Upper GI and Hepatobiliary System

Question 1

Primary Biliary Cirrhosis

Answer 1

Autoimmune liver disorder with progressive destruction of intrahepatic bile ducts, leading to cholestasis, cirrhosis and liver failure.

Question 2

In what population should you suspect primary biliary cirrhosis in when they present with unexplained pruritus, fatique, RUQ pain, and/or jaundice?

Answer 2

Middle-aged women

Question 3

What are some classic S/SX of primary biliary cirrhosis?

Answer 3

Insidious fatigue, pruritus, dry mouth, and RUQ pain

Question 4

What is the work-up for primary biliary cirrhosis and what would you expect?

Answer 4

Labs: GGT (elev.), ALP (elev.), minimally abnormal AST and ALT, anti-mitochondrial antibodies (elev.)
Procedure: biopsy

Question 5

Diffuse liver damage due to inadequate blood or O2?

Answer 5

Ischemic hepatitis

Question 6

What are possible causes of ischemic hepatitis?

Answer 6

Heart failure or acute hypotension, resp. failure or CO2 toxicity, inc. metabolic demand (e.g. sepsis)

Question 7

What are the s/sx of ischemic hepatitis?

Answer 7

N/V, tender hepatomegaly

Question 8

What is the work-up for ischemic hepatitis and what would you expect to see?

Answer 8

Labs: LFT (very high AST and ALT), bilirubin (mod. inc.), LDH (inc. within hour of ischemic event)
Imaging: U/S. MRI, or arteriography to I.D. obstructed vessel

Question 9

Focal damage to the biliary tree due to disrupted flow from the hepatic artery via peribiliary arterial plexus?

Answer 9

Ischemic cholangiopathy

Question 10

What is the main cause for ischemic cholangiopathy?

Answer 10

Vascular injury during surgical procedures

Question 11

What are the s/sx of ischemic cholangiopathy?

Answer 11

Pruritis, pale stool

Question 12

What is a typical work-up in a pt with suspected ischemic cholangiopathy?

Answer 12

Labs: reveal cholestasis; UA
Imaging: U/S initially; follow-up with MRCP and/or ERCP to r/o cholelithiasis or cholangiocarcinoma

Question 13

Diffuse venous congestion in the liver that results from RCHF which leads to increased central venous pressure?

Answer 13

Congestive hepatopathy

Question 14

What are the s/sx of congestive hepatopathy?

Answer 14

Most are asx, can see RUQ discomfort, severe congestion can result in jaundice

Question 15

What would a PE on a pt presenting with congestive hepatopathy reveal?

Answer 15

Ascites, hepatomegaly, (+) hepatojugular reflex

Question 16

What would a work-up look like in a pt suspected to have congestive hepatopathy?

Answer 16

Moderately elevated LFTs

Question 17

Obstruction of the hepatic venous outflow from small hepatic veins inside the liver to the inferior vena cava and R atrium?

Answer 17

Budd-Chiari Syndrome

Question 18

What is the most common cause of Budd-Chiari Syndrome?

Answer 18

Patients who are in hypercoagulable states

Question 19

What are the s/sx of Budd-Chiari Syndrome?

Answer 19

Acute obstruction: fatigue, RUQ pain, N/V, mild jaundice
Chronic obstruction: may be asx in some patients until it progresses and may cause fatigue, abdominal pain, and hepatomegaly, lower extremity edema

Question 20

What does a work-up in a pt with possible Budd-Chiari Syndrome look like?

Answer 20

Labs: LFTs
Imaging: vascular imaging

Question 21

Endothelial injury, leading to non-thrombotic occlusion of the terminal hepatic venules and hepatic sinusoids?

Answer 21

Veno-occlusive disease

Question 22

What are some common causes of Veno-occlusive disease?

Answer 22

Irradiation, transplant rejection, hepatotoxins

Question 23

Increased resistance to blood flow, which commonly arises from dz within the liver itself or uncommonly from blockage of the splenic or portal vein?

Answer 23

Portal HTN

Question 24

What are the common causes of portal HTN?

Answer 24

Cirrhosis (developed countries), Schistosomiasis (in endemic areas), hepatic vascular abnormalities

Question 25

What are some concomitants with a Dx of portal HTN?

Answer 25

Esophageal varices, portal-systemic encephalopathy

Question 26

What are the s/sx of portal HTN?

Answer 26

Often asx | Sx arise from acute variceal bleeding --> sudden, painless, upper GI bleed

Question 27

What are the PE findings in a pt who may have portal HTN?

Answer 27

Low systolic BP, splenomegaly, ascites, peripheral edema, dilated abdominal wall veins, and caput medusae. May present with jaundice and spider angiomas

Question 28

What is a typical work-up for portal HTN?

Answer 28

US or CT that reveals dilated intraabdominal collateral arteries

Question 29

Neuropsychiatric syndrome where absorbed products that would otherwise be detoxified through the liver end up in systemic circulation where they may be toxic to the brain?

Answer 29

Portal-systemic encephalopathy

Question 30

What are s/sx of portal-systemic encephalopathy?

Answer 30

Constructional apraxia, characteristic flapping tremor (asterixis)

Question 31

What is a typical work-up for a pt with portal-systemic encephalopathy?

Answer 31

Psychometric eval CMP: electrolytes, albumin, LFT EEG shows diffuse slow-wave activity

Question 32

What are the three types of postoperative dysfunctions?

Answer 32

Postoperative jaundice Postoperative hepatitis Postoperative cholestasis

Question 33

What are some types of solid benign liver tumors?

Answer 33

Hepatocellular adenoma, focal nodular adenoma, hemangiomas, lipomas, and fibromas

Question 34

What is the most common type of primary liver cancer and where is it most prevalent?

Answer 34

Hepatocellular carcinoma | Most prevalent in E. Asia and sub-Saharan Africa

Question 35

What are the s/sx of hepatocellular carcinoma?

Answer 35

Previously stable cirrhosis pt presents with RUQ pain, wt loss, RUQ mass, and unexplained deterioration.

Question 36

What is a typical work-up in a pt with suspected hepatocellular carcinoma?

Answer 36

Labs: Alpha-Fetoprotein measurement (elev.) Imaging: CT, U/S, or MRI Procedure: biopsy

Question 37

What are the more common primary sites of metastatic liver cancer?

Answer 37

GI, breast, lung, pancreas, leukemia

Question 38

What is the work-up for someone with suspected metastatic liver cancer?

Answer 38

CT or MRI with contrast | Biopsy of nodule is definitive

Question 39

Presence of one or more calculi (gallstones) in the gallbladder

Answer 39

Cholelithiasis

Question 40

What are the five F's for developing cholelithiasis?

Answer 40

Female, fat, forty, fertile, family hx

Question 41

What are the three types of gallstones and which is most common?

Answer 41

Cholesterol stones - most common Black pigment stones Brown pigment stones

Question 42

What are the s/sx of cholelithiasis?

Answer 42

80% are asx | RUQ pain that radiates to back or down the arm, episodic, N/V with episode

Question 43

What is the work-up for a pt suspected to have cholelithiasis?

Answer 43

U/S

Question 44

Inflammation of the GB that develops over hours, usually because a gallstone obstructs the cystic duct?

Answer 44

Acute cholecystitis

Question 45

What are the s/sx of acute cholecystitis?

Answer 45

Pain similar to cholelithiasis, but lasts longer. Vomiting is common.

Question 46

What are any significant PEs that one may find in acute cholecystitis?

Answer 46

Right subcostal tenderness, (+) Murphy's sign, fever

Question 47

What is a typical work-up for acute cholecystitis?

Answer 47

Abdominal U/S; CT to help identify complications

Question 48

Longstanding GB inflammation almost always due to stones

Answer 48

Chronic cholecystitis

Question 49

What is it called when there is extensive damage done to the GB due to fibrosis?

Answer 49

Porcelain gallbladder

Question 50

What is the difference in PE for acute vs. chronic cholecystitis?

Answer 50

In chronic, pt is afebrile

Question 51

Biliary colic without gallstones, resulting from structural or functional disorders?

Answer 51

Acalculous biliary pain

Question 52

The occurrence of abdominal sxs after cholecystectomy?

Answer 52

Postcholecystectomy Syndrome

Question 53

What are the s/sx of postcholecystectomy syndrome?

Answer 53

Dyspepsia, non-specific biliary sxs, persistent abdominal pain

Question 54

What is a work-up for PCS?

Answer 54

Biliary manometry

Question 55

Presence of stones in the bile ducts causing biliary colic, biliary obstruction, gallstone pancreatitis, or cholangitis?

Answer 55

Choledocholithiasis

Question 56

What are the four types of stones in choledocholithiasis?

Answer 56

Primary stones Secondary stones (most common) Residual stones Recurrent stones

Question 57

Bile duct obstruction allows bacteria to ascend from duodenum resulting in infxn and inflammation

Answer 57

Acute cholangitis

Question 58

What is Charcot's triad in acute cholangitis?

Answer 58

Abdominal pain, Jaundice, Fever or chills

Question 59

What are pertinent PE findings in acute cholangitis?

Answer 59

RUQ tenderness, tender and enlarged liver, confusion and hypotension (PANIC!)

Question 60

Intrahepatic brown stone formation leading to repeating cycles of obstruction, infxn, and inflammation?

Answer 60

Recurrent Pyogenic Cholangitis

Question 61

Chronic choleastatic syndromes characterized by patchy inflammation, fibrosis, and strictures of the intrahepatic and extrahepatic bile ducts?

Answer 61

Primary Sclerosing Cholangitis

Question 62

What are the s/sx of primary sclerosing cholangitis?

Answer 62

Progressive fatigue then pruritus, jaundice later, steatorrhea and deficiencies of fat-soluble vitamins, symptomatic gallstones and choledocholithiasis in 75%

Question 63

What is the work-up for sclerosing cholangitis?

Answer 63

Lab: ALP and GGT elev., Globulins and IgM elev., antimitochondrial Ab is NEGATIVE Imaging: U/S to r/o biliary obstruction, MRCP

Question 64

Biliary obstruction secondary to biliary tract strictures caused by various opportunistic infxns in AIDS pts?

Answer 64

AIDS Cholangiopathy

Question 65

What are the three main types of GB and bile duct malignancies?

Answer 65

Cholangiocarcinoma GB Carcinoma GB Polyps

Question 66

What is the PE sign for peritonitis?

Answer 66

Blumberg sign

Question 67

What are the PE signs for appendicitis?

Answer 67

McBurney's point tenderness Rovsing's sign Psoas sign Obturator sign

Question 68

What is the PE sign for cholecystitis?

Answer 68

Murphy's sign

Question 69

What are the four major mechanisms of diarrhea?

Answer 69

Osmotic Secretory Exudative Motility

Question 70

What are the 5 main PE findings in diarrhea?

Answer 70

``` Dehydration Failure to thrive and malnutrition Abdominal pain Borborygmi Perianal erythema ```