Dit Set 5:lectures 53 - 65

Front 1

Lecture 53: Biochem 4

Back 1

Glycogen

Front 2

What are the arterial branches off the common hepatic artery?

Back 2

Gastroduodenal artery
•Branches
-Right gastro-omental artery
-Anterior superior pancreaticoduodenal artery

Right gastric artery

Proper hepatic artery
•Branches
-Left hepatic artery
-Right hepatic artery

Front 3

What cell type secretes the histamine that stimulates the histamine receptor on parietal cells thereby increasing gastric acid production?

Back 3

Enterochromaffin like cells (ECL cells)

Front 4

An infant present to the ER with vomiting. What presenting features would lead you to suspect congenital pyloric stenosis?

Back 4

Age: 2-6 weeks old

Male > female
-Classic presentation – 1st born male

Nonbilious, projectile vomiting

Dehydration

Metabolic alkalosis (hypokalemic, hypochloremic)

Palpable olive-like bulge on abdominal exam (***HIGH YIELD***)

Front 5

Pompe Disease

Back 5

Infantile form: Cardiomegaly and Early death

Adult form: Diaphragm weakness and Respiratory failure

Due to: Deficiency of lysosomal a-1,4-glucosidase

Front 6

What is the rate-limiting enzyme for the following metabolic pathway?

Glycolysis

Back 6

Phosphofructokinase-1

Front 7

RLE (Rate-limiting enzyme): Gluconeogenesis

Back 7

Fructose-1,6-bisphosphatase

Front 8

RLE: Glycogenesis

Back 8

Glycogen synthase

Front 9

RLE: Glycogenolysis

Back 9

Glycogen phosphorylase

Front 10

Which enzyme converts glucose-6-phosphage to glucose?

Back 10

Glucose-6-phosphatase

Front 11

Which glycogen storage disease matches each of the following phases? (***HIGH YIELD***)

Glycogen phosphorylase deficiency

Back 11

Type V (McArdle disease)

Front 12

Glucose-6-phosphatase deficiency

Back 12

Type I (von Gierke disease)

Front 13

Lactic acidosis, hyperlipidemia, hyperuricemia (gout)

Back 13

Type I (von Gierke disease)

Front 14

α-1,6-glucosidase deficiency

Back 14

Type III (Cori disease)

Front 15

α-1,4-glucosidase deficiency

Back 15

Type II (Pompe disease)

Front 16

Cardiomegaly

Back 16

Type II (Pompe disease, infantile type)

Front 17

Diaphragm weakness leading to respiratory failure

Back 17

Type II (Pompe disease, adult type)

Front 18

Increased glycogen in the liver; severe fasting hypoglycemia

Back 18

Type I (von Gierke disease)

Front 19

Hepatomegaly, hypoglycemia, hyperlipidemia (normal kidneys, lactate, and uric acid)

Back 19

Type III (Cori disease)

Front 20

Painful muscle cramps, myoglobinuria with strenuous exercise

Back 20

Type V (McArdle disease)

Front 21

Severe hepatosplenomegaly, enlarged kidneys

Back 21

Type I (von Gierke disease)

Front 22

Lecture 54: Biochem 5

Back 22

Energy

Front 23

What are the different RNA polymerases in eukaryotes?

Back 23

RNA polymerase I: makes rRNA

RNA polymerase II: makes mRNA

RNA polymerase III: makes tRNA

Front 24

In which order of elimination (zero order first) would you see a linear decrease in the plasma concentration of a substance when plotted against time?

Back 24

Zero order

*Rate of elimination is INDEPENDENT of the concentration of the substance

Front 25

What is the equation for Gibbs free energy?

Back 25

Delta G = Delta H – T Delta S

G = Gibbs’s free energy
H = energy/enthalpy (heat change in constant pressure rxns)
S = entropy/disorder/randomness

Front 26

Why are alanine and glutamine found in such high concentrations in the blood?

Back 26

They are the 2 major carriers of nitrogen from tissues

Front 27

What is generally involved in transamination?

Back 27

Transfer of the amino group of an amino acid to
a- ketoglutarate to form glutamate

The remaining deaminated amino acid is a keto-acid (such as pyruvate) that is used in energy metabolism

Front 28

What enzyme catalyzes transamination in the alanine cycle cycle?

Back 28

Aminotransferase

Front 29

How are aminotransferases named?

Back 29

By donor of the amino group (alanine aminotransferase converts alanine to pyruvate and forms glutamate)

Front 30

In addition to substrates, what is required by all aminotransferases?

Back 30

Pyridoxal phosphate (a derivative of vitamin B6)

Front 31

What are the 2 most important aminotransferase enzymes? What reaction do they catalyze?

Back 31

Alanine aminotransferase (ALT):
-alanine + a- ketoglutarate <--- ---> glutamate + pyruvate

Aspartate aminotransferase (AST):
-glutamate + oxaloacetate <---- ----> a-ketoglutarate + aspartate

Front 32

Cofactors of Pyruvate Dehydrogenase
*** HIGH YIELD ***

Back 32

“Tender loving care for no one”

Thiamine (vit. B1) to make TPP

Lipoic acid

CoA (comes from vit. B5)

FAD (comes from vit B2)

NAD (comes from vit. B3, Niacin)

Front 33

Arsenic exposure
KNOW!

Back 33

Arsenic inhibits Lipoic acid

Manifests as:
-Garlic breath
-Vomiting
-Rice water stool

Look like gastritis with garlic breath

Front 34

TCA cycle 3 enzymes to KNOW***

Back 34

Citrate synthase

Isocitrate dehydrogenase (very heavily regulated)

a-ketoglutarate Dehydrogenase
-Same cofactors as Pyruvate Dehydrogenase
-“Tender loving care for no one”

Front 35

Substances the inhibit Complex I, NADH reductase

Back 35

Amytal (barbiturate)

Rotenone (fish poison)

Methy-phenylpyridinium (MPP)
-Comes from MPTP (which is created when druggies try to may make synthetic Meperidine/ Opoids)
-Causes Parkinson’s symptoms

Front 36

Substances that inhibit Complex III

Back 36

Inhibited by: Antimycin A

Front 37

Substances that inhibit Complex IV

Back 37

Cyanide (CN-)

Sodium azide (N3-)

Carbon monoxide (CO)

Hydrogen sulfide (H2S)

Front 38

What are the possible products of pyruvate?

Back 38

Acetyl-CoA

Lactic acid

Alanine

Oxaloacetate

Front 39

What are the two main nitrogen transporters in the blood? (***KNOW***)

Back 39

Alanine

Glutamine

Front 40

What are the major regulatory enzymes of citric acid cycle?

Back 40

Citrate synthase

Isocitrate dehydrogenase (rate-limiting step)

a-ketoglutarate dehydrogenase

Front 41

What substances are known to inhibit the complexes of the electron transport chain?

Back 41

Complex I, NADH reductase inhibited by:
-Amytal (barbiturate)
-Rotenone (fish poison)
-Methy-phenylpyridinium (MPP) (Comes from MPTP)

•Complex III
Inhibited by: Antimycin A

Complex IV inhibited by:
-Cyanide (CN-)
-Sodium azide (N3-)
-Carbon monoxide (CO)
-Hydrogen sulfide (H2S)

Front 42

What substances can increase the permeability of the inner mitochondrial membrane, thereby decreasing ATP synthesis but increasing heat generation?

Back 42

Aspirin

Thermogenin

2,4-DNP

*These are uncoupling agents

Front 43

5 cofactors that are required to complete the Pyruvate Dehydrogenase Complex

Back 43

Thiamine (vit. B1) to make TPP

Lipoic acid

CoA (comes from vit. B5)

FAD (comes from vit B2)

NAD (comes from vit. B3, Niacin)

Front 44

Lecture 55: Biochem 6

Back 44

HMP Shunt and Other Sugars

Front 45

What are the features of Plummer-Vinson syndrome?

Back 45

Dysphagia from esophageal webs

Glossitis

Iron deficiency

Front 46

Explain why methanol is such a toxic substance.

Back 46

Methanol metabolized to formaldehyde leading to:

-Metabolic acidosis

-Retinal damage and blindness

Front 47

A woman commonly develops intense muscle cramps and darkening of her urine after exercise. What is her diagnosis?

Back 47

McArdle disease (classic presentation)

* Causes myoglobinura with strenuous exercise

Front 48

What are the components of the HMP shunt (pentose phosphate pathway)

Back 48

G6p-----> (2) NADPH + Ribulose- 5- Phosphate

Via Glucose -6- P- dehydrogenase

Ribulose-5-P----> PRPP (final product of pathway)

Front 49

What to the enzymes NADPH oxidase, Superoxide dismutase, and Myeloperoxidase catalyze?

Back 49

The oxidative burst:

NADPH oxidase: O2 ----->O2-

Superoxide dismutase: O2- -----> H2O2

Myeloperoxidase: H2O2 ----> HOCl

*Chronic Granulomatous Disease
-Caused by: Deficiency in NADPH oxidase
-Catalse + organisms especially dangerous

Front 50

Describe essential fructosuria

Back 50

Fructokinase defect/deficiency

Can't use fructose as an energy source

Benign condition resulting in increase fructose in the blood and urine

Cause dieresis because fructose acts as an osmole and brings more water into the urine

Front 51

Describe Fructose intolerance

Back 51

Caused by Aldolase B deficiency

Accumulation of fructose-1- phosphate

Inhibits glycosis and glyconeogenesis

Results in :
-Hypoglycemia
-Vomiting
-Jaundice
-Cirrhosis

Front 52

Describe Galactoskinase Deficiency

Back 52

Galactose accumulates if galactose is present in the diet

Can cause cataracts in infants

Front 53

Describe Classic Galactosemia

Back 53

Absence of Galactose-1-phosphate uridyltransferase

S/S:
-Severe cataracts
-Hepatomegaly
-Cirrhosis
-Failure to thrive
-Mental retardation

Tx: exclude galactose and lactose from diet

Front 54

What is the rate-limiting step of the pentose phosphate pathway?

Back 54

Glucose-6-phosphate dehydrogenase (G6PD)

Front 55

Which tissue of the body use the pentose phosphate pathway?

Back 55

Red blood cells

Liver

Adrenal cortex

Mammary glands

Front 56

Explain why a deficiency of the enzyme that is the rate-limiter for the HMP shunt can result in hemolytic anemia?

Back 56

G6PD generates NADPH

NADPH is used to produce reduced glutathione

Without G6PD -- RBC oxidative damage-- hemolysis

Front 57

What are the symptoms of classic galactosemia?

Back 57

Failure to thrive

Mental retardation

Hepatomegaly

Jaundice

Infantile cataracts

Front 58

Galactokinase deficiency

Back 58

Galactokinase deficiency... Well that's an easy one

Front 59

Aldolase B Deficiency

Back 59

Fructose intolerance

Front 60

Lactase deficiency

Back 60

Lactose intolerance

Front 61

Galactose-1-phosphate uridyltransferase deficiency

Back 61

Galactosemia

Front 62

Fructokinase deficiency

Back 62

Essential fructosuria

Front 63

G6PD Deficiency

Back 63

X-linked recessive

Stressors:
-Infection
-Drugs: Antimalarials, Sulfonamides, Fava beans

Causes: Non-immune hemolytic anemia

Front 64

Lecture 56: Biochem 7

Back 64

Fuel Use

Front 65

What substances are well known for causing hemolytic anemia in patients with G6PD deficiency?

Back 65

Fava beans

Sulfa drugs

Primaquine

Isoniazid

High-dose aspirin

Ibuprofen

Nitrofurantoin

Dapsone

Naphthalene

Front 66

What are the 4 important pharmacokinetic equations?

Back 66

Vd = (amount of drug given in IV form/ concentration of drug in plasma)

Clearance (CL) = (0.7 x Vd/ t ½)

Loading dose (LD) = Css x Vd

Maintenance dose (MD) = Css x CL

Front 67

What is seen histologically in patients with celiac sprue?

Back 67

Blunting of villi

Hyperplastic crypts

Lymphoctyes in the lamina propria

Front 68

What fuels are produced and used in the post-absorptive period?

Back 68

Produced: Glucose from gluconeogenesis & Fatty acids from adipose tissue

Used: Muscle, brain, and other tissues predominantly glucose

Front 69

How does the pattern of fuel production and usage change in early starvation (24 hours after the last meal)?

Back 69

•Produced: Glucose from gluconeogenesis & Fatty acids from adipose tissue

•Used: Brain uses predominantly glucose & Muscles and other tissues use some glucose but predominantly fatty acids

Front 70

In intermediate starvation (48 hours after the last meal), how does the pattern of fuel production and consumption change?

Back 70

•Produced:
-Glucose from gluconeogenesis
-Fatty acids from adipose tissue
-Ketone bodies from the liver

•Used:
-Brain uses predominantly glucose but also some ketone bodies
-Muscles and other tissue use predominantly fatty acids but also some ketone bodies

Front 71

What metabolic scenario favors the synthesis of ketone bodies?

Back 71

When production of acetyl-CoA from  oxidations of fatty acid exceeds the oxidative capacity of the TCA cycle

Front 72

What is the pattern of fuel utilization and production in prolonged starvation (5 days after last meal)?

Back 72

•Produced
-Glucose from gluconeogenesis
-Faty acids form adipose tissue
-Ketone bodies from the liver

•Used
-Brain uses predominantly ketone bodies
-Muscles and other tissue use predominantly fatty acids but also some ketone bodies

Front 73

Kwashiorkor

Back 73

Protein malnutrition

Causes skin lesion, edema, liver malfunction, fatty change of liver (because APOB 100 is not made and put on chylomicrons to leave the liver), skin and hair depigmentation

Porker’s—skinny child with protubrent belly because of edema and hepatomegly

Front 74

Marasmus

Back 74

Energy malnutrition

Front 75

What is the primary energy source in a patient that has not eaten in two days?

Back 75

Fatty acids

Front 76

What hormone stimulates the storage of lipids in the fed state?

Back 76

Insulin

Front 77

What is the rate-limiting enzyme in ketone body synthesis?

Back 77

HMG CoA Synthase

Front 78

A stressed physician comes home from work, consumes 7 or 8 shots of tequila in rapid succession before dinner, and becomes hypoglycemic. Why did she become hypoglycemic?

Back 78

•Ethanol metabolism generates NADH
-Pyruvate --- lactate
-Oxaloacetate -- malate

•No longer have pyruvate or oxaloacetate to undergo gluconeogenesis ---- hypoglycemia

Front 79

What are some of the hallmark features of kwashiorkor?

Back 79

Protein malnutrition
Edema and ascites
Anemia
Malfunction of the liver (fatty change)
Skin lesions
Skin and hair depigmentation

Front 80

It’s the process of generating glucose for energy during periods of fasting or starvation.

Back 80

Gluconeogenesis

Front 81

It’s the ketone body that causes the breath of DKA patients to smell sweet or fruity.

Back 81

Acetone

Front 82

It’s the ketone body used for energy in the CNS for energy during periods of prolonged starvation.

Back 82

Beta-hydroxybutyrate

Front 83

It’s the only source of energy that RBCs can metabolize.

Back 83

Glucose

Front 84

Its metabolism yields 9 kilocalories of energy per gram, more than either protein or carbohydrates.

Back 84

Fat

Front 85

Lecture 57: Endocrine 10

Back 85

Obesity

Front 86

What co-factors are required for the function of pyruvate dehydrogenase? What other enzyme requires the same co-factors?

Back 86

•Co-factors required for the function of Pyruvate dehydrogenase
-Thiamine pyrophosphate
-Lipoic acid
-CoA
-FAD
-NAD

•Other enzyme that requires the same co-factors
a–ketoglutarate dehydrogenase

Front 87

What medications are used to shrink prolactinomas?

Back 87

•Dopamine agonists:
Bromocriptine
Cabergoline

Front 88

What are the functions of interleukins 1-5?

Back 88

•“Hot T-bone stEAk”

Hot : IL-1 causes fever

T-bone:
- IL-2 is a T-cell stimulator
-IL-3 is a bone marrow stimulator

stEAk
-IL-4 enhances production of IgE and IgG
-IL-5 is associated with IgA and eosinophils

Front 89

BMI Ranges (***HIGH YIELD***)

Back 89

≥ 30 – obese

25.0-29.9 – overweight

18.5-24.9 – normal, healthy weight

< 18.5 – underweight

Front 90

Hypothalamus Nuclei involved in Regulating Hunger and Food Intake

Back 90

Paraventricular nuclei

Dorsal medial nuclei

Arcuate nucleus

Lateral hypothalamus

Ventromedial nuclei

Front 91

What stimulates and inhibits the lateral hypothalamus?
KNOW***

Back 91

Stimulated by hunger

Inhibited by anorexia

Inhibited by Leptin (KNOW)

Front 92

What stimulates and inhibits the Ventromedial nuclei

Back 92

Stimulated by satiety

Destroyed by hyperphagia

Stimulated by Leptin (KNOW)

Front 93

Hormonal Regulation of Fat Utilization (***HIGH YIELD***)

Back 93

Both cause – INCREASED sympathetic stimulation
-Heavy exercise
-Stress: Increase NE & glucocorticoids

Front 94

Causes of Lipodystrophy
High Yield***

Back 94

Leptin deficiency

HIV

HIV medications (protease inhibitors

Sites of insulin injection

Front 95

Metabolic Syndrome
3 Stars ***

Back 95

Diagnosis based on any 3 of the following:

-Abdominal Obesity: waist circumference > or = 40in (102cm), or > or = 35in (88cm) in women (recognize that not all metabolic syndromes patients are overweight)

-Triglycerides > or = 150 mg/dL

-HDL <40 mg/dl in men, <50 mg/dl

-BP > or = 130/85

-Fasting serum glucose > or = 100 mg/dl (or 2hr post oral glucose > or = 140 mg/dl)

Front 96

What are the five categories of criteria for the diagnosis of metabolic syndrome? (HIGH YIELD)

Back 96

Abdominal obesity

Triglycerides

Low HDL

BP

Glucose

Front 97

What liver disease is associated with obesity? (HIGH YIELD)

Back 97

Non-alcoholic steatohepatitis (NASH)

*Suspect with chronically elevated LFTs

Front 98

At what BMI is a patient considered obese? (HIGH YIELD)

Back 98

BMI ≥ 30

Front 99

Lecture 58: GI 9

Back 99

Hepatocytes and Cirrhosis

Front 100

What immunodeficiency matches each of the following statements?

Neutrophils fail to respond to chemotactic stimuli

Back 100

Job syndrome (hyper IgE syndrome)

Leukocyte adhesion deficiency syndrome

Front 101

Adenosine deaminase deficiency

Back 101

SCID

Front 102

Failure of endodermal development

Back 102

DiGeorge syndrome

Front 103

Defective tyrosine kinase gene

Back 103

Bruton agammaglobulinemia

Front 104

Associated with high levels of IgE

Back 104

Job syndrome (hyper IgE syndrome)

Front 105

How does hexokinase differ from glucokinase?

Back 105

•Hexokinase
-Found – everywhere
-Insulin: doesn’t induce
-Low Km
-Low Vmax

•Glucokinase
-Found – Liver, pancreas ( cells)
-Insulin: induces
-High Km
-High Vmax

Front 106

MOA: Protease inhibitors

Back 106

Inhibit viral assembly

Block the protease enzyme

Front 107

MOA: Nucleoside reverse transcriptase inhibitors

Back 107

Nucleic acid analogs

Inhibit reverse transcriptase

Prevent incorporation of the viral genome into the host DNA

Front 108

MOA: Non-nucleoside reverse transcriptase inhibitors

Back 108

Inhibit reverse transcriptase by binding directly and non-competitively to the enzyme

Irreversible inhibitors

Prevent the incorporation of the viral genome into the host DNA

Front 109

MOA: Fusion inhibitors

Back 109

Bind viral glycoprotein 41

Inhibit fusion with CD4 cells

Front 110

Gilbert syndrome

Back 110

Slight problem with bilirubin uptake

Mild deficiency in UDP-GT

Higher levels of indirect bilirubin

Clinically insignificant

Front 111

Crigler-Najjar syndrome Type I

Back 111

Complete absence of UDP-GT

S/S—jaundice in first few days of life -- kernicturis

Treatment:
•Phototherapy (bili light)
•Plasmapheresis
•Liver transplant

Front 112

Crigler-Najjar syndrome Type II

Back 112

Mutated UDP-GT (less severe than Type 1)

Front 113

Alcoholic Liver Disease

Back 113

“A Scotch and Tonic”
AST > ALT

2:1

Depletion of ALT possibly due to B6 deficiency

Front 114

What enzyme is responsible for the conjugation of bilirubin?

Back 114

UDP-glucoronyl transferase

Front 115

Mildly decreased UDPGT

Back 115

Gilbert syndrome

Crigler-Najjar syndrome, type 2

Front 116

Completely absent UDPGT

Back 116

Crigler-Najjar syndrome, type 1

Front 117

Grossly black liver

Back 117

Dubin-Johnson syndrome

Front 118

Responds to Phenobarbital

Back 118

Crigler-Najjar syndrome, type 2

Gilbert syndrome

Front 119

Treatment includes plasmapheresis and phototherapy

Back 119

Crigler-Najjar syndrome, type 1

Front 120

Asymptomatic unless under physical stress (alcohol, infection)

Back 120

Gilbert syndrome

Front 121

What are the signs of portal hypertension?

Back 121

Esophageal varices
Hematemesis
Melena
Hemorrhoids
Caput medusa
Splenomegaly
Ascites

Front 122

Triglyceride accumulation in hepatocytes

Back 122

Fatty liver disease

Front 123

Eosinophilic inclusions in the cytoplasm of hepatocytes

Back 123

Mallory bodies

Front 124

Cancer closely linked to cirrhosis

Back 124

Hepatocellular carcinoma

Front 125

Severe hyperbilirubinemia in a neonate

Back 125

Crigler-Najjar syndrome, type 1

Front 126

Mild, benign hyperbilirubinemia

Back 126

Gilbert syndrome

Front 127

What common disorder is associated with benign asymptomatic elevation of indirect bilirubin?

Back 127

Gilbert syndrome

Front 128

Lecture 59: GI 10

Back 128

Liver Pathology

Front 129

After the loss of his job, a 35 year old man has diarrhea and hematochezia. Intestinal biopsy shows transmural inflammation. What is the diagnosis?

Back 129

Crohn’s disease

Front 130

What effect will a competitive antagonist have on efficacy and potency?

Back 130

Increase Km

Decrease potency

No change in efficacy

Front 131

What effect does stress have on adipocytes?

Back 131

Stress increases sympathetic tone

Activates hormone-sensitive TG lipase in fat cells:
-Rapid breakdown of TGs
-Mobilization of fatty acids

Anterior pituitary releases ACTH
-Adrenal cortex secretes more Glucocorticoids

Front 132

Budd-Chiari Syndrome

Back 132

Hepatomegaly, ascities, abdominal pain, liver failure

Portal hypertension

Absence of JVD (KEY)

Congestion of liver

Front 133

Reye’s Syndrome

Back 133

Seen in Children given Aspirin
Child also has a Viral Infection
Hepatoencephalopathy (Liver & )Brain
Damage to mitochondria

Front 134

Wilson Disease

Back 134

Cooper not incooperated into the bile

Copper accumulation in: Liver, Brain, Cornea, Kidneys, Joints

Clinical presentation:
-Cirrhosis
-Corneal deposits (Kayser-Fleischer ring)
-Hepatocellular carcinoma
-Hemolytic anemia
-Basal ganglia degeneration
-Parkinsonian symptoms
-Hepaticencephalopathy
-Dementia
-Fanconi’s syndrome (Proximal tubule dysfunction)

Treatment: Penicillamine

Front 135

Hemochromatosis (bronze diabetes) Triad
KNOW***

Back 135

Cirrhosis
Diabetes mellitus
Skin pigmentation

Front 136

Hemochromatosis primary, secondary, and treatment

Back 136

•Primary hemochromatosis: Autosomal recessive disorder

•Secondary hemochromatosis: Transfusions (chronic)

•Treatment: Phlebotomy, Deferoxamine

Front 137

Hepatocellular Carcinoma risk factors

Back 137

Hepatitis B
Hepatitis C
Wilson disease
Hemochromatosis
a 1-antitrypsin deficiency
Alcoholic cirrhosis
Carcinogen exposure

Front 138

Hepatocellular Carcinoma Serum Marker
KNOW***

Back 138

a–fetoprotein (AFP)

Front 139

Alcoholic cirrhosis

Back 139

AST:ALT will be >2:1

Front 140

Biliary tract

Back 140

Increased GGT
Increased Alk Phos

Front 141

Bone formation

Back 141

Increased Alk Phos

Front 142

Budd-Chiari

Back 142

NO JVD
Ascites

Front 143

Reye’s syndrome

Back 143

ASA
Ped’s
Virus

Front 144

Wilson’s Disease

Back 144

Copper
Decreased cerulopasmin
Kayser-Fleischer ring
Penicillamine

Front 145

Hemochromatosis

Back 145

Iron
Bronze diabetes
Increased ferritin
Decreased TIBC
Increased transferrin saturation
Phlebotomy
Deferoxamine

Front 146

a1-antitrypsin deficiency

Back 146

Liver
Lung: Emphysema (panaciner)

Front 147

What is seen in Budd-Chiari syndrome? What conditions are associated with Budd-Chiari syndrome?

Back 147

Occulusion of the IVC of hepatic veins

Associated with:
-Polycythemia vera
-Pregnancy
-Hepatocellular carcinoma

Front 148

What is the mechanism by which aspirin can cause Reye syndrome?

Back 148

Metabolites of aspirin inhibit mitochondrial enzymes
Decreased B-oxidation

Front 149

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?

Back 149

Wilson Disease

Treatment – penicillamine

Front 150

What is the underlying problem in Wilson disease? What are the characteristics of Wilson disease? What is the treatment for Wilson disease?

Back 150

Underlying problem:
-Impaired copper excretion
-Body does not put copper into bile appropriately

Characteristics:
-Asterixis
-Basal ganglia degeneration
-Decreased ceruloplasmin
-Cirrhosis
-Kayser-Fleischer rings
-Copper accumulation
-Dementia
-Dyskinesia
-Dysarthria

Treatment – penicillamine

Front 151

What is the classic triad of symptoms in hemochromatosis? What lab tests are used to diagnose hemochromatosis? What is the treatment?

Back 151

Classic triad:
-Diabetes
-Bronzing of the skin
-Cirrhosis

Lab tests:
-Elevated ferritin
-Elevated transferring saturation
-Elevated serum iron
-Decreased TIBC

Treatment
-Phlebotomy
-Deferoxamine

Front 152

What are the risk factors for the development of hepatocellular carcinoma?

Back 152

Hepatitis B
Hepatitis C
Wilson disease
Hemochromatosis
a1-antitrypsin deficiency
Alcoholic cirrhosis
Carcinogen exposure

Front 153

Hepatomegaly, abdominal pain, ascites

Back 153

Budd-Chiari syndrome

Front 154

Green/yellow/ brown corneal deposits

Back 154

Keyser-Fleisher rings

Front 155

Low serum ceruloplasmin

Back 155

Wilson disease

Front 156

Cirrhosis, diabetes and hyperpigmentation

Back 156

Hemochromatosis

Front 157

Lecture 60: GI 11

Back 157

Hepatitis

Front 158

What is the result of a glycolytic enzyme deficiency? What is the result of a deficiency in pyruvate dehydrogenase?

Back 158

•Glycolytic enzyme deficiency
-RBC hemolysis

•Deficiency in pyruvate dehydrogenase
-Neurologic defects

Front 159

Which substance serves as chemotactic agents for leukocytes?

Back 159

IL-8
Leukotriene B4
C5a
Kallikrein

Front 160

What enzyme is inhibited by PPIs? Name 2 different PPIs

Back 160

Hydrogen potassium ATPase

Common PPIs:
-Omeprazole
-Lansoprazole
-Pantoprazole
-Exomeprazole

Front 161

Identify the hepatitis B status of each of the following patients based on their hepatitis B serologic markers.

HepBsAg- Negative

HepBsAB- Positive

HepBcAb- Positive

Back 161

Recovery

Front 162

HepBsAg- Negative

HepBsAB- Negative

HepBcAb- Positive

Back 162

Window

Front 163

HepBsAg- Positive

HepBsAB- Negative

HepBcAb- Positive IgM

Back 163

Acute infection

Front 164

HepBsAg- positive

HepBsAB- negative

HepBcAb- positive IgG

Back 164

Chronic infection

Front 165

HepBsAg- negative

HepBsAB- positive

HepBcAb- negative

Back 165

Immunized

Front 166

Early acute infection

Back 166

HBsAg: positive

HBsAb: negative

HBcAb: +IgM

HBeAg: positive

HBeAb: negative

Front 167

Window period

Back 167

HBsAg: negative

HBsAb: negative

HBcAb: +IgM

Front 168

Chronic infection with Increased transmissibility

Back 168

HBsAg: positive

HBsAb: negative

HBcAb: +IgG

HBeAg: POSITIVE

HBeAb: NEGATIVE

Front 169

Chronic infection with Decreased transmissibility

Back 169

HBsAg: positive

HBsAb: negative

HBcAb: +IgG

HBeAg: NEGATIVE

HBeAb: POSITIVE

Front 170

Past infection

Back 170

HBsAg: negative

HBsAb: positive

HBcAb:+IgG

HBeAg: negative

HBeAb: positive

Front 171

Vaccinated

Back 171

HBsAg: negative

HBsAb: positive

HBcAb: +IgG

HBeAg: negative

HBeAb: negative

Front 172

What medications are used in the treatment of chronic hepatitis B and C?

Back 172

•Interferon alpha: Chronic hepatitis B and C

•Rabavirin: Chronic hepatitis C

Front 173

What antibodies can be used to help make the diagnosis of autoimmune hepatitis?

Back 173

•ANA (+), Anti-smooth muscle Ab (+)

•Anti-liver-kidney microsomal Ab (+)

•Anti-mitochondrial Ab (-)

Front 174

Rapid Fire Fact: Treatment for chronic hepatitis

Back 174

Interferon alpha

Front 175

What serologic marker for hepatitis B indicates contagiousness?

Back 175

“BE Ware”
Hepatitis “B” “E”nvelope Antigen (HBeAg)

Front 176

Lecture 61: GI 12

Back 176

Biliary Tract

Front 177

What cocktail of medications is commonly taken by patient suffering from severe cirrhosis and what do they do?

Back 177

Lactulose--- treats hepatic encephalopathy by decreasing ammonia

Vitamin K--- Maximizes clotting potential

Diuretics--- prevent ascities and edema

B-blocker--- to prevent bleeding from esophageal varices

Front 178

What is the cause of chronic granulomatous disease? What are the consequences of chronic granulomatous disease?

Back 178

Causes: Lack of NADPH oxidase activity; Phagocytes can engulf bacteria, but cannot generate free radicals (Impotent phagocytes)

Consequences: Susceptible to opportunistic infections
-S. aureus
-Salmonella
-Klebsiella
• Aspergillus

Front 179

Which immunoglobulin isotype is associated with eosinophilia? Which cytokine is associated with differentiation of eosinophils?

Back 179

Immunoglobulin -- IgE

Cytokine -- IL-5

Front 180

DDx of Unconjugated Hyperbilirubinemias

Back 180

Increased bilirubin production:
-Hemolytic anemia
-Sickle cell disease
-Hematoma breakdown

Impaired bilirubin uptake and storage
-Post viral hepatitis
-Rifampin

Decreased UDP-GT activity
-Gilbert syndrome
-Crigler-Najjar syndrome, type I and II
-Neonatal physiologic jaundice

Front 181

DDx of Conjugated Hyperbilirubinemias

Back 181

Impaired transport
-Dubin-Johnson syndrome
-Rotor syndrome

Biliary epithelial damage
-Hepatitis
-Cirrhosis
-Liver failure

Intrahepatic biliary obstruction
-Primary biliary cirrhosis
-Sclerosing cholangitis
-Chlorpromazine and arsenic

Extrahepatic biliary obstruction
-Pancreatic neoplasm
-Pancreatitis
-Cholangiocarcinoma
-Choledocholithiasis

Front 182

Describe Primary Sclerosing Cholangitis (HIGH YIELD FACTS TO KNOW)

Back 182

“Beads on a string” on ERCP (endoscopic retrograde cholangiopancreatography)

Men around age 40

60% have a positive pANCA

Associated with
-Ulcerative colitis
-Cholangiocarcinoma

Front 183

Primary Biliary Cirrhosis

Back 183

90% of patients are middle-aged women
Positive anti-mitochondrial antibody (AMA)
Associated with other autoimmune disorders
Treat with ursodil

Front 184

Cholelithiasis classic risk factors

Back 184

Fat (overweight/obesity)
Fertile
Female
Forty

Dx: RUQ ultrasound

Front 185

Charcot’s Triad of Cholangitis (KNOW)

Back 185

Jaundice
Fever
Right upper quadrant pain

Front 186

Reynold’s Pentad of Cholangitis

Back 186

Jaundice
Fever
Right upper quadrant pain
Hypotension
Altered mental status

Front 187

What is the fate of bilirubin after it is conjugated and secreted into the GI tract?

Back 187

•Bacteria convert it to urobilinogen
•Most is excreted in the stool as stercobilin
•Some is reabsorbed/recycled in bile
•Tiny amount excreted in the urine as urobilin

Front 188

What enzyme is functioning suboptimally in newborns with physiologic jaundice?

Back 188

UDP-Glucuronyl Transferase

Front 189

What are some of the intrahepatic and extrahepatic causes of biliary obstruction?

Back 189

•Intrahepatic
-Primary biliary cirrhosis
-Primary sclerosing cholangitis
-Drugs

•Extrahepatic
-Pancreatic neoplasm
-Choledocholithiasis
-Pancreatitis
-Cholangiocarcinoma

Front 190

What is the difference between primary biliary cirrhosis and primary sclerosing cholangitis?

Back 190

Primary Biliary Cirrohosis
-Positive AMA
-Middle-aged females
-Autoimmune disease (CREST scleroderma)

Primary Sclerosing Cholangitis
-Positive pANCA
-Males over 40
-Ulcerative colitis and cholangiocarcinoma
-“Beads on a string” on ERCP

Front 191

What are some of the risk factors for the development of cholesterol gallstones?

Back 191

Fat
Fertile
Female
Forty

Front 192

Lecture 62: Biochem 8

Back 192

Lipids

Front 193

What are the 3 most common types of thyroid cancer?

Back 193

Papillary carcinoma (most common)

Follicular carcinoma

Medullary carcinoma (3rd most common)

Front 194

What are the side effects of orlistat?

Back 194

Steatorrhea

GI discomfort

Reduced absorption of fat-soluble vitamins

Front 195

What are the arterial branches off of the celiac trunk and what branches come off of those?

Back 195

Common hepatic artery
-Gastroduodenal artery
-Right gastric artery
-Proper hepatic artery

Splenic artery

Left gastric artery

* Common hepatic -----> Gastroduodenal---->
- Right gastro-omental artery
-Anterior superior pancreaticoduodenal artery

Front 196

Familial Hypercholesterolemia

Back 196

Type 2 A hypercholesterolemia--- accelerated atherosclerosis

Autosomal dominant

Double dominant mutation—MI at age 20

Front 197

What is the substrate and RLE for cholesterol synthesis? What what inhibits the RLE?

Back 197

Substrate -- Acetyl CoA

Rate-limiting enzyme -- HMG-CoA reductase

Statins inhibit HMG-CoA reductase

Front 198

What is the substrate and RLE for fatty acid metabolism? Where does degradation of FA occur and what is the RLE for this?

Back 198

Substrate: Acetyl CoA

Rate-limiting enzyme: Acetly-CoA carboxylase

Degradation:
-Occurs in Mitochondrial maxtrix
-Rate-limiting enzyme: Carnitine acyltransferase

Front 199

RLE: Fatty acid synthesis
KNOW***

Back 199

Acetyl-CoA carboxylase

Front 200

RLE: B-oxidation of fatty acids
KNOW***

Back 200

Carnitine acyltransferase

Front 201

RLE: Ketone synthesis
KNOW***

Back 201

HMG-CoA synthase

Front 202

RLE: Cholesterol synthesis
KNOW***

Back 202

HMG-CoA reductase

Front 203

What deficiency causes familial hypercholesterolemia?

Back 203

Deficiency of LDL receptors

Front 204

Activates LCAT

Back 204

Apo A-1

"Put A1 steak sauce on a Cat"

Front 205

Mediates chylomicron secretion

Back 205

Apo B-48

Front 206

Mediates VLDL secretion

Back 206

Apo B-100

Front 207

Binds to LDL receptors

Back 207

Apo B-100

Front 208

Cofactor for lipoprotein lipase

Back 208

C-II

Front 209

Mediates uptake of remnant particles

Back 209

Apo E

Front 210

What is the rate-limiting enzyme for each of the following metabolic pathways? (HIGH YIELD)

• Hexose monophosphate pathway

• Fatty acid synthesis

• B-oxidation of fatty acids

• Ketone body synthesis

• Cholesterol synthesis

Back 210

Hexose monophosphate pathway: Glucose-6-phosphate dehydrogenase

Fatty acid synthesis: Acetyl-CoA carboxylase

B-oxidation of fatty acids: Carnitine acyltransferase

Ketone body synthesis: HMG-CoA synthase

Cholesterol synthesis: HMG-CoA reductase

Front 211

Which group of medications inhibits the rate limiting enzyme of cholesterol synthesis?

Back 211

HMG-CoA reductase inhibitors (statins)

Front 212

Where in the cell would you find each of the following enzymatic processes taking place?

• Fatty acid degradation

• Fatty acid synthesis

•Glycolysis
• TCA cycle
• Electron transport chain (oxidative phosphorylation)
• Gluconeogenesis

Back 212

Fatty acid degradation: Mitochondria

Fatty acid synthesis: Cytoplasm

Glycolysis: Cytoplasm

TCA cycle: Mitochondria

Electron transport chain (oxidative phosphorylation): mitochondria

Gluconeogenesis: Mitochondria and cytoplasm

Front 213

Lecture 63: Biochem 9

Back 213

Amino Acids and nitrogen

Front 214

A 2 year old girl has an increase in abdominal girth, failure to thrive, and skin/hair depigmentation. What is her diagnosis?

Back 214

Kwashiorkor

*Skin/ hair depigmentation is a "big hint"

Front 215

What enzyme is deficient in each of the following diseases?

• Fructose intolerance
• Essential fructosuria
• Classic galactosemia

Back 215

Fructose intolerance: Aldolase B

Essential fructosuria: Fructokinase

Classic galactosemia: Galactose-1-phosphate uridyltransferase

Front 216

What structures run through the cavernous sinus?

Back 216

Oculomotor nerve (CN III)

Trochlear nerve (CN IV)

Abducent nerve (CN VI)

Ophthalmic division of trigeminal nerve (CN V1)

Maxillary division of trigeminal nerve (CN V2)

Front 217

Name the Essential Amino Acids (***HIGH YIELD – 3 stars***)

Back 217

“PVT TIM HALL”

Phenylalanine

Valine

Threonine

Tryptophan

Isoleucine

Methionine

Histidine

Arginine

Leucine

Lysine

Front 218

Name the basic AA

Back 218

Lysine

Arginine
-Produces:Creatine, Urea, Nitric oxide

Histidine

Front 219

Acidic AA

Back 219

Aspartate – aspartic acid

Glutamate – glutamic acid

Front 220

What organ does the urea cycle take place in? What are the 2 enzymes involved and where are they found?

Back 220

Liver

Rate-limiting enzyme: CPS-1 (carbamoyl phosphate)
- Found in mitochondriaCPS-1
- Gets it's N from ammonia

Ornithine transcarbamoylase
- Found in the mitochondria

Front 221

What does Ornithine Transcarbamoylase Deficiency cause?

Back 221

Hepatoencephalopathy
-Slurring of speech
-Somnolence
-Vomiting
-Cerebral edema
-Blurring of the vision

Treatment:
-Phenylbutyrate (urea cycle problem)
-Benzoate
-Lactulose (hyperammonemia, liver disease)

Front 222

Compare carbamoyl phosphate synthetase I to carbamoyl phosphate synthestase II.

Back 222

•CPS-I
-Urea cycle
-Mitochondria
-Gets nitrogen from ammonium

•CPS-II
-Pyrimidine synthesis
-Cytosol
-Gets nitrogen from glutamine

Front 223

What is the mechanism of action of lactulose?

Back 223

Digested by bacteria in the colon

Creates an acidic environment

NH3 is converted to NH4+ which is now trapped in the lumen of the colon

NH4+ excreted in the stool

Front 224

Lecture 64: Biochem 10

Back 224

Amino Acid disorders

Front 225

Which three enzymes are required to convert phenylalanine to DOPA?

Back 225

Phenylalanine hydroxylase

Tyrosine hydroxylase

Dihydropterin reductase

Front 226

What are the byproducts of MAO and COMT enzymatic activity on dopamine, Norepinephrine, and epinephrine?

Back 226

Dopamine: HVA

Norepinephrine: VMA

Epinephrine: metanephrine

Front 227

What substrate and cofactor is required of the generation of GABA?

Back 227

Substrate: Glutamate

Cofactor: B6

Front 228

PKU (***HIGH YIELD TOPIC – 4 stars***)

Back 228

Deficiency of either Phenylalanine hydroxylase or a deficiency of the cofactor (Tetrahydrobiopterin) for phenylalanine hydroxylase

Patients cannot make tyrosine

Phenyl Ketones
-Phenylacetate
-Phenyl-lactate
-Phenylpyruvate

Treatment:
Avoid phenylalanine- Aspartame
Augment: tyrosine & tetrahydrobiopterin

Front 229

Alkaptonuria

Back 229

Deficient in the enzyme homogentisic acid oxidase

Autosomal recessive

Ochronosis

Sclera – dark brown

Urine turns black when exposed to air

Debilitating arthralgias (due to accumulation of homogentisic acid in the joint)

Front 230

Cystinuria

Back 230

Defect of the renal proximal tubular transporter for: "COLA"

Cysteines

Ornithine

Lycine

Arginine

Front 231

Hartnup Disease

Back 231

•Tryptophan is excreted in the urine and not absorbed in the gut

Due to – an autosomal recessive defect in a transport found in the intestine and kidneys

Front 232

Vitamin B3 Deficiency (Pellagra)

Back 232

The “3 Ds” of Vitamin B3 deficiency

Dermatitis

Diarrhea

Dementia

Front 233

A full term neonate becomes mentally retarded and hyperactive and has a musty odor. What is the diagnosis?

Back 233

PKU

*Musty oder – big clue

Front 234

A patient with PKU should have diet low in phenylalanine. What other dietary modifications should a patient with PKU make?

Back 234

Increasing tyrosine in the diet

Replacing tetrahydrobiopterin cofactor

Front 235

A middle aged man has dark spots on his sclera and has noted that his urine turns black when left sitting for a period of time. What’s the diagnosis?

Back 235

Alkaptonuria

Front 236

What is the underlying cause of maple syrup urine disease?

Back 236

Deficiency of a-ketoacid dehydrogenase

Front 237

Maple Syrup Urine Disease

Back 237

Caused by deficiency of the enzyme a-ketoacid dehydrogenase

Lead to a buildup of the 3 branched amino acids
-Isoleucine
-Leucine
-Valine

Can cause:
-Mental retardation
-Death

Front 238

Lecture 65: Pharm Basics 7

Back 238

Drug Metabolism

Front 239

What effect does changing Km and Vmax have on potency and efficacy?

Back 239

Km
- Lower Km → higher potency
- Km is inversely related to potency
- Changing Km does not affect efficacy

Vmax
- Higher Vmax → higher efficacy
- Vmax is directly related to efficacy
- Changing Vmax does not alter potency

Front 240

What are the 4 main pharmacokinetic equations? (KNOW)

Back 240

Vd = (amount of drug given in IV form)/([drug] plasma)

Clearance (CL) = (0.7 x Vd)/(t1/2)

Loading Dose (LD) = Css x Vd

Maintenance Dose (MD) = Css x CL

Front 241

What enzyme is deficient in PKU? What are the symptoms?

Back 241

Deficient enzyme: Phenylalanine hydroxylase

Symptoms:
-Musty body odor
-Growth retardation
-Mental retardation
-Seizures
-Eczema
-Fair skin

Front 242

What are the reactions that occur in Phase I metabolism? What are the properties of the metabolites?

Back 242

Reactions: Reduction, Oxidation, Hydrolysis

Metabolites: Slightly polar, Water-soluble, Active

Cytochrome P450 enzymes perform – Phase I reactions

Elderly patients lose Phase I first

Front 243

What are the reactions that occur in Phase II metabolism? What are the properties of the metabolites?

Back 243

Reactions: Acetylation, Methylation, Sulfation, Glucuronidation

Metabolites: Very polar, Inactive

Front 244

P450 Inhibitors (***HIGH YIELD***)

Back 244

“CRACK AMIGOS”

Cimetidine

Ritonavir (protease inhibitor)

Amiodarone

Ciprofloxacin

Ketoconazole

Acute alcohol use

Macrolides

Isoniazid

Grapefruit juice

Omeprazole

Sulfonamides

Front 245

P450 Inducers (***HIGH YIELD***)

Back 245

“Ken Rides Shotgun in Barbie’s Girl Car”

Kenytoin – Phenytoin

Rides – Rifampin

Shotgun – St. John’s Wort

Barbie’s – Barbiturates

Girl – Griseofulvin

Car – Carbamazepine

Front 246

What enzyme does Disulfiram inhibit?

Back 246

Acetaldehyde dehydrogenase

Front 247

What enzyme does Fomepizole inhibit?

Back 247

Alcohol dehydrogenase

Front 248

What 3 drugs are know to act through Zero-order kinetics? (KNOW)

Back 248

Phenytoin

Ethanol

Aspirin

Cp (plasma concentration) decreases linearly with time

Front 249

First-order kinetics

Back 249

Cp decreases proportionally with time

Front 250

Which hepatic phase of metabolism is lost first by geriatric patients? Which phase is mediated by cytochrome p450?

Back 250

Lost first by geriatric patients: Phase I

Mediated by cytochrome p450: Phase I

Front 251

Which medication overdose can be treated with sodium bicarbonate?

Back 251

Weak acids

Front 252

Which medication overdose can be treated with ammonium chloride?

Back 252

Weak bases

Front 253

What medication inhibits alcohol dehydrogenase?

Back 253

Fomepizole

Front 254

Which medications inhibit acetaldehyde dehydrogenase?

Back 254

Disulfiram

Front 255

Is conjugation a Phase I or Phase II reaction?

Back 255

Phase II

Front 256

Which takes place first: Phase I metabolism, or Phase II?

Back 256

Any order

Front 257

In what enzyme deficiency will Heinz bodies and Bite cells be seen in?

Back 257

Glucose 6 phosphate deficiency

*Heinz bodies-- oxidixes Hemoglobin precipitated within RBCs

*Bite cells-- results from phagocytic removal of Heinz bodies by splenic macrophages

"Bite into some Heinz ketchup"

Front 258

What is the equation for Serum- Ascites: Albumin Gradient (SAAG)

Back 258

SAAG= [albumin] serum - [albumin] ascites

SAAG > or = 1.1 in portal hypertension

SAAG < 1.1 in cancer, nephrotic syndrome, TB, pancreatitis, biliary disease, connective tissue

Front 259

How is Hep A transmitted, what is used to Dx it, and what are the S/S associated with it?

Back 259

ssRNA

Transmission: fecal- oral route

Dx: IgM HepA antibodies

S/S: mild
- Fatigue
- Abdominal pain
- Jaundice

Front 260

What is Hep A's carrier state? Does it cause chronic infection? Is there a vaccine available for it? How long is it's incubation period?

Back 260

No carrier state

Does not cause chronic infection

Vaccine is available

2 week incubation period

Front 261

Whats a good mnemonic to remember Hep A?

Back 261

3 A's:

Asymptomatic (usually)

Acute

Alone (no carrier)

Front 262

How is Hep E transmitted? Does it have a carrier? Is a vaccine available for it?

Back 262

Transmission: Fecal- oral route

No carrier

No vaccine

Front 263

Hep E is usually very mild and treatment is only supportive. In what population can Hep E be potentially fatal?

Back 263

Pregnant women

Front 264

What are the key characteristics of Hep D?

Back 264

Detective virus: incomplete genome of ssRNA

Can not infect unless a person already has hepatitis B

Transmission: sexual, parenteral, trans-placental

Severe infection

Front 265

What is the genome of Hep C and how is it transmitted?

Back 265

ssRNA

Transmission: mostly parenterally, can be passed sexual and trans-placental

*Hep C is the most common cause of transfusion mediated hepatitis

Front 266

Does Hep C cause chronic disease? Does it increase the risk for hepatocellular cancer?

Back 266

Chronic disease will occur in 80-90%

Carrier state increases the risk for hepatocellular cancer

Front 267

What drugs are used to treat Hep C? Is there a vaccine available?

Back 267

Treatment: Interferon and Ribavirin

No vaccine

Front 268

What is the genome of Hep B? How is it transmitted?

Back 268

dsRNA

Transmission: mostly sexually, can be through trans-placental and parenterally

Front 269

Can Hep B causes a chronic infection?

Back 269

It can, but less so than Hep C

If infected as an adult--- 10%

If infected trans- placentally or also have immune deficiency--- 80%

Front 270

Does Hep B have a carrier state?

Back 270

Yes

Increases risk of HCC

Front 271

Is there a vaccine for Hep B? How long is Hep B's incubation period?

Back 271

Yes, one of first vaccines given to a newborn

Incubation period= 60-90 days

Front 272

When will HB surface antigen (HBsAg) be positive?

Back 272

During active infection

Front 273

When will you have positive HBsAb (surface antibody)

Back 273

When you do not have active disease:

You had HepB and recovered

You were immunized against HepB

Front 274

What does positive Hep B core antibody indicate?

Back 274

A history of Hep B infection

Acute infection (early on) ---> +IgM

Chronic infection ----> +IgG

Full recover from infection ---> +IgG

Front 275

What does positive HB e antigen indicate?

Back 275

Virus is actively replicating and there is a high transmissibility

Front 276

What does positive HB e antibody indicate?

Back 276

Low transmissibility

Front 277

Describe Autoimmune Hepatitis Type 1 and 2

Back 277

Type 1: ANA (+) and/ or anti- smooth muscle antibody (+)

Type 2: Liver/ kidney microsomal antibody (+) and/ or liver cytosol antigen (+)

Front 278

What is the pathology of Primary sclerosing cholangitis? How does it present?

Back 278

Unknown cause of concentric "onion skin" bile duct fibrosis---> alternating strictures and dilation

Presentation:
- Pruritus
- Jaundice
- Dark urine
- Light stools
- Hepatosplenomegaly

Front 279

What is the pathology of Primary biliary cirrhosis?

Back 279

Autoimmune reaction---> Lymphocytic infiltrate + granulomas

*Lymphocytic infiltrate= T lymphocytes attacking the bile ductules in the liver parenchyma with granuloma formation

Front 280

What is the MOA of ursodiol?

Back 280

It is a naturally occuring bile actid

Decreases synthesis of cholesterol in the liver

Changes the composition of bile

In PBC is delays disease progressive and enhances survival

*Only approved treatment of PBC

Front 281

What is the pathology of secondary biliary cirrhosis?

Back 281

Extrahepatic biliary obstruction (gallstone, biliary stricture, chronic pancreasitis, carcinoma of the pancreatic head) ---> Increases pressure in intrahepatic ducts ---> injury/ fibrosis and bile stasis

Front 282

Cholelithiasis

Back 282

Gallstones

Front 283

Cholecystitis

Back 283

Inflammation/ infection of the gallbladder

Front 284

Cholangitis

Back 284

Inflammation/ infection of the biliary tree

Front 285

Choledocholithiasis

Back 285

Gallstones in the bile ducts

Front 286

What is clonorchis sinensis and what can it cause?

Back 286

Liver fluke that infects the biliary

Can cause cholangiocarcinoma and pigemented gallstones

Front 287

Phenylalanine is the precursor for _______. What enzyme enzymes this reaction?

Back 287

Phenylalanine -----> Tyrosine

via Phenylalanine hydroxylase

Front 288

What is cofactor is needed for phenylalanine hydroxylase?

Back 288

Tetrahydrobiopterin

Front 289

Tyrosine is converted to ______ via what enzyme?

Back 289

Dopa

Tyrosine hydroxylase

Front 290

Dopa is converted to dopamine via what enzyme? What cofactor is required?

Back 290

Dopa Decarboxylase

Vitamin B6

*Dopa decarboxylase is inhibited by Carbadopa

Front 291

Dopamine is converted to NE via what vitamin?

Back 291

Vitamin C

Front 292

What converts NE to Epinephrine?

Back 292

Cortisol

Front 293

Histidine is the precursor of ________

Back 293

Histamine

Front 294

Glycine is the precursor of _____ and _______

Back 294

Porphyrin

Heme

Front 295

Glutamate is the precursor of __________ and_________

Back 295

GABA

Glutathione

Front 296

Methionine is the precursor of _______

Back 296

S- adenosyl- methionine (SAM)

Front 297

Arginine is the precursor of________, _______, & ________

Back 297

Creatine

Urea

NO

Front 298

Tryptophan is the precursor of ______ and _______

Back 298

Niacin

Serotonmin

Front 299

What amino acid derivatives require Vit B6?

Back 299

Histidine ---> Histamine

Tryptophan ---> Niacin

Glycine ----> Porphyrin

Glutamate ----> GABA