan with regards to HBV treatment?
A. Start Entacavir NOw
B. Start tenofovir Now
C: Check Hep B DNA in 2nd trimester and treat if DNA > 200,000
D: C section
E: Give Hep B IVIG after delivery
B. Start tenofovir now.
If a patient has an indication for HBV treatment, she should be treated whether or not she is pregnant.
This patient has immune-active chronic HBV.
Tenofovir disoproxil fumarate is preferred in pregnancy.
The mainstay of prevention of mother-to-child transmission is HBV vaccination of the infant.
500
A 39-year-old man, with an 8-year history of chronic obstructive pulmonary disease (COPD), is found to have the following laboratory results shown below in Figure 1.
He is a current smoker (1 pack per day) and drinks 2 to 3 beers most days within increased use on weekends.
Right upper quadrant ultrasound revealed a normal gallbladder with no gallstones, common bile duct was 3 mm, liver was of small size with a nodular contour, no liver masses were seen, and Doppler study showed normal flow in the hepatic and portal veins.
Moderate splenomegaly was present.
Hepatitis serologies, antinuclear antibody, anti-smooth muscle antibodies, iron studies, and serum ceruloplasmin are all negative, but an alpha-1-antitrypsin level (A1AT) is less than 10.
SERPINA 1 protein testing was negative
Which of the following tests will reveal the likely cause of this patient's liver disease?
A. 24 hour urine copper
B. A1AT phenotype
C. AMA
D. C282Y mutation anaylsis
E. PETH level
laboratory testing and imaging consistent with cirrhosis.
Although in this case, the patient does have pulmonary disease from A1AT deficiency likely exacerbated by smoking, the absence of detectable SERPINA1 protein suggests a null mutation. In patients with A1AT deficiency from null mutation, pulmonary disease exists in the absence of liver disease since no protein is present.
This differs from PI*ZZ or other abnormal mutation compound heterozygotes for which serum levels of A1AT remain low with accumulation of abnormal protein in the liver that drives hepatic inflammation.
In this case, his alcohol use has been significant and is the likely cause of his underlying liver disease, a finding that could be confirmed with phosphatidylethanol testing.
Phosphatidylethanol is a metabolite of ethanol that is incorporated in red blood cell membranes and is sensitive enough to detect moderate-heavy alcohol use in the past 30 days.
Given normal iron studies, hemochromatosis is unlikely and additional HFE gene mutation testing is not indicated. Liver enzymes are not consistent with primary biliary cholangitis and therefore antimitochondrial antibody testing is not indicated.
Given normal ceruloplasmin, additional 24-hour urine copper testing is not indicated.
500
A 34-year-old woman undergoes a hematopoietic stem cell transplant (HSCT) for acute myeloid leukemia.
She receives myeloablative-conditioning therapy with busulfan and cyclophosphamide.
On day 15 after HSCT, she develops jaundice, right upper quadrant pain, and ascites.
She undergoes transjugular liver biopsy with normal hepatic vein venogram and hepatic venous pressure gradient of 18 mmHg.
Histology reveals congestion in zone 3 with associated hemorrhage and collagen deposition within the central vein.
What is the best treatment for this patient?
A. Defibrotide
B. Heparin
C. Thrombolysis
D. Liver Transplant
E. TIPS
This patient presents with classic symptoms of sinusoidal obstruction syndrome (SOS) following high-intensity myeloablative-conditioning therapy for HSCT.
Busulfan and cyclophosphamide are both associated with higher risk of SOS compared with lower intensity, nonmyeloablative-conditioning regimens.
SOS develops within the first month after HSCT, and in the case of cyclophosphamide conditioning, typically within 20 days.
This patient meets diagnostic clinical criteria based on Seattle Criteria (at least 2 of following criteria within 20 days of HSCT: total bilirubin >2 mg/dL, hepatomegaly or right upper quadrant pain, >2% weight gain).
In addition, hepatic venous pressure gradient (HVPG) more than 10 mmHg is highly specific for SOS (91%) and histology is consistent showing classic early findings of zone 3 congestion and early collagen deposition that subsequently progresses to nonthrombotic occlusion of central veins and sinusoids.
Aside from diuretic therapy, treatment options are limited. Anticoagulation alone or in conjunction with thrombolysis has been studied with high rates of bleeding and little clinical benefit (29% survival) and is not recommended. Transjugular intrahepatic portosystemic shunt (TIPS) is effective for treating portal hypertension (mean reduction in HVPG from 20.2 mmHg to 6.4 mmHg) but does not improve survival (3-month mortality, 87.5%) and is not recommend for severe SOS after HSCT. There is limited evidence to support TIPS in SOS following solid organ transplant.
Liver transplantation for SOS can be considered in patients with benign conditions or malignancy with favorable prognosis but would not be considered in this patient having just undergone therapy for acute myelogenous leukemia.
Defibrotide is a single-stranded polydeoxyribonucleic acid with fibrinolytic, antithrombotic, and antiischemic properties. Defibrotide (6.25 mg/kg) given for at least 14 days led to complete remission in up to 55% of patients and survival past day 100 after HSCT of 43%, both significantly improved compared with historical controls.
The US Food and Drug Administration approved defibrotide for the treatment of severe SOS in April 2016.
There is also data to support defibrotide for prophylaxis of HSCT though this is currently limited to the pediatric population.
500
53 YOM with ETOH cirrhosis and chronic pancreatitis comes in for hematemesis. Before this visit, he was decompensated by HE, but was no longer drinking. He is rescucitated and started on octreotided and ppi IV BID. Urgent EGD is performed that shows medium EV without any high risk stigmata and Large IGV-1 with stigmata of recent bleeding. Which of the following would be best suited for this patient?
A. EVL
B. TIPS
C. NSBB
D. BRTO
D. BRTO
EVL not as succeful as Glue injection under EUS given submucosal location of GV's. TIPS would be helpful for both EV and GV, however his hx of HE would be a relative contraindication. BB alone would not be sufficient given high risk nature of his GV. BRTO would address both the GV and lower the incidence of HE given decreased portosystemic shunting of blood