Hepatobiliary worksheet with answers-Paulson (exam 3) Flashcards

1
Q

Primary sclerosing cholangitis is a progressive disease of the liver and _______, characterized by?

A
  • gallbladder

- characterized by inflammation and scarring/fibrosis of intrahepatic and/or extrahepatic bile ducts

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2
Q

Primary sclerosing cholangitis:

-Sx?

A
  • Patients may be asymptomatic, or complain of jaundice, pruritis, fatigue, and RUQ pain
  • Hepatomegaly and splenomegaly may also be seen
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3
Q

Primary sclerosing cholangitis:

  • Labworks shows a ______ pattern
  • positive for which antibody?
A
  • cholestatic pattern is seen, with **ALP usually predominantly elevated
  • Positive P-ANCA (Perinuclear Anti-Neutrophil Cytoplasmic Antibody), elevated bilirubin, and elevated ALT/AST are also likely
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4
Q

Primary sclerosing cholangitis:

-Diagnosis is typically made by _____

A

**MRCP or ERCP–> Multifocal stricturing and dilation of intrahepatic and/or extrahepatic bile ducts can be seen

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5
Q

Primary sclerosing cholangitis is strongly associated with ______

A
  • *inflammatory bowel disease, 90% with ulcerative colitis

- +/- Crohn disease

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6
Q

Primary sclerosing cholangitis:

Pts with PSC are at an increased risk for developing _______

A

cholangiocarcinoma (type of cancer)

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7
Q

Primary sclerosing cholangitis:

tx?

A
  • The best definitive treatment is liver transplantation.

- Meds: UDCA(ursodeoxycholic acid) often used, though no meds have been shown to slow the disease process

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8
Q

Primary Biliary Cirrhosis is an autoimmune disease of _______

A

**intrahepatic small bile ducts

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9
Q

Primary Biliary Cirrhosis: pathophysiology

-T cells attack the intrahepatic small bile ducts and they ____

A

**they are gradually destructed and disappear–> leads to cholestasis–> cirrhosis/liver failure

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10
Q

Primary Biliary Cirrhosis:

-Most Pts are _____

A

**middle aged women

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11
Q

Primary Biliary Cirrhosis:
Are most patients symptomatic?
-Sx?

A

NO! Symptoms include fatigue, pruritis RUQ discomfort, jaundice, hepatomegaly.

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12
Q

Primary Biliary Cirrhosis:

  • Labs?
  • almost all Pts have positive _______ antibodies
A
  • *cholestatic pattern. ALP and GGT usually very elevated

- **Almost all patients have positive antimitochondrial antibodies (AMA)

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13
Q

Primary Biliary Cirrhosis:

-1st line tx?

A

UDCA (ursodeoxycholic acid)

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14
Q

Autoimmune Hepatitis= a chronic disease with continuing _________

A

**hepatocellular inflammation and necrosis that tends to progress to cirrhosis

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15
Q

Autoimmune Hepatitis:

-Sx?

A

-Patients may be asymptomatic, OR may have Sx such as: hepatosplenomegaly, stigmata of chronic liver disease, jaundice, or acute liver failure.

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16
Q

Autoimmune Hepatitis:

  • Labs?
  • positive for which antibodies?
A
  • Transaminases and bilirubin are elevated

- Smooth muscle antibodies and a positive ANA

17
Q

Autoimmune Hepatitis:

-what establishes the diagnosis?

A

**Liver biopsy

18
Q

Autoimmune Hepatitis:

-tx?

A

prednisone and azathioprine. (Then taper prednisone down)

19
Q

Acute Liver Failure definition

A

The development of severe acute liver injury with encephalopathy and elevated INR (>1.5) in a patient without cirrhosis or preexisting liver disease.

20
Q

Acute Liver Failure:
what is the MC cause?
-other common causes?

A
  • acetaminophen toxicity= MC

- Other very common causes= drug reactions and viral hepatitis

21
Q

Acute Liver Failure:

manifestations? (list 6) (pain in which quadrant?)

A
  • encephalopathy (by definition)
  • jaundice
  • hepatomegaly
  • RUQ tenderness
  • renal failure
  • thrombocytopenia
22
Q

Acute Liver Failure: which 2 things often complicate acute liver failure?

A

-Adrenal insufficiency and cardiac injury often complicate acute liver failure

23
Q

Acute Liver Failure: Labs? (what is elevated)

A
  • PT/INR >1.5
  • also ↑ ammonia
  • elevated LFTs
24
Q

Acute Liver Failure:

  • tx for tylenol overdoses?
  • It is KEY to prevent the development of _____
A
  • acetylcysteine is used

- ***Prevention of the development of cerebral edema is key

25
Acute Liver Failure: | -definitive treatment?
*liver transplantation
26
NAFLD(nonalcoholic fatty liver disease) is subdivided into 2 categories:
- NAFL (nonalcoholic fatty liver) | - NASH (nonalcoholic steatohepatitis)
27
Difference b/w NAFL and NASH?
-In NAFL, hepatic steatosis is present WITHOUT any significant inflammation, but in NASH, hepatic steatosis is associated with inflammation.
28
NASH may be histologically indistinguishable from _______
**alcoholic steatohepatitis
29
NAFLD may progress to _______
cirrhosis.
30
NAFLD: major risk factors?
obesity, DM2, dyslipidemia, metabolic syndrome
31
NAFLD: Sx?
Most patients are asymptomatic. They might have fatigue, malaise, vague RUQ discomfort.
32
NAFLD: Labs show mild-moderate elevations of ____
mild-mod elevations of AST/ALT
33
NAFLD: definitive diagnosis made by ______ | -tx?
-liver biopsy. Treatment: Weight loss. This has been associated with histologic improvement. -Patients should also avoid heavy alcohol consumption.
34
Hemochromatosis: definition
Excess iron absorption and deposition in parenchymal cells of the heart, liver, pancreas, adrenals, testes, pituitary, & kidneys.
35
Hemochromatosis: MC in which population?
Men (usually begin after age 40-50) | -Autosomal recessive.
36
Hemochromatosis: clinical Sx?
- weakness/lethargy - skin hyperpigmentation - arthralgia - cardiac enlargement +/- heart failure - diabetes - ED/impotence
37
Hemochromatosis: Labs---> serum _____ is elevated with increased _____ -which test can eliminate the need for a liver bx?
- **Serum iron is elevated, with INCREASED transferrin saturation and ferritin - **Genetic testing can eliminate the need for liver biopsy
38
Hemochromatosis: tx?
-**Therapeutic phlebotomy until iron stores are depleted, then begin maintenance (less frequently)
39
Hemochromatosis: tx if Pt CANT tolerate phlebotomy? | -what should these Pts avoid?
- chelation therapy is recommended | - These patients should avoid iron, vitamin C, and alcohol.