List drugs to avoid in patients with ascites due to cirrhosis

AASLD Guidelines

1. angiotensin-converting enzyme inhibitors

2. angiotensin receptor blockers

3. Propranolol

4. Prostaglandin inhibitors (like NSAIDS) due to their effect on lowering sodium excretion and risk of bleeding

Reason is that arterial pressure independently predicts survival in patients with cirrhosis. MAP >82 mmHg have a 1 yr survival of 70% versus 40% in those with <82 mmHg

What are midodrine's effects on ascites?

AASLD Guidelines

increase urine volume,
urine sodium excretion, MAP, and survival

can be
added to diuretics to increase blood pressure and convert
refractory ascites back to diuretic sensitive.

What is the effect of albumin infusion after large volume paracentesis?

 AASLD Guidelines

Lowers the odds ratio of death of 0.64 in albumin group

Albumin infusion (6-8 g/liter of fluid removed) is recommended when
more than 5 L of ascitic fluid are removed.

What change in bacterial flora has resulted from increased use of quinolones in prevention of spontaneous bacterial peritonitis

 AASLD Guidelines

There are more Gram-positives and extended-spectrum
B-lactamase-producing Enterobacteriaceae in recent
years.

Increased resistant organisms which is associated with a higher mortality and post transplant complications

What are the primary tests to be done on ascitic fluid

 AASLD Guidelines

Cell count and differential

albumin

total protein

Optional tests as indicated:

culture in blood culture bottle

glucose

LDH

amylase

gram's stain

What are symptoms suggesting spontaneous bacterial peritonitis?

 AASLD Guidelines

fever, abdominal pain, or unexplained encephalopathy,
acidosis, azotemia, hypotension, or hypothermia

So generally, if someone with ascites comes to clinic looking pretty sick or sudden change, think SBP.

What type of ascites is present if the serum-ascites albumin gradient (SAAG) 1.3?

 AASLD Guidelines

If > 1.1 --> most likely portal hypertension or cardiac

First line treatment of cirrhosis and ascites

 AASLD Guidelines

• Sodium restricted diet and diet education

  ---

• Cessation of alcohol use, when present
• Dual diuretics, usually spironolactone and furosemide, orally with single daily dosing
• Discontinue non-steroidal anti-inflammatory drugs
• Evaluation for liver transplantation

Second line treatment of cirrhosis and ascites?

AASLD Guidelines

• Discontinue beta blockers, angiotensin converting enzyme inhibitors, and angiotensin receptor blockers
• Consider adding midodrine especially in the profoundly hypotensive patient
• Serial therapeutic paracenteses
• Evaluation for liver transplantation
• Transjugular intrahepatic portasystemic stent-shunt (TIPS)

What is the goal urinary sodium excretion in treatment of cirrhosis with ascites?

AASLD Guidelines

increase urinary excretion of sodium so that
it exceeds 78 mmol per day (88 mmol intake per day -
 10 mmol nonurinary excretion per day)

random spot urine: sodium concentration is greater than the potassium concentration (ratio of urinary sodium/urinary potassium >1)

hyponatremia treatment

2016 Transplant Review Course

• Vaptans (vasopressin receptor antagonists)
• can correct mild hyponatremia
• not useful in treatment of ascites
• most patients can tolerate mild hyponatremia
• fluid restriction if there is severe hyponatremia

Which diuretics in cirrhosis with ascites?

AASLD Guidelines

• Start with both spironolactone and lasix (100:40 mg ratio)
• if there is significant hypokalemia, start with just spironolactone
• if renal disease, then may need less spironolactone due to hyperkalemia risk

What is the treatment of refractory ascites?

AASLD Guidelines

• sodium restriction
• high dose spironolactone and lasix (400/160)
• oral midodrine increases urine volume, urine sodium, MAP, survival. can be added to

Name the diagnostic criteria for spontaneous bacterial peritonitis: 

AASLD Guidelines

• presence of an elevated ascitic fluid absolute polymorphonuclear leukocyte (PMN) count (i.e., 250 cells/mm3 [0.25  109/L])
• without an evident intra-abdominal, surgically treatable source of infection

define culture negative neutrocytic ascites:

AASLD Guidelines

• ascitic fluid PMN count of >250 cells/mm³
• culture negative
• no prior antibiotic treatment
• no other explanation like hemorrhagic ascites, peritoneal carcinomatosis, pancreatitis, or peritoneal tuberculosis

Define monomicrobial nonneutrocytic bacterascites:

AASLD Guidelines

• positive bacterial ascitic culture
• low or no neutrophil response (count <250 cells/mm³)

  ---

  Most patients (62%) will resolve the colonization without antibiotics and without a neutrophil response. If there are signs and symptoms of infection at the time of paracentesis, then treatment should be started until culture results are known regardless of PMN count.

What antibiotic choice is best for initial empiric treatment of spontaneous bacterial peritonitis?

AASLD Guidelines

Cefotaxime or a similar third-generation cephalosporin
appears to be the treatment of choice for suspected
SBP; it used to cover 95% of the flora including
the 3 most common isolates:

Escherichia coli, Klebsiella
pneumoniae, and Streptococcal pneumoniae

5 days of treatment is as efficacious as 10 days.

What are the three most common bacteria associated with SBP?

AASLD Guidelines

E. coli

Klebsiella pneumoniae

Streptococcal pneumoniae

Should patients with SBP be treated with albumin in addition to antibiotics?

AASLD Guidelines

• serum creatinine is > 1 mg/dL
• BUN >30 mg/dL
• TB >4 mg/dL

How do you separate SBP from secondary peritonitis due to a surgically treatable intraabdominal source?

AASLD Guidelines

• PMN count greater than or equal to 250 cells/mm3 (usually many thousands)
• multiple organisms (frequently including fungi and enterococcus) on Gram’s stain and culture

Is a follow up paracentesis necessary in the treatment of bacterial peritonitis?

AASLD Guidelines

Repeat paracentesis is only indicated if the setting, symptoms, analysis, organism(s), or response are atypical, repeat paracentesis can be helpful in raising suspicion for secondary peritonitis and further evaluation. In secondary peritonitis the PMN count rises after 48 hours of treatment in perforation and nonperforation secondary peritonitis.

What can be done to prevent SBP?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• Patients with previous bouts of SBP are at risk for recurrence in 1 yr (69%). Use of PPIs has been shown to increase risk, so PPIs should be discontinued if there is not a clear indication.
• Treatment with antibiotics at time of variceal bleeding (either norfloxacin po 400 mg BID for 7 days or ceftriaxone IV 1 g/d for 7 days)

What are the diagnostic criteria for

hepatorenal syndrome?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

(1) cirrhosis with ascites

(2) serum creatinine greater than 1.5 mg/dL

(3) no improvement of serum creatinine (decrease to a level of 1.5 mg/dL or less) after at least two days with diuretic withdrawal and volume expansion with albumin (The recommended dose of albumin is 1 g/kg/day up to a maximum of 100 g/d

(4) absence of shock

(5) no current or recent treatment with nephrotoxic drugs

(6) absence of parenchymal kidney disease as indicated by proteinuria >500 mg/day, microhematuria (>50 red blood cells per high power field), and/or abnormal renal ultrasonography.

Explain the two types of HRS?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Type 1: rapidly progressing (within 2 weeks) reduction in renal function defined by doubling of initial serum creatinine to >2.5 mg/dL or a 50% reductiton of creatinine clearance to <20 mL/min.

Type 2: not a rapidly progressive course more commonly associated with death.

Histologic lesion associated iwth HRS: glomerular tubular reflux

What biomarker is useful for making the diagnosis of HRS in patients with cirrhosis?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Urinary neutrophil gelatinase-associated lipocalin

 20 ng/mL creatinine in normal controls

20ng/mL in pre-renal azotemia

50 ng/mL in chronic kidney disease

105 ng/mL in hepatorenal syndrome

325ng/mL in acute kidney injury

(recently the US manufaturer ceased production)

What treatments can help to prevent HRS?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

1. Albumin infusions in the setting of SBP can prevent HRS

2. Pentoxifylline: helpful in preventing HRS in patiens with cirrhosis ascites, ad CrCl between 41-80 mL/min.

What are possible therapies for HRS?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• Hemodialysis: hemodialysis can often be associated with hypo-tension. Continuous venovenous hemodialysis/hemofiltration causes less hypotension
• Drug combination: albumin along with octreotide and midodrine. 20 grams of intravenous albumin per day for 20 days, plus octreotide with a target dose of 200 mg sub-cutaneously 3 times per day, and midodrine titrated up to a maximum of 12.5 mg orally 3 times per day to achieve an increase in mean blood pressure of 15 mm Hg.
• TIPS: possibly effective. needs more studies.
• Transplantation

What are the hallmark histologic findings in AIH?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• Interface hepatitis
• plasma cell infiltration is typical
  (Though, neither histological finding is specific for AIH)
• Eosinophils, lobular inflammation, bridging necrosis, and multiacinar necrosis may be present.
• Granulomas rarely occur.
• portal lesions generally spare the bile ducts.
• In all but the mildest forms, fibrosis is present, with advanced disease, bridging fibrosis or cirrhosis

What autoantibodies are typically seen in AIH?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

ANA, SMA, anti-LKM1, and anti-LC1 constitute the conventional serological repertoire for the diagnosis of AIH. In North American
adults, 96% of patients with AIH have ANA, SMA, or both, and 4% have anti-LKM1 and/or anti-LC1.

Anti-LKM1 are deemed more frequent in European AIH patients and are typically unaccompanied by ANA or SMA.

Anti-SLA are highly specific for the diagnosis of autoimmune liver
disease, and their detection may identify patients with more severe disease and worse outcome.

What is the only hereditary condition that is associated with AIH?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

AIH may be present in patients with multiple endocrine
organ failure, mucocutaneous candidiasis, and
ectodermal dystrophy. [genetic disorder autoimmune polyendocrinopathy-candidiasis- ectodermal dystrophy (APECED)], caused by a single-gene mutation located on chromosome 21q22.3 that affects the generation of the autoimmune regulator (AIRE) protein. Autosomal recessive pattern of inheritance and lacks HLA DR associations and female predilection.

What are the most common autoimmune disorders that can occur concurrently with AIH?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Autoimmune thyroiditis, Graves’ disease, synovitis and ulcerative colitis are the most common immune- ediated disorders associated with AIH in North American adults,

Type I diabetes mellitus, vitiligo, and autoimmune thyroiditis are the most common concurrent disorders in European anti-LKM1+ AIH patients.

What percentage of children at the time of diagnosis of AIH type 1 had bile duct abnormalities?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are the absolute indications for corticosteroid therapy in AIH?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

1. serum AST of at least 10x ULN or > 5x ULN in conjunction with a serum gamma-globulin level >2x ULN have a high mortality (60% at 6 month) if untreated.

2. bridging necrosis or multilobular necrosis at pre-sentation progress to cirrhosis in 82% of untreated patients and associated with a 5-year mortality of 45%.

3. Incapacitating symptoms associated with hepatic inflammation, such as fatigue and arthralgia, are also absolute indications for treatment regardless of other indices of disease severity

What are indications for prednisone alone in AIH?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Prednisone is appropriate as the sole medication in individuals with severe cytopenia, those undergoing a short treatment trial (duration of therapy, <6 months), individuals who are pregnant or contemplating pregnancy, patients with some active malignancies, and individuals with known complete thiopurine methyltransferase deficiency

In which patients is combination therapy (steroids and azathioprine) recommended in the treatment of AIH?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Combination regimen is appropriate in patients who will be treated continuously for >6 months or who are at increased risk for drug-related complications, (postmenopausal women, emotional instability, osteo-porosis, brittle diabetes, labile hypertension, or obesity).

Patients on prednisone should undergo eye examinations for cataracts and glaucoma periodically, and those receiving azathioprine in any dose should be monitored at 6 month intervals for leukopenia and thrombocytopenia

What is the definition of remission in AIH?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• complete normalization of transaminases

• disappearance of symptoms

• normal bilirubin

• normal gamma gobulin levels

• normal hepatic tissue or inactive cirrhosis

Gradual withdrawal of prednisone over 6 week period. Serum AST or ALT, total bilirubin, and gamma globulin levels determined q 3 weeks during and for 3 months after drug withdrawal Repeat lab assessments q 6 months for a 1 year, and then every year life long

What is the recurrence rate of AIH post transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Recurrent AIH in LT allografts occurs in ~30% of adult and pediatric patients (range 12%-46%) with an average time to recurrence of 4.6 years. The incidence accelerates after discontinuation of steroids.

Diagnostic criteria for recurrence include:

(1) elevation of serum AST or ALT levels;

(2) persistence of autoantibodies;

(3) hypergammaglobulinemia and/or elevation of IgG level;

(4) compatible histopathological findings;

(5) exclusion of alternative etiologies; and

(6) responsiveness to steroids

What are contraindications to liver transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

MELD Score<15
Severe cardiac or pulmonary disease
AIDS
Ongoing alcohol or illicit substance abuse
Hepatocellular carcinoma with metastatic spread
Uncontrolled sepsis
Anatomic abnormality that precludes liver transplantation
Intrahepatic Cholangiocarcinoma
Extrahepatic malignancy
Fulminant hepatic failure with sustained ICP >50 mm Hg or CPP <40 mm Hg*
Hemangiosarcoma
Persistent noncompliance
Lack of adequate social support system

What are the obesity recomendations prior to transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

- Obese patients (WHO class 1 and greater) require dietary counseling prior to LT

- Class 3 obesity (BMI 40) is a relative contraindication
to LT

What mmHg is pulmonary hypertension noted and what levels are of prognostic significance in liver transplantation?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Pulmonary hypertension, elevation of the mean
pulmonary artery pressure (MPAP) >25 mmHg, occurring
in the presence of portal hypertension, is referred to as portopulmonary hypertension (PoPH).

Mild POPH, MPAP <35 mmHg, is not of major concern but moderate (MPAP 35 mmHg) and severe POPH (MPAP 45 mmHg) are predictors of increased mortality following LT

It is not correlated with the severity of or etiology of portal hypertension.

Does hepatopulmonary syndrome significantly affect mortality in LT patients?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Hepatopulmonary syndrome (HPS) resulting from
intrapulmonary microvascular dilation in the setting of chronic liver disease and/or portal hypertension leads
to arterial deoxygenation. Intrapulmonary shunting
demonstrated by contrast echocardiography or by 99mTC macro aggregated albumin (MAA) lung/brain
perfusion scanning.  LT offers a survival benefit in HPS, with 76% of LT recipients at the Mayo Clinic surviving 5 years compared to 26% of matched patients with  equivalent severity of hypoxemia and liver disease who were not transplanted. LT reverses HPS in almost all patients who survive more than 6 months, although perioperative mortality appears to be high in those with severe HPS, with a preoperative PaO2 <50 mmHg alone or in combination with an MAA shunt scan of greater than 20% predictors of increased mortality after LT.  experience indicates that more severe hypoxemia predicts the need for longer-term supplemental oxygen and a longer recovery rather than increased mortality

What MELD exception points are given to patients with HPS?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

When is combined liver kidney transplant recommended?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Simultaneous LK was sanctioned for:

(1) endstage renal disease (acute HRS etiology excluded) with cirrhosis;

(2) liver failure with chronic kidney disease (CKD) and GFR <30 mL/min,

(3) acute kidney injury or HRS with creatinine 2.0 mg/dL and dialysis for 8 weeks; or

(4) liver failure with CKD and renal biopsy demonstrating >30% glomerulosclerosis or >30% fibrosis.

What adverse events are seen in LT patients who smoke tobacco?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Cigarette smoking is implicated in a number of
adverse outcomes in LT recipients including cardiovascular
mortality and an increased incidence of hepatic artery thrombosis.

Oropharyngeal and other neoplasms following LT are also linked to cigarette smoking

What are rsk factors for osteoporosis in patients with cirrhosis?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Osteoporosis is frequent in patients with cirrhosis, up
to 55% in some studies.

This reflects risk factors common
in patients with cirrhosis including inactivity, inadequate
nutritional status, hypogonadism, chronic
cholestasis, and alcohol excess.

Can patients with HIV be transplanted?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Patients with HIV infection need to have a CD4 count >100/lL with a viral load anticipated to be completely suppressed at time of LT. Overall survival rates are similar to non-HIV-infected recipients, with the exception of HCV coinfected patients, in whom recurrent HCV leads to inferior outcomes. Factors implicated in the latter include BMI <21, combined liver/kidney transplant, and older donor age.

 AIDS is a contraindication.

Wha psychosocial determineants are necessary to be evaluated for a liver transplant recipient?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

evidence of compliance with medical directives, adequate support from able caregivers especially in the peri-operative period, and an absence of active psychiatric disorders with the potential to impact compliance or include behaviors harmful to health (e.g., alcohol, tobacco, or illicit drug use).

Depressive symptoms in the early postoperative period are associated with poorer outcomes post LT but is not an absolute contraindication to transplant.

Prior to the use of DAAs in the treatment of HCV inffection, what was the outcome in LT in this population?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• was the most common indication for LT for the same indications as others, decompensated cirrhosis and HCC
• tempo of HCV infection is accelerated post LT
• progression to cirrhosis in 20-30% of patients with graft failure due to recurrent HCV in 10% of HCV-infected recipients within 5-10 years of LT

What was the major factor(s) in decreasing recurrence of HBV post transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• HBV immune globulin (HBIG) as immunoprophylaxis post transpltant (prior to routine use of HBIG, recurrence rates were 80%)
• combination of HBIG with oral antivirals for HBV-infected patients to evolve from having the poorest posttransplant outcomes to rates among the best of all recipients. With this regimen, the 5-year graft survival for those transplantedfor HBV is 85% and retransplantation for recurrent HBV cirrhosis is rare

What are factors associated with poor outcomes in AIH and need for LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• younger age at presentation
• more acute presentation
• delayed response (eg aminotransferase) to therapy
• MELD score >12
• multiple relapses

What are indications for LT in patients with primary sclerosing cholangitis?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• decompensated cirrhosis
• cholangiocarcinoma
• recurrent bacterial cholangitis

What is the recommended cancer screening post LT in patients with PSC?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What is the recommended period of abstinence from alcohol for patients with alcohol related decompensated cirrhosis prior to transplant evaluation?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

A 6-month minimum period of abstinence is commonly
enforced on the basis that this period allows
addiction issues to be addressed, and in patients with
recent alcohol consumption or acute alcoholic hepatitis,
may allow for spontaneous recovery and obviate
the need for LT as well as reduce the risk of alcohol
relapse if LT remains necessary.

It is critical that the requirement for addiction rehabilitation not be neglected during this time.

What etiologies of acute liver failure (encephalopathy and INR >1.5) are the most likely to have spontaneous survival?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• acetaminophen
• pregnancy related liver disease
• hepatitis A
• shock liver

What are the criteria for Status 1 UNOS listing?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Admission to an ICU and one of the following:

What are contraindications to transplant in a Status 1 patient?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• uncontrolled infection
• CPP <40 for 2 hours or more
• worsening ventilatory settings with increasing FiO2
• intracerebral bleed or other irreversible neurological complications
• high dose pressor requirements

What are the Milan criteria for HCC prognosis post liver transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Milan indicated that the 4-year survival after
transplant was 75% and the recurrence-free survival
was 83% provided the tumor burden was either one
lesion 5 cm, or three lesions each 3 cm without
metastatic spread at the time of LT.

Automatic MELD score of 22

What are the criteria for HCC diagnosis?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• Imaging:
• late arterial phase: enhancement
• portovenous phase
• rim enhancement (pseudocapsule enhancement)
• washout
• growth on serial images
• OR biopsy documentation of HCC

Is cholangiocarcinoma transplantable and what are the criteria?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Two single-center reports of protocols incorporating neoadjuvant chemoradiation therapy, rigorous assessment for extrahepatic (nodal and/or metastatic) disease, avoidance of direct transperitoneal biopsy, and LT describe 5-year patient survival rates of nearly 80%.

UNOS granted exception status in June 2009 to unresectable, early stage, peri-hilar cholangiocarcinoma with an initial award of MELD exception score commensurate with a 10% 3-month mortality risk and escalation  every 3 months.

Which phenotype is expressed after transplant for alpha 1 antitrypsin deficiency?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are the historic reasons for poorer outcomes post liver transplant in patients with hemochromatosis related cirrhosis or HCC?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Hereditary hemochromatosis is a relatively uncommon indication for LT, ( 0.5-1% of all transplants) despite the frequency of the HFE gene. LT is indicated for HCC or decompensated liver disease.

Cardiovascular events, most notably arrhythmias and
infectious complications, are increased after LT in hereditary hemochromatosis, resulting in outcomes inferior to other indications for LT.

Judicious use of iron reduction therapy pretransplant, careful selection and follow-up appear to have resulted in improved outcomes after LT, similar to other indications for LT in more recent analyses.

Is living related donor transplantation appropriate for Wilson's disease?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Living related donor liver transplant (LDLT) from parents (obligate heterozygotes) to children has also been  reported to be successful

Hepatic manifestations of Wilson’s disease include acute or chronic hepatitis, cirrhosis, and acute liver failure. The disease may also present with neuropsychiatric dysfunction, hemolytic anemia, and renal impairment.

The ratio of alkaline phosphatase to bilirubin combined with aspartate aminotransferase to alanine aminotransferase ratio has a high sensitivity and specificity.

What is the most common hereditary amyloidosis disorder that requires transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Most common disorder where LT is used is familial amyloid polyneuropathy resulting from mutations in the transthyretin gene inherited in an autosomal dominant fashion. Associated with the Val30Met mutation.

Common clinical findings include sensory-motor polyneuropathy,
autonomic dysfunction, and frequent cardiac and ocular involvement. Renal dysfunction occurs in less than 50% of patients. LT does not alter the course of cardiac or ocular involvement and may stabilize but does not reverse neuropathy

what is the most common amyloid mutation associated with liver transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Val30Met mutation

Approximately 80% of all patients who have undergone
LT have the Val30Met mutation in the transthyretin
gene, but many mutations have been identified

What is the recommended treatment of patients with hyperoxaluria type 1?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are the prognostic bilirubin levels in patients with biliary atresia at 3 months?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

 2 year transplant free survival determined from bilirubin at 3 months:

16% if bilirubin is >6 mg/dL

84% if bilirubin is <2 mgdL

Do children with chronic liver disease require more calories?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Yes

Children with chronic liver disease are at risk for
malnutrition as they require 20%-80% more calories
than normal children to achieve adequate growth.
Increased caloric requirements result from a hypermetabolic state coupled with malabsorption.

Other than structural cardiac disease, what type of cardiac disease in children with biliary atresia may affect morbidity with LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Structural cardiac disease can be seen in children with BA and Alagille syndrome. Cirrhotic cardiomyopathy (CC), characterized by increased cardiac output, impaired diastolic relaxation, myocardial hypertrophy, and repolarization abnormalities, carries a high risk of post-LT mortality in adults.

Evidence of cardiomyopathy, as determined by two-dimensional echocardiography (2-DE), can also be found in children with cirrhosis as well as those with cardiomyopathy associated with glycogen storage disease or systemic mitochondrial disease.  70% of children with BA had evidence of CC. While those with CC experienced a longer ICU and hospital stay.

What are clinical signs of hepatopulmonary syndrome?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Nonspecific clinical findings include digital
clubbing, facial telangiectasia, dyspnea, wheezing, and syncope.

Screening for HPS is performed by pulse oximetry detection of oxygen desaturation when in the
sitting or standing position; pulse oximetry less than
97% on room air should be considered for further
evaluation.

What are tests for hepatopulmonary syndrome?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

HPS is confirmed with 2-D echo during
infusion of agitated saline with the appearance of
saline bubbles in the left atrium within 3-6 cardiac
cycles.

A 99mTechnetium-macroaggregated albumin
(MAA) perfusion lung scan can be used to quantify
and follow the degree of intrapulmonary shunting; an
MAA shunt fraction of 27.8% was highly specific for
intrapulmonary shunting associated with hypoxia.

What are the best tests to determine pre-LT renal function in children?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are risk factors for nonadherence in pediatric LT patients?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Risk factors for nonadherence include:

history of resistance to taking medications,

substance abuse,

physical or sexual abuse,

school absenteeism,
single parent home,

having received public assistance.

What risk factors correlate with poorer cognitive functioning in pediatric patients post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Cognitive measures have revealed reduced global
cognitive functioning in children following LT,
and specific weaknesses in motor skills and receptive
language development following LT.

Poorer nutritional status early in life, reduced head circumference, poor weight gain and growth, and low vitamin E levels correlate with poor cognitive functioning  before and after transplantation.

What comorbidities may affects morbidity and mortality in LT for Alagille's syndrome (AGS)?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

How does the outcome post LT differ for alagille's patients versus Biliary atresia patients?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

A review of the UNOS database revealed 5-year graft and patient survival was worse for AGS compared BA patients, 61.5% versus 70% (P50.02) and 78.4% versus 84%, respectively. 

Risk factors for poor outcome among AGS patients included neurological and cardiac complications. Renal disease associated with AGS will require a renal-sparing immunosuppressive regimen to minimize the risk of renal dysfunction following LT.

What are the criteria for pediatric acute liver failure?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Those entry criteria include:

1) absence of a known, chronic liver disease;
2) liver-based coagulopathy that is not responsive to parenteral vitamin K

3) International Normalized Ratio (INR) between 1.5 and 1.9 with clinical evidence of encephalopathy or >2.0 regardless of the presence of clinical encephalopathy.

What are contraindications to LT in pediatric acute liver failure?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Contraindications to LT in PALF include:

• severe multisystem mitochondrial disease, particularly those due to with valproic acid toxicity,
• uncontrolled sepsis
• irreversible cerebral edema with uncal herniation

What is the outcome for hepatoblastoma?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Which PRETEXT groups in hepatoblastoma have poorer outcomes?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are good parameters for prognosis in patients with hepatoblastoma and pulmonary metastases?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Are the majority of HCC in pediatric patients associated with or without cirrhosis?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What is the most common liver tumor in pediatrics?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Infantile hemangioma, most common pediatric tumor

Three categories:

1) focal lesions,

2) multifocal lesions, and
3) diffuse lesions.

All focal and most multifocal lesions are asymptomatic and involute spontaneously. However, some multifocal lesions present with high output cardiac failure and can lead to a fatal outcome in the first year of life. Multifocal and diffuse lesions express GLUT-1, which may biologically distinguish them from focal lesions.

What are common complications of diffuse hepatic hemangioendotheliomas?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

respiratory insufficiency due to an abdominal mass effect,
abdominal compartment syndrome,

coagulopathy (Kasabach-Merritt syndrome),

multiorgan system failure,

hypothyroidism (due to overproduction
of type 3 iodothyronine deiodinase which converts
thyroid hormone to its inactive form)

What diseases can mimic PSC in children?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Langerhan's cell histiocytosis

Cystic fibrosis

primary and secondary immunodeficiency states

What organ systems are affected by FIC1 disease?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What is the primary protein involved in FIC2 disease?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What is the primary defect in MDR3 disease?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Multidrug resistance protein-3 (MDR-3) disease,
formerly PFIC-3, results from a mutation in the
ABCB4 gene that codes for the MDR3 glycoprotein
which serves as a phosphatidylcholine flippase, transferring the lipid from the inner to the outer leaflet of
the canalicular membrane.

(MDR-3 disease is associated with cholelithiasis, intrahepatic cholestasis of pregnancy, transient neonatal cholestasis, drug-induced cholestasis, and an autosomal recessive cholestatic liver disease associated with a high GGT)

What are clinical and laboratory findings consistent with bile acid synthesis disorders?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• cholestasis
• lack of itching
• low or normal GGT

  ---

  These diseases are characterized by a failure to produce normal bile acids and an accumulation
  of unusual bile acids and bile acid intermediaries.

How is a diagnosis of bile acid synthesis disorder made?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• clinical and lab findings
• fast atom bombardment-mass spectometry

What are clinical findings of neonatal tyrosinemia type 1?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Which forms of glycogen storage disease (GSD) are most frequently transplanted?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Which organ systems are most commonly affected by GSD type I?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Most commonly affects liver, kidney, and intestine.

Glycogen storage disease type I (GSDI) is comprised of two major subtypes290: GSD type Ia (glucose-6-phosphatase deficiency) and GSD 1b (glucose-6-phosphate translocase deficiency) that affect the liver, kidney, and intestinal mucosa causing excessive accumulation of glycogen and fat in these organs. GSD type Ib has additional features that include neutropenia and impaired neutrophil function, resulting in recurrent bacterial infections and oral and intestinal mucosa ulceration that resembles inflammatory bowel disease, particularly Crohn’s disease.

What are frequent clinical manifestations of GSD-I and what helps?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

With good metabolic control, clinical manifestations such as growth retardation, hepatomegaly, hypoglycemia, lactic acidemia, hyperuricemia, and hyperlipidemia can be managed. Nephrocalcinosis, glomerular hyperfiltration, proteinuria, and endstage renal disease can occur. Hepatic adenomas (HA) are common and the prevalence of HCC increases with age reaching an estimated 50%-80% by the third decade of life.

Which organs are most commonly affected by glycogen storage disease type III (debrancher)?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

The majority of patients with GSD III (debranching
deficiency) have a disease that is generalized (type IIIa,
80% of cases) to involve liver, muscle, cardiac muscle,
erythrocytes, and fibroblasts and a minority having disease that is restricted to the liver (Type IIIb).

 The presence of fibrosis, ranging from minimal to cirrhosis, occurs in GSD III but not GSD I. Type 1 frequently has adenoma and malignant degeneration.

GSD IV  is a  disorder resulting in accumulation of insoluble amylopectin-like polyglucosan in the liver heart, muscle, nervous system, and skin.

The most common form in children appears to be predominantly hepatic with rapid progression to cirrhosis and liver failure, with death by 5 years of age. HCC can occur. LT for GSD IV suggest a favorable outcome. systemic progression of amylopectin-like deposits in the heart and muscle can occur post-LT resulting in  dysfunction and, death.

What are key features of clinical presentation in disorders of fatty acid oxidation?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Why can liver explants from maple sugar urine disease patients be transplanted into other patients (domino transplant)?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Due to the ubiquitous presence of BCKDH complex in non- MSUD patients, explanted livers from patients receiving a transplant for MSUD may be transplanted to a non-MSUD patient (“domino” transplant).

In classic variant maple syrup urine disease (MSUD), a severe mitochondrial deficiency of the branch chain keto acid dehydrogenase (BCKDH) complex associated with volatile metabolic derangements with impaired brain development or unpredictable risk of neurologic crisis.

Does liver transplant completely correct the metabolic derangement in methylmalonic acidemia?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are the thee main disorders associated with mitochondrial defects (respiratory chain defects)?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

The three main RC defects associated with liver disease are deficiencies of RC enzymes, mtDNA depletion syndrome, and Alper’s syndrome. The natural history of all three disorders is almost always fatal.

While RC defects can involve any organ, those with high-energy requirements such as brain, liver, and muscle are more commonly affected.

Does neurologic disease progress post LT for mitochondrial disorders?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Absence of evidence for extrahepatic mitochondrial
disease prior to LT does not exclude its development
after LT; the family of potential LT candidates should be well informed of this possibility.

Children with severe, life-threatening extrahepatic
multiorgan mitochondrial disease are contraindicated
for LT evaluation, as they have had uniformly poor posttransplant neurological outcomes.

Name three ductal plate disorders:

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Caroli's disease

ARPKD

Isolated congenital hepatic fibrosis

LT for biliary ductal plate malformations (DPM) associated with autosomal recessive polycystic kidney disease (ARPKD), Caroli’s disease, and isolated congenital hepatic fibrosis is not often required in the pediatric age group.

What are complications of ductal plate disorders?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Most stem from issues from portal hypertension and infection.

Complications associated with DPM include recurrent  cholangitis, biliary sepsis, and portal hypertension complicated by variceal hemorrhage or pulmonary conditions (e.g., hepatopulmonary syndrome, pulmonary hypertension).

What is one of the leading risk factors of parenteral nutrition associated liver disease?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Prolonged administration of a soy-based lipid exceeding 1 gm/kg/d in the management of pediatric intestinal failure has been implicated as an important factor in the development of cholestasis

PNALD results from myriad factors including prematurity,
sepsis, lack of enteral feeding, intestinal failure,
abdominal surgery, as well as various component of PN including protein, glucose infusion rate, and in particular lipid administration.

What is the most appropriate treatment for patients with Abernethy malformations?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Why is LT contraindicated in acute hemophagocytic lymphohistiocytosis?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Contraindicated due to high relapse risk in the transplanted organ.

HLH is a disorder of cellular immunity characterized by reduced or absent cytotoxic T cell and NK cell activity. Primary (familial) HLH is an autosomal recessive disorder, while secondary (acquired) HLH occurs following systemic infection or due to immunodeficiency. Most cases (80%) occur within the first year of age. Familial HLH reported in neonates as early as the first days, and even in preterm infants. Symptoms from  infiltration of various organs by hyper activated macrophages and lymphocytes, and diffuse intravascular hemo-phagocytosis. Infantile acute liver failure remains a rare presentation of HLH, but is critically important to recognize, as chemotherapy and bone marrow transplantation (BMT) may reverse.

What are the components of PELD score?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

INR

Total bilirubin

Growth

Age

Albumin

What liver diseases get automatic PELD/MELD scores of 30?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Hepatoblastoma

Urea cycle defects

Organic acidemia

Candidates with other metabolic diseases may apply to the RRB for an appropriate PELD (less than 12 years old) or MELD (12-17 years old) score. RRB will accept or reject the center’s requested MELD/PELD score based on guidelines developed by each RRB.

When do most deaths occur post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are the causes of death and graft loss after the first year post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are the long term morbidities post LT immunosuppression?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• increased risk of bacterial, viral, and fungal infections;
• metabolic complications such as hypertension, diabetes
  mellitus (DM), hyperlipidemia, obesity, and gout;
• hepatobiliary or extrahepatic de novo cancers [including
  posttransplant lymphoproliferative disorder (PTLD)].
• kidney failure or the development of endstage renal disease (ESRD)] was 18% at 5 years and 25% at 10 years.
• cardiovascular profiles with a high prevalence of hypertension requiring antihypertensive medications, recurrent DM and new-onset diabetes mellitus (NODM), and hyperlipidemia

What are the components of the metabolic syndrome?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Three of the following:

central obesity

hypertension

diabetes

dyslipidemia

What are the long term signs of hepatic artery thrombosis?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Hepatic artery thrombosis (HAT) or stenosis may present
clinically after 3 months, as :

• intrahepatic non-anastomotic strictures and/or
sterile or infected fluid collections within the liver,
sometimes referred to as bilomas,
• ischemic cholangiopathy or
• biliary cast syndrome.

What are the major complications of the CNI class of immunosuppression?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Renal toxicity

DM (especially with tacro)

hypertension

What are the major complications of mTOR inhibitors?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

hyperlipidemia

kidney injury (proteinuria)

pulmonary fibrosis

delayed wound healing

hypertension

What are the major complications of mycophenolate mofetil?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

GI bleeding and distress

bone marrow suppression

What are the two types of late rejection?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Late rejection is defined as rejection that has its onset
more than 90 days after transplantation.

Traditionally, 2 forms have been recognized:

cellular rejection (also known as acute cellular rejection and late-onset rejection)

ductopenic rejection (also known as vanishing bile duct syndrome).

What are the histological characteristics of cellular rejection?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are the causes of late cellular rejection?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Risk factors include the following:
• Reduction of immunosuppression (iatrogenic
or due to noncompliance).
• Pre-LT autoimmune liver disease.
• Concurrent interferon (for HCV treatment).

The differential diagnosis includes infection, recurrent and de novo autoimmune disease, and drug toxicity; it may sometimes be difficult to distinguish cellular ejection from HCV infection, and indeed, the two often coexist.

What are the treatment recommendations for late acute cellular rejection?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Based on the degree of inflammation:

Mild: increase in baseline immunosuppression levels

Mod-severe: prednisone 500 mg/day or prednisolone 200 mg/day for 3 days and an increase in baseline immunosuppression

What is the most likely cause of late onset ductopenic rejection?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Humoral alloreactivity mediated by antibodies
against donor human leukocyte antigen (HLA) molecules,
acting in concert with cellular mechanisms, may
play a role in the development of ductopenia (a process
known as antibody-mediated rejection)

Most commonly seen within the first year post transplant.

What are the histologic features of ductopenic or ab mediated rejection?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

early: possible cellular infiltrate

progressive loss of bile ducts (need to look at 10 portal triads), cholestasis, 

Later:foamy macrophages

What are the risk factors for ductopenic, late rejection?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• recurrent and/or unresponsive ACR
• transplantation for autoimmune disease
• exposure to interferon
• loss of previous graft to ductopenic rejection

What are risk factors for chronic kidney disease post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

•  immunosupressive meds
• hypertension
• athersclerosis
• Diabetes
• hyperlipidemia
• chronic HCV
• pretransplant kidney dysfunction
• perioperative acute kidney injury

What is the treatment of hypertension (chronic systolic >130 or diastolic >80) in LT patients?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• lower immunosuppression as much as possible
• low salt diet
• weight loss if overweight
• calcium channel blockers to improve renal blood flow
• Beta blockers
• ACE or ARB and direct renin inhibitors if there is significant proteinuria, CKD or DM. Watch potassiums
• consider adding in diuretics for volume control

What is the most common form of cancer post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Cutaneous malignancies (squamous cell and basal cell) are the most common form of malignancy in LT.

• Cigarette smokers are at increased risk of developing lung cancer and oropharyngeal cancer
• rate of colon cancer is increased in patients under-going transplantation for PSC associated with IBD.
• viral infections [eg, EBV leading to PTLD]

What are the pregnancy outcomes post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• higher incidence of prematurity (29%-50%) and low birth weight (17%-57%).
• Neonatal deaths or birth defects are not more frequent in comparison with the general population (except when the mother is on mTOR inhibitors or MMF).

What are the recommendations regarding pregnancy post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

The National Transplant Pregnancy Registry guidelines recommend female LT recipients postpone conception until

• At least 1 year after LT.
• Allograft function is stable.
• Medical comorbidities such as diabetes and hypertension are well controlled.
• Immunosuppression is at a low maintenance level.

What is the high risk period for opportunistic infections post liver transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What is the most common clinical syndromes of CMV post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are risk factors for CMV post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• CMV-seropositive donor organ (especially in absence of prior immunity, ie, a CMV-seronegative recipient).

• Augmented immunosuppression (especially with use of anti-lymphocyte antibodies or highdose mycophenolate).
• Allograft rejection.
• Coinfection with other immunomodulating viruses (eg, human herpesviruses 6 and 7), bacteria, or fungi.

What are risk factors for EBV associated PTLD post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are manifestations of PTLD?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

what are risk factors for fungal infection post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• massive blood transfusion
• prior fungal infection
• choledochojejunostomy
• reopertion or retransplantation
• hepatic iron overload
• renal replacement therapy
• and extended time in the ICU prior to LT.

Which are the most frequent fungi identified as infections in LT patients?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What infection is trying to be prevented by long term sulfa prophylaxis in LT patients?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

P. jirovecii (formerly called P. carinii) is an uncommon
pathogen in LT recipients, primarily because of the
widespread use of antimicrobial prophylaxis after LT.

Pneumocystis should be suspected in individuals  presenting with respiratory symptoms, hypoxemia (often exacerbated by exercise), and fever. Classic radiographic findings include bilateral interstitial infiltrates. Diagnosis confirmed by identification of the organism by a cytological examination of induced sputum or bronchoalveolar lavage fluid.

What is the treatment for Pneumocystis?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are risk factors for TB post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Several risk factors for the development of symptomatic
TB after LT have been identified:

• a prior infection with TB;
• intensified immunosuppression (especially anti–T lymphocyte therapies);
• DM;
• coinfections with CMV, mycoses, P. jirovecii, and
  Nocardia.

What problems are encountered in the drug treatment of TB post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Hepatoxicity and drug-drug interactions

There is a risk of a marked reduction in CNI and mTOR inhibitor levels with rifampin coadministration, the doses of CNIs need to be increased 2- to 5-fold at  initiation of treatment. Rifabutin may be substituted for rifampin to reduce the impact on drug levels, or non–rifampin-containing regimens can be considered, although the duration of treatment will need to be extended.

What are risk factors for higher morbidity in patients transplanted for HCV?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

• older donor --> higher graft loss risk
• acute rejection episodes -- higher cirrhosis risk
• CMV hepatitis
• Anti-lymphocyte agents
• DM and insulin resistance
• steatosis.

What are the effects of LT in pediatric patients on liinear growth?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Linear growth failure is common in children with cirrhosis because of malnutrition secondary to fat malabsorption, abnormal nitrogen metabolism, and increased energy expenditure and possibly growth hormone resistance.

After LT and nutritional restitution, growth hormone and insulin-like growth factor 1 levels return to normal, and linear growth improves. Catch-up growth is dependent on steroid usage and may not occur til the second year; this plateaus after 2 to 3 years, and up to 25% of patients have heights < 5% for their age over the long term.

What effect does ESLD and subsequent LT have on neurocognitive functioning?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Studies comparing neurocognitive function before and after LT have noted that many patients’ delays persist after physical rehabilitation. Approximately 30% require special education after transplantation.

What are complications of late hepatic artery thrombosis?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Most series have reported HAT rates between 3% and
10%. Early HAT commonly leads to early graft failure, retransplantation, or death. Collateralized arterial flow into the transplanted liver may minimize late HAT. 

For symptomatic late HAT with cholangitis,
hepatic abscesses, or diffuse biliary stricturing,
retransplantation is frequently required

What does IVC or hepatic vein obstruction present with post liver transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What types of biliary strictures are seen after LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What routine vaccines should be given to LT patients?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Routine vaccinations should be given before transplantation; these include immunoprophylaxis against varicella, measles, pneumococcal diseases, influenza viruses, hepatitis A and B, and travel-related infections. Live attenuated vaccines are generally contraindicated after transplantation. Varicella vaccination is not  recommended in children receiving long-term immunosuppression. Live vaccines, with the exception of polio, may be given to family members.

What are the features of chronic rejection on biopsy?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What is the target tacro level in pediatric patients more than 1 year post LT?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

For patients >1 year after transplantation with normal liver blood tests, maintain tacrolimus therapy with target immunosuppression levels<6 ng/mL.

>5 years after transplantation, immunosuppression minimization (defined as a CNI once daily) may be considered if there is no history of CR, liver tests are normal, and a biopsy sample shows minimal or no portal inflammation and less than stage 3 fibrosis.

Does colectomy prior to LT reduce the recurrence of post PSC?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

In adults, colectomy prior to LT reduced the recurrenc of PSC in the transplanted liver. It is unknown whether this is true for children.

Is it better to attempt any resection of hepatoblastoma prior to liver transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Children with unresectable hepatoblastoma who  underwent primary transplantation (without an attempt at resection) fared better than those who underwent inadequate resection before transplantation.

Otherwise, with chemotherapy and good resection (tumor free margins), LT is not indicated.

Can PFIC 1 or 2 recur post transplant?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Progressive Familial Intrahepatic Cholestasis 2

Children who underwent transplantation for a deficiency of the canalicular bile salt export pump (adenosine triphosphate–binding cassette B11). Features of recurrent bile salt export pump deficiency (jaundice and pruritus) developed up to 12 years after trans-plantation. Patients developed anti–bile salt export pump antibodies and liver cellular infiltrations against this epitope, which essentially acts as a neoantigen.

Which disease entity has the highest risk of post transplant diabetes in pediatrics?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are risk factors for PTLD?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Primary infections and high/repetitive doses of anti-lymphocyte globulin are recognized risk factors for early PTLD.

EBV:an important cause of morbidity and mortality, with symptomatic EBV infections and posttransplant lymphoproliferative disorder (PTLD) more common after primary EBV infections (disproportionately affecting children, often EBV seronegative before transplantation).

What is the definition of hepatic encephalopathy?

   Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Which patients have the highest rates of hepatic encephalopathy?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What happens to the deep tendon reflexes in hepatic encephalopathy?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What are precipitating factors of hepatic encephalopathy?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

Infections

GI bleeding

Diuretic overdose

Electrolyte disorder

Constipation

What is the mechanism of action of lactulose in encephalopathy?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

What is the recommended treatment approach for hepatic encephalopathy?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

A four-pronged approach to management of HE is
recommended (GRADE II-2, A, 1):
1. Initiation of care for patients with altered consciousness
2. Alternative causes of altered mental status should be sought and treated.
3. Identification of precipitating factors and heir correction
4. Commencement of empirical HE treatment

What are the dietary recommendations for patients with Hepatic encephalopathy?

Questions generated from review of AASLD practice guidelines at http://www.aasld.org/publications/practice-guidelines-0

1. Daily energy intakes should be 35-40 kcal/kgideal body weight
2. Daily protein intake should be 1.2-1.5 g/kg/day
3. Small meals or liquid nutritional supplements evenly distributed throughout the day and a latenight snack should be offered
4.  Oral BCAA supplementation may allow recommended nitrogen intake to be achieved and maintained in patients intolerant of dietary protein

How is the survival of HCV infected patients post transplant different from other groups?

2016 Transplant Review Course

What are risk factors for worse outcomes in patients transplanted for HCV?

2016 Transplant Review Course

1. Older donor age
2. prior treatment of acute rejection
3. HIV coinfection
4. CMV infection
5. Prolonged cold ischemia time
6. African-American race
7. high pre-LT viral load
8. post transplant diabetes

What are the histologic features favoring HCV over acute rejection?

2016 Transplant Review Course

• Predominant mononuclear portal  and parenchymal based infiltrate
• minimal to mild bile duct injury
• lack of endotheliitis
• presence of acidophilic bodies

Which direct acting antivral agent category should not be used in decompensated cirrhosis?

2016 Transplant Review Course

Protease inhibitors (the -previrs)

Which DAA should not be used with patients who have a CrCL <30 mL/min?

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What are the conditions to monitor in post LT patients being treated with direct-acting antiviral agents?

2016 Transplant Review Course

drug-drug interactions of protease inhibitors and CNIs/mTORs

need for ribavirin in all regimens

potential risk for rejection

Whats happening re incidence trends with regards to decompensation due to HCV and to the rates of HCC since the start of use of DAAs?

2016 Transplant Review Course

Which age groups carry the highest burden in HCV?

1. 35-45
2. 45-55
3. 55-65
4. 65-75

   ---

Which group of patients transplanted is at higher risk of mortality at 5 years post LT?

1. cholestatic liver disease
2. autoimmune hepatitis
3. HCV
4. HBV

   ---

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How can you differentiate by the drug name, what type of DAA you are using?

-previr

-asvir

-buvir

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PREvir = PRotEase inhibitor

Asvir = NS5A inhibitor

BUvir = NS5B nUcleotide/non-nUcleotide inhibitor

Which class of DAAs should not be used in renal disease?

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Which class of DAAs should not be used if the patient has decompensated cirrhosis (Childs-Pugh Class B or C)?

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What drug needs to be included with any DAA regimen post LT?

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Which DAA class has significant drug-drug interactions with CNIs and mTORi?

2016 Transplant Review Course

Protease inibitors

interact with both CNIs and mTORi due to effect on CYP3A/4. With simeprevir and CNIs, simeprevir levels rise with cyclosporin (but not with tacro). With the other -previrs, watch for elevated CNI and mTORi levels.

What is the most common indication for LT in HBV infected patients?

2016 Transplant Review Course

HCC is the most common indication.

with the advent of nucleos(t)ide analogues (specifically starting with lamivudine) and resulting viral suppression, disease progression is limited.

In which settings should anti-HBV therapy be started?

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• severe acute hepatitis B
• severe exacerbation of chronic hepatitis B
• decompensated HBV cirrhosis

  ---

Which factors contribute to higher risk of HBV recurrence post transplant?

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• detectable HBV DNA in the serum
• HBV antiviral drug resistance
• HDV or HIV coinfection
• HCC at transplant

  ---

  These groups need antiviral therapy with nucleos(t)ide anaogues with the addition of HBIG post transplant. Duration and dosing unclear

Is HBIG indicated in the prevention of recurrence of HBV post transplant if the donor is HBsAg - / HBcAb + (indicative of a past history of HBV infection but with clearance)?

2016 Transplant Review Course

Which 2 nucleos(t)ide analogues are associated with the lowest rates of HBV resistance?

2016 Transplant Review Course

Entecavir and Tenofovir

Resistance is 0-1% up to 6-8 years of therapy.

Which nucleos(t)ide analogue has been associated with lactic acidosis?

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Which antiviral should be used in HBV patients?

2016 Transplant Review Course

What is the outcome of transplanting a HBsAg- / HBcAb+ liver into a HBsAg- recipient prophylaxed with a NUC analogue alone?

2016 Transplant Review Course

What is the best therapeutic regimen for post transplant if the patient has low or undectable HBV DNA levels at the time of transplant?

2016 Transplant Review Course

What if the most common cause of recurrent HBV in LT patients?

2016 Transplant Review Course

The most common cause (>90%) is due to the development of antiviral with or without HBIG resistance. Other causes include:

improper prophylaxis regimen

poor/decreased compliance

Rescue therapy should be tailored to the suspected drug resistance, genotypic resistance testing whenever possible.

What is the second leading cause of adult liver transplantation in 2016, thought to increase to top leading cause in 2020?

2016 Transplant Review Course

What do most transplant centers feel is the upper BMI cutoff for LT?

2016 Transplant Review Course

Does the outcome differ for LT for NASH versus other causes of cirrhosis?

2016 Transplant Review Course

What is the rate of NAFLD in the USA in adults and children?

2016 Transplant Review Course

In adults, it is dependent on the means of measure. Roughly 30% of adults have NAFLD by MRI. With US, the rate drops to 15-17%, and with transaminase elevation it is around 7-12%.

In children, the rate is reported to be 10-15% of children have NAFLD

What is the difference between simple fatty liver (steatosis) and steatohepatitis histologically?

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What are the 4 major risk factors for NAFLD?

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1. type 2 diabetes
2. dyslipidemia
3. obesity
4. metabolic syndrome

   ---

   In most of these groups, more than 50% will have NAFLD.

What diagnosis should be considered in patients in their 30-40s, presenting with cirrhosis, hepatopulmonary syndrome who had radiation RX due to craniopharyngeoma?

2016 Transplant Review Course

What are the 3 most common causes of death in NAFLD?

2016 Transplant Review Course

1. Cardiovascular disease and stroke
2. malignancy
3. cirrhosis

   ---

   Many also will become frank diabetics if not already insulin resistant.

What are possible tests to rule in or out advanced fibrosis in patients with NAFLD?

2016 Transplant Review Course

• FIB-4
• NAFLD fibrosis score
• Fibroscan
• CK18 and ELF not available in the USA

What adverse outcomes are more common in obese patients transplanted than other groups?

2016 Transplant Review Course

Due to obesity, increased adverse outcomes seen are:

1. Primary nonfunction
2. cardiovascular events (especially A-fib)
3. infections

   ---

   Rejection is NOT increased in obese LT recipients.

What is one of the predictors of poor outcome in obese patients undergoing transplant?

2016 Transplant Review Course

Sarcopenia

It is the loss of muscle mass associated with aging.

What is the most common arrhythmia in obese patients about to undergo LT?

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What is a possible cause of low Hgb A1c in obese patients even with high glucoses?

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Does NASH recur post LT?

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What are causes of recurrent NAFLD post LT?

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1. underlying metabolic conditions
2. metabolic complications post transplant
3. side effects of immunosuppression

   ---

   graft failure is not high so far for recurrent NAFLD.

What are risk factors for de novo NAFLD post transplant?

2016 Transplant Review Course

• alcoholic cirrhosis
• graft steatosis
• tacrolimus use
• PNPLA3 G/G phenotype

What disorders are commonly associated with PBC?

2016 Transplant Review Course

• sicca syndrome
• thyroiditis
• rheumatoid arthritis
• celiac disease
• scleroderma

What if the treatment for PBC?

2016 Transplant Review Course

UDCA at 13-15 mg/kg/day.

Monitor alk phosphatase and other biochemistries.

What treatment should be started if there is no response or an incomplete response to UDCA for PBC?

2016 Transplant Review Course

Which disease(s) are more common in females?

PBC

PSC

Autoimmune hepatitis

Overlap syndromes

IgG-4 Sclerosing cholangitis

Sarcoidosis

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Which disease(s) are more common in males?

PBC

PSC

Autoimmune hepatitis

Overlap syndromes

IgG-4 Sclerosing cholangitis

Sarcoidosis

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What diseases are frequently seen with autoimmune hepatitis?

2016 Transplant Review Course

Other autoimmune conditions such as:

Hashimoto thyroiditis

Graves disease

alopecia

vitiligo

rheumatoid arthritis

IBD

lupus

celiac disease

What classic findings are seen on histology with AIH?

2016 Transplant Review Course

interface hepatitis

plasma cell infiltrates

rosette formation

hepatocyte swelling

What is the biggest risk factor for recurrence of AIH post end of treatment?

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What malignancies occur commonly in PSC?

2016 Transplant Review Course

• cholangiocarcinoma
• gallbladder cancer
• colon cancer (especially in IBD patients).

How does small duct PSC differ from large duct on imaging?

2016 Transplant Review Course

Large duct PSC can be seen on imaging (cholangiogram) whereas small duct PSC cannot.

laboratory parameters and histologically they otherwise appear the same.

Is large or small duct PSC seen more commonly in overlap syndrome with AIH?

2016 Transplant Review Course

Large duct disease is seen more commonly, especially in children and young adults.

Should always get an MRCP done at the time of AIH diagnosis in children.

How often is colonoscopy recommended in IBD/PSC patients?

2016 Transplant Review Course

Annually

PSC/IBD patients are at much higher risk of colon cancer.

Is IgG4 sclerosing cholangitis seen more commonly in children or adults?

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What is the treatment for IgG4 sclerosing cholangitis?

2016 Transplant Review Course

Steroids.

Due to high rate of relapse, long term immunosuppression with azathioprine recommended.

what other organs/areas are affected with IgG4-Sclerosing cholangitis?

2016 Transplant Review Course

pancreatitis

sialadenitis

retroperitoneal fibrosis

interstitial nephritis

What types of strictures are seen with IgG4 sclerosing cholangitis: single or multiple?

2016 Transplant Review Course

What is a histologic finding helpful to diagnose IgG4 sclerosing cholangitis?

2016 Transplant Review Course

Is IgG4 sclerosing cholangitis more common in men or women?

2016 Transplant Review Course

More frequent in males (8:1)

specifically in their 60s, presenting with obstructive jaundice.

Celiac disease is associated with what liver diseases?

2016 Transplant Review Course

what is the hallmark antibody seen in PBC?

2016 Transplant Review Course

Can you have PBC without a positive AMA?

2016 Transplant Review Course

What 3 criteria are used to make a diagnosis of PBC?

2016 Transplant Review Course

Positive AMA >1:40

chronic cholestasis with unexplained elevation of alk phos for >6 months

Histology showing non-suppurative destructive cholangitis and duct loss (florid duct lesions)

Need 2 of the 3 for diagnosis

What are the most common symptoms in patients with PBC?

2016 Transplant Review Course

Pruritus

Fatigue

Sicca symptoms

Often the patients are asymptomatic.

What is the treatment for PBC?

2016 Transplant Review Course

UDCA 10-15 mg/kg/day

If non responsive, try obeticholic acid.

What therapy is available for PBC patients with poor response to UDCA?

2016 Transplant Review Course

Obeticholic acid 5 mg/day

A good response to therapy is a drop of Alk phos to <1.6x ULN.

What are possible treatments for pruritus secondary to cholestasis?

2016 Transplant Review Course

rifampin

sertraline

naltrexone

cholestyramine

What are the 1 and 5 year oucomes rates in patients transplanted for PBC?

2016 Transplant Review Course

Rates are 90% and 83%, respectively.

of note, recurrent PBC occurs in up to 60% at about 15 yrs. UDCA post transplant may decrease the rate of recurrence.

When is the most common age of occurrence of AIH?

2016 Transplant Review Course

What factors confirm the diagnosis of AIH?

2016 Transplant Review Course

interface hepatitis

presence of autoantibodies (ANA, SMA, LKM, SLA)

Hypergammaglobulinemia

Association with HLA DR3 or DR4

Favorable response to imunosuppression

How often is cirrhosis present at the time of diagnosis of AIH?

2016 Transplant Review Course

30% in adults

50% in children

Why is it difficult to diagnose AIH in acute hepatitis or acute liver failure?

2016 Transplant Review Course

Emperilopoiesis is frequently seen in AIH histology. What is that?

2016 Transplant Review Course

What are the components of the Scoring for Autoimmune Hepatitis Criteria?

2016 Transplant Review Course

- Elevation of the IgG

- Presence of autoantibodies

- Histologic criteria

- Absence of viral hepatitis

Score =6 is probable AIH; Score >7 is definite AIH.

[image]

What is the definition of remission in AIH?

2016 Transplant Review Course

Normalization of all the below:

symptoms

transaminases

bilirubin

IgG

Histology (no inflammation)

Why is the benefit of combination therapy using azathioprine with steroids versus steroids alone in the treatment of AIH?

2016 Transplant Review Course

What are laboratory parameters indicative of a successful withdrawal from therapy for AIH?

2016 Transplant Review Course

Transaminases <1/2 ULN

IgG <1.2 g/dL

How long do you treat AIH?

2016 Transplant Review Course

At least 3 years.

Should have at least 2 years of normal transaminases and IgG.

When do you use MMF in the treatment of AIH?

2016 Transplant Review Course

When do you use CNIs in the treatment of AIH?

2016 Transplant Review Course

What is the mechanisms of action of Calcineurin inhibitors?

2016 Transplant Review Course

What are the indicators of a high mortality prognosis in AIH?

2016 Transplant Review Course

multilobular necrosis

hyperbilirubinemia

MELD >12 at presentation

HLA DR3

How do yo differentiate drug induced "AIH" versus true AIH?

2016 Transplant Review Course

What criteria is needed to make the diagnosis of AIH/ PBC overlap?

2016 Transplant Review Course

Need 2/3 criteria in each disease:

PBC: cholestasis, lymphocytic cholangitis, AMA ab

AIH: interface hepatitis, IgG >2 mg/dL or +SMA, ALT >5x ULN

The interface hepatitis has to be at least moderate in severity.

Which portions of the biliary tree are affected by PSC?

2016 Transplant Review Course

Both the intra and extra hepatic biliary tree is affected.

[image]

What are common laboratory findings in PSC?

2016 Transplant Review Course

- elevated alk phos or GGT

- high IgG

- high IgM (50%)

- elevated IgG4 (10%)

Elevation of the IgG4 is associated with a faster progression.

What is the liver disease shown below?

 [image]

PSC

Findings of concentric fibrosis around the bile duct and inflammation.

2016 Transplant Review Course

Does PSC and IgG4 sclerosing cholangitis differ in response to steroids?

2016 Transplant Review Course

What is the recurrence rate at 5 years of PSC post LT?

2016 Transplant Review Course

20%.

5 year survival is 80-85%. UC post transplant and younger age are risk factors for recurrence.

What are risk factors for PSC recurrence post LT?

2016 Transplant Review Course

What is the classic histologic findings in sarcoidosis?

2016 Transplant Review Course

Non-caseating granulomas. Noted in 50-65%.

Most patients with liver involvement of their sarcoid are asymptomatic.

What are surveillance strategies for monitoring for cholangiocarcinoma or gallbladder cancer in patients with PSC?

2016 Transplant Review Course

(Very little data and low quality)

• cross sectional imaging every 6-12 months
• serum CA 19-9 every 6-12 months
• cholecystectomy of GB polyps >8 mm

What lab test is done for sarcoidosis?

2016 Transplant Review Course

ACE will be elevated in 75% of patients

would also look to see if transaminases are elevated. Patient may have abdominal pain, hepatomegaly and pruritus. CT may show numerous hypodense nodules. Can present with venous obstruction (PV thrombosis, Budd Chiari, portal hypertension)

What HLA types are associated with celiac disease?

2016 Transplant Review Course

What is the most common liver disease associated with celiac disease?

2016 Transplant Review Course

PBC is seen in up to 10% patients with celiac disease.

It is also seen with PSC, AIH, and congenital hepatic fibrosis.

Which cholestatic disease(s) is associated with ABCB11?

This codes for the bile salt excretory pump (BSEP) and is associated with PFIC type 2.

BSEP pumps bile salts from a low concentration in the hepatocytes to a higher concentration in the bile ducts (thus against the concentration gradient).

In the PFIC disorders, are the serum bile salts high or low?

Which PFIC disorder has a high GGT?

1. PFIC1
2. PFIC2 (BSEP deficiency)
3. PFIC3 (MDR# defect)

Which PFIC disorder has a high rate of HCC?

1. PFIC1
2. PFIC2 (BSEP deficiency)
3. PFIC3 (MDR# defect)

Which PFIC disorder is associated with the defect in ATP8B1?

1. PFIC1
2. PFIC2 (BSEP deficiency)
3. PFIC3 (MDR# defect)