Liver Disease and Transplantation Flashcards

1
Q

Rates of fulminant hepatic failures in HAV, acute HBV, and HBV and HDV coinfection

A

HAV = 0.1%
Acute HBV = 1%
HBV and HDV Confection = 20%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Percent of individuals who develop chronic HBV when exposed perinatally? When exposed in childhood? When exposed in adulthood?

How about chronicity in patients exposed to HCV?

A

Chronicity is 90% when exposed perinatally, 50% if exposed during childhood, and 5% in adulthood.

HCV becomes chronic in 60-85% of patients exposed regardless of age.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

When to screen HBV carriers without cirrhosis for HCC?

A

Africans >20, Asian males >40, asian females >50, family history of HCC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

2 extrahepatic complications of chronic HCV? How to improve symptoms?

A

Essential mixed cryoglobulinemia, membranoproliferazive glomerulonephritis (MPGN).

Can improve renal function and proteinuria with eradication of HCV RNA.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Extrahepatic manifestation of chronic hepatitis B infection? How to improve symptoms?

A

Polyarteritis nodosa (positive for HBsAg).

Can improve symptoms with antivirals, as well as mortality.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

HBV genotype that is most responsive to peginterferon? Least responsive

A

Most responsive is genotype A with high ALT or low HBV DNA.

Least responsive is genotype D.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Symptoms of chronic HBV spontaneous seroconversion

A

Where HBeAg is suppressed by immune system, HBV DNA is lost, and HBeAb appears. Can cause elevations in AST/ALT and sometimes acute liver failure. These patients can still get HCC, but not cirrhosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Risk of fulminant liver failure from HEV?

A

Very low, less than 1% but as high as 15-25% in pregnant women

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

HCV genotype most likely to progress to cirrhosis?

A

Genotype 3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Most challenging HCV genotype to treat? How to treat more effectively?

A

Genotype 3 is the most challenging to treat. Can treat most effectively by increasing therapy to 24 weeks.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the risk of recurrent HCV after transplant?

A

25-33% of recurrent HCV patients after transplant can develop fibrosis or cirrhosis again.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the features and lab tests for Type II AIH?

A

Autoimmune hepatitis which is rapid onset usually seen in children under 14. Test for Anti-LKM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Most common cause of drug-induced autoimmune hepatitis? How to treat?

A

Nitrofurantoin and minocycline
Cause interface hepatitis and abundance of plasma cells
Stop drug, sometimes will need acute course of steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Which beta blocker is best for variceal ppl during pregnancy?

A

Propranolol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Management of HEV in solid organ transplant?

A

Reduction of immunosuppression, if that doesn’t work, then ribavirin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Ribavirin major side effect

A

Hemolytic anemia (dose dependent)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Treatment options for HCV in patients without cirrhosis

A

Glecaprivir/pibrentasvir for 8 weeks (Mavyret)

Sofosbuvir/velpatasvir for 12 weeks (Epclusa)

Ledipasvir/Sofosbuvir for 12 weeks (Harvoni)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Difference between treating HCV in patients with compensated cirrhosis and without cirrhosis?

A

None, can still use Glecaprevir/pibrentasvir for 8 weeks, though need to increase to 12 weeks if connected with HIV.

Can still use sofosbuvir/velpatasvir for 12 weeks.

Can still use ledipasvir/sofosbuvir for 12 weeks.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Critical lab test to check before starting HCV therapy? Why?

A

HBV serologies because risk of HBV reactivation increases with DAA therapy, even for patients who are core positive.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

How to treat HCV in patients with decompensated cirrhosis?

A

Need to increase duration of treatment in most cases, consider addition of ribavirin, and start transplant eval because may be beneficial to transplant before tx

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Do you need to dose-reduce DAAs for HCV in patients with CKD

A

No

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Should you treat acute HCV?

A

Monitor HCV q4-8 weeks for up to 12 months to determine if spontaneous clearance occurs.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

How to treat chronic hepatitis D infection?

A

PEG-IFN alpha for 12 months, can also add Bulevirtide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How to prevent HBV infection in transplant recipients?

A

All HBsAg positive patients undergoing transplant should receive entecavir, TDF, or TAF, with or without HBIG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
How to treat HBsAg negative patients who receive HBsAg negative, HBcAb positive donor livers?
Long term prophylactic antivirals with entecavir TDF or TAF
26
How to diagnose CMV hepatitis?
Biopsy is needed
27
Which patients are most susceptible to HSV hepatitis?
Immunosuppressed patients or pregnant patients in 2nd or 3rd trimester
28
How to treat HSV hepatitis
IV acyclovir is preferred, start before diagnosis is confirmed because delay can be catastrophic
29
Which drugs require prophylaxis for HBV reactivation?
Very high risk: Rituximab and stem cell transplant All others just require monitoring with HBV DNA. High risk: >20mg pred, cytokine inhibitors Moderate risk: Chemotherapy, antiTNF, anti rejection meds Low risk: Azathioprine, methotrexate
30
3 groups for which to treat chronic HBV
1. Cirrhosis and low viremia (<2000) 2.HBeAg+ immune-active if HBV DNA >20,000 and ALT >2x ULN 3. HBeAg- Immune-active if HBV DNA >2000 and ALT > 2x ULN
31
Why is TAF superior to TDF
Decreased chance of bone and renal abnormalities
32
When to stop oral meds for chronic HBV with HBeAg+
12 months after seroconversion from HBeAg to HBeAb Lifelong if no seroconversion
33
When to stop oral meds for chronic HBV with HBeAg-?
With 3 years of viral load suppression if they can be monitored closely
34
Microvesicular steatosis vs Macrovesicular steatosis
Microvesicular steatosis seen in rare conditions (Reye syndrome, mitochondrial disorders, drug toxicity [tamoxifen, amiodarone, methotrexate]) Macrovesicular steatosis seen with NAFLD and alcoholic steatosis
35
Features of alcoholic hepatitis on pathology
Ballooning degeneration, apoptosis, giant mitochondria, mallory bodies (not specific). Damage most prominent in perivenular region
36
Presentation and treatment for acute Wilsonian hepatitis
Elevated LFTs, jaundice, hemolysis due to copper Transplant
37
How to diagnose hemochromatosis?
Ferritin, transferrin saturation, hemochromatosis genetics. BUT ultimately liver biopsy IS necessary
38
Features of metabolic syndrome
High visceral fat, dyslipidemia, insulin resistance, hypertension Waist circumference Fasting triglycerides >150 HDL <40 BP>130/85 Fasting BG >110
39
Likely cause of elevated ALT if no obvious cause
75% of these patients will have NASH
40
Acute Fatty Liver of Pregnancy presentation, cause, and treatment
Rapidly progressive rise in AST/ALT, diffuse micro vesicular steatosis. Can cause liver failure. Caused by toxic intermediates of fatty acid oxidation. Need immediate termination of pregnancy and sometimes transplant
41
Difference between Dubin-Johnson and Rotor Syndrome
Both cause conjugated hyperbilirubinemia, liver turns black in Dubin-Johnson syndrome
42
PFIC
Progressive familial intrahepatic cholestasis can cause cirrhosis in infancy. Likely will need transplant
43
BRIC
Benign recurrent intrahepatic cholestasis. Intermittent, non progressive, normal liver function
44
CF associated liver disease, and treatment to delay progression
Causes progressive cholestasis due to thick bile, can give patients ursodiol, can progress to biliary cirrhosis
45
Hereditary Tyrosinemia Type 1 and treatment
Enzyme missing leading to toxic intermediates in pathway of phenylalanine and tyrosine metabolism. Causes childhood fibrosis and cirrhosis. NTBC inhibits early step in pathway and can be used for treatment. Some patients may need transplant.
46
Glycogen storage diseases and hepatic consequences
Type 1 (Von Girke) Type 3 (Cori) Changes in G6P (type 1) and debranching enzyme (type 3) Causes hepatic adenomas and HCC Some can have ALF and require transplant
47
Gaucher Disease
Lipid storage disease (glucocerebrosidase deficiency). Most common deficiency in jews Causes hepatosplenomegaly, bone fractures, anemia, thrombocytopenia Some patients can develop progressive fibrosis and portal hypertension
48
Role of hepcidin
Hepcidin regulates iron release from macrophages and intestinal absorption by binding to ferroportin in enterocytes
49
What manifestations of hemochromatosis usually don't improve with chelation of phlebotomy
Testicular atrophy and arthropathy Risk of HCC does not improve either
50
What does ATP7B do?
Secretes copper into bile for excretion. Mutation causes Wilson disease
51
How to diagnose Wilson disease?
Check ceruloplasmin, if low, eye exam and 24h copper extretion
52
How to treat Wilson disease
Copper chelation with Trientine and prevent absorption with oral zinc. Can also use d-penacillamine but has side effects
53
Treatment for ALF due to wilson's
Transplant, if normal ATP7B in graft then chelation is not needed
54
A1AT Alleles
M is normal S is modest deficiency Z is severe deficiency MM normal ZZ is severe deficiency, SZ also relatively severe
55
Diseases that often accompany PBC?
Sjogrens Syndrome, CREST. Inflammatory destruction of small bile ducts causing cholestasis and biliary cirrhosis.
56
PBC presentation
Elevated AP, pruritis, fatigue, xanthomas/xanthelasmas, fat soluble vitamin deficiencies
57
Treatment of PBC
13-15mg/kg ursodiol or OBCA for ursononresponders
58
Histology of PBC
Florid duct lesion
59
How to diagnose AIH?
Serologies are helpful but need to biopsy to confirm
60
AIH histology
Plasma cell infiltration, interface hepatitis
61
How to treat AIH
40-60mg pred, transition to 1.5-2mg/kg azathioprine can also use mycophenolate or tacrolimus
62
Which treatment for AIH cannot be used in pregnancy?
Mycophenalate
63
Presentation of acute cellular rejection
Elevations in transaminases and alc phos weeks to months after transplant
64
What condition has hepatocyte injury, ductulitis, venular Endothelitis?
Acute cellular rejection
65
How to treat ACR?
Mild: Conservative Severe: IV steroids and anti-lymphocyte globulin
66
What condition usually follows ACR?
Chronic ductopenic rejection -- marked by progressive loss of bile ducts
67
Schistosomiasis causes...
isolated pre-sinusoidal portal hypertension
68
ABCB11
Gene implicated in BRIC PFIC and intrahepatic cholestasis of pregnancy
69
Jaundice and transaminase elevation in the setting of chemotherapy or bone marrow transplant?
Hepatic sinusoidal obstruction syndrome
70
Cholestasis with hilar biliary structures in a patient with a history of shock
Ischemic cholangiopathy
71
An insulin sensitizing peptide secreted by adipose tissue whose levels decline as fat accumulates, low levels characterize metabolic syndrome
Adiponectin
72
A phospholipase enzyme associated with the propensity to hepatic steatosis and fibrosis in both NASH and alcoholic liver disease
Adiponutrin
73
A circulating signalling peptide that participates in the up regulation of hepcidin production
Bone morphogenic protein 1
74
Hepatic co-receptor for bone morphogenic protein 1
Hemojuvelin
75
What is the antibody against the lipoid antibody moiety of the pyruvate dehydrogenase complex in bile duct epithelial cells
Anti-mitochondrial antibody
76
What is the antibody against hepatic enzyme cytochrome P450 2E6
Anti liver kidney microsomal antibody (AIH type 2)
77
How to calculate HVPG? What is considered elevated
Hepatic wedge pressure - free hepatic pressure. More than 5 is elevated, varies tend to happen over 10
78
Paradigm for primary prevention of varices
Look at liver stiffness and platelet count. If PLT >150,000 or liver stiffness <20, no need for primary prevention. If LS>20 and platelet <150K, or LS>25 then start NSBB. If not tolerated, do EGD. If no varices, EGD q2y. If large varices, band.
79
Important treatment of variceal hemorrhage
CTX 1g/day for up to 5 days Octreotide PPI is not important if variceal bleed
80
GOV1 vs GOV2 vs IGV1 vs IGV2?
GOV1 extend along lesser curve GOV 2 extend from esophagus to the fundus IGV1 are isolated in the fundus IGV2 are in the antrum
81
Which patients benefit from early tips and variceal bleeding?
CP C after hemorrhage or CP B with active hemorrhage
82
How to treat portal hypertensive gastropathy
Iron and beta blocker
83
Two different types of high SAAG (>1.1) ascites?
Portal hypertension (low ascites albumin <2.5) Cardiac ascites (higher ascites albumin >2.5)
84
Who should be on SBP ppx? What to use
Those who have ever had SBP -- use cipro
85
What causes persistent encephalopathy in patients that are otherwise compensated?
Splenorenal or other shunt
86
How to diagnose hepatic sarcoid
Biopsy
87
Hepatopulmonary syndrome cause, presentation, treatment
Caused by dilated pulmonary capillaries and shunting causing hypoxemia. Presentation is platypnea and orthodeoxia. Tx with transplant
88
Portopulmonary hypertension
Pulmonary vasoconstriction due to unprocessed mediators from splanchnics causes severe pulmonary hypertension
89
Typical imaging features of HCC?
Arterial hypervascularity and early washout in venous/portal/delayed phase
90
How to treat early stage HCC in healthy patient?
Resection if single mass <5cm is curative Otherwise will need RFA +/- TACE or TARE
91
Milan criteria
Patients can be transplanted for HCC if they have 3 or fewer tumors <3cm, or 1 tumor <5cm provided no vascular invasion or extra hepatic disease
92
What must be included in transplant workup for HCC patients?
CT chest and bone scan
93
How to treat advanced stage HCC in Childs A or B patients?
Atezolizumab/bevacizumab >>> sorafenib
94
How to treat advance stage HCC in Childs C patients?
Supportive care, median survival is 3 months
95
What is the MELD to consider TXP?
15, but patient should be referred to txp center after their first decompensation
96
Does AIH recur post transplant?
Yes -- do not taper steroids completely
97
What are the signs of primary liver graft nonfunction?
No bile production, liver failure, renal failure
98
What causes hepatic venous outflow obstruction after transplant?
Stenosis of the anastomosis of the hepatic vein and the IVC
99
What is the baseline IS regimen after liver transplant?
A CNI (tac/cyclo), immunomodulator (MMF, everolimus, azathioprine), and steroids
100
What is long term IS regimen after liver transplant?
Tacrolimus
101
Why is an immunomodulator added to the baseline IS regimen?
To reduce doses of the calcineurin inhibitor (tac/cyclo) and prevent nephrotoxicity
102
What is the colonoscopy interval for post liver transplant patient with UC/PSC
Every year
103
Should you treat HBV with cirrhosis?
Yes Always treat
104