2- Hepatic/liver Disease Flashcards
1
Q
An AST/ ALT > 1.5 (but traditionally ≥ 2) indicates what condition?
A
Alcoholic liver disease
2
Q
An AST/ ALT < 1.0 indicates what condition?
A
NASH (often acute or chonic viral hepatitis)
3
Q
An AST/ ALT > 10 indicates what condition?
A
Cirrhosis
4
Q
An ALT/ AST < 1.0 indicates what condition?
A
Fatty liver
5
Q
What conditions are indicative of low to high ALT values? (ranging from ~40 to 10,000)
A
Hepatitis B + C chronic → EtOH → Hepatitis C, A + B acute → “shock liver” or acetaminophen toxicity
6
Q
What is defined as inflammation of the liver by fat deposition?
A
Steatohepatitis
7
Q
Predominantly elevated ALT and AST (liver transaminases) indicate what?
A
Hepatocellular pattern
8
Q
What is the spectrum of diseases included as part of NAFLD (non- alcoholic fatty liver disease)?
A
NAFL → NASH → NASH cirrhosis
9
Q
How is NAFL (aka isolated steatosis) defined?
A
Fatty liver without injury or fibrosis of hepatocytes on bx
10
Q
How is NASH defined?
A
Fatty liver + inflammation = hepatocyte injury
Risk of progression of fibrosis, cirrhosis is significant
11
Q
The following are RFs for what condition?
Abd obesity, DMT2, hyperlipidemia, metabolic syndrome, PCOS
A
NAFLD
(metabolic syndrome = strongest predictor)
12
Q
How does NAFLD typically present clinically?
A
Asx, often incidental finding
13
Q
What tools are used to aid in identifying those with NAFLD that are at risk for advanced fibrosis?
A
NAFLD fibrosis score, fibrosis-4 index, vibration-controlled transient elastography (VCTE)
14
Q
On fibroscan (VCTE) you note ≥ 5% liver fat on imaging or liver bx, US notes fat present on the liver, labs show hepatocellular pattern with elevated ferritin, lipids, and glucose. What are you concerned for?
A
NAFLD
15
Q
When is it recommended to obtain a liver biopsy in pts with NAFLD?
A
Increased risk for advanced fibrosis, metabolic syndrome/ elevated LFTs, need to r/o competing etiologies
16
Q
What conditions must be r/o when evaluating suspected NAFLD?
A
Significant alcohol consumption, competing etiologies for hepatic steatosis, coexisting causes of chronic liver disease (CLD)
17
Q
Pts with NAFLD are at a high risk for what?
A
CV morbidity/ mortality
(aggressive modification of CVD RFs should be considered)
18
Q
What is used in the treatment of dyslipidemia in patients with NAFLD and NASH, and when should this treatment be avoided?
A
Statins; avoided in pts with decompensated cirrhosis
19
Q
What is considered the “cornerstone” of NASH management?
A
Exercise and weight loss
(should also minimize EtOH, control underlying conditions, vaccinate)
20
Q
What is the relationship between body weight reduction and histological improvement of NASH?
A
>3% improves steatosis
7-10% NASH resolution
≥10% fibrosis regression
21
Q
What are the hereditary liver disorders? (3)
A
Hereditary hemochromatosis, alpha-1 antitrypsin deficiency, Wilson’s disease
22
Q
What is hereditary hemochromatosis?
A
Hereditary disorder of iron metabolism
23
Q
What is the pathophysiology of hereditary hemochromatosis?
A
Genetic mutation → increased GI absorption of iron → accumulation of iron in tissues
(liver, pancreas, heart, adrenals, testes, pituitary, skin, kidney)
24
Q
How does hereditary hemochromatosis present clinically (early stages)?
A
FH or incidentally noted increased AST and ALT
Initially non-specific sxs (fatigue, malaise, RUQ discomfort)
25
The following are complications of what disease?
Hepatomegaly, hepatic insufficiency, cirrhosis, DM, impotence, arthralgia, bronze pigmentation of skin, cardiomegaly +/- CHF, cardiac arrhythmia
Hereditary hemochromatosis
26
What is the triad a/w Bronze Diabetes (rare) and what condition can it be seen with?
Can be seen with hereditary hemochromatosis
Triad: DM, bronze pigmentation of skin, cirrhosis
27
Early diagnosis of hereditary hemochromatosis is important. What lab studies should be ordered?
Abd chem panel, iron studies and screening if (+) FH
28
Pt with concern for liver disease shows elevated liver tests/ LFTs, along with transferrin sat ≥ 45 and/ or ferritin \> 200 (men) OR \> 150 (women). What condition should you be concerned for?
Hereditary hemochromatosis
29
How is a diagnosis of hereditary hemochromatoma confirmed?
Genetic testing (GI mutation analysis) +/- liver bx
30
What is the goal of treatment for hereditary hemochromatosis and how is this done?
Goal is to prevent cirrhosis from iron load → therapeutic phlebotomy and q 6 months HCC screening w/ cirrhosis
31
What lifestly recommendations should be made for a pt with hereditary hemochromatosis to prevent cirrhosis from iron overload?
Avoid vit C, iron containing supplements, uncooked shellfish, EtOH
32
What is recommended for all 1st degree relatives of a pt with hereditary hemochromatosis?
HFE genotype and iron testing
33
According to AASLD guidelines, who should be screened for hereditary hemochromatosis?
Elevated liver tests, abd iron studies, 1st degree relative dx, evidence of liver disease, suggestive sxs
34
What condition is a very rare hereditary disorder of copper metabolism that leads to decreased excretion of copper into bile and accumulation of copper in the liver?
Wilson's disease
35
Hepatic presentation of Wilson's disease includes what?
Acute hepatitis, chronic liver disease, acute or chronic liver failure
36
In Wilson's disease, once the liver's capacity for copper is exceeded, copper is released into the bloodstream and starts to accumulate where?
Brain, cornea, joints, kidney, heart, pancreas
37
What are the common clinical manifestations of Wilson's disease? (systems)
Hepatic, neurologic, psychiatric
(neuro/ psych: confusion, dysarthria, incoordination/ ataxia, Parkinsonism, seizures, dystonia, spasticity, personality/ behavior changes)
38
What is pathognomonic for Wilson's disease?
Kayser-Fleischer ring + neuro manifestations
39
Pt presents with a brownish or gray-green color to their eye and you note neurologic manifestations as well. What are you concerned for and what should you do?
Wilson's disease/ Kayser-Fleisher ring
Get ophthamology exam
40
What lab diagnostics should be ordered with suspicion of Wilson's disease?
Serum ceruloplasmin (low, \<5 = strong evidence), 24 hr urinary copper (inc), liver bx +/- molecular testing (dx confirmation), AST/ ALT (N/ low)
41
If available, what is recommended for 1st degree relatives of pts with Wilson's disease?
Screening w/ genetic analysis
42
What is the treatment for Wilson's disease?
Chelating agents and transplant if liver failure
43
Alpha-1 antitrypsin deficiency is a genetic disorder characterized by decreased levels of alpha-1 antitrypsin in circulation. What is the pathophysiology of this condition?
Liver cells make alpha-1 antitrypsin → abn shape get stuck in liver cells → accumulation in hepatocytes
44
What is the role of alpha-1 antitrypsin in the body?
Protects against tissue injury
45
What levels of alpha-1 antitrypsin in circulation indicate severe disease?
\< 11 micromole/ L
46
Adults with an alpha-1 antitrypsin deficiency are at risk for what?
Severe lung disease and chronic liver disease
47
Who are at an increased risk for alpha-1 antitrypsin def and who is more likely to present with severe liver disease?
Increased risk w/ cigarette smoking (and accelerated onset)
Infants and children more likely to present w/ severe liver disease
48
What disease might you suspect in a non-smoker with emphysema at a young age (\< 45 yo), emphysema with predominant basilar changes on CXR, or in neonatal cholestasis/ childhood cirrhosis?
Alpha-1 antitrypsin def
49
Who should be screened for alpha-1 antitrypsin def?
Emphysema in abn population, adult onset asthma, clinical findings/ hx or unexplained chronic liver disease, FH of emphysema/ liver disease, hx of panniculitis
50
What skin manifestations can be a/w alpha-1 antitrypsin def?
Panniculitis
51
What lab diagnostics should be ordered if suspicion for alpha-1 antitrypsin def?
AST/ ALT (mild elevation) serum alpha-1 antitrypsin (dec), alpha-1 antitrypsin phenotype/ genotype, +/- liver bx
52
What is the tx for alpha-1 antitrypsin def?
Tx of liver disease = liver transplant
Genetic counseling (risk of passing to children)
53
What disease can manifest with variable presentation ranging from asx → acute/ chronic liver inflammation → acute liver failure +/- hx of autoimmune related disease?
Autoimmune hepatitis (AIH)
54
How will hutoimmune hepatitis (AIH) present with acute onset (\<30 days)? (4)
Hepatomegaly/ tenderness, jaundice, splenomegaly, fever
55
What labs should be ordered first in the dx of autoimmune hepatitis (AIH)?
**ANA, SMA, IgG, LKMA-1, liver bx**
(LMKA = liver kidney microsomal antibody, order for children initially and to secure dx in adults)
(other tests: LC-1, anti-SLA, LKMA-3, pANCA, IAIHG scoring system)
56
How is autoimmune hepatitis (AIH) diagnosed?
Serological markers w/ exclusion of viral/ other causes and histologic features (interface hepatitis) on bx
57
What is the management for autoimmune hepatitis (AIH)?
* **Refer to gastroenterologist/ hepatologist**
* **Prednisone +/- Azothioprine @ lowest dose ≥ 2 yrs (continue until remission)**
* Liver transplant if liver failure
* Monitor DEXA (give calcium + vit D)
* Vaccinate
* Screen for autoimmune conditions
58
What types of viral hepatitis are only acute?
A and E
(although B, C and D **can** present acutely)
\*only **A**cut**E**
59
What types of viral hepatitis are transmitted via fecal-oral routes?
E and A
\***E**at **A**ss
60
For which types of viral hepatits can you get a vaccine?
A and B
**AB**le to get vaccine
61
What type of viral hepatitis typically presents chronically?
C
62
Hep D is depdenent on what?
Hep B
63
In what population can Hep E be fatal in?
Pregnancy
64
In what areas is HAV common in due to transmission via fecal-oral routes from person to person or contaminated food or water?
Areas with inadequate sanitation
(can also be seen w/ homeless- outbreaks in USA)
65
What population is more likely to be sx/ asx with Hep A?
Children \< 6 yo - asx
Adults- sx
66
Pt presents with **jaundice, dark urine, pruritus, and light colored stool**. Hx of fever, malaise, anorexia, N/V, RUQ pain. On PE you note hepatomegaly. What are you concerned for?
Hep A (icteric phase)
67
In addition to elevated LFTs, what antibodies will be positive with Hep A?
IgM anti-HAV (+) = acute infection (sx onset)
IgG anti-HAV (+) = immunity
68
What is the typical management for Hep A?
Fluids and rest (most recovered by 6 mos)
69
Who should be hospitalized with Hep A?
Elderly, multiple comorbidites, underlying liver disease, fulminant liver failure
70
What is included in the management of Hep A aside from supportive care?
Infection precautions, notify local health dept, post-exposure prophylaxis for non-immune close contacts, prevention with vaccine
71
How is Hep B transmitted?
Person to person via: blood, sexual contact, parenteral contact, peri-natal transmission
72
What type of hepatitis is a leading cause of cirrhosis and hepatocellular carcinoma worldwide?
HBV
73
Is acute hep B typially sx or asx?
Asx
(if sx: N/V, RUQ pain, jaundice, malaise, arthralgias, fever)
74
What lab findings are a/w acute hep B?
Elevated bilirubin, ALP, and transaminases
(ALT \> 15x)
75
What % of pts with acute hep B develop chronic disease?
\<5% of adults will become chronic, 80-90% of infants will become chronic
76
Although rare, what complication is a/w acute hep B?
Fulminant hepatic failure
(severe and sudden)
77
What is the management for hep B?
Supportive care (majority recover w/ immunity)
+/- antiviral therapy if acute liver failure/ prolonged disease
78
When should pts with hep B be admitted to the hospital?
Underlying liver disease, mulitple comorbidities, signs of liver failure, older/ elderly (more severe if \> 60 yo)
79
Hep B vaccine results in presence of what antibody?
Anti-HBs (surface)
(core antibody will be negative)
80
Hep B infection (current or previous) results in presence of what antibody?
Anti-HBc and anti-HCs (surface and core)
81
What lab findings are a/w chronic hep B?
HepB surface Ag (HBsAg) \> 6 months, elevated transaminases
82
What are the complications of chronic hep B?
Cirrhosis, HCC (hepatocellular carcinoma) (can occur w/o cirrhosis)
83
Acute hep B most often becomes chronic in what pt populations?
IMC adult or if exposure as an infant or child \< 5 yo
84
If lab work shows + IgM anti-HBc, what does this indicate?
Acute hep B infection
85
If lab work shows + IgG anti-HBc or total anti-HBc, what does this indicate?
Previous infection
86
What is used as an index of infectivity and marker of replication for Hep B?
HepB e-antigen (HBeAg), a/w higher levels of HBV DNA
87
Antibody to hep B e-antigen (anti-HBe) w/ HB e Ag negative status indicates what?
Lower levels of HBV DNA, predictor of long term clearance of HBV
88
If interpretation of Hep B serologic test results are unclear (HBsAg (-), total anti-HBc (+), anti-HBs (-))... this indicates 4 possibilies: resolved infection, false +, low level chronic infection, resolving acute infection. What is the management?
Diagnose and refer to GI
89
What is the general management for a pt acutely infected with Hep B?
Monitor and ensure immunity developed
90
What is the management for chronic Hep B?
* HCC surveillance (increased risk)- all pts with cirrhosis, and q 6 months for pts w/o cirrhosis
* Vaccinate for Hep A
* Counsel on prevention precations
91
**IV drug use, intra-nasal drug use, tattoos and piercings** are RFs for what type of hepatitis?
(others: recipient of clotting factors, blood transfusion, HIV infection, hemodialysis, incarceration, known exposure, children born to infected mother, sexual contact)
Hep C
92
Is acute HCV typically sx or asx?
Asx
(if sx: jaundice, fatigue, fever, nausea, vomiting, RUQ discomfort)
93
Aside from AST/ ALT in 100s (usually \< 300) and elevated bilirubin, what other lab values will be found for Hep C? (serology)
(-) Hep C Ab w/ (+) RNA viral load
OR
(+) Hep C Ab prior had (-) Hep C Ab
94
When evaluating for chronc Hep C, if HCV Ab and HCV RNA are negative, what should you do?
Repeat HCV RNA w/ + RF in \> 3 weeks
95
When evaluating for chronc Hep C, if HCV Ab and HCV RNA are positive, what should you do?
DAA (direct acting antiviral), do not await spontaneous resolution
96
What is recommended due to many new cases of chronic HCV in 20's age group due to IVDU?
1 time testing for all 18 yo or older
97
When do most pts with chronic Hep C present?
Late in disease course, signs of cirrhosis
98
What is included in the management of Hep C?
Avoid EtOH intake, screen for HAV/ HBV/ HIV, vaccinate, non-infected sexual partner testing, check for pregnancy
99
If after "cure" of Hep C with direct acting antiviral (DAA), ALT/ AST is elevated w/o cause, what should you do?
Cure does not prevent re-infection
Refer to GI for tx with DAA x 8-12 weeks
100
Due to the risk for HCC in a pt with Hep C, what is routine management?
Regular imaging + AFP for HCC screening, monitor for cirrhosis
101
Oral regimens for DAA prescribed after GI referral is based on what? (4)
Genotype/ previous tx, +/- cirrhosis, +/- compensation, +/- co-infection with HBV before tx
102
Is DAA (direct acting antiviral) approved for treatment during pregnancy?
NO
103
HDV Abs and HDV RNA with + HBsAg is associated with what?
More severe Hep D
104
How is Hep D prevented?
Hep B vaccination
105
What is the tx for Hep D?
Interferon (IFN) alfa/ weekly x 1 year +/- tx for Hep B
(no specific tx for acute Hep D- defer tx to liver transplant facility)
106
How does Hep E present?
Acute hepatitis sxs
107
How is Hep E diagnosed?
Hep E RNA
108
In what population can Hep E be fatal?
Pregnant women (more severe/ greater mortality in 2nd and 3rd trimester)
109
What is the management of Hep E?
Supportive
