Pathoma

Question 1

Genetic disease associated with Apo-E allele mutation

Answer 1

Late set Alzhemiers

Question 2

Disease caused by Amyloid precursor protein, presenilin 1 gene on chromosome 14, presenilin 2 gene on chromosome 1

Answer 2

Early onset Alzheimers

Question 3

There is a killed (Salk) and live (Sabin) vaccine against the polio virus. Which one is better at prolonged synthesis and secretion of local mucosal IgA?

Answer 3

Live virus

Question 4

What kind of test is used to compare the means of 2 groups of subjects?

Answer 4

Two-sample T-test

Question 5

What does acetylcholinesterase in the amniotic fluid typically indicate?

Answer 5

Failed fusion (Neural tube defect) 
Note:  Alpha-fetoprotein (AFP) in the amniotic fluid suggests the same thing.  Lack of close of the neural tube allows for these things to leak out into the amniotic fluid.

Question 6

TLR found on macrophages that recognizes the PAMP (lipopolysaccharide) on the outer membrane of gram negative bacteria.

Answer 6

CD-14

Question 7

TLR activation results in upregulation of ______, a nuclear transcription factor that activates immune response genes leading to production of multiple immune mediators.

Answer 7

NF-kappaB

Question 8

Enzyme that causes arachidonic acid release from the phospholipid cell membrane.

Answer 8

Phospholipase A2.

Remember, AA precursor of prostaglandins and thromboxanes (via cyclooxygenase COX) or leukotrienes (via 5-lipooxygenase).

Question 9

What function do the prostaglandins PGI2, PGD2, and PGE2 have?

Answer 9

They all three mediate vasodilation and increased vascular permeability.

Note: PGE2 also causes fEver and pain.

Question 10

What type of immune cells will you find toll-like receptors?

Answer 10

innate immune cells (macrophages and dendritic cells).

Question 11

What type of leukotriene will attract and activate neutrophils?

Answer 11

LTB4

Note: C5a (complement), IL-8 and bacterial products also attract and activate neutrophils.

Question 12

Which type of leukotrienes will contraction of smooth muscle (vasoconstriction and bronchospasm), as well as increased vascular permeability?

Answer 12

LTC4, LTD4, and LTE.

Question 13

What type of cell is activated by one of these three methods:

1. Tissue trauma
2. Complement proteins C3a and C5a
3. Cross linking the cell surface IgE by antigen

Answer 13

Mast cells-which are widely distributed throughout connective tissue.

Note: when they are activated, they release preformed histamine granules, which mediate vasodilation of the arterioles and increased vascular permeability.

Question 14

Three ways to activate complement are:

Classical pathway
Alternative pathway
Mannose-binding lectin pathway

What is each ones of these about?

Answer 14

1. Classical: C1 binds IgG or IgM that is bound to antigen.
   * ***Mneumonic: GM makes classic cars.
2. Alternative: microbial products directly activate complement.
3. MBL: MBL binds to mannose on microorganisms, which activates complement.

Question 15

All complement pathways result in production of C3 convertase (mediates C3–>C3a and C3b), which in turn produces C5 convertase (mediates C5–>C5a and C5b). C5b complexes with C6-C9 to form the membrane attack complex (MAC).

What do the following cause to happen:

1. C3a and C5a
2. C5a
3. C3b
4. Mac

Answer 15

1. C3a & C5a: anaphylatoxins: trigger mast cell degranulation, resulting in histamine-mediated vasodilation and increased vascular permeability.
2. C5a: Chemotaxis for neutrophils
3. C3b: Opsonin for phagocytes
4. MAC: Lyses microbes by creating a hole in the cell membrane.

Question 16

Inactive proinflammatory protein produced in the liver which is activated upon exposure to subendothelial or tissue collagen, which in turn activates:

1. Coagulation of fibrinolytic systems
2. Complement
3. Kinin System—>cleaves kininogen to bradykin, which mediates vasodilation and increased vascular permeability (similar to histamine), as well as pain

Answer 16

Hageman Factor (Factor XII)

Question 17

What are the five cardinal signs of inflammation?

Answer 17

1. Redness (rubor)
2. Warmth (calor)
3. Swelling (tumor)
4. Pain (dolar)
5. Fever

Question 18

In inflammation, rubor and calor (redness and warmth) are due to vasodilation, which results in increased blood flow. What are the key mediators of this?

Answer 18

Histamine, Prostaglandins (PGI2, PGD3, and PGE2), and bradykinin.

Question 19

Inflammation during swelling occurs due to leakage of fluid from POSTCAPILLARY venues into the interstitial space (exudate). What are are the key mediators?

Answer 19

1. Leukotrienes: cause endothelial cell contraction.

2. Tissue damage: results in endothelial cell disruption

Question 20

You will see pain (dolar) involved with inflammation. What mediates this pain?

Answer 20

Bradykinin and PGE2 sensitize sensory nerve endings.

Question 21

Macrophages are responsible for fever during inflammation. This occurs when pyrogens (LPS from bacteria) cause macrophages to release ________ and _________, which increase COX activity in per vascular cells of the hypothalamus, resulting in PGE2, which raises the temp set point.

Answer 21

IL-1 and TNF

Question 22

In acute inflammation, you will neutrophils will:

1. Marginate (due to vasodilation)
2. Roll
3. Adhere
4. Transmigrate and chemotax
5. Phagocytose

What causes the rolling?

Answer 22

P-selectin (released from Weibel-Palade bodies–>from histamine)
E-sectin (induced by TNF and IL-1)

Remember TNF and IL-1 are released from macrophages and also cause fever.

Question 23

In acute inflammation, you will neutrophils will:

1. Marginate (due to vasodilation)
2. Roll
3. Adhere
4. Transmigrate and chemotax
5. Phagocytose
6. What are the molecules that cause cellular adhesion?
7. What molecules cause the upregulation of these adhesion molecules?

Answer 23

1. ICAM and VCAM: on endothelium upregulated by TNF and IL-1.
2. Integrin: on leukocytes upregulated by C5a and LTB

Interaction between the CAMs and Integrins result in firm adhesion of leukocytes to the vessel wall.

Question 24

Autosomal recessive defect of integrins (CD18 subunit)

**Clinical features include delayed separation of umbilical cord, increased circulating neutrophils, and recurrent bacterial infections that lack pus formation.

Answer 24

LAD: Leukocyte adhesion deficiency

Question 25

In acute inflammation, you will neutrophils will:

1. Marginate (due to vasodilation)
2. Roll
3. Adhere
4. Transmigrate and chemotax
5. Phagocytose

What two opsonins are important in phagocytosis of pathogens or necrotic tissue?

Answer 25

IgG

C3b

Question 26

Autosomal recessive protein trafficking defect (caused by microtubule defect) characterized by impaired PHAGOLYSOSOME FORMATION.

Characterized by:

-Increased risk of pyrogenic infection-
Neutropenia (due to intramedullary death of neutrophils)
-Giant granules in leukocytes (due to fusion of granules arising from the Golgi)
-Defective primary hemostasis (due to abnormal dense granules platelets).
-Albinism (pigment can’t move into keratinocytes).
-Peripheral neuropathy (can’t traffic protein from nerve to nerve ending distally).

Answer 26

Chediak-Higashi syndrome

Question 27

What is the most effective method of phagocytosis?

Answer 27

O2-dependent killing.

(HOCL- generated by oxidative burst in phagolysosomes destroys phagocytosed microbes.

Question 28

Name that enzyme:

1. O2 —->O2-
2. O2—->H2O2
3. H2O2—->HOCL- (bleach): found in lysosomes in neutrophiles

Answer 28

1. NADPH oxidase (oxidative burst)
2. superoxide dismutase (SOD)
3. Myeloperoxidase (MPO)

Question 29

1. Disease caused by NADPH oxidase defect (so no O2—>O2—->H2O2—>HOCl-). It is either X-linked or autosomal recessive). Leads to recurrent infection and granuloma formation with catalase-positive organisms.
2. Two catalase-+ organisms that are typically recurrent in this diease.

Answer 29

1. Chronic granulomatous disease (CGD)

2. S. aureus, PSEUDOMONAS CEPACIA.

Question 30

Chronic granulomatous disease (CGD) is characterized by poor O2 dependent killing and is typically due to NADPH oxidase defect. What test is used to screen for CGD?

Answer 30

Nitroblue tetrazolium test-
Leukocytes are incubated with NBT dye, which turns blue if NADPH oxidase can convert O2 to O2- but remains colorless if NADPH oxidase is defective.

Question 31

What is the difference between chronic granulomatous disease and MPO deficiency?

Answer 31

Chronic granulomatous disease: NADPH oxidase deficiency-positive NBT (nitroblue terazoium test…will be clear results). No O2 to O2-.

Myeloperoxidase deficiency: defective conversion of H2O2 to HOCl- INCREASED RISK OF CANDIDA INFXN.–>NBT test will turn blue (b/c NAPDH oxidase is fine).

Question 32

What happens to neutrophils after resolution of infection?

Answer 32

Apoptosis within 24 hours after resolution of inflammatory stimulus.

Question 33

In acute inflammation, neutrophils will be on the scene first. But macrophages predominate after neutrophils and peak 2-3 days after inflammation begins. They arrive in the tissue the same way neutrophils do.

After macrophages ingest organisms via phagocytosis, how do they kill them?

Answer 33

O2 INDEPENDENT killing: use enzymes in secondary granules (such as lysozyme).

Question 34

Macrophages manage the next next of inflammatory process (after neutrophils). They can facilitate:

1. Resolution and healing.
2. Continued acute inflammation (IL-8 recruits more neutrophils)
3. Abscess (macrophages mediate fibrogenic growth factors and cytokines)
4. Chronic inflammation: macrophages present antigen to activate CD4+ helper T cells, which secrete cytokines that promote chronic inflammation.

What are the anti-inflammatory cytokines that cause resolution and healing after an acute inflammation?

Answer 34

1. IL-10 and TGF-beta

Question 35

Progenitor T-cells are formed in the bone marrow, but are further developed in the thymus where the T-cell receptor (TCR) undergoes rearrangment and becomes CD4+ helper T-cells or CD8+ cytotoxic T cells.

Which MHC do CD4+ t-cells recognize?
Which MHC do CD8+ t-cells recognize?

Answer 35

1. CD4+ (Helper T’s): MHC II
2. CD8+ (Cytotoxic T’s): MHC I

Remember MHC II is antigen from outside of the cell (bacteria, for example.
MHC I is antigen from inside of the cell (either self antigen or viral antigen).

Question 36

Activation of T-cells require two steps:

1. Activation of antigen/MHC complex.
   2 Additional signal.
2. In the case of CD4+ T-cells, they will bind MHC II. What is the second step in activation?
3. In the case of CD8+ T-cells, they will bind MHC I. What is the second step in activation?

Answer 36

1. B7 (antigen presenting cells)—>binds CD28 (on CD4+ helper T’s).
2. IL-2 from CD4+ helper T’s is the second step in activation of CD8+ cells.

Question 37

CD4+ T-Helpers can be divided into two categories:

TH1
TH2

What do each of these types of cells “help”?

Answer 37

TH1: stimulates CD8+ T-cells (via T-cell growth factor) and macrophages (via IFN-gamma.)

TH2: stimulates B-cell class switching to IgG and IgE via IL-4, eosinophil chemotaxis and activation, as well as maturation of B cells to plasma cells and class switching to IgA via IL-5, as well as secrete IL-10 (which inhibits TH1 phenotype).

Question 38

How do cytotoxic T-cells kill? (2 ways)

Answer 38

1. Secretion of perforin and granzyme; perforin creates pores that allow granzyme to enter the target cell, activating apoptosis.
2. Expression of FasL, which bind to Fas on target cells, activating apoptosis.

Question 39

Immature B cells are produced in the bone marrow and undergo immunoglobulin rearrangements to become naive B cells that express surface IgM and IgD. What two ways does B-cell activation occur?

Answer 39

1. Antigen binds surface IgM or IgD, resuling in maturation to IgM or IgD-secreting plasma cells.
2. B-cell antigen presentation to CD4+ helper T-cells via MHC class II.

Question 40

B lymphocytes are activated either by antigen binding surface IgM or IgD which results in a IgM or IgD plasma secreting cell OR a B-cell antigen presentation to CD4+ helper T’s via MHC Class II.

If the activation occurs in the latter way, what else has to happen to activate the B-cell after it presents antigen to the Th2 cell?

Answer 40

CD40 R (on Bcell) binds CD40L (on T cells), providing the second activation signal.

*Helper T then secretes IL-4 and IL-5, which mediates B-cell isotype switching, hypermutation, and maturation into plasma cells).

Question 41

A collection of epithelioid histiocytes (macrophages with abundant pink cytoplasm, usually surrounded by giant cells and a rim of lymphocytes.

Answer 41

Granuloma.

Question 42

Common etiologies include reaction to foreign material, sarcoiosis, beryllium exposure, Crohn disease, and cat scratch disease

Answer 42

Non-caseating granulomas (lack central necrosis)

Question 43

Tb and fungal infections cause this

Answer 43

Caseating granulomas (central necrosis)

Question 44

What do macrophages secrete that induce CD4+ helper T cells to differentiate into TH1 subtype?

Answer 44

IL-12

Question 45

What do Th1 cells secrete, which will convert macrophages into epithelioid histiocytes and giant cells which characterize granulomas?

Answer 45

IFN-gamma

Question 46

Present with T-cell deficiency (lack of thymus), hypocalcemia (lack of parathryoid hormone), abnormalities of heart, great vessels and face.

Answer 46

DiGeorge Syndrome (22q11 microdeletion): developmental failure of the 3rd and 4th pharyngeal pouches.

Question 47

Characterized by defective T-cell and B-cell immunity which results from adenosine deaminase (ADA) deficiency.

Answer 47

SCID: Since ADA is necessary to deaminiate adenosine and deoxyadenosine for exretion as waste products, buildup of adenosine and deoxyadenosine is toxic to lymphocytes.

Question 48

X-linked disease caused by mutated Bruton tyrosine kinase

Presents with recurrent bacterial, enterovirus and Giardia lamblia infections after 6 months of age.

Answer 48

x-linked agammaglobulinemia: disordered B cell maturation.

Avoid live vaccinations

Remember that there is no antibody to opsinize the bacteria, and no IgA to protect the mucosa (enterovirus and Giardia affect GI tract).

Question 49

Common variable immunodeficiency (CVID) is characterized by low Ig due to B-cell or T-cell defects. There is an increased risk for bacterial, enterovirus and Giardia infections.

What disorders does having CVID increase the risk of?

Answer 49

Autoimmune and lymphoma

Question 50

Most common immunoglobulin deficiency.

Answer 50

IgA: increased risk for mucosal infection, esp. viral; however, most patients are asymptomatic.

Question 51

Hyper IgM syndrome is caused by _______.

Answer 51

Mutated CD40L (on helper T cells) or CD40 (on B cells).

Second signal can not be delivered to helper T cells during B-cell activation. Consequently, cytokines necessary for Ig class switching are not produced.

Low IgA, IgG, and IgE result in recurrent pyogenic infections, due to poor opsonization, esp. at mucosal sites.

Question 52

Characterized by thrombocytopenia (increased bleeding), eczema, and recurrent infections (defective humoral and cellular immunity)

Answer 52

Wiskott-aldrich syndrome: due to mutation in the WASP gene (X-linked)

Question 53

What do C5-C9 deficiencies increase the risk for?

Answer 53

Neisseria infection (gonorrhea and meningititis)

Question 54

What will C1 inhibitor deficiency result in?

Answer 54

Hereditary angioedema (edema of skin and mucosal surfaces).

Nothing to inhibit complement.

Question 55

Sjogren’s cancer association

Answer 55

B-cell lymphoma

Suspect if patient has unilateral growth of parotid.

Question 56

SLE: characterized by ANA (sensitive but not specific). What antibody is specific?

Answer 56

Anti-ds DNA

Question 57

Drug-induced lupus antibody

Answer 57

Antihistone antibody

Drugs that cause are hydralazine, procainamide, and isoniazid. Removal of drugs = remission of symptoms.

Question 58

SLE can lead to false + syphillis test and falsely elevated PTT labs. Why?

Answer 58

Antiphospholipid antibody syndrome, where you have:

Anticardiolipin and lupus anticoagulant.

Question 59

1 of death in lupus patients

Answer 59

Diffuse proliferative glomerulonephritis

Question 60

Sjogren’s syndrome is lymphocyte mediated damage (type IV hypersensitivity) with fibrosis. Dry eyes, dry mouth because autoimmune destruction of lacrimal glands.

Characterized by ANA and anti-SSA and anti-SS-B. What is the target of these two anti-bodies?

Answer 60

Ribonucleotide proteins.

Question 61

Sjogren’s is associated with other AI disorders. Which one is most common?

Answer 61

RA

Question 62

Scleroderma is an disorder characterized by autoimmune tissue damge with activation of fibroblasts and deposition of collagen (fibrosis). Along with ANA, you will have Scl-70 antibody. What is this antibody against?

Answer 62

DNA topoisomeriase

Question 63

What is crest syndrome?

Answer 63

Type of localized scleroderma.

Crest:

Calcinosis
Reynaud's Phenomenon
ESOPHAGEAL DYSMOTILITY
Sclerodactyly
Telangectasia

Note: Has Anti-Centromere antibody

Question 64

If you see features of SLE, systemic sclerosis, and maybe a little bit of polymyositis mixed together, what kind of serum antibodies will you look for to help make a dx of mixed connective tissue disease?

Answer 64

anti-U1 ribnonuceloprotein antibodies.

Question 65

There are 3 types of wound healing based on tissue regenerative capacity.

1. Labile tissues: constant regeneration
2. Stable tissues: Have quietscent G0 cells that can reenter the cell cycle to regenerate when necessary
3. Permanent tissues: lack significant regenerative capacity.

What type of tissues fall in each?

Answer 65

1. Labile:
   Skin (basal cell stem cells)
   Small and large bowel (stem cells in mucosal crypts
   Bone marrow (hematopoietic stem cells.
2. Stable:
   Liver regeneration by compensatory hyperplasia after partial resection.
3. Permanent: myocardium, skeletal muscle and neurons.

Question 66

During wound healing, when will you get repair rather than replacement?

Answer 66

Repair is replacement of damaged tissue with a fibrous scar.

It occurs when regenerative stem cells are lost (deep skin cut) or when a tissue lacks regenerative capacity (after myocardial infarction).

Question 67

During wound healing, when regeneration can’t occurs and repair must happen, granulation tissue occurs first. What does granulation tissue include (3 things)?

Answer 67

Fibroblasts (depositis type III collagen)
Capillaries (provides nutrients)
Myofibroblasts (causes wound contraction)

Note: Type III collagen will be eventually replaced with Type I collagen via collagenase (with zinc cofactor).

Question 68

Type 1 collagen includes:

Answer 68

bONE
skin
tendons
most organs

Question 69

Type III collagen includes:

Answer 69

Remember, it is pliable, so it includes:

Blood vessels, granulation tissue, embryonic tissue

Question 70

Type II collagen includes:

Answer 70

CarTWOlidge

cartilage

Question 71

Type IV collagen is found in the:

Answer 71

basement membrane

Question 72

Keloids are formed from what type of collagen

Answer 72

Type III (pliable)–>remember type III is the type used in the initial granulation tissue of wound repair.

Question 73

Enzyme used to catalyze reaction from TYPE III to Type I collagen in wound repair? What is needed as cofactor of this enzyme?

Answer 73

Collagenase (needs zinc as a cofactor)

Question 74

Tissue regeneration and repair is mediated by paracrine signaling via growth factors. The growth factor interaction with its receptor will result in gene expression and cellular growth. Here are a few examples:

TGF-alpha
TGF-beta
PDGF:
Fibroblast growth factor
VEGF

Which of these is:

1. an important fibroblast growth factor while also inhibiting inflammation?
2. Important in angiogenesis?

Answer 74

1. TGF-B (remember that when macrophages secrete this, they call off the acute inflammation and start the healing response).
2. Fibroblast Growth Factor and VEGF are both important in angiogenesis.

Question 75

What is the difference between primary and secondary intention when relating to cutaneous healing??

Answer 75

1. Primary intention: wound edges are brought together (suturing of a surgical incision); leads to minimal scar formation.
2. Secondary intention: edges are not approximated. Granulation tissue (fibroblasts, capillaries, and myofibroblasts), then a scar forms.

Question 76

You would have delayed wound healing with infection, vitamin C, copper and zinc deficiencies as well.

Why would vitamin c, copper and zinc deficiencies cause delayed healing?

Answer 76

1. Vitamin C:cofactor for hydroxylation of proline and lysine procollagen residues. This hydroxylation is necessary for eventual collagen cross-linking.
2. Cooper: cofactor for lysyl oxidase, which cross-links lysine and hydroxylysine to form stabile collagen.
3. Zinc is cofactor for collagenase, which replaces Type III collagen with Type I.

Question 77

Dihiscence

Answer 77

Rupture of a wound, most commonly seeen after abdominal surgery.

Question 78

What is the difference between a hypertrophic scar and a keloid?

Answer 78

Hypertrophic scar is excess production of scar tissue that is localized to the wound. Keloid is excress scar tissue that is out of proportion to the wound (Excess type III collagen, AA, classically earlobes, face and UE).

Question 79

Kallman Syndrome

Answer 79

Defective migration of GnRH cells and formation of olfactory bulbs; Decreased Synthesis of GnRH in hypothalamus; anosmia, lack of secondary sexual characteristics. Decreased GnRH, FSH, LH, testosterone and sperm count.

FA page 525

Question 80

Three phases of gastric activity are cephalic, gastric and intestinal. Which phase(s) has/have increased gastric acid secretion and which have reduced?

Answer 80

Cephalic and gastric: increased (release of gastrin —>H+)

Intestinal phase reduces gastric acid secretion

Question 81

When doing drug trials, how do you determine the Number Needed to Harm (NNH)?

Answer 81

Find attributable risk:
Event rate of treatment - Event rate of placebo.

NNH = 1/attributable risk

page 52 FA

Question 82

In the final step of insulin synthesis, the pancreas B-cell cleaves away a portion of the proinsulin polypeptide chain, forming insulin. What happens to the other part of proinsulin that is cleaved from insulin?

Answer 82

C peptide is put in secretory granules with insulin and they are both secreted in eqimolar concentrations.

Question 83

Pulmonary fibrosis is a restrictive lung disease whereas COPD and asthma are obstructive lung diseases. How do people breathe in each one (rate and depth)?

Answer 83

Restrictive lung disease: Rapid, shallow breaths.

Obstructive: slow, deep breaths

Question 84

Obstructive of what vein will cause the following symptoms:

Right slided face and arm swelling
Engorgement of subcutaneous veins on the right side of the neck?

Answer 84

Right Brachiocephalic vein

Note: If symptoms were bilateral, this would be SVC Syndome.

Question 85

Autosomal recessive disorder caused by a deficiency in the enzyme homogentisic acid oxydase, which causes arthritis and deposits in sclera and ear cartilage. Urine turns black when hits air.

Answer 85

Alkaptonuria: This enzyme normally breaks down the tyrosine byproduct homogentysic acid (also called alkapton). This acculated homogentesic acid causes pigment deposits in connective tissues throughout the body.

Question 86

Name that pharyngeal pouch:

Epithelium of middle ear and auditory tube

Answer 86

1st pharyngeal arch

Question 87

Name that pharyngeal pouch: thymus and inferior parathyroid glands

Answer 87

3rd arch

Question 88

Name that pharyngeal arch:

Superior parathyroid glands, ultimobranchial body

Answer 88

4th pharyngeal arch

Question 89

Myasthenia gravis is associated with abnormalities of the _____________.

Answer 89

thymus

Question 90

What is the difference between suppression and repression as relates to coping skills?

Answer 90

Suppression: CONSCIOUS, mature defense mechanism involving troublesome thoughts or impulses.

Repression: UNCONSCIOUS removal of disturbing psychological material from conscious awareness.

Question 91

What heart abnormality is associated with Downs Syndrome?

Answer 91

Endocardial cushion defects (Ostium primum ASD, regurgant atrioventricular valves)

Question 92

Heart defect associated with DiGeorge Syndrome.

Answer 92

Tetrology of Fallot and interrupted aortic arch.

Question 93

What heart defect is associated with Marfan Syndrome?

Answer 93

cystic medial necrosis of aorta.

Question 94

Heart defect associated with Turner Syndrome:

Answer 94

Coarctation of the aorta.

Question 95

What heart defect is associated with Tuberous sclerosis?

Answer 95

Valvular obstruction due to cardiac rhabdomyomas. (TS = benign tumors in a lot of different tissues including brain and kidneys).

Question 96

Heart problems associated with Friedreich’s ataxia.

Answer 96

Hypertrophic cardiomyopathy.

Question 97

What is defective in kertagener syndrome?

Answer 97

Dynein in the microtubules, which means so movement of cilia. Infertility, recurrent sinusitis and bronchiectasis result. Is associated with situs inversis.

Question 98

Most common three cancers in women?

Answer 98

Breast, lung, colon

Note: When looking at mortality, it is in the following order: Lung, Breast, Colon

Question 99

What is the best way to prevent neonatal tetanus?

Answer 99

Give maternal vaccine during pregnancy. This will allow the transfer of protective IgG antitoxin antibodies across the placenta.

Question 100

What is the difference between brief psychotic disorder, schizophreniform disorder and schizophrenia?

Answer 100

The amount of times that symptoms last.

Brief psychotic disorder: < 1 month
Schizophrenifom: 1 month - 6 months
Schizophrenia: > 6 months

Question 101

What happens if there is incomplete obliteration of the processus vaginalis?

Answer 101

You get indirect inguinal hernia (hernia through the deep inguinal ring, but not in the inguinal triangle as with direct hernia).

BUT, if you don’t have a herniation but still have incomplete obliteration, fluid can leak into the testicles causes hydrocele of the testicle.

Question 102

Fetus with flat face, abnormal ears, slanted palpebral fissures, redundant skina t nape of neck, hypotonia, pelvic dysplasia and single transverse palmar crease.

Answer 102

Down Syndrome (Trisomy 21)

Question 103

Microcephaly, low-set ears, prominent occiput and small mandible. Clenched fist and fingers overlap. What is it?

Answer 103

Trisomy 18 (Edwards Syndrome)

Question 104

Cleft lip and palate, polydactyly and omphlocele

Answer 104

Trisomy 13 (patau syndrome)

Question 105

How do you attempt to prevent acute organ rejection after a transplant?

Answer 105

Calcineurin inhibitors: cyclosporine and tacrolimus. If in active rejection, give steroids.

Question 106

In the female reproductive tract, androgens and progesterone are synthesized from cholestrol in the theca interna. Where are these androgens converted to estradiol?

Answer 106

The granulosa cells have aromatase, which can convert androgens to estradiol. This occurs under the stimulation of FSH.

Question 107

Polyhydramnios refers to excessive accumulation of amniotic fluid and is typically related to either decreased fetal swallowing of increased fetal urination. What fetal anomalies are associated with polyhydramnios?

Answer 107

Gastrointestinal obstruction due to duedenal, esophageal, or intestinal atresia and ANACEPHALY.

Question 108

What causes Potter’s Syndrome?

Answer 108

Severe oligohydramnios. You will get renal a genesis and fetal compression by the uterus, which results in fetal deformities.

Question 109

Connection observed at the apices of glandular cells and consist of two closely adherent cytoplasmic membranes without an intervening space. The first component of the junctional complex.

Answer 109

Tight junctions

Question 110

Delicate network of cytplasmic filaments that radiate from teh cell membrane to hold adjacent cells together. They are the second component of the junctional complex.

Answer 110

Intermediate junctions

Question 111

Small, circular, adherent patches circumferentially palced around cellst hat comprise the third component of the junctional complex. They are common in stratified squamous epithelium and contribute significantly to the structural cohesiveness of tissues subject to mechanical stressors.

Answer 111

Desmosomes

Question 112

Extend from basal surfaces of keratinocytes in the stratified squamous epithelium to attach to the basal lamina.

Answer 112

Hemidesmosomes.

Question 113

Osteocytes have long intracanalicular processes that extend through the ossified bone matrix. These processes send signals to and exchange nutrients and waste products wtihin the neighboring lamallae via _______________ junctions. Osteocytes can sense mechanical stresses and send signals to modulate the activity of surface osteoblasts, therby helping to regulate the bony remodeling.

Answer 113

Gap

Question 114

In a normal bell-shaped distribution, what % falls within:

1 std
2 std
3 std

Answer 114

1: 68%
2. 95%
3. 99.7%

Question 115

Chronic granulomatous disease is an X-linked disorder resulting in the deficiency of what enzyme?

What test is used to diagnose this disease?

Answer 115

1. NADPH oxidase (O2 —> O2- can not be done, meaning that HOCL- can not be created to kill the bacteria in the neutrophil.)
2. Nitroblue tetrazoium test: If it DOES NOT CHANGE COLOR, then there IS a deficiency of NADPH oxidase.

Question 116

S. aureus has an enzyme on its surface that reacts with prothrombin , which results in converting fibrinogen to fibrin. This process is believed to result in fibrin-coating of the organsims and resistance to phagocytosis. What is the molecule on the surface of S. aureus?

Answer 116

Caoagulase

Question 117

What is the enzyme produced by clostridium bacteria that is capable of destroying erythrocytes, platelets, leukocytes and endothelial cells? It is responsible for the necrosis observed in gas gangreen (closteridium myonecrosis).

Answer 117

Lecinthinase, also called Phospholipase C or alpha toxin

Question 118

A promoter region that binds transcription factors and RNA polymerase II during initiation of transcription. It is located appx. 25 bases upstream from teh beginning of the coding region.

Answer 118

TATA box

Note: The other promoting codon is the CAAT box, which is loacted 60-80 bases upstream on the 5’ end of the coding region.

Question 119

What is the purpose of DNA methylation?

Answer 119

To silence the gene it is affecting. It is part of the epigenetic code.

Question 120

1. ___________________ is produced by Sertoli cells and is the physiologic inhibitor of FSH secretion.
2. LH concentration is primarily controlled by levels of what hormone?

Answer 120

1. Inhibin B: FSH stimulates the release of inhibin B from the sertoli cells of the testes.
2. Testosterone (also DHEA and dihydotestosterone)==>all part of the LH feedback inhibition loop. LH stimulates the release of testosterone from the Leydig cells of the testes.

Question 121

CO binds to hemoglobin with a much higher affinity than O2, thus preventing O2 binding to hemoglobin. It also reduces teh unloading from hemoglobin into the tissues.

Does CO poisoning affect the PaO2, and does it precipitate methemoglobinemia?

Answer 121

1. No: The PaO2 (partial pressure of oxygen dissolved in the plasma) does not change. It affects only the amount of oxygen that can bind to hemoglobin. Thus PaO2 in CO poisoning is normal.

No. Methemoglobin is formed with the Fe2+ (ferrous iron) in heme is oxidized to Fe3+ (ferric iron). This results from drug exposures (dapsone, nitrites), as well as enzyme deficencies and hemoglobinopathies.

Question 122

1. How does CSF get from the lateral ventricles to the third ventricle?
2. From the third to the fourth ventricle?
3. From the fourth ventricle to the subarachnoid space?

Answer 122

1. Interventricular foramen of monroe (lateral to third)
2. Cerebral aquaduct (third to fourth)
3. Lateral foramina or Luschka or Medial foramen of Magendie (fourth to subarachnoid space).
   * Note: CSF is returned to the venous circulation via the arachnoid villi (granulations)

Question 123

No blockage to CSF flow within the brain or brainstem, but a blockage in the subarachnoid space where flow may be restricted by fibrosis following inflammation

Answer 123

Communicating hydrocephalus

Question 124

Hydrocephalus where CSF is not absorbed by the arachnoid villi

Answer 124

Normal pressure hydrocephalus

Question 125

Increased CSF volume occurring in conditions of brain atrophy

Answer 125

Hydrocephalus ex vacuo

Question 126

A condition that classically occurs in overweight young females and is related to decreased CSF outflow of the arachnoid villi

Answer 126

Pseudotumor cerebri

Question 127

The RAS-MAP kinase pathway is a signal tranduction pathway that leads to gene transcription. RAS is a G-protein that exists in active and inactive forms.

What makes RAS inactive?
What makes RAS active?

Answer 127

Inactive RAS: attached to GDP
Active RAS: bound to GTP (occurs after the tyrosine kinase receptor on the membrane autophosphorylates).

Note: Activated RAS starts a phosphorylation cascade tht starts with activation of RAS kinase. This results in activation of MAP (mitogen-activated protein) kinase, which enters the nucleus to influence gene transcription.

Question 128

Annular pancreas is when the pancreas encircles the descending part of the duodenumand may lead to symptoms of duodenal obstruction in neonates. Embyologically, what causes annular pancreas?

Answer 128

The abnormal migration of the ventral pancreatic buds.

Ventral pancreatic bud gives rise to a portion of the head, the ucinate process adn the main pancreatic duct.

Question 129

What is t(15:17) associated with?

*What type of cancer and what receptor is messed up?)

Answer 129

The M3 variant of Acute Myelogenous Leukemia, called Acute PROMYELOTCYTIC leukemia.

The gene for RETINOIC ACID RECEPTOR alpha from chromosome 17 is transferred to chromosome 15 in a location adjacent to the PML gene. The fusioni causes an abnormal retinoic acid receptor, inhibiting myeloblast differentiation.

Question 130

Abnormal epidermal growth factor receptors are associated with what type of cancers?

Answer 130

ERB1: Non-small cell lung carcinoma

ERB2 (aka HER2/neu): breast cancer

Question 131

Defective platelet derived grwoth factor receptor plays a role in what type of cancer?

Answer 131

CML: Chronic myelomonocytic leukemia

Question 132

Abnormal Rb gene prediposes individuals to what two types of cancer?

Answer 132

Retinoblastoma and osteosarcoma

Question 133

In the kidney, what does the ureteric bud ultimately give rise to?

Answer 133

The collecting system, including the collecting ducts, minor calyces, major calyces, the renal pelvis and ureters.

Question 134

In the kidney, what does the metanephric mesoderm give rise to?

Answer 134

Glomerulus, Bowman’s space, the proximal tubule, loop of Henle, distal tubule and the collecting tubule.

Question 135

What is associated renal angiomylipoma? (Benign tumor of blood vessels, smooth muscle and fat)

Answer 135

Tuberous sclerosis (autosomal dominant), so will present with brain harmartomas and ash-leaf skin patches. Patient can have seizures from the harmartomas.

Question 136

What is the primary site of regulation of potassium concentrations in the tubular fluid and urine?

Answer 136

Collecting duct.

Note: 2/3 of filtered potassium is absorbed in the proximal convoluted tubule.
20% of that filtered potassium load will then occur in the loop of henle by the Na/K/2Cl transporter.

The rest occurs in the collecting duct and depends on state of kalemia in the body.

Question 137

The left kidney lies immediately deep to the tip of the ______ rib.

Answer 137

12th

Question 138

What cells would be expected to undergo hyperplasia and hypertrophy in the event of decreased kidney perfusion?

Answer 138

Modified smooth muscle cells of the afferent arteriole, which secrete renin from the juxtaglomerular apparatus. These cells are located in the afferent and efferent arterioles. In the event of low perfusion, the afferent JG cells will synthesize and secrete renin.

Question 139

What essential protein is necessary to promote the growth and differention of T-cells?

Answer 139

Calcineurin, a protein phosphatase will work on NFAT (nuclear factor of activated T-cells), dephosphorylating it. Then, the NFAT will cuase IL-2 promotion.

IL-2 stimulates growth and differential of T-cells.

Question 140

What do the immunosuppressant drugs, cyclosporine and tacrolimus, inhibit?

Answer 140

Calcineurin, which causes upregulation of IL-2 and therefore causes T-cell differentiation.

These drugs are commonly used as immunosuppressants in kidney transplantation.

Question 141

Chronic NSAID use can cause what in the kidneys?

Answer 141

Chronic interstitial nephritis. Usually gets better when medication is stopped.

Question 142

Amphotericin B works by binding ergosterol in fungal cell membranes to form holes. It is the drug of choice for mucormycosis. Considering the side effects of this med, what should be routinely monitored?

Answer 142

This drug is NOTORIOUS for renal toxicity.

Severe HYPOKALEMIA and HYPOMAGNESIA

*Note: May often relay daily supplementation.

Question 143

A 64-year old male hospitalized with severe abdominal pain and hypotension begins to hyperventilate. Lab testing reveals metabolic acidosis, an increased anion gap and high plasma lactate levels. This patient’s findings are best explained by a low activity of what enzyme?

Answer 143

Pyruvate dehydrogenase.

Hypoxia induced lactic acidosis is typically caused by low activity of this enzyme because oxidative phosphorylation can’t take place and there is a high activity of lactase dehydrogenase.

Question 144

When will you see someone with renal osteodystrophy?

Answer 144

End-stage renal disease: Causes hypocalemia via renal retention of phosphate and decreased renal synthesis of calcitriol (1,25-dihydroxycholecalceriferol vitamin D. Both hypocalcemia and the resultant secondary hyperPTH contribute to renal osteodystrophy.

Question 145

A drug used to treat intraocular pressure is also a diuretic.

What is the drug and what part of the tubule does it work?

Answer 145

Acetazolamide: Carbonic anhydrase inhibitor.

It works in the proximal tubule.
Note; will cause alkaline urine because CA blocks NaHCO2 and water reabsorption in the proximal tubule. If CA is block, you pee out the NaHCO2. (alkaline urine)

Question 146

Aldosterone antagonist commonly used in treating class III and IV heart failure patients. It is structually similar to steroids, so can cause gynecomastia, decreased libido and impotence.

Answer 146

Spironolactone.

NOTE: Eplerenone is a newer and more selective aldosterone antagonist that may produce less endocrine effects.

Question 147

DNA-dependent RNA polymerase that incorporates short RNA primers into replicating DNA.

Answer 147

Primase

Question 148

Topisomerases are enzymes that relieve the resultant strains from when helicase separates and unwinds, causing supercoiling.
Another name for topoisomerase.

Answer 148

Gyrase

Question 149

During kidney transplantation, after connecting graft and host vessels, the kidney becomes cyanotic and mottled immediately. What type of hypersensitivity is this?

Answer 149

Type II - There are preformed recipient antibodies against antigens on the host organ.

Question 150

Type I hypersensitivity (immediate) in cases of anaphylaxis, urticaria and some forms of seasonal allergies and asthma. What is happening on a cellular level in Type I?

Answer 150

Antigen leads to IgE class switching on B-lymphocytes.

Question 151

Type of sensitivity reaction where antibody-antigen complexes along with complement bind and attract neurophils to cause increased inflammation and tissue damage at site?

Answer 151

Type III: Immune complex mediated.

Question 152

Graft versus host disease is a type of what kind of hypersensitivity?

Answer 152

Type IV: Cell mediated, caused by competent donor T-cells form a donor attack host.

Question 153

Anti-GBM ab can react with the type IV collagen in the glomerular basement membrane and alveoli (hematuria and hemoptysis) This is found in Goodpasture’s Disease. On light microscope, what will you see?

What about on IF?

Answer 153

Light microscope: Crescents (Rapidly progressive Glomerulonephritis, Type 1)

IF: linear deposits of IgG and C3 along the glmoerular basement membrane.

Question 154

What is the first way to tell if diabetes is injury the kidneys?

Answer 154

Microalbuminuria

Question 155

HIV patient has visual problems.

What is probably causing it?
What drug do you give/

Answer 155

CMV-causes retinitis in AIDS patients.

Foscarnet-an analog of pyrophophate that can chelate calcium and promote nephrotoxic renal magnesium wasting. These toxicities can result in symptomatic hypocalcemia and hypomagnesemia.

Question 156

What happens to the GFR and FF (filtration fraction) when you have severe vasoconstriction of the EFFERENT arteriole?

Answer 156

GFR: Increases (to a certain extent)
FF: Always increases.

Question 157

Spike and dome appearance on electron microscopy?

Answer 157

Membranous glomerulopathy: most common cause of nephrotic syndrome in adults.

It can be secondary to underlying malignant tumors, infections and certain mediations. Diffuse thickening of the glomerular basement membrane (on light microscopy) without increased cellularly.

Remember, has “membranous” in the name, so is Ig mediated.

Question 158

Caucasian male undergoing treatment with Amphotericin B for disseminated histoplasmosis complains of weakness and palpitations. ECG reveals frequent premature ventricular beats. What does drug toxicity of this drug cause?

Answer 158

Renal tubular dysfunction. So, hypokalemia and hypomagnesia cause the problem in the heart.

Question 159

What does ureteral constriction or obstruciton do to the GRF and FF?

Answer 159

Decreases both.

Question 160

Why do uric acid crystals tend to precipitate out in the collecting ducts or DCT?

Answer 160

This is more acidic. They will be soluble in a normal pH. They need a more acidic environment to precipitate out.

Question 161

Wegener’s Granulomatosis

Answer 161

c-ANCA

Upper respiratory (sinus infection)
Pulmonary systems (hemoptysis)
Renal:  hematuria (Rapidly progressing glomerulonephritis-crescentric)

Question 162

Renal Blood Flow (RBF) refers tot the volume of blood that flows through the kidney per unit time and can be calculated by dividing the

RPF/(1-hematocrit)

How do you determine renal plasma flow (RPF)

Answer 162

RPF = PAH clearance

PAH clearance is determined by:

(urine [PAH] X urine flow rate)/plasma [PAH]

Question 163

Coca-cola colored urine and facial edema after a step infection is poststreptococcal glomerulonephritis. What characteristic is most likely to affect prognosis of these patients.

Answer 163

Age: children recover 95% of the time.

Adults: only 60% will resolve completely.

Question 164

Where will you find the most concentrated urine in the absence of ADH?

Answer 164

The junction between the descending and ascending limbs of the loop of Henle.

Question 165

A patient presents with periorbital edema, hematuria, and a has a history of impetigo (or cellulitis or pharyngitis). What is mediating the damage to the patient’s kidneys?

Answer 165

Immune complexes (type III hypersensitivity). Causing poststreptococcal glomerulonephritis.

Question 166

More than 60% of fluid filtered into the glomerulus is reabsorbed in the ______________, regardless of the patient’s hydration status.

Answer 166

Proximal tubule.

Question 167

What effect does aldosterone have on the following:

Na+
N+
K+
H2O

Answer 167

Na+: reabsorbed
H+: secretion (follows K+)
K+: secretion
H2O: reabsorption

Question 168

Alcoholic (homeless) man is brought in to the ER with vomiting and prolonged oliguria. Renal biopsy shows multiple oxylate crystals observed in the tubule lumen. What happened?

Answer 168

Ethylene glycol is rapidly absorbed in the GI tract and metabolized to glycolic acid, which is toxic to renal tubules, and to oxalic acid, which precipitates as calcium oxylate crystals in the renal tubules.

High anion gap and metabolic acidosis and osmolar gap are typical.

Question 169

What is the normal kidney filtration fraction in a normal healthy individual?

Answer 169

20%

Question 170

A 45-year old female with polyuria receives a vasopressin injection. Her urinary output decreases signficantly shortly afterward. Renal clearance of which of the following substances would be most significantly reduced from baseline after the injection?

Glucose
Creatinine
Urea
Para-amino hippuric acid
Calcium

Answer 170

Urea

Vasopressin (ADH) produces a V2 receptor-mediated increase in permeability to water and urea at the luminal membrane of the inner medullary collecting duct. The increase in urea reabsorption corresponds to a decrease in the fractional excretion of urea and a decrease in renal clearance of urea from plasma.

Question 171

Which type of diuretic (loop or thiazide) are more likely to cause hypercalcemia?

Answer 171

Thiazide (which work in the distal convulated tubule)

They work by blocking Na-Cl symporters. Since only a small amount of Na+ reaches the distal tubules, thiazides are not as efficacious as loop diuretics.

Question 172

Kimmelstiel-Wilson nodules in the kidneys indicate nodular glomerulosclerosis. What are K-@ nodules pathognomonic for?

Answer 172

Diabetes nephropathy

Note: ACE inhibitors and ARBs can prevent progression to diabetic nephropathy!!!

Question 173

What happens to most people who experience acute tubular necrosis?

Answer 173

They experience tubular re-epithelization and regain renal function.

Question 174

42-year-old white guy with hypertension and hematuria undergoes renal biopsy. Light microscopy reveals cellular proliferation, focal necrosis, and crescent formation of most glomeruli. On IF, THERE ARE NO IG COMPLEMENT DEPOSITS. What additional findings would you expect?

Answer 174

Serum anti-neurophil cytoplasmic bodies
(c-ANCA Wegeners)
p-ANCA-Churg Strauss

Note: There are not Igs to bind complement, so nothing on IF.

Question 175

What kind of drug is furosemide?

Answer 175

Loop diuretic: inhibits Na-K-2Cl symporter in the ascending loop of henle.

Question 176

What would be the common findings of the following in chronic renal failure?

1. PTH
2. Calcium
3. Phosphate
4. Calcitriol (vitamin D)

Answer 176

1. PTH: high
2. Calcium: low
3. Phosphate: high
4. Calcitriol: low

Question 177

What happens to renal plasma flow and filtration fraction when you constrict the efferent arteriole of the kidneys?

Answer 177

Renal plasma flow ill decrease
FF will increase

RPF: is the volume of plasma that is delivered to the kidney per unit time.

Question 178

Transitional cell carcinoma of the bladder typically presents as gross hematuria i nan elderly man. A history of smoking or occupational exposure to rubber, plastics, aromatic amine-containing dyes, textiles or leather increases the risk of developing transitional cell carcinoma. So does smoking.

Answer 178

n/a

Question 179

Cimetidie is a H2-receptor antagonist. What is it used to treat and what is a common side effect?

Answer 179

Treats peptic ulcer disease: Decreases hydrochloric gastric acid production.

Gynecomastia (like spironolactone, the aldosterone antagonist(.

Question 180

What vitamin increases peripheral metabolism of levadopa, decreasing its effectiveness?

Answer 180

B6

Question 181

Acute salicylate intoxication

Answer 181

1. Resp. alkalosis first (hyperventilation)
2. Anion gap acidosis begins to develop, due to accumulation of organic acids in the blood. At high concentrations, salicylates increase lipolysis, uncouple oxidative phosphorylation, and inhibit the citric acid cycle resulting in metabolic intermediates like ketoacids, lactate and pyruvate.

Question 182

Horseshoe kidney is a congenital abnormality that occurs when both kidneys are fused at the poles in early embryonic life. What does the isthmus get trapped on that prevents it from ascending to its normal place?

Answer 182

Inferior mesenteric artery.

Question 183

Nephrotic syndrome: proteinuria, edema

What does the liver synthesize to help bulk up the blood since protein is getting secreted out?

Answer 183

Lipoproteins.

Question 184

Inherited deficiency of alpha-glactosidase A that causes accumulations of ceramide trihexosides in tissue. The earliest manifestations are angiokeratomas, hypodirosis and acroparesthia.

1. What is this disease?
2. What is the patient at great risk of developing if enzyme therapy is not given?

Answer 184

1. Fabry Disease

2. Renal failure

Question 185

Ultrasound examination in utero reveals bilateral absence of the kidneys in a fetus at 24 weeks. The baby is born alive at term, but dies 12 hours after birth. What is responsible for the death?

Answer 185

Lung immaturity.

Renal agenesis = potter’s syndrome due to oligohydramniose. Potters is low set ears and flat face, pulmonary hyperplasia, absence of kidneys.

Question 186

The most common renal malignancy is clear cell carcinoma, which arises from the renal tubule cells (usually proximal tubule). What is in the cells that makes them stain clear?

Answer 186

Glycogen and lipids.

Question 187

Why would a person taking lithium complain of excessive thirst and urination?

Answer 187

Lithium-induced diabetes insipidus is the result of lithium’s inhibitory action on vasopressin in receptors in the collecting ducts.

Question 188

Substance A is freely filtered in the glomeruli and reabsorbed int he renal tubules. A healthy volunteer receiving substance A has the following findings:

Inulin clearance; 100 mL/min
PAH clearance: 500 mL/min
[Plasma] A: 0/5 mg/mL
Tubular reabsorption of A: 25 mg/min

What is the expected excretion rate of substance A in this volunteer?

Answer 188

Net excretion for A = Total Filtration Rate - Total tubular reabsorption rate.

Total filtration rate of A =GFR X [PlasmaA]
GFR is appx. equal to inulin clearance b/c inulin is neither secreted or reabsorbed in the renal tubules. So…

Total filtration rate of A = (100 ml/min)(0.5 mg/mL) - 25 mg/min

Excretion rate of A: 25 mg/min

Question 189

Diabetic ketoacidosis.

Will each of these decrease or increase in the urine?

pH
HCO3-
H2PO4-

Answer 189

pH will decrease: as more acidic compounds accumulate in the bloodstream, H+ excretion increases throughout the nephron.

HCO3- bicarb will be recycled, so it will decrease in the urine

H2PO4-: Increase, because there is a limit to urinary excretion of free H+ (minimum urinary pH is 4.5), so some H+ is bound by HPO4^2- and NH3 to form the titratable acids H2PO4- and NH4+ respectively. Binding of H+ by these buffers allows for the excretion of more acid without a rapid decrease in urinary pH.

Question 190

Where do the most potent class of diuretics work in the kidney? (What symporter and what part of the tube?)

Answer 190

Loop diuretics work by inhibiting the Na+K+2Cl- symporter in the thick ascending loop of henle and effectively block Na+ and Cl- transport resulting in increased Na+, Cl- and H2O excretion.

Common side effects: hypokalemia, hypomagnesemia and hypocalcemia.

Loopes: Furosemide torsemide, bumetanide and ethacrynic acid. Used to treat pulmonary edema, venous and pulmonary congestion secondary to CHF, and peripheral edema. Note: less common side effect is ototoxicity.

Question 191

What type of drug is acetazolamide?

Answer 191

Diuretic that works in the proximal tubule inhibiting carbonic anhydrase. Carbonic anhydrase inhibitors block the reabsorption of NaHCO3 in the proximal tubule.

Question 192

Mannitol is an osmotic diuretic.

Where does it work?

Answer 192

Descending limb of loop of Henle and proximal tubule.

The descending limb is very permeable to water, allowing water to diffuse into the interstituim to produce a more concentration tubular fluid. This diuretic reduces the absorption of water (keeps in in the tubule to be pee’d out).

Question 193

Where do thiazide diuretics work?

Answer 193

Distal tubule, which is almost impermable to water So, these diuretics are not near as potent.

Question 194

What kind of diuretics work in the collecting duct system of the kidney?

Answer 194

Aldosterone inhibiting diuretics (spironolactone) and sodium channel blockers (amiloride)

Note: Both types are POTASSIUM SPARING.

Question 195

A mallory weiss tear is caused from repetitive, forceful vomiting. Would a person with a mallory weiss tear be in metabolic acidosis or alkalosis?

Answer 195

Alkalosis.

Question 196

Why would starting an ace inhibitor cause a GFR reduction?

Answer 196

Angiotensin II preferentially constricts the EFFERENT arteriole, thereby maintaining the GFR. When you give an ACE-inhibitor, you will have less angiotensin II, so this will promote arteriolar dilation, causing GFR reductions.

Question 197

Acyclovir is a antiviral that is often used to treat herpes. If you give high dose IV acyclovir, what do you need to make sure and do along the the treatment?

Answer 197

Aggressive IV hydration. Acyclovir can cause crystalline nephropathy if adequate hydration is not also provided.

Question 198

Where is PAH concentrention lowest in the nephron?

Answer 198

In Bowman’s space. PAH is not reabsorbed in any portion of the nephron, yet secretion into the tubule occurs via carrier-mediated organic acid active transport.

Question 199

What will you note about glucose excretion at low loads?

What about at higher loads?

Answer 199

Normal renal handling of plasma glucose is characterized by complete reabsorption of low filtered loads at low plasma concentration. (You don’t pee any out).

Increasing fractional excretion of glucose is observed at high plasma concentrations.

Question 200

Ischemic ATN is one of the most common causes of intrinsic renal failure (acute renal failure) in hospitalized patients. Muddy brown casts are pathognomonic for ATN. What part of the nephron is most susceptible to renal ischemia?

Answer 200

PROXIMAL TUBULES and thick ascending loop of henle.

Question 201

It is estimated that for PAH, the extraction ratio (arterial plasma PAH minus venous plasma PAH divided by arterial plasma PAH) is near 90% at arterial plasma concentrations lower than 20 mg/dl. Once the plasma concentration of PAH is increased above this level, the extraction ration decreases progressively. What explains that?

Answer 201

PAH is freely filtered from the blood in the glomerular capillaries to the tubular fluid in Bowman’s space. It is also secreted from teh blood into the tubular fluid by the cells of the proximal tubule by a carrier protein-mediated process. The secretion of PAH can be saturated at high blood levels.

Question 202

An 8-year old male is brought tot he doctor with a puffy face and coca-colored urine. He had experienced a skin infection three weeks ago. A kidney biopsy is performed and IF is done. What will IF show?

Answer 202

Granular deposits of IgG,IgM and C3 in the mesangium and basement membranes. A ‘starry sky’ appearance.

Question 203

A 60-year-old male with painless hematuria is found to have a right-sided renal mass. There is no significant family history. Cytologic evaluation of the mass shows malignant cells with chromosome 3p deletion. The deletion most likely involves what gene?

Answer 203

VHL-Patients with both sporadic and hereditary (associated with Von Hippel-Lindau disease) renal carcinomas are found to have deletions of the VHL gene on chromosome 3p

Question 204

If you see imaging of a kidney and liver full of cysts, there is a genetic problem. What is most likely mode of inheritance?

Answer 204

Autosomal Dominant

ADult polycystic kidney disease

Most frequent genetic cause of renal failure in adults.

Question 205

If an elderly patient has a normal creatinine, would you want to decrease the dose in mediations that are renally cleared, such as digoxin?

Answer 205

Because elderly patients exhibit age-related renal insufficiency, even in the presence of normal creatinine levels.

Side note: Digoxin is a cardiac glycoside and toxicity will present with visual changes and GI disturbances.

Question 206

These are side effects for what type of drug?

Decreased GFR
Hyperkalemia
Cough
Angioedema is a rare, but life-threatening side effect.

Answer 206

ACE-inhibitors (pril)

Question 207

If you have an individual who is in diabetic ketoacidosis and their PaCO2 is not low, what is going on?

Answer 207

They are in respiratory failure. They should be breathing off CO2 in a compensatory response to the metabolic acidosis. They can do this for awhile, but eventually will have respiratory failure if they do not receive medical care.

Question 208

How do you determine filtration fraction (FF) if you are given the GFR (which can be inulin rate), renal blood flow (RBF) and Hct?

Answer 208

FF=GFR/[(1-Hct)(RBF)]

Question 209

1. Where does the highest tubular fluid osmolarity occur in the nephron? (High osmolarity = most concentrated fluid)? Note: This question is referring to when there is no secretion of vasopressin.
2. Where does the lowest osmolarity (most dilute fluid) occur when ADH is secreted?

Answer 209

1. Osmolarity around 1200 at the bottom of the loop.

2. Osmolarity about 100 in the distal convoluted tubule.

Question 210

What will you see on electron microscopy if you have hematuria, mild proteinuria and ocassional RBC casts following facial impetigo three months prior on electron microscopy?

Answer 210

Discrete subepithelial humps on EM.

On IF, you will see “lumpy-bumpy” Ig deposits (not linear). These desposits are of IgG and C3.

Question 211

What type of diuretic (class and name) has the toxicity of pulmonary edema.

Answer 211

Osmotic diuretics-mannitol

Question 212

Class of diuretics used to treat edema secondary to heart failure, renal disease or liver disease?

Answer 212

Thiazide diuretics & Loop diuretics (furosemide, bumetanide, ethacrynic acid)

Side effects: Hypokalemia and hypomagnesemia

Question 213

Where do the loop diuretics, like bumetanide, work?

Answer 213

Inhibit NaK2Cl symporters in the ascending loop, so these will be excreted (along with water). Commonly used to treat pulmonary edema.

Hypokalemia, Hypomagnesmia and hypocalcemia are side effects.

Question 214

Triamterene is what kind of diuretic?

Answer 214

Potassium sparing that works in the distal tubule/collecting duct blocking Na+ channels.

Question 215

Characterized by facial edema with massive proteinuria. It is the most common cuase of nephrotic syndrome in children 2-8. It may be associated with respiratory infections, immunization, or atopic disorders (urticaria). Responds to corticosteroid treatment.

1. What is the disease?
2. How would you get a definite diagnosis? (IF, Light microscopy, electron microscopy)

Answer 215

1. Minimal change disease.
2. EM-effacement of foot processes.

Remember that Focal Segmental Glomerular Sclerosis also has effacement of foot processes, but occurs in older people and does not respond to steroid therapy.

Note: NOTHING shows up on IF! No complement or Ig deposits. Weird!

Question 216

Three things that determine the amount of molecules that can cross a semipermeable membrane.

Answer 216

1. Difference of the molecule’s concentration across the membrane.
2. Total membrane surface area
3. Solubility of the substance.

Note: Diffusion is inversely proportional to the total membrane thickness and the molecular weight of the molecule.

Question 217

What happens to the GFR, RPF, and FF in the case of a severely hypovolemic individual with a BP around 90/60?

(Do each go up or down)

Answer 217

RPF: Down a lot (low blood volume)
GFR: Down, but not as much. (B/C the auto regulatory mechanisms kick in (renin and constriction of efferent arterial).

FF is a function of GFR/RPF, so since RPF goes down a lot more than GFR, the FF will increase.

Question 218

What structure is at risk of injury during pelvic surgeries (particularly cervix and hysterectomies)?

Answer 218

Uterine aa. can be accidentally ligated. This would result in hydronephrosis.

Question 219

Acute tubular necrosis has three stages:

Initiation stage: ischemic injury to renal tubules precipitaed by hemorrhage, acute MI, sepsis, surgery, etc.

Maintenance stage: Decreased urine output, fluid overload, increasing creatine/BUN

Recovery Phase: Gradual increase in urine output leading to high-volume diuresis. Electrolyte abnormalities may include decreased concentrations of K, Mg, PO4, and Ca due to slowly recovering tubule function.

What might you see hyperkalemia?

When will you see hypokalemia?

Answer 219

Hyperkalemia: Maintenance stage
Hypokalemia: Recovery stage

During the recovery stage (polyuric phase) of ATN, patients can become dehydrated and can develop severe hypokalemia due to high volume, hypotonoic urine.

Pathognomonic for ATN–>muddy brown casts

Question 220

Man has muscle weakness and periodic headaches. He has a bp of 200/120. He has high renin AND high aldosterone. Where is his tumor?

Answer 220

Juxtaglomerular cells in the kidney (which synthesize and secrete renin)

Question 221

A patient has a transplant and then about three weeks later starts feeling generalized malaise. A histopathologic image of the transplanted kidney shows lymphocytic infiltration of the renal parenchyma. What type of hypersensitivity reaction is this?

Answer 221

Cell-mediated hypersensitivity (Type IV-delayed type)

Question 222

How are Anti-CD3 antibodies (such as muromanab-CD3) useful clinically?

Answer 222

They inhibit T-lymphocytes, which is useful in the treatment of acute rejection in patients with kidney, heart and liver transplants.

Note: CD4+ Th lymphocytes express MHC class II receptor and IL-2 receptors. IL-2 receptor stimulation induces T-lymphocyte proliferation. CD3 is an antigen required for the proper function of T-cell receptors.

Question 223

Why might an individual develope a varacocele or any other type of clot if they have nephrotic syndrome?

Answer 223

They have lost a lot of protein (albumin, antithrombin III, etc). The loss of factors that prevent clotting (antithrombin III allows for clotting to take place).

Question 224

Which occurs in adults versus newborns?

1. Autosomal dominant PKD
2. Autosomal recessive PKd

Answer 224

1. Adults - AD

2. Babies - AR

Question 225

In post glomerular strep glomerulonephritis, you will see subepithelial “humps” on EM. What are these made of?

Answer 225

IgG, IgM and C3

Question 226

Medullary sponge kidney (MSK) is a relatively common and benign congenital disorder characterized by cystic dilatations of the medullary collecting ducts. Cysts usually do not involve the cortex. What is the most common complication of this disease?

Answer 226

Kidney stones

Question 227

You have to know the chart with PAH, creatinine, inulin, urea, Na/K+, HCO3, a.a. and glucose.

Answer 227

N/A

Question 228

Nephrotic syndrome where a child might be slightly puffy and can have proteinuria, but the proteinuria is predominantly albumin. What is this disease?
Is it tubular, functional ,selective, or isolated?

Answer 228

Minimal change disease

Selective proteinuria - albumin!

Question 229

Antibodies detected in the serum that react to the alpha 3 chain of Type IV collagen.

1. What does this patient have?
2. What are the symptoms?

Answer 229

1. Goodpasture syndrome - develop RPGN (rapidly proliferating glomerulonephritis).
2. Hemoptysis (because ab reacts against basement membrane in alveoli)
   Hematuria (reacts against basement membrane in glomerulus), oliguria.

Note: Linear IgG and c3 deposition on IF

Question 230

How might you distinguinish between PSGN and IgA nephropathy?

Answer 230

Post strep glomerulonephritis will occur appx. 3 weeks after the infection. You will see subepithelial humps of IgM, IgG and C3 on EM). Grainy IF.

IgA (which the most common type of nephropathy) will occur TWO TO THREE DAYS after URI. Dx made via detection of IgA deposits in mesangium of glomeruli on IF.

Note: If you have IgA nephropathy with extra renal symptoms (ab pain, purpuric skin leasions), the dx is Henoch-Schonlein purport.

Question 231

A 7-year old boy with 2-day history of colicky ab pain develops bloody stools and his urine appears red, too. PE reveals palpable skin lesions on his buttocks. What additional finding with be most consistent with this patient?

Answer 231

Class signs of Henoch-Schonlein purport (the most common vasculitis in children)

1. GI tract (severe intermittent ab pain)
2. Kidneys (IgA nephropathy)
3. Skin (palpable purpura)
4. Joints: self-limited migratory arthralgias and arthritis most commonly seen in the large joints of the LE.

Question 232

What part of the nephron is normally IMPERMEABLE TO WATER?

Answer 232

Ascending limb of loop

Question 233

Minimal change disease is a nephrotic syndrome where the main protein lost is albumin. What is the current theory of how this occurs.

Answer 233

Loss of negatively charged components in the glomerular basement membrane. The loss of negative particles destroys the negative-negative repulsion between the GBM and albumin.

Question 234

The research division of a large pharm company focuses their efforts on studying renal tubule transport proteins. A substance is identified that specifically and completely inhibits glucose transport in the proximal renal tubules. In a healthy volunteer receiving this substance, glucose clearance will best approximate the clearance of what substance?

Answer 234

Inulin

Question 235

What does this describe:

Easy fatigability, constipation, back pain, and azotemia in an elderly patient. On kidney biopsy, large eosinophilic casts are seen in tubular lumen.

Answer 235

The casts are composed of Bence-Jones proteins.

Multiple Myeloma

Question 236

Crescent formation on light microscopy is diagnostic for rapidly progressing glomerulonephritis. What do crescents consist of?

Answer 236

Glomerular parietal cells, monocytes and macrophages, and abundant FIBRIN.

Note: Crescents eventually become sclerotic, disrupting glomerular function and causing irreversible renal injury.

Question 237

Characterized by a triad of acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia.

Answer 237

HUS (Hemolytic-uremic syndrome), which is a common cause of acute renal failure in children. It is associated with shiga toxin-producing organisms like E. Coli and Shigella dysenteriae.

Question 238

Where does renal cell carcinoma (clear cell type) typically originate?

Answer 238

Proximal renal tubules.

Question 239

What causes diplopia, dystonia, and dysphagia?

Answer 239

Botulinum toxin, which you can get from wounds or home-canned food consumption.

Prevents release from ACh from nerve terminals at the NMJ, thereby preventing muscular contraction.

Question 240

Where are serotonergic (serotonin-releasing) neurons found in the CNS?

What do lesions in these areas cause?

Answer 240

The raphe nuclei of the brainstem (midbrain, pons and medulla). The axons from these cells bodies project widely throughout the CNA to synpase on structure such as the cerebral cortex, thalamus, hypothalamus, cerebellum, hippocampus, and spinal cord.

2. Lesions cause insomnia and depression. These neurons play a role in the sleep-wake cycle and the patient’s level of arousal.

Question 241

The caudate nucleus and the putamen from the striatum in the brain which functions in motor activities. In what disease will there be a loss of cholinergic and GABA-releasing neurons in the striatum?

Answer 241

Huntington’s

Question 242

What do the neurons in the red nucleus, which is located in the anterior midbrain, participate in?

Answer 242

The motor activities of the upper extremities.

Question 243

The nucleus basalis of Meynert houses the cell bodies of cholinergic neurons. In Alzheimer’s disease, these neurons secrete in adequate amount of ______________.

Answer 243

acteycholine.

Question 244

Basic interviewing techniques include facilitation, reflection, confrontation, support, empathy, silence and direct and indirect questioning. What is the primary difference between support and empathy?

Answer 244

Support involves expressing concern independent of understanding. With empathy, the physician expresses understanding and varcarious experiencing of a patient’s situation.

Question 245

A resident is belittled by his attending on rounds who later angrily berates his medical student. What mechanism is this?

Answer 245

Displacement

Question 246

Acute stress disorder and PTSD present with identical symptoms (recurrent nightmares and flashbacks, potential memory loss, and exaggerated startle response). What is the difference between the two?

Answer 246

Acute stress disorder can last no more than 4 weeks. PTSD lasts longer than 4 weeks.

Question 247

what defense mechanism does this describe:

Symbolically nullifying an unacceptable or guilt-provoking thought, idea or feeling (by confession or atonement)

Answer 247

Undoing

Question 248

Mature defense mechanism that converts unacceptable feelings or drive into more socially accepable ones.

I.e. Agressive tendencies, so they join the military.
Mad at wife, so they go run 5 miles

Answer 248

Sublimation

Question 249

Replacement of an unpleasant or unacceptable thought or desire with its opposite.

Homosexual desires, so talk about how homosexuality is wrong.

Answer 249

Reaction formation

Question 250

Mature defense mechanism that alleviates guilty feelings through selfless service or giving.

Answer 250

Altruism

Question 251

Female athlete triad

Answer 251

eating disorder, amenorrhea, and osteoporosis

Question 252

WHat does parotid gland hypertrophy along with anterior tooth enamel erosion demonstrate?

Answer 252

significant vomiting history (perhaps with bulimia/anorexia)

Question 253

Demential, dermatitis and diarrhea

Answer 253

Niacin deficiency

Question 254

DSM-IV criteria

1. Body weight at least 15% below normal and refusal to maintain normal.
2. Amenorrhea for three months.
3. Body image distortion
4. Fear of gaining weight or becoming fat despite being underweight.

Answer 254

Anorexia nervosa

Question 255

Withdrawal from this drug is described as “flu like”.

Includes abdominal pain, nausea, vomiting, diarrhea, piloerection, pupillary DILATION, diarphoresis and fevere.

Answer 255

Opiate withdrawal-heroine for example

Question 256

Withdrawal of this drug:

psychomotor agitiation
psychosis
nystagmus
tachycardia
ataxia
homicidality

Answer 256

PCP-This”withdrawal” is actually reintoxication due to GI tract reabsorption.

Question 257

Drug withdrawal that causes hypersomnolence, fatigue, depression, suicidality and strong cravings

Answer 257

Cocaine

Question 258

PCP, OR PHENCYCLIDINE, is a hallicinogen that works by inhibiting NMDA receptor activation, which is receptor for the excitatory neurotransmitter glutamate. What do moderate amounts of PCP cause in a person?

Answer 258

Feelings of detachment and distance. Additionally, it can produce ataxia, involuntary movements, exaggerated gait, NYSTAGMUS, paranoia and hallucinations (like schizo). Most users become hostile and aggressive.

Question 259

Street drug that blocks the reuptake of dopamine, norepi and serotonin

Answer 259

Cocaine:

Causes high HR, high BP, blurry vision, tremos, twitching, chest pain, irritability, hallucinations and delusions.

Question 260

Illegal drugs that is a sympathomimetic stimulant that mimics the actions of norepi on the body.

Answer 260

Amphetamine

Question 261

Can you give patient information to a loved one if asked?

Answer 261

Not without the explicit consent of the patient. , which should be documented in the patient’s chart.

Question 262

Eating disorder characterized by binge-eating and either restrictive or purging compensatory behaviors.

Answer 262

Bulimia nervosa:

If patient is <85% of ideal BMI, this would be anorexia nervosa instead of bulemia.

Question 263

What does this describe:

mild eurphoria with laughing behavior, slowed reflexes, dizziness, impaired coordination, and short term memory loss.

Rapid heart rate and CONJUNCTIVAL INJECTION are the two most immediate symptoms.

Answer 263

Marijuana use.

Remains in tissues for a significant amount of time and can be detected up to 30 days after use.

Question 264

SSRI inhibitor (like paroxetine) is considered a first line therapy for both generalized anxiety disorder and panic disorder.

However, SSRI’s take about 4 weeks to notice any therapeutic effect. Benzos can be given in the interim. There are short, intermediate and long-term benzos. Which category do each of the fall into?

Chlordiazapoxide
Diazepam
Triazolam
Loarazepm
Fluazerpam

Answer 264

Short: Traizolam

Intermiediate: Lorazepam

Long: Diazepam, Flurazepam, and Chlordiazepoxide

**Triazolam is a benzo that can be useful for treating insomnia in patients employed in a mission-critical positions, as its short half-life minimizes undesirable daytime side effects.

Question 265

Major depression is diagnosed when at least 5 of the following symptoms are present for at least two weeks. (one of them must be depressed mood or anhedonia (inability to find pleasure in things you used to enjoy). What are the list of symptoms?

Answer 265

SIGECAPS

Sleep disorder
Interest deficit
Guilt
Energy deficit
Concentration deficit
Appetite Disorder
Psychomotor retardation or agitation
Suicidality

Question 266

Dysthmic disorder is a chronic, low-intensity mood disorder that responds well to antidepressant medications. Its symptoms are less severe than those of major depressive disorder. How long must the symptoms be present for a diagnosis to be made?

Answer 266

at least 2 years.

Question 267

Unconscious manifestation of neurologic symptoms when pathophysciological explanations for the symptoms cannot be found. it occurs more commonly in women and often occurs after a significant life stress.

Answer 267

Conversion disorder

Question 268

Causes of erectile dysfunction:

Answer 268

Psychogenic stressors (marital problems)
Performance anxiety or depression
Medications (SSRIs and sympathetic blockers like clonidine, methydopa and beta blockers)
Vascular or neurological impairment (would not get morning wood)
Genitourinary trauma

Question 269

What is the difference between suppression and repression as defense mechanisms?

Answer 269

Suppression: intentional withholding of distressing unconscious material. Mature defense mechanism.

Repression: Unconscious removal of disturbing psychological material from conscious awareness. (i.e. adult who was sexually abused as a child and does not even remember until they see a movie involving sexual abuse).

Question 270

If you suspect elder abuse, you get ask anyone else to leave the room and ask waht three questions?

Answer 270

1. Do you feel safe where you live?
2. Who prepares your meals?
3. Who handles your checkbook.

Note: Clinicians have ethical and moral obligations to report elder abuse, neglect and exploitation. If there is reason to suspect abuse or neglect, the patient should be interviewed alone to avoid intimidation by possible abusers.

Question 271

Defense mechanism:

Unconscious belief that people are either wholly good or wholly bad.

Answer 271

Splitting

borderlines have this alot.

Question 272

A worker accusing his boss of having sexual desires fro him when the worker himself has the sexual desire.

Answer 272

Projection

Question 273

Defense mechanism:

Former smoker vigorously enforcing a smoking ban.

Answer 273

Reaction formation.

Question 274

Defense mechanism

A patient with sexually explicit thoughts becoming a sex therapist.

Answer 274

Sublimation

Question 275

Throwing a tantrum

Answer 275

Acting out

Question 276

Suppressing one’s feelings by thinking about the problem. Example: a surgeon who avoids his feeling of failure after a patient dies by thinking about every step of the surgery in meticulous detail.

Answer 276

Intellectualization.

Question 277

Delirium vs. Dementia

Onset-
Consciousness
Course:
Prognosis
Memory impairment

Answer 277

DELIUM
onset:  Acute
Consciouness:  impaired 
Course:  Fluctuating
Prognosis:  Reversible
Memorty impairment:  Global

DEMENTIA:
onset:  gradual
Consciouness:  Intact
Course:  Progressive decline (think Alz)
Prognosis:  irreversible
Memory:  remote memory spared.

Question 278

In an emergency situation, if there is any doubt int he clinician’s mind concerning the wishes of a patient who is Jehovah’s Witness, what the best course of action?

Answer 278

Treat according to accepted standard of care. In an emergency, the physician should ALWAYS provide potentially life-saving therapy to a minor.

Question 279

In a non-emergent setting, what do you do if there is a language barrier with a patient (either foreign language or patient is deaf)?

Answer 279

Always used a trained, objective interpreter!

No friends, no writing things down, no checklist, etc.

Question 280

What is the most common medical cause of daytime sleepiness in the U.S?

Answer 280

Obstructive sleep apnea: occurs due to poor oropharyngeal tone and results in daytime sleepiness, MORNING HEADACHES, and DEPRESSION.

Question 281

A 54 year old man with insulin-dependent diabetes mellitus visits his physician for a routine fu. His HbA1c is 11%, way above his goal. Although the patient is resistant to change his regimen, the physician increases his dose of insulin and insists on tighter diet control. For the next several months, the patient either arrives late for his appointments or cancels them. What defense mechanism is he exhibiting.

Answer 281

Passive aggressive behavior: expressing hostility toward his physician in a passive manner by missing his appointments.

Question 282

Where would you find the enzyme elastase in the lungs?

Answer 282

Macrophage lysosomes and in the large, azurophil (or primary) granules of neutrophils.

Normally, elastase release from infiltrating neutrophils and macrophages is balanced by the antielastase activity of serum alpha1 antitypsin. Excess elastase activity in lung acini is thought to be a major factor in the development of centriacinar and panacinar emphysema.

Question 283

Constitute over 95% of the epithelial lining of the alveoli. These end-differentiated squamous cells not known to be a major source of alveolar fluid secretory products.

Answer 283

Type I Pneumocytes

Question 284

Dipalmitoyl phosphtidycholine. What is it?

Answer 284

Phospholipid that is secreted from TYPE II pneumocytes. It is the major component of pulmonary surfactant.

Question 285

What type of cells are the germ cells in the alveoli of the lung?

Answer 285

Type II pneumocytes

Question 286

Where are clara cells found?

Answer 286

Terminal respiratory epithelium.

They are not affiliated, secret Tory constituents of the terminal respiratory epithelium. They secrete Clara cell secretory protein (CSSP), which inhibits neutrophil recruitment and activation.

Question 287

Do bronchioles of the lung have goblet cells?

Answer 287

No, bronchioles terminal bronchioles respiratory bronchioles and lung acini generally lack goblet cells.

Question 288

What drug increases the peripheral metabolism of levodopa and decreases its effectiveness?

Answer 288

Vitamin B6

Question 289

Cimetidine is a histamine-2 receptor antagonist that decreases hydrochloric gastric acid production. What can submitted Dean cause?

Answer 289

Gynecomastia, and this is a very frequently asked USMLE step one question.

Question 290

Most patients with hyperthyroidism in the United States are treated with ablating doses of radioactive iodine. The concentration of iodine levels with in the thyroid gland is much higher compared to anywhere else in the body because thyroid follicles transport inorganic iodide against a concentration gradient. What drugs do you want to make sure and not use prior to ablating with iodide because they will decrease the effects of therapy by competitive inhibition.

Answer 290

Anion inhibitors (perchlorate and pertechnetate). These drugs block ioside absorption by thyroid gland via competitive inhibition.

Question 291

How do drugs like methimazole and propylthiouracil treat hyperthyroidism?

Answer 291

These thionamides inhibit thyroid peroxidase.

Question 292

What drug is known to cause Ebstein’s anomaly in infants exposed in utero?

Answer 292

Lithium, which is a common drug used to treat bipolar disorder.

Epstein’s anomaly includes apical displacement of the tricuspid valve leaflets, decreased right ventricular volume, and atrialization of the right ventricle.

Question 293

There are three phases that classically stimulate acid secretion within the stomach. Cephalic, gastric, and intestinal.

What stimulates the cephalic and gastric phases?

Answer 293

The cephalic phase is mediated primarily by cholinergic and vagal mechanisms, and is triggered by the thought, sight, smell, and taste of food. The gastric phase is mediated by the presence of gastrin, which stimulates histamine secretion and therefore indirectly acid secretion, and his triggered by the chemical stimulus of food and distention of the stomach.

Note: The intestinal phase is initiated when protein containing food enters the duodenum, but this phase plays only a minor role of stimulating gastric acid. In fact, intestinal influences are effective in down regulating gastric acid secretion after a meal.

Question 294

And gastric function, the ileum and colon-released peptide YY, which binds to receptors on the endocrine cells described as enterochromaffin like cell which counteracts be cephalic and gastric phases of acid secretion by inhibiting gastrin stimulated histamine release from ECL. What other factors inhibit acid secretion?

Answer 294

Somatostatin and prostaglandins

Question 295

83 month old male undergoes surgery for a sublingual math that seems to impair his breathing. The event is brought to the ER several weeks after the surgery with lethargy, breathing problems, constipation, and dry skin. what embryologic process failed?

Answer 295

Migration.

Failure of migration of the thyroid gland can called lingual thyroid. Surgeons should be careful when removing any mass along that thyroglossal duct usual path, as the mass could be the only thyroid tissue present in the patient.

Question 296

The classical complement pathway can be activated after C1 by either IgM or IgG. Which one is more effective at activating complement, and why?

Answer 296

IgM, because it circulates in pentameric form, whereas IgG circulates in monomeric form. The C1 module binds Fc portion of the heavy immunoglobulin chain in the region near the hinge point.

Question 297

What will the levels of serum calcium and parathyroid hormone be like in an individual here is in renal failure?

Answer 297

Serum calcium will be low, and parathyroid hormone will be high.

Deficiency of 1-alpha hydroxylase in the kidney will prevent Vitamin D from getting it its active form (1, 25-dihydroxy Vitamin D). So, low calcium. The compensatory response is high PTH.

Question 298

What neurotransmitter is implicated in opioid tolerance?

Answer 298

Gluatmate: binds and activates NMDA receptors, which can phosphoyrlate opioid receptors, including NO levels that ultimately leads to opioid tolerance.

Question 299

What nerve roots are associated with saddle anesthesia (weakness in butt and perianal area), as well as loss of reflex around the ass pipe?

Answer 299

S2-S4

Question 300

Gastric cancer tends to metastasize to one of three different areas. What are those?

Answer 300

Ovaries: Krukenberg tumor (mucus producing cell in the ovary)

Sister Mary Joseph nodule: periumbilical region

Virchow’s node: left supraclavicular sentinel node

Question 301

Where are the are the osmoreceptors and baroreceptors that will activate the synthesis of ADH?

Answer 301

Osmoreceptors: Hypothalamic nuclei
Baroreceptors: carotids and atria

Note: If there is damage to these areas, you will get central diabetes insipidus. If you give the person exogenous ADH, their urine mOsm/L will go up. If it is nephrogenic DI (the kidney doesn’t respond to ADH in the collecting duct), the mOsm/L will NOT go up. This is how you differentiate between the two.

Question 302

What adrenergic receptor causes constriction of the peripheral vasculature?

What adrenergic receptor causes vasodilation of the peripheral vasculature?

Answer 302

1. Vasoconstriction: alpha-1
2. Vasodilation: beta-2

Note:

Alpha-a also causes contraction of the internal urethral sphincter of the bladder, and contraction of pupillary dilator muscle (mydraisis-or dilation)

Beta-2 also causes bronchodilitation of the bronchi, and uterus relaxation.

Question 303

What adrenergic receptor inhibits uterine contractions?

Answer 303

Beta-2 (tocolysis of uterine)

Question 304

What adrenergic receptor causes contraction of the ocular pupillary dilator muscle, resulting in pupillary dilation?

Answer 304

Alpha-1

Question 305

Langhans giant cells are characteristic of the caseating granulomas associated with M. Tuberculosis infection and have multiple nuclei preferably organized in the shape of a horseshoe. What are these granulomas composed of?

Answer 305

Macrophages, which are activated by CD4+ Th1 lymphocytes.

Question 306

What is the primary purpose of CD8+ T lymphocytes?

Answer 306

Orchestrate the death of virally infected cells.

Question 307

Radiation therapy is often used for cancer. How does radiation therapy shrink cancer tumors? Two ways.

Answer 307

Induces DNA damage through DNA double-stand fractions.

Formation of free radicals.

Question 308

Polycythemia vera presents with dizziness, headaches, and Proadis after showering. What is the most likely cause of polycythemia vera?

Answer 308

Increased bone marrow sensitivity to growth factors (erythropoietin and thrombopoietin) due to a JAK2 mutation

Question 309

Rate limiting enzyme in the urea cycle?

Answer 309

Carbamoyl phosphate synthestase I (CPSI), which is activated by N-acetylgultamte.

Question 310

Where do the nitrogen atoms in the urea molecule from? (2 places?

Answer 310

NH3 and aspartate

Question 311

What is a major risk of neisseria gonorrhea and chlamydia?

Answer 311

Infertility. Both of these bacteria can cause pelvic inflammatory disease which can lead to scarring of the fallopian tubes.

Question 312

Where is ribosomal RNA synthesized?

Answer 312

In the nucleolus

Question 313

If a patient in diabetic ketoacidosis has facial pain, headache, and black necrotic eschar in the nasal cavity:

1. What do ou need to do to diagnose it?
2. What is the likely diagnosis?
3. How do you treat?

Answer 313

1. Mucosal biopsy
2. Mucormycosis-will see broad nonseptate hype with right angle branching.
3. Surgery and Amphotericin B

Question 314

WHat does this describe:

Neontal defects of the head (microcephaly, MR)
Eyes (cataracts)
Ears (Deafness)
Heart/CV (patient ductus arterioles, peripheral pulmonic stenosis

Answer 314

Congenital rubella syndrome.

To decrease the risk of this syndrome, the CDC currently recommends the vaccination of children and NON-PREGNANT females of childbearing age with live, attenuated rubella virus vaccine.

Question 315

A 20-year-old male is evaluated for trimmer and gait instability. Laboratory studies revealed elevated serum transaminases. A serum ceruloplasmin concentration decreased what medication do you use to treat this patient?

Answer 315

Penicillamine.

This is Wilson’s Disease, which involves an excess of non-ceruloplasmin-bound serum copper, leading to injurious accumulation of this element in the liver, CNA lenticular nucleus and cornea. Chelation therapy with penicillamine is indicated.

Question 316

Rubella typically presents with a low-grade fever and maculopapular rash. The rash spreads from the face to the chest to the trunk and extremities overtime. Usually over 48 hours. If a woman is pregnant, what is she at risk for developing? And what is the fetus at risk for developing?

Answer 316

Mom: polyarthralgia

Baby: deafness, cataracts and cardiac malformation.

Question 317

In the lungs, what happens to perfusion as you move from the apex to the base of the lungs? What happens to ventilation as you move from the apex to the base?

Answer 317

Think: Gravity

Perfusion (Q) increases A LOT.
Ventilation (V) increases A LITTLE.

For this reason V/Q ratio DECREASES from apex to base.

Question 318

What drugs will induce P450?

What drugs will inhibit P450?

Answer 318

Inducers of P450: Barbituates, RIFAMPIN, CARBAMAZEPINE, GRISEOFULVIN, CHRONIC ALCOHOL

Inhibitors: Isoniazid, cimetidine, macrolides, azole antifungals, grapefruit juice.

Question 319

Hydralazine and minoxidil are drugs used to prevent severe hypertension that is resistant to other drugs.

What is its mechanism of action and side effects?

Answer 319

Mechanism: Arteriolar vasodilation

Side effect: because there is significant vasodiation in the arterioles, the baroreceptor mediated activation of the sympathetic system is activated. This results in increased heart rate, increased renin activity, resulting in SODIUM AND FLUID RETENTION!

Question 320

Renal cell carcinoma (both hereditary and sporadic) are associated with this mutation.

Answer 320

VHL-There are deletions of the VHL gene on chromosome 3p.`

Question 321

Retinoblastoma and Osteosarcoma mutation association

Answer 321

RB anti-oncogene-Chromosome 13

Question 322

Dark, intraellular inclusions that stain with crystal violet?

Answer 322

Heinz bodies–>G6PD deficiency–>usually becomes known after a stressor (such as malaria prophylactics)

Question 323

What does this describe:

Hypogonadism
Eunuchoid habitus (underdeveloped sexual organs where sexual hormones are not produced)
Small firm testes
Genotype 47 XXY

Answer 323

Klinefelter syndrome

Note: You will have LOW serum testosterone and LOW sperm count, but you will have high LH and FSH due to loss of feedback inhibition.

Question 324

Severe retrosternal pain that radiates to the back is the classic presentation of an aortic dissection. What is thought to be the initiating process in a dissecting aortic aneurysm?

Answer 324

Intimal tear.

Question 325

A patient comes in with flulike symptoms or chronic pneumonia. Tissue samples showed spherules containing endospores. What is your diagnosis?

Answer 325

Coccidiodes immitus

Question 326

Fungus that commonly causes this pulmonary disease in immunocompromised patients. In tissue specimens, it is seen as septate hyphae that branch at 45° angles.

Answer 326

Aspergillus fumigatus

Question 327

Round yeast with broad based budding.

Answer 327

Blastomyces

Question 328

Why do individuals with Crohn’s disease develop gallstones?

Answer 328

Decreased bile acid reabsorption in the terminal ilieum and its loss via feces increases the lithogenecity of bile. Cholesterol precipitates informs gallstones.

Question 329

What is the most common hepatic neoplasia?

Answer 329

Metastatic liver cancer from either the breast, lung, or colon. This is 20 times more frequent than hepatocellular carcinoma. Note that with metastases, the liver will have multiple nodules throughout. Even with significant metastatic involvement, patients may have no clinical or laboratory signs suggestive of hepatic insufficiency until their cancer gets really bad.`

Question 330

What are the risk factors of hepatocellular carcinoma?

Answer 330

Histories of cirrhosis and for infection with hepatitis B or C.

Question 331

Niemann pick disease is an autosomal recessive disorder that affects patients in their first year of life. What enzyme is deficient in this disease that causes foamy histiocytes?

Answer 331

Sphingomyelinase-causes abnormal accumulations of sphingomylin in phagocytes.

Question 332

Autosomal recessive condition in which a deficiency of the enzyme B-hexosaminidase A causes Gm2 ganglioside to accumulate within neurons

Answer 332

Tay-Sachs

Question 333

834-year-old male is found to have an intracranial tumor. Many cells within the tumor demonstrate outdated uptake of bromodeoxyuridine, a thymidine analogue. The finding most likely suggest that the tumor has what characteristic?

Answer 333

High grade: these tumors are displaying cellular and nuclear pleomrophism and a high number of mitoses. the high uptake indicates there a lot of cells in the S cycle getting ready to divide.

Note: Staging of brain tumors is not that important because these tumors rarely go outside of the brain AND even a tiny tumor can compress a vital center and be lethal.

Question 334

What class of drug does this describe:

Causes hepatocytes to increase their LDL receptor density as ameans of increasing their uptake of circulating LDL

Answer 334

Statins

Question 335

Testes drains to what lyphh nodes?

Scrotum to what lymph nodes?

Answer 335

Testes: para-aortic nodes

Scrotum: superficial inguinal lymph nodes

Question 336

What disorder:

• Recurrent skin and resp infection
• Light skin and silvery hair
• Horizontal nystagmus
• Peripheral blood smear demonstrates GIANT CYTOPLASMIC GRANULES in neutrophils and monocytes.

Answer 336

Chediak-Higashi Syndrome.

Defect in neurtrophil phagosome lysosome fusion.

Question 337

Hormone responsible for gallbladder contraction that is made in the duodenum and jejunum in response to fatty acids and amino acids.

Answer 337

Cholecystokinin (CCK)

Question 338

How does decreased levels of CCK cause biliary sotnes?

Answer 338

CCK is responsible for gallbladder contractility. So, if there is no contractility, there is stasis. Stasis=stones.

Question 339

What hormones does somatostatin inhibit?

Answer 339

secretin, CCK, glucagon, insulin and gastrin

Question 340

Why does an individual become “lactose intolerant” after a battle with a stomach bug (like rotavirus)?

Answer 340

The lactase enzyme (B-galactosidase) is concentrated in the brush border of the epithelial cells of the small intestine. If these cells are lost, then there is a lactase deficiency. Remember lactose = galacto syl B-1,4-glucose)

Question 341

Baby has not been feeling well, has mild jaundice, an enlarged tongue, general hypotonia, and umbilical hernia.

Answer 341

Hypothyroidism: FLOPPY BABY.

Need to treat stat or baby is going to develop profound and irreversible MR.

Question 342

Mousy body odor, fair skin MR and developmental delay

Answer 342

PKU (deficiency of phenylalanine hydroxyase that leads to inability to metabolize phenylalanine.

Question 343

What type of drugs are best to deal with the negative symptoms (decrease in emotional range, poverty of speech, loss of interest in living) of schizophrenia?

Answer 343

Atypical (2nd gen) neuroleptics (clozapine, risperidone, olanzapine, quetiapine). These are ATYPICAL antipsychotics and improve both positive and negative symptoms of schizophrena.

Question 344

Birth defect caused by valproate?

Answer 344

Meningocele or neural tube defect. Valproate inhibits intestinal folic acid absorption.

Question 345

Major virulence factor of strep pyogenes. It inhibits phagocytosis and complement activation, mediates bacterial adherence, and the target of type-specific humoral immunity to S. pyogens.

Answer 345

Protein M

Question 346

_________________ is a glucose sensor within pancreatic beta cells. Inactivating mutations of the enzyme result in mild hyperglycemia that can be exacerbated by pregnancy.

Answer 346

Glucokinase

Question 347

Injury to what cranial nerves will make a person unable to tolerate everyday sounds?

Answer 347

VII

Stapedium muscle is innervated by the stapedius nerve, which is a branch of the facial nerve.

Question 348

What the typical method of contracting tetanus?

Answer 348

Wound–>retrograde transport up motor neuron where it causes inhibition of inhibitory interneurons and unregulated firing of primary motor neurons.

Clostridium tetani is responsible for tetanus, a toxin-mediated disease that causes uncontrolled muscle spasms and respiratory failure.

Question 349

What virus takes this route:

wound—>neuron axons—>salivary glands

Answer 349

Rabies virus.

Question 350

Food—>systemic circulation—>meninges.

What bacteria takes this route?

Answer 350

Listeria monocytogenes: Gram + food born pathogen that causes meningitis in neonates and immunocompromised patients.

Question 351

What is the best drug to use for patients with both absence and associated tonic-clonic seizures?

Answer 351

Valproate for BOTH.

If it is just absence seizures alone, use ethosuximide.

Question 352

How is depolarization (phase 0) of a cardiac pacemaker cell different from a non-pacemaker cell (cardiomyocytes and purkinje fibers)

Answer 352

Pacemaker: depolarization is due to Ca2+ rushing in.

Non-pacemaker: depolarizaiton is due to inward sodium current.

Question 353

What kind of staining uses aniline dye (carbolfuchsin? What type of organisms does this stain?

Answer 353

Acid-fast stain: Used to stain organisms that have mycolic acid present in their cells walls.

It stains mycobacterium and some nocardia species.

Question 354

On gram + or gram - bacteria:

Teichoic acid
LPS

Answer 354

1. Teichoic acid: Gram + (antigenic target for human immune system and antigenic determinant for organism identification in the lab)
2. LPS: gram -: component of out cell envelope

Question 355

Why do patients with tetraology of fallot squat during episodes of severe cyanosis?

Answer 355

This decreases the right to left shunt (squatting increases the total systemic vascular resistance).

Question 356

What side effect might the combination of statins + fibrates (atorvastatin and gemfibrozil) cause?

Answer 356

Myopathy. Note that stain use is also associated with hepatoxicity.

Question 357

The combination of fibric acid derviatives (anticholesterol drug) and bile acid resins (cholestyramine) cause?

Answer 357

Gallstones (increased cholesterol int he gallbladder)

Question 358

Most common infectious agents in neonatal meningitis?

Answer 358

Group B strep, E. coli and Listeria monocytogenes

Question 359

Most common infectious agent that causes meningitis in children and teens?

Answer 359

Neisseria meningitis

Goes in through nose, gets in blood, then goes into meninges.

Question 360

What is the MCC of meningitis in adults and elderly?

Answer 360

Strep pneumoniae

Question 361

Why is it highly encouraged to get children vaccinated against H. influenza?

Answer 361

It can cause meningitis in nonvaccinated infants.

Question 362

Most common virus that causes meningitis?

Answer 362

Coxsacievirus (children: fecal-oral)

Question 363

Who gets a fungal meningitis?

Answer 363

Immunocompromised people.

Question 364

Where do you do a LP?

Answer 364

Between L4 and L5 (spinal cord ends at L2, but CSF goes down to S2)

Question 365

What kind of meningitis?

-Neurophils with decrease CSF glucose

Answer 365

Bacterial:

Group B strep, e.coli, listeria (neonates)
N. meningitis (children)
Strep pneumoniae (elderly and adults)
H. influenza (non vaccinated infants)

Question 366

What kind of meningitis:

In CNS
Lymphocytes and normal glucose

Answer 366

Viral

Question 367

What kind of meningitis
In CNS

-Lymphocytes and low glucose

Answer 367

Fungal

Question 368

A left side mass compressing the renal vein will most like cause what?

Answer 368

Varicocele (b/c the testicular vein drains into the left renal renal vein ). Note: On right side, test. vein drains into the inferior vena cava, so a mass on that side would not cause varicocele.

Question 369

If you have overuse of diuretics, are you more likely to be acidotic or alkalotic?

Answer 369

In alkalosis.

Diuretics cause Na+ and water reabsorption at the expense of K+ and H+, which will be excreted.

Question 370

What do the following indicate:

1. Bleeding time
2. aPTT (activated partial thromboplastin time)
3. Prothrombin time

Answer 370

1. Bleeding time: platelet hemostatic function.
2. aPTT: Defect in INTRINSIC coagulation system.
3. PT: Indicates defect in EXTRINSIC system at a step not shared with the intrinsic system (Factor VII)

Question 371

WHat do these chemicals have in common?

Leukotriene B4, H-HETE (the leukotriend precursor, complement component C5a and IL-8?

Answer 371

They all induce chemotaxis and phagocytosis in neutrophils.

Note: IL-8 is released by macrophages.

Question 372

In a neonate, this triad is associated with what?

1. Hydrocephalus
2. Intracranial calcification
3. Chorioretinitis

Answer 372

Toxplasmosis (in utero infection because it is transmitted transplacentally).

Expecting mothers should avoid cat feces to help prevent exposure to toxoplasma.

Question 373

Sullfhydryl group supplementation (N-acetyl cystein) can be used to treat what kind of pharmaceutical toxicity?

Answer 373

Acetaminophen

N-acetyl cysteine (NAC) acts as a glutathione substitute and binds to the toxic metabolite. NAC also provides sulfhydryl groups to enhance the non-toxic sulfation elimination of acetominaphen.

Note: In therapeutic doses, tylenol is metabolized by the liver and glucoronide conjugation. When this system is overwhelmed, more goes to the c-P$%) pathway and a toxic metabolite is formed (NAPQI). This can cause hepatocellar injury and centrilobular necrosis.

Question 374

Patient eats fish and then goes to ED complaining of weakenss, dizziness, and paresthesias involving his face and extremities. Muscle strength and deep-tendon reflexes are decreased on neurological exam.

What is going on?

Answer 374

Pufferfish–>Tetrodotoxin

Tetrodoxtoxin binds to voltage-gated sodium channels in nerve and cardiac tissue, preventing sodium influx and DEPOLARIZATION.

Question 375

GM2 ganglioside metabolites accumulated in system.

Cherry red spot in choroid.

Baby

What is the disorder?

Answer 375

Tay-Sacs

Deficiency in B-hexosaminidase A that results int he accumulation of the cell membrane glycolipid GM2 ganglioside within cell lysosomes.

Autosomal recessive

Question 376

Enzyme deficiency is sphingomyelinase, so you get accumulation of spingomyelin. Is autosomal recessive.

You will see hepatosplenomegaly, red spot in macula, foam cells, progressive neurodegeration.

Answer 376

Neiman-Pick Disease

Question 377

X-linked recessive disorder causes by a deficiency in alpha-galactosidase.

Physical signs and symptoms include angiokeratomas, cardiac and renal involvement and pain neuropathy.

Answer 377

Fabry disease

Question 378

Macrophages that look like crumbled paper, hepatosplenomegaly, pancytopenia, severe bone and joint pain.

What is the disease and what is the deficient enzyme?

Answer 378

Gaucher disease (the macrophages are called gaucher cells).

B-glucocerbrosidase

Question 379

If you see an MRI of an HIV patient’s brain and you see multiple ring-enhancing lesions, along with oral thrush and lymphadenopathy, what is the diagnosis?

Answer 379

Toxoplasmosis.

Question 380

Blind as a bat, mad as a hatter, red as a beet, hot as a hair, dry as a bone, the bowel and bladder lose their tone, the heart runs alone.

What kind of toxicity does this describe and how would you counteract it?

Answer 380

Atropine (antimuscarinc drug)

To counteract, you need to increase the concentration of ACh in the synaptic cleft. This can be done by a cholinesterase inhibitor. PHYOSTIGMINE counteracts atropine poisoning.

Question 381

What do deficiency of the complement factors that form the membrane complex (C5b-C9) cause recurrent infections of?

Answer 381

Neisseria species.

Remember, N can cause bacterial meningitis (common in college dorms). Present with high fevers, chills, altered mentaiton, petechial skin rash from Neisseria-induced small vessel vasculitis (especially affecting palms and soles) and ultimately septic shock

TREATMENT: Ceftriaxone for at least 2 weeks.

Question 382

Absence of what would cause recurrent viral and fungal infections?

Answer 382

T-cells.

Remember DiGeorge–>T-cell dysfunction due to lack of thymus (3rd and 4th pharyngeal arch did not form properly). Also no parathyroid glands and facial abnormalities with DiGeorge)

Question 383

These people suffer current infections with catalase-positive organisms (like staph)

Answer 383

Chronic granulomatous disease (X-linked and results from inability of phagocytes to nynthesize NADPH oxidase, which is neceesary of the lysosomal oxidative burst).

Question 384

N. meinigitidis has pili. What are these used for?

Answer 384

Epithelial attachment of the bacteria to the nasopharynx.

Question 385

Trigeminal nerve questions:

1. Where does CNV2 (maxillary nerve) exit the skull?
2. Where does CNV3 (mandibular nerve) exit the skull?

Answer 385

1. V2: Foramen rotundum
2. V3: Foramen ovale (V3 innervates the muscle of mastication–>massester, medial and lateral pterygoids, and temporalis muscles).

Question 386

If you have a patient who shows classical signs of meningitis (fever, headache, photophobia, and painful extraocular movements), but the CSF has viral RNA, what can you assume?

Answer 386

90% of viral menigitis cases are from enterovirus infection, so they will get the infection via the fecal-oral route.

Note: Enteroviruses are coxsackieviruses, echoviruses, and polioviruses. Note: poliovirus can cause LMN injury in addition to meningitis, especially in non-immunized individuals from endemic regions.

Question 387

A 24-year old male is evaluated for chronic headaches and visual changes. An intracranial calcified mass in detected on MRI. The mass is surgically removed. Gross inspection shows cystic spaces filled iwth thick brownish fluid that is rich in cholesterol. The mass most likely shares a common origin with which of the following?

Answer 387

Craniopharyngiomas-calcified cystic tumors

Arise from remnants of rathke’s pouch, which is the embryonic precursor of the anterior pituitary. The commonly cause headaches, growth failure and bitemporal hemianopia.

Question 388

You are inspecting a swimmer’s ear for external ear infection. While inspecting, you insert a speculum into the external auditory meatus in close contact with its posterior wall. The patient faints during the procedure. What the hell happened?

Answer 388

Vasovagal syncope: stimulated vagal nerve and parasympathetic outflow via the nerve (CN X) leads to decreased HR and blood pressure.

Note: The rest of the external auditory canal (including the external portion of the TM) is innervated by V3 (mandibular). The inner surface of the TM is innervated via glossopharyngeal nerve (CN IX) via its tympanic branch.

Question 389

What carries afferent taste fibers tot he anterior 2/3 of the tongue as well as efferent parasympathetic preganglionic fibers that will ultimately innervate submandibular and sublingual salivary glands?

Answer 389

Facial Nerve (VII).

Question 390

Mousy body odor and fair skin = PKU

What amino acid becomes essential in this case?

Answer 390

tyrosine.

PKU patients can not synthesize tyrosine from phenylalanine.

Question 391

Patient has acidosis and a high methylamalonic acid level. The enzyme that converts methylmalonc acid is not working, thus not converting it to succinyl coA so that it can enter the TCA cycle.

What does the enzyme do?

Answer 391

Methylmalonyl CoA is an isomer of Succinyl CoA, so the enzyme isomerizes the molecule.

Question 392

Bilateral hemorrhagic destruction of both adrenals can be caused by?

Answer 392

N. Meningitis (can present with DIC and destruction of both adrenals)–>Waterhouse-Friderichsen syndome.)

Question 393

Claw hand deformity:

Ape hand deformity:

Wrist drop:

Answer 393

Claw hand: Ulnar nerve (and anesthesia over medial 1.5 digits).

Ape hand: Median nerve

Wrist drop: Radial nerve

Question 394

Beta-endorphin (an endogenous opioid peptide) is derived from proopiomelanocortin (POMC). This molecule is also an enzyme is also a precursor for what molecules?

Answer 394

ACTH and MSH:

The fact that beta-endorphin and ACTH are derived from the same precursor suggest htat there may be a close physiological relationship between the stress axis and the opoid system.

Question 395

A baby who has hydrocephaly will present with irritability, poor feeding, increased head circumference and enlarged ventricles on CT. What do you see long-term, hypotonicity or hypertonicity of the muscles?

Answer 395

Hypertonicity-because there is upper motor neuron damage that results from stretching of the periventricular pyramidal tracts.

Question 396

What the first line of treatment for the following type of seizure:

Brief arrhythmic jerking movements that last

Answer 396

Valproic acid is used to treat myoclonic seizures.

Question 397

First line drugs for the following type of seizure:

Generalized tonic extension of the extremitites, followed by clonic rhythmic movements; loss of consciousness and prolonged postictal confusion.

Answer 397

Tonic-clonic seizure:

Treated with:

1. Phenytoin
2. Carbamazepine
3. Valproate

Question 398

First line treatment for both of these types of seizures:

1. One body part is involved; no loss of consciousness and no post-ictal state are present.
2. Almost always temporal lobe (mood changes, illusions, hallucinations); imparied consciouness and postictal state present.

Answer 398

These are:

1. Simple partial
2. Complex partial

Both treated with carbamazepine.

Note: Aplastic anemia is a dreaded (but rare) side effect.

Question 399

How do you treat the following type of seizures?

Brief episodes of staring, but no postictal confusion?

Answer 399

Absence seizure

1st line: treat with ethosuximde
2nd line: treat with valproate (if person has absence along with tonic-clonic or myoclonic, treat with valproate.)

Question 400

A 3-year-old male is hospitalized with prgressive spastic paresis of his lower extremities and choreoathetoid movements. Comprehensive laboratory testing reveals very high arginine levels in both the child’s plasma and the CSF. THe enzyme deficient in this patient is normally involved in the production of what?

Answer 400

Urea: Patient has arginase deficiency.

Arginase in an enzyme in the urea cycle that produces urea and ornitine from arginine. This deficiency is underdiagnosed because it looks like cerebral palsy.

Question 401

If a patient has a major surgical procedure and then has an increase bp, increased HR, and fever and muscle rigidity after the surgery (along with skin mottling), what do you need to do for that patient to improve his condition?

Answer 401

Give him a drug that will inhibit intracellular calcium release in skeletal muscles. (DANTROLENE)

Malignant hyperthemia is syndrome that occurs due to hypersensitivity of skeletal muscles due to inhalation of anethetics (like halothane) and muscle relaxant succinylcholine. (It is an autosomal dominant trait causing a defect of ryanodine receptors on the surface of the sarcoplasmic reticulum, which is a calcium channel, causing the receptor to release A LOT of calcium).

Question 402

How do the drugs physostigmine, neostigmine, myridostigmine and edrophonium used to treat myasthenia gravis?

Answer 402

Inhibit acetylcholinesterase degradation in the cholinergic synapses.

Question 403

What is the mechanism of action for the following drugs:

Atrophine, scopolamine, ipratropium

Answer 403

Cholinergic antagonists that bind to receptors to prevent their interaction with acetylcholine.

Question 404

How do you treat pheochromacytoma?

Answer 404

alpha-adrenergic antagonists (phentolamine and phenoxybenzamine) which inhibit alpha-receptor mediated sympathetic activity.

Question 405

Jaw pain that starts in the middle of a meal is characteristic for temporal arteritis. What should you do if temporal arteritis is suspected?

Answer 405

Get ESR–>it is uniformly elevated in patients with temporal arteritis. Then do biopsy. If confirmed, start corticosteroids immediately to prevent possible vision loss.

Question 406

What is the drug primidone (antiepileptic drug) metabolized to?

Answer 406

Phenobarbital and phenylethymalanoamide. (All three are active anticonvulsants)

Question 407

What would causes bilateral wedge-shaped bands of necrosis over the cererbral convexity, a few centimeters lateral to the interhemistpheric tissue?

Answer 407

Global cerebral ischemia.

These watershed area are at risk during hypoxemia of the brain.

Question 408

Cystic tumor in the cerebellum.

Well differentiated neoplasm comprised of SPINDLE CELLS with HAIR-LIKE GLIAL PROCESSES, associated with microcysts. These cells are mixed with rosenthal fibers and granular eosinophilic bodies. Waht type of lesion is it?

Answer 408

Pilocytic astrocytoma.

Note: A cystic tumor of the cerebellum of a child is most likely pilocytic astrocytoma.

Question 409

Ragged red muscle fibers are seen in what type of disease?

Answer 409

Mitochondrial.

Remember, only women pass on the mitochondria, so if a guy has the disease, there is a 0% chance he will pass to his offspring.

Question 410

First line treatment for both of these types of seizures:

1. One body part is involved; no loss of consciousness and no post-ictal state are present.
2. Almost always temporal lobe (mood changes, illusions, hallucinations); imparied consciouness and postictal state present.

Answer 410

These are:

1. Simple partial
2. Complex partial

Both treated with carbamazepine.

Note: Aplastic anemia is a dreaded (but rare) side effect.

Question 411

How do you treat the following type of seizures?

Brief episodes of staring, but no postictal confusion?

Answer 411

Absence seizure

1st line: treat with ethosuximde
2nd line: treat with valproate (if person has absence along with tonic-clonic or myoclonic, treat with valproate.)

Question 412

A 3-year-old male is hospitalized with prgressive spastic paresis of his lower extremities and choreoathetoid movements. Comprehensive laboratory testing reveals very high arginine levels in both the child’s plasma and the CSF. THe enzyme deficient in this patient is normally involved in the production of what?

Answer 412

Urea: Patient has arginase deficiency.

Arginase in an enzyme in the urea cycle that produces urea and ornitine from arginine. This deficiency is underdiagnosed because it looks like cerebral palsy.

Question 413

If a patient has a major surgical procedure and then has an increase bp, increased HR, and fever and muscle rigidity after the surgery (along with skin mottling), what do you need to do for that patient to improve his condition?

Answer 413

Give him a drug that will inhibit intracellular calcium release in skeletal muscles. (DANTROLENE)

Malignant hyperthemia is syndrome that occurs due to hypersensitivity of skeletal muscles due to inhalation of anethetics (like halothane) and muscle relaxant succinylcholine. (It is an autosomal dominant trait causing a defect of ryanodine receptors on the surface of the sarcoplasmic reticulum, which is a calcium channel, causing the receptor to release A LOT of calcium).

Question 414

How do the drugs physostigmine, neostigmine, myridostigmine and edrophonium used to treat myasthenia gravis?

Answer 414

Inhibit acetylcholinesterase degradation in the cholinergic synapses.

Question 415

Claw hand deformity:

Ape hand deformity:

Wrist drop:

Answer 415

Claw hand: Ulnar nerve (and anesthesia over medial 1.5 digits).

Ape hand: Median nerve

Wrist drop: Radial nerve

Question 416

Beta-endorphin (an endogenous opioid peptide) is derived from proopiomelanocortin (POMC). This molecule is also an enzyme is also a precursor for what molecules?

Answer 416

ACTH and MSH:

The fact that beta-endorphin and ACTH are derived from the same precursor suggest htat there may be a close physiological relationship between the stress axis and the opoid system.

Question 417

A baby who has hydrocephaly will present with irritability, poor feeding, increased head circumference and enlarged ventricles on CT. What do you see long-term, hypotonicity or hypertonicity of the muscles?

Answer 417

Hypertonicity-because there is upper motor neuron damage that results from stretching of the periventricular pyramidal tracts.

Question 418

What the first line of treatment for the following type of seizure:

Brief arrhythmic jerking movements that last

Answer 418

Valproic acid is used to treat myoclonic seizures.

Question 419

First line drugs for the following type of seizure:

Generalized tonic extension of the extremitites, followed by clonic rhythmic movements; loss of consciousness and prolonged postictal confusion.

Answer 419

Tonic-clonic seizure:

Treated with:

1. Phenytoin
2. Carbamazepine
3. Valproate

Question 420

First line treatment for both of these types of seizures:

1. One body part is involved; no loss of consciousness and no post-ictal state are present.
2. Almost always temporal lobe (mood changes, illusions, hallucinations); imparied consciouness and postictal state present.

Answer 420

These are:

1. Simple partial
2. Complex partial

Both treated with carbamazepine.

Note: Aplastic anemia is a dreaded (but rare) side effect.

Question 421

How do you treat the following type of seizures?

Brief episodes of staring, but no postictal confusion?

Answer 421

Absence seizure

1st line: treat with ethosuximde
2nd line: treat with valproate (if person has absence along with tonic-clonic or myoclonic, treat with valproate.)

Question 422

A 3-year-old male is hospitalized with prgressive spastic paresis of his lower extremities and choreoathetoid movements. Comprehensive laboratory testing reveals very high arginine levels in both the child’s plasma and the CSF. THe enzyme deficient in this patient is normally involved in the production of what?

Answer 422

Urea: Patient has arginase deficiency.

Arginase in an enzyme in the urea cycle that produces urea and ornitine from arginine. This deficiency is underdiagnosed because it looks like cerebral palsy.

Question 423

If a patient has a major surgical procedure and then has an increase bp, increased HR, and fever and muscle rigidity after the surgery (along with skin mottling), what do you need to do for that patient to improve his condition?

Answer 423

Give him a drug that will inhibit intracellular calcium release in skeletal muscles. (DANTROLENE)

Malignant hyperthemia is syndrome that occurs due to hypersensitivity of skeletal muscles due to inhalation of anethetics (like halothane) and muscle relaxant succinylcholine. (It is an autosomal dominant trait causing a defect of ryanodine receptors on the surface of the sarcoplasmic reticulum, which is a calcium channel, causing the receptor to release A LOT of calcium).

Question 424

How do the drugs physostigmine, neostigmine, myridostigmine and edrophonium used to treat myasthenia gravis?

Answer 424

Inhibit acetylcholinesterase degradation in the cholinergic synapses.

Question 425

Claw hand deformity:

Ape hand deformity:

Wrist drop:

Answer 425

Claw hand: Ulnar nerve (and anesthesia over medial 1.5 digits).

Ape hand: Median nerve

Wrist drop: Radial nerve

Question 426

Beta-endorphin (an endogenous opioid peptide) is derived from proopiomelanocortin (POMC). This molecule is also an enzyme is also a precursor for what molecules?

Answer 426

ACTH and MSH:

The fact that beta-endorphin and ACTH are derived from the same precursor suggest htat there may be a close physiological relationship between the stress axis and the opoid system.

Question 427

A baby who has hydrocephaly will present with irritability, poor feeding, increased head circumference and enlarged ventricles on CT. What do you see long-term, hypotonicity or hypertonicity of the muscles?

Answer 427

Hypertonicity-because there is upper motor neuron damage that results from stretching of the periventricular pyramidal tracts.

Question 428

What the first line of treatment for the following type of seizure:

Brief arrhythmic jerking movements that last

Answer 428

Valproic acid is used to treat myoclonic seizures.

Question 429

First line drugs for the following type of seizure:

Generalized tonic extension of the extremitites, followed by clonic rhythmic movements; loss of consciousness and prolonged postictal confusion.

Answer 429

Tonic-clonic seizure:

Treated with:

1. Phenytoin
2. Carbamazepine
3. Valproate

Question 430

First line treatment for both of these types of seizures:

1. One body part is involved; no loss of consciousness and no post-ictal state are present.
2. Almost always temporal lobe (mood changes, illusions, hallucinations); imparied consciouness and postictal state present.

Answer 430

These are:

1. Simple partial
2. Complex partial

Both treated with carbamazepine.

Note: Aplastic anemia is a dreaded (but rare) side effect.

Question 431

How do you treat the following type of seizures?

Brief episodes of staring, but no postictal confusion?

Answer 431

Absence seizure

1st line: treat with ethosuximde
2nd line: treat with valproate (if person has absence along with tonic-clonic or myoclonic, treat with valproate.)

Question 432

A 3-year-old male is hospitalized with prgressive spastic paresis of his lower extremities and choreoathetoid movements. Comprehensive laboratory testing reveals very high arginine levels in both the child’s plasma and the CSF. THe enzyme deficient in this patient is normally involved in the production of what?

Answer 432

Urea: Patient has arginase deficiency.

Arginase in an enzyme in the urea cycle that produces urea and ornitine from arginine. This deficiency is underdiagnosed because it looks like cerebral palsy.

Question 433

If a patient has a major surgical procedure and then has an increase bp, increased HR, and fever and muscle rigidity after the surgery (along with skin mottling), what do you need to do for that patient to improve his condition?

Answer 433

Give him a drug that will inhibit intracellular calcium release in skeletal muscles. (DANTROLENE)

Malignant hyperthemia is syndrome that occurs due to hypersensitivity of skeletal muscles due to inhalation of anethetics (like halothane) and muscle relaxant succinylcholine. (It is an autosomal dominant trait causing a defect of ryanodine receptors on the surface of the sarcoplasmic reticulum, which is a calcium channel, causing the receptor to release A LOT of calcium).

Question 434

How do the drugs physostigmine, neostigmine, myridostigmine and edrophonium used to treat myasthenia gravis?

Answer 434

Inhibit acetylcholinesterase degradation in the cholinergic synapses.

Question 435

How do you treat pheochromacytoma?

Answer 435

alpha-adrenergic antagonists (phentolamine and phenoxybenzamine) which inhibit alpha-receptor mediated sympathetic activity.

Question 436

Jaw pain that starts in the middle of a meal is characteristic for temporal arteritis. What should you do if temporal arteritis is suspected?

Answer 436

Get ESR–>it is uniformly elevated in patients with temporal arteritis. Then do biopsy. If confirmed, start corticosteroids immediately to prevent possible vision loss.

Question 437

What is the drug primidone (antiepileptic drug) metabolized to?

Answer 437

Phenobarbital and phenylethymalanoamide. (All three are active anticonvulsants)

Question 438

What would causes bilateral wedge-shaped bands of necrosis over the cererbral convexity, a few centimeters lateral to the interhemistpheric tissue?

Answer 438

Global cerebral ischemia.

These watershed area are at risk during hypoxemia of the brain.

Question 439

Cystic tumor in the cerebellum.

Well differentiated neoplasm comprised of SPINDLE CELLS with HAIR-LIKE GLIAL PROCESSES, associated with microcysts. These cells are mixed with rosenthal fibers and granular eosinophilic bodies. Waht type of lesion is it?

Answer 439

Pilocytic astrocytoma.

Note: A cystic tumor of the cerebellum of a child is most likely pilocytic astrocytoma.

Question 440

Ragged red muscle fibers are seen in what type of disease?

Answer 440

Mitochondrial.

Remember, only women pass on the mitochondria, so if a guy has the disease, there is a 0% chance he will pass to his offspring.