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153 Cards in this Set

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What are the extrahepatic effects of liver failure?
Coma (hepatic encephalopathy)
Scleral icterus
SPider nevi (estrogen effect)
Gynceomastic (estrogen effect)
Jaundice
Testicular atrophy
Coarse hand tremor
Bleeding tendency (dec'd prothrombin and clotting factors)
Ankle edema
What are the lab values of liver failure?
Low PLT
Elevated PT, INR
Dec'd lipids
Micro vs Macronodular Cirrhosis:
Criteria
Causes
Micronodular--nodules <3mm; uniform size
Due to metabolic insult (EtOH, hemochromatosis, Wilson's Dz)

Macronodular: Nodules >3mm, varied size

Due to significant liver injury leading to hepatic necrosis (postinfectious or drug-induced hepatitis); inc'd risk HCC
Micronodular cirrhosis of liver; from EtOH pt.
Cirrhosis; regenerative lesions surrounded by fibrotic bands of collagen (bridging fibrosis), forming characteristic nodularity
Serum Albumin: Ascites Gradient:
What is it?
What does it tell you?
SAAG + serum albumin - ascites albumin

If SAAG >1.1-->portal HTN

If SAAG <1.1-->cancer, nephrotic syndrome, TB, panc/biliary dz, CT dz
When is alkaline phosphatase elevated?
Obstructive liver dz (HCC), bone dz, bile duct dz
When is GGT elevated?
GGT =gamma-glutamyl transpeptidase; elevated with verious liver dz; esp heavy EtOH consumption
AST/ALT:
Changes based on dz
ALT > AST - viral hepatitis
AST > ALT - EtOH
AST elevated - MI
Reye's Syndrome;
Pathophys
Presentation
Childhood hepatoencephalopathy

Presents with mitochondrial abnlts, fatty liver dz, hypoglycemia, coma

Assocd w/viral infeciton (VZV, H flu B) treated with ASA

ASA metabolites dec beta-oxidation by inhibiting mitochondrial enzyme
Fatty liver; early reversible change assocd w/EtOH consumption. Fat-filled vacuoles, no inflammn due to fibrosis of more serious alcoholic liver damage.
Fatty liver, gross specimen showing enlarged yellow appearance.
When do mallory bodies present?
After sustained, long-term EtOH consumption.

(note: fatty liver dz is reversible)
Hepatocellular carcinoma:
Lab values
Presentation
Method of spread
Inc'd AFP

Presents with jaundice, tender hepatomegaly, ascites, polycythemia, hypoglycemia

Spread via hematogenous route

May lead to Budd-Chiari syndrome
Nutmeg liver:
Cause
Due to backup of blood in liver

Commonly caused by right-sided heart failure and BUdd-Chiari syndrome
Budd-Chiari Syndrome:
Pathophys
Associated with?
Occlusion of IVC or hepatic veins-->centrilobular congestion and necrosis

-->Congestive liver dz (hepatomegaly, ascites, abdominal pain, liver failure)

Assocd w/hypercoag state, polycythemia vera, pregnancy, HCC
alpha1-antitrypsin deficiency:
Pathophys
Effects
Diagnostics
Misfolded gene protein product accumulates in hepatocellular ER-->dec'd elastic tissue in lungs-->panacinar emphysema

Leads to elevated PAS-positive globules in liver
What are causes of increased bilirubin production?
Hemolytic anemia
Sickle cell anemia
Hematoma breakdown (newborn delivered by vacuum delivery and develops hematoma on scalp)
What are causes of impaired bilirubin uptake and storage?
Postviral hepatitis
Drug rxns
What are causes of decreased UDP-GT activity?
Gilbert's
Crigler-Najjar
Neonatal Jaundice--more common in premies
When is impaired bilirubin transport seen?
Dubin-Johnson Syndrome
Rotor Syndrome
When is biliary epithelial damage seen?
Hepatitis
Cirrhosis
Liver Failure
When is extrahepatic biliary obstruction seen?

Presentation?
Labs?
Pancreatic neoplasms
Choledocholithiasis
Pancreatitis
Cholangiocarcinoma

All result in inc'd pressure in intrahepatic ducts-->injury/fibrosis and bile stasis


Presents as pruritus, jaundice, dark urine, HSM, light-colored stool

Elevated conj'd bilirubin, inc'd cholesterol, inc'd alk phos
When is intrahepatic biliary obstruction seen?
Primary biliary cirrhosis
Sclerosins cholangitis
Drugs
Primary Biliary Cirrhosis:
Pathophys
Labs
Presentation
Associated conditions
Autoimmune reaction-->lymphocytic infiltrate + grnaulomas

Leads to inc'd serum mitochondrial Ab including IgM
60% positive p-ANCA


Presents as pruritus, jaundice, dark urine, HSM, light-colored stool

Ascod w/CREST, RA, Celiac Dz
Primary Sclerosing Cholangitis:
Pathophys
Labs
Presentation
Associdated conditions
Unkonwn cause of concentric onion skin bile duct fibrosis-->strictures and dilation with beading of intra- and extrahepatic bile ducts on ERCP

BEADING


Presents as pruritus, jaundice, dark urine, HSM, light-colored stool

Labs show elevated IgM

Assocd w/ulcerative colitis; can lead to secondary biliary cirrhosis
Wilson's Disease:
Patphophys
Presentation
Treatment
inadequate hepatic copper excretion

Presents with:
Asterixis

Dec'd Ceruloplasmin
Corneal deposits
Cirrhosis
Carcinoma
Dementia

Tx: Copper pennies-->Penicillamine
Kayser-Fleischer ring in Wilson's Dz
Hemochromatosis:
Pathophys
Presentation
Labs
Iron deposition

Bronze diabetes (skin pigmentaiton and DM)
Results in CHF, inc'd risk HCC

May be primary (AR inheritance) or secondary to chronic transfusion tx (beta-hthal major)

Labs:
Inc'd ferritin
Inc'd Iron
Dec'd TIBC
Inc'd transferrin saturation
Hemochromatosis with cirrhosis. Prussian blue iron stain shows hemosiderin in liver parenchyma.
A young man presents with ataxia and tremors. He has brown pigmentation in a ring around the periphery of his cornea.

What treatment should he receive?
Penicillamine
An 80 year-old woman comes to your clinic because her family is concerned about her yellowing skin. Exam reveals yellowing of the skin including the palms and soles but no scleral icterus.

What question could you ask the patient that would most likely identify the cause of the yellowing?
Jaundice always results in scleral icterus.

Sounds like carotenemia, ask if eating carrots.
A 20 year-old man contracts influenza then presents with an idiopathic hyperbilirubinemia.

Cause?
Gilbert's
What is the fate of bilirubin after it's conjugated and secreted into the GI tract?
Acted upon by gut bacteria, forming urobilinogen

Some urobilinogen excreted, some reabs'd
A patient presents complaining of pain in the right upper quadrant that he can point to with one finger. The area is tender to light touch and pain is worsened when the patient is asked to raise his arms above his head.

Diagnosis?
Points to with one finger-->musculoskeletal pain, rectus abdominis tear
Diagnose:
Most common cause of acute RLQ pain
Appendicits
Diagnose:
50 year-old female presents with pruritis without jaundice, lab reveals (+) AMA
PBC
Diagnose:
Most common cause of acute LLQ pain
Diverticulitis
Diagnose:
Gluten sensitivity
Celiac Sprue
Diagnose:
A patient with Gl bleeding has buccal pigmentation
Peutz-Jeghers
Diagnose:
Colonoscopy reveals very friable mucosa extending from the rectum to the distal transverse colon
Ulcerative colitis
Diagnose:
A small intestinal mucosa laden with distended macrophages in the lamina propria (that are filled with PAS(+) granules and rod-shaped bacilli seen by
electron microscopy)
Whipple Dz
Diagnose:
Most common cause of RUQ pain
Cholecystitis
Diagnose:
Liver biopsy on a 23 year-old female with elevated levels of LKM-1 antibodies, no alcohol history, and no viral serologic markers reveals infiltration of the
portal and periportal area with lymphocytes
Autoimmune hepatitis
Diagnose:
Diarrhea, fever, and abdominal cramps following a course of antibiotics
Pseudomembranous colitis
Diagnose:
Fatal disease of unconjugated bilirubin resulting from a complete lack of UDPGT activity
Crigler-Najjar I
Diagnose:
Radiography reveals a "string-sign" in the terminal ileum
Crohn's
Diagnose:
Nonfatal disease of unconjugated bilirubin resulting from low levels of UDPGT activity
Gilbert's or Crigler-Najjar II
Diagnose:
Elevated levels of serum ferritin and increased transferrin saturation
Hemocrhomatosis
Diagnose:
Alpha-fetoprotein levels >1 000 pg/mL
HCC
Diagnose:
Elevated serum copper, decreased serum ceruloplasmin, and elevated 24-hr urinary copper
Wilson's
Diagnose:
Liver disease+ lung emphysema
alpha1-antitrypsin dz
Diagnose:
ERCP reveals alternating strictures and dilation
Primary Sclerosing Cholangitis
Diagnose:
60 year-old female with rheumatoid arthritis and no alcohol history presents
with fatigue and right abdominal pain, lab studies reveal high levels of ANA and ASMA, elevated serum lgG levels, and no viral serologic markers
Autoimmune hepatitis
Diangose:
Total or subtotal atrophy of the small bowel villi, plasma cells and lymphocyte infiltration into the lamina propria and epithelium, and hyperplasia/elongation of crypts
Celiac Sprue
Cholelithiasis:
Risk Factors
Presentation
Effects
4F's:
Female, fat, fertile, forty
(Feathers--Pima Indians)

Presents with jaundice, fever, RUQ pain; positive Murphy's sign--inspiratory arrest on deep palpation due to pain

Can cause cholecystitis; ascending cholangitis, acute pancreatitis, bile stasis
Biliary colic:
Pathophys
Obstruction of common duct by gallstones causes biel duct contraction--cause bile duct obstruction which results in bile duct contraction
Cholesterol vs Pigment Gallstones:
Appearance on ultrasound
Frequency
Causes
Cholesterol--radiolucent with 10-20% opaque due to calcifications

Assocd w/obesity, Crohn's, CF, age, fibrates, estrogen, Native American origin

Pigment stones: radiopaque; seen in pts w/chronic hemolysis, alcoholic cirrhosis, age, biliary infection
Acute pancreatitis:
Causes
Presentation
Effects (acute vs chronic)
Gallstones, EtOH***
Trauma
Steroids
Mumps
ERCP**
Sulfa Drugs, some HIV drugs

Presentation: Epigastric abdominal pain radiating to back

Labs: elevated amylase, lipase (higher specificity), can lead to DIC--this is life-threatening!

If chroniic-->pancreatic insuff; steatorrhea, fat-soluble vitamin def., DM
What is the most common cause of chronic pancreatitis?
ALCOHOL
Chronic pancreatitis; CT shows punctate calcifications in head, body, and tail of pancreas
Pancreatic adenocarcinoma:
Most common site
Presentation
Most common in pancreatic head-->obstructive jaundice

Presents with PAINLESS JAUNDICE; abdominal pain radiating to back

Weight loss
Migratory thrombophlebitis--redness and tenderness on palpation of extremities
Ondansetron:
MOA
Use
5-HT3 antagonist.

Powerful central-acting antiemetic. Controls postop vomiting and in pts undergoing chemo.

You will not vomit with ONDANSetron, so you can go ON DANCing.
What are some of the risk factors for esophageal cancer?
Achalasia, Alcohol
Barrett's
Corrosive esophagitis, Cigarettes
Diverticuli
Esophageal web
Familial
GERD
Hot dogs (nitrosamines)
What are the risk factors for colon cancer?
Tubular adenoma
Tubulovillous adenoma
Villous adenoma
High-fat, low-fiber diet
Chronic IBD
Age
Cancer syndrome (FAP, HNCC, Peutz-Jeghers, juvenile polyposis)
What are the signs of portal hypertension?
Esophageal varices
Hemorrhoids
Caput medusa
Ascites
Hematemesis, melena
Splenomegaly
What is seen in Budd-Chiari syndrome?

What conditions are a/w Budd-Chiari syndrome?
Occlusion of IVC or hepatic veins

A/W:
Polycythemia vera
Pregnancy
What is the underlying problem in Wilson's disease?

What are the characteristics of Wilson's disease?

What is the treatment for Wilson's disease?
Impaired copper excretion through bile

Asterixis
PD syx
Dec'd serum ceruloplasmin
Cirrhosis
Kayser-fleischer rings
Copper accumuln
HCC
Dementia
Dyskinesia
Dysarthria

Tx: penicillamine
What is the classic triad of symptoms in hemochromatosis?

What lab tests are used to diagnose hemochromatosis?

What is the treatment for hemochromatosis?
Bronze diabetes:
-Skin pigmentation
-DM
Cirrhosis

Labs;
Inc'd ferritin, inc'd transferrin saturation
Inc'd serum Fe, dec'd TIBC

Tx: Phlebotomy
What are the possible etiologies of acute pancreatitis?
GET SMASHED
Gallstones
EtOH
Trauma
Steroids
Mupms
Autoimmune
Scorpion stings
Hypercalcemia
Hyperlipidemia
Drugs
What is the typical presentation of a pt with pancreatic insufficiency?

What is the treatment for pancreatic insufficiency?
Diarrhea, steatorrhea, weight loss, weakness, fat soluble vitamin def (ADEK)

Tx: limit fat intake, replace pancreatic enzymes
What are the risk factors for the development of hepatocellular carcinoma?
HBC, HCV
Wilson's
Hemochromatosis
alphta1-antitrypsin def
Hepatic adenoma
Alcoholic cirrhosis
What is the difference between primary biliary cirrhosis and primary sclerosing cholangitis?
Primary Biliary Sclerosis:
+AMA
Middle-aged female
Auto-immune dz
Assocd w/other auto-immune dz

Primary Sclerosing Cholangitis:
Etiology UNK
60% assocd w/+P-ANCA
Males >40
Assocd w/UC and cholangiocarcinoma
ERCP findings of BEADING and STRICTURING
What is the most common salivary gland tumor?

What is the second most common salivary gland tumor?

What is the most common location for a salivary gland tumor?
1) Pleomorphic adenoma
2) Mucoepidermoid carcinoma

Most common location = parotid gland
What are the five 2's of Meckel's diverticulum?
2 inches long
2 feet from ileocecal vlve
2% of populn
Commonly presents in first 2 years of life
2 types of epihelium--gastric and pancreatic
What are the tumor markers for pancreatic cancer?
CEA
CA19-9
What is the typical histological neoplastic progression of colon cancer?
Tubular adenoma-->
Tubulovillous adenoma-->
Villous adenoma-->
Carcinoma
List the names of the B vitamins.
The Rich Never Pan Pyrite Filled Creeks
B1 - Thiamine
B2 - Riboflavin
B3 - Niacin
B5 - Pantothenic acid
B6 - Pyridoxine
B9 - Folic acid
B12 - Cobalamin
Name of vitamin A.
Retinol, retinal
Name of vitamin E.
alpha-tocopherol
Name of vitamin C.
Ascorbic acid
What is the most common vitamin deficiency in the US?
Folic Acid--vit B9
What is the functionally active form of thiamine?
Thiamine = vit B1
Active form = Thiamine pyrophosphate (TPP)
In what reactions does thiamine pyrophosphate have a role? List enzymes.
Pyruvate-->acetyl coA (Pyruvate DH)

alpha-ketoglutarate-->succinyl CoA (alpha-ketoglutarate DH)--TCA

Ribose 5-P<-->Glyceraldehyde 3-P (Transketolase)--HMP
How is thiamine deficiency diagnosed?
By an increase in RBC transketolase activity observed upon addition of thiamine
What characterizes dry beriberi?
Presentation?
Nonspecific peripheral neuropathy with myelin degeneration

Toe dorp, wrist drop, food drop

Muscle weakness, hyporeflexia, areflexia
What characterizes wet beriberi?
Peripheral vasodilation-->high output cardiac failure-->peripheral edema

Cardiomegaly

(wet = blood)
What are the biologically active forms of riboflavin?

What is the role of these molecules?
Riboflavin = vit B2

Active forms:
Flavin mononucleotide (FMN)
Flavin adenine dinucleotide (FAD)

Used for red-ox rxns
What are some of the symptoms associated with riboflavin deficiency?
Riboflavin = vit B2

Syx:
Dermatitis
Cheilosis--fissuring of corners of mouth
Glossitis (smooth, purple tongue)
What is angular stomatitis?

What population is this common in?
Inflammn of corners of mouth

Common in those who wear dentures
What nutrient deficiencies are a/w cheilosis, glossitis, and stomatitis?
Iron
Riboflavin
Niacin
Folate
B12 (cobalamin)
What are the biologically active forms of niacin?
Niacn = vit B3

Active forms:
NAD+ (nicotinamide adenine dinucleotide)
NADP+ (nicotinamide adenine dinucleotide phosphate)
What amino acid is required for the generation of niacin?
Tryptophan
What disease is caused by niacin deficiency? What are the symptoms of this disease?
Pellagra: Dermatitis, diarrhea, dementia

Can cause death!
Why might pellagra be seen in a population that primarily eat corn?
Tryptophan can be metabolized to form niacin, and corn lacks tryptophan.
How is niacin effective in treating type IIb hyperlipoproteinemia?
Inhibits lipolysis in adipose tissues-->less circulating FFA's-->less FA's to liver-->less VLDL made-->less LDL prouced
What is the biologically active form of pyridoxine?
Pyridoxal phosphate

Pyridoxine = B6
What is the metabolic function of pyridoxal phosphate?
Coenzyme for enzymes of amino acid metabolism (transaminations and deaminations)
What drug can lead to a deficiency of B6 in addition to B3?
Isoniazid
What are the clinical findings of B6 deficiency?
Same as riboflavin deficiency + convulsions***, hyperirritability, peripheral neuropathy
What is the biologically active form of folic acid?
Tetrahydrofolate
What are the characteristics of folic acid deficiency?
NT defects
Growth failure
Megaloblastic anemia
What are the steps involved in the intake and absorption of B12?
Pepsin in stomach releases B12 from protein-bound form
B12 binds salivary vitamin B12 binding proteins (cobalophilins)
R-B12 complex broken down in duodenum by panc protease

Unbound B12 (in duodenum) binds IF secreted by parietal cells from stomach

IF-B12 complex binds IF-specific receptors on cells of terminal ileum
B12 transverses mucosal cell and picked up by plasma protein called transcobalamin II
What specific organs are involved in the absorption of B12?
Salivary glands
Stomach
Pancreas
Distal Ileum
Descibe the stages of the Schilling test and their diagnostic utility.
1. Administration of radiolabeled 812 without intrinsic factor (to determine if there is a problem absorbing B12)

2. Administration of radiolabeled B12 with intrinsic factor (to determine if a lack of intrinsic factor is the cause of the problem)

3. Administration of radiolabeled B12 with pancreatic supplements (to determine if a lack of enzymatic degradation of R protein is the problem)

4. Administration of radiolabeled B12 after administration of antibiotics (tetracycline) or anti-inflammatory drugs (prednisone) (to determine other causes of B12 malabsorption such as bacterial overgrowth)
What is the metabolic role of biotin?
Apoenzyme in carboxylation reactions (buy-a-tin of CO2)
What can cause a deficiency of biotin?
Glycoprotein avidin found in egg whites prevents absorption of biotin (would need 20 egg whites per day)

Antibiotic use--gut bacteria make biotin
What is the main metabolic reaction that vitamin C is involved in?
Hydroxylation of prolyl and lysyl residues
What are the major sources of vitamin C deficiency-scurvy?
Sore, spongy gums
Loose teeth
Fragile BVs-->hemorrhages
Swollen joints--bleeding into joint spaces
Impaired wound healing
Anemia
What are the different forms of Vitamin A?
Retinol, retinal (used by body)
beta-carotene (cleaved in intestine to yield retinal)
Retinoic acid (can't be reduced; unusable by body)
What are the signs of vitamin A deficiency?
Night blindness
Xerophthalmia--pathologic dryness of conjunctiva and cornea-->corneal ulceration and blindness

Keratomalacia--wrinkling, clouding of cornea

Bitot's spots--dry, silver-grey plaques of bulbar conjunctiva
What are the signs of hypervitaminosis A?
HA, n/v, stupor
Skin--dry, pruritic
Liver--enlarged (possibly cirrhotic)
Bone, joint pain
Inc'd ICP
T/F: Lack of vitamin A in pregnant women has the potential for causing congenital malformations.
False, excessive Vit A in pregnant women has potential for causing congenital malformations including:
-hydrocephalus
-inhibited migration of NCC
In which patient populations is vitamin A supplementation a bad idea?
Pregnancy-->teratogenic
Smokers-->inc'd risk lung ca
What are the names for vitamin D deficiency in adults and in children?
Adults--osteomalacia
Children--rickets
What is the principle role of vitamin K?
Post-translation modification of various clotting factors where it serves as a co-enzyme in the carboxylation of certain glutamic acid residues present in these proteins.
Which proteins are vitamin K-dependent?
Protein C
Protein S
Prothrombin
Factors 2,7,9,10

ALL require vitamin K for synthesis
What is the primary function of vitamin E?
Antioxidant--prevents nonenzymatic oxidation of cell components (esp on RBCs) by molecular oxygen free radicals
What is another name for vitamin E?
alpha-Tocopherol
A patient presents with convulsions and irritability.

What vitamin deficiency is causing these symptoms in this patient?
B6
Which vitamin deficiency results in gum bleeding, bruising, anemia, and poor wound healing?
C
Vitamin C is necessary for the hydroxylation of which amino acids in collagen synthesis?
Proline and Lysine
What vitamin in excess can cause hypercalcemia?
Vitamin D, also Vitamin A
What vitamins have a function similar to reduced glutathione?
(anti-oxidant = glutathione)

A, C, E = anti-oxidants
An alcoholic develops a rash, diarrhea, and altered mental status.

What is the vitamin deficiency?
B3 (niacin)
Which vitamin deficiency:
Increased RBC fragility
E
Which vitamin deficiency:
Dermatitis, cheilosis, glossitis
B2, B3, folate, B12, Fe
Which vitamin deficiency:
Peripheral neuropathy, angular cheilosis, glossitis
B12
Which vitamin deficiency:
Hemorrhagic disease
K
Which vitamin deficiency:
Neural tube defects
Foilc acid
Which vitamin deficiency:
Dermatitis, diarrhea, dementia
B3--Niacin
Which vitamin deficiency:
Megaloblastic anemia
Folate or B12
Which vitamin deficiency:
Pernicious anemia
B12
Which vitamin deficiency:
Bitot's spots, keratomalacia, xerophthalmia
A
Which vitamin deficiency:
Osteomalacia
D
Which vitamin deficiency:
Rickets
D
Which vitamin:
Can be used to treat acne and psoriasis
A
Which vitamin:
Used in oxidation/reduction reactions
B2 (riboflavin)--FAD/FMN
Niacin--NAD, NADP
Which vitamin:
Used in carboxylation reactions
Biotin
Which vitamin:
Involved in the hydroxylation of prolyl residues
C
Which vitamin:
Requires intrinsic factor for absorption
B12
Which vitamin:
Deficiency may result from kidney disease
D
Which vitamin:
Used by pyruvate dehydrogenase and a-ketoglutarate dehydrogenase
B1, b2, b3, b5, lipoic acid
Which vitamin:
Given prophylactically to newborns
Vit K
Which vitamin:
Can be used to elevate HDL and lower LDL
B3--niacin
Which vitamin:
Deficiency can be caused by isoniazid use
B6
Which vitamin:
Cobalt is found within this vitamin
B12--cobalamine
Which vitamin:
Critical for DNA synthesis
B12, Folate
What are the symptoms of zinc deficiency?
poor wound healing
What enzyme is inhibited by the drug fomepizole?
Alcohol DH
What is the difference between kwashiorkor and marasmus?
Kwashiorkor--protein malnutrition, skin lesions, edema, dec'd apolipoprotein synthesis; swollen belly

Marasmus--tissue wasting, global manlutrition
What vitamins should vegetarians supplement in their diet?
B12
What are the symptoms of vitamin A toxicity?
Bitot's spots
Keratomalacia
Xerophthalmia