UW 2/8-2/14

Question 1

Why do athletic women miss periods?

Answer 1

Reduced Leptin levels inhibit pulsatile GnRH

Question 2

A fetal autopsy reveals fused cerebral hemispheres with an absent forebrain fissure and a single intracranial ventricle. What is the disease? What is the mechanism of these findings?

Answer 2

Disease: Holoprosencephaly
(may have proboscis)

Mechanism: Developmental Field Defect

Question 3

What is an Association Defect? Give some examples

Answer 3

Multiple anomalies without a known cause that occur together frequently.

Examples VACTERL
Vertebral defects.
Anal Atresia
Cardiac Defects
Tracheoesophageal fistulas
Renal Anomalies
Limb Abnormalities

Question 4

What is a deformation? Give examples

Answer 4

Fetal structural anomalies due to extrinsic mechanical forces.

Low amniotic fluid levels can cause bone growth abnormalities.

Question 5

What is Dysplasia with regards to development? Give an example of dysplasia.

Answer 5

Abnormal organization of cellular architecture in a tissue.

OI- Defective type 1 collagen

Question 6

What is the treatment for improperly controlled asthma (SABA use currently)?

How can you prevent the major side effect of ICS?

Answer 6

Long acting inhaled cortical steroids.

Oral rinsing and using a spacer on the inhaler help prevent ORAL CANDIDIASIS

Question 7

What are the symptoms of a Manic Episode?

Answer 7

DIGFAST
Distractibility
Impulsivity/Indiscression
Grandiosity
Flight of Ideas
Activity Inc
Sleep Dec
Talkativeness

Question 8

Can a patient be Bipolar 1 without major depressive episodes?

Answer 8

YES

Question 9

How do you differentiate Brief Psychotic Disorder from Bipolar I with Psychotic features?

Answer 9

Prominent mood symptoms (DIGFAST) occur more in Bipolar I, not BPD.

Question 10

What is schizophreniform disorder? How is it different from schizophrenia and Brief Psychotic Disorder?

Answer 10

Schizophreniform disorder lasts 1-6 months. It shares features of BPD and schizophrenia (psychosis, disturbed behavior, decline in function.)

BPD is less than 1 month
-phrenia is more than 6 months

Question 11

Male Pattern Baldness has what kind of inheritance pattern? What are a couple other notable examples of diseases with the same inheritance?

Answer 11

Polygenic

it depends on androgens, and other key genes.

Ischemic Heart Disease
Diabetes
Epilepsy

Question 12

Why is a single shot of hCG given to people trying to conceive? What is administered before hCG?

Answer 12

It mimics the LH surge. Menotropins (FSH analogs)

Question 13

How do fibrates work? What other treatment has similar MOA?

Answer 13

They activate PPAR-Alpha and LPL activity.
This primarily lowers Triglyceride levels.

Fish oil dec VLDL production.

Question 14

Why do mycobacteria grow as “serpentine” cords on enriched media?

Answer 14

Due to cord factor (virulence), a mycoside.

Cord factor inhibits neutrophils, mitochondrial destruction and induced release of TNF.

Question 15

What 3 paracrine signals stimulate Gastric Acid Secretion?

Answer 15

Histamine (from ECL cells)
Gastrin (G Cells)
AcH (Vagus Nerve)

Question 16

A man has fatigue, rashes, flushing and abdominal cramps for a few months. His rash gets worse in a hot shower and with itching. He has clusters of mast cells on biopsy. What is going on?

Answer 16

Histamine secretion due to mast cell proliferation (SYSTEMIC MASTOCYTOSIS).

In addition to these symptoms a patient would also have increased gastric acid secretion as histamine is secreted by ECL cells to stimulate GA.

Question 17

Which disease is associated with excessive mast cell proliferation and increased histamine release?

Answer 17

Systemic Mastocytosis. Usually have mutated KIT gene

Question 18

What is Hemaglobin C?

Answer 18

Mutation in B-globin chain that causes glutamate to be replaced by lysine.

Question 19

What is the most common cause of pericarditis causing a holosystolic murmur in young kids?

Answer 19

Group A Strep–> Acute Rheumatic Fever.

Question 20

What is a Aschoff body and for which disease is it pathognomonic?

Answer 20

It is an interstitial myocardial granuloma (lymphocytes and macrophages in myocardium)–> Acute Rheumatic fever.

Question 21

How can you tell the difference between viral and bacterial myocarditis caused by ARF?

Answer 21

Viral myocarditis lacks Aschoff bodies

Question 22

What are the hallmarks of Pure Red Cell Aplasia and what is the pathogenesis of it?

Answer 22

Hypoplasia of Erythroid cells with normal granulopoesis and thrombopoesis.

Associated/Caused by THYMOMAS, lymphocytic leukemias and B19 Parvovirus

Question 23

Do Renal Cell Carcinomas selectively suppress erythroid precursors in bone marrow?

Answer 23

No.

Question 24

What do Entacapone and Tolcapone do in the setting of parkinsons? Which is Central acting, which is peripheral?

Answer 24

Inhibit Catechol-O-Methyl Transferase (which degrades L-Dopa)

Entacapone is peripheral only
Tolcapone is both central and peripheral.

Question 25

What are 3 drugs that prevent the breakdown of L-Dopa?

Answer 25

Carbidopa (dopa decarboxylase inhibitor) Entacapone ( COMT in hibitor) Tolcapone (COMT)

Question 26

Which drug used in the treatment of Parkinsons increases endogenous dopamine effect?

Answer 26

Amantadine (inc synth/release dec reuptake)

Question 27

Which nerves enter the orbit through the Superior Orbital Fissure?

Answer 27

III, IV, V1, VI The Superior ophthalmic vein also runs here. V2 and V3 enter via the Foramen Rotundum and Ovale respectively.

Question 28

What do IL 1-6 do?

Answer 28

``` 1-Fever 2-Stims T Cell 3- Stim Bone marrow 4- IgE switch 5- IgA 6- aKute phase reactants Hot T-Bone stEAK ```

Question 29

What does interferon Gamma do?

Answer 29

Stimulates Macrophages (from helper T cells)

Question 30

What is the main vascular concern for pts with marfans? What gene is defective?

Answer 30

Aortic Aneurism | Fibrillin-1 (AD inheritance)

Question 31

Which connective tissue disease is associated with Berry Aneurisms?

Answer 31

Ehlers-Danlos (type 3 collagen disorder)

Question 32

What is Myxomatous degeneration

Answer 32

Pathologic weakening of connective tissues

Question 33

What are the effects of CFTR mutation on Respiratory/intestinal epithelia, and sweat glands?

Answer 33

Respiratory/intestinal: Decreased Cl- SECRETION into lumen Sweat: Decreased ABSORPTION of Cl back to body. (body doesn't want to lose all its salt)

Question 34

What two types of infections can lead to poststreptococcal glomerulonephritis? What is seen on immunochemistry staining?

Answer 34

Streptococcal infections of SKIN or pharynx. Think of impetigo. Lumpy bumpy immune complexes (IgG, IgM, C3) on the epithelial side of the BM.

Question 35

What is the histological finding of Rapidly progressive glomerulonephritis?

Answer 35

Fibrin deposition

Question 36

What is the classic histological findings of Diabetic nephropathy?

Answer 36

GBM thickening and Kimmelstiel-Wilson Nodules (due to Hyaline deposition)

Question 37

Rapidly progressive glomerulonephritis may be caused by deposition of Anti GBM-IgG antibodies. What is the name of this syndrome and what kind of hypersensitivity reaction is it?

Answer 37

Goodpasture's Type II hypersensitivity *differentiate between II vs III= 2- is Tissue specific 3= is free to roam body.

Question 38

What is pancreatic divisum? What are the origins of the structures involved?

Answer 38

Main pancreatic duct fails to form. It is derived from the Ventral pancreatic bud. The Dorsal bud forms the majority of pancreatic tissue including MOST of the head.

Question 39

What type of RBC forms with a pyruvate kinase deficiency? Why is splenomegaly seen in pts with this?

Answer 39

Echinocyte (urchin like) RBCs-->inc hemolysis by splenic reticuloendothelial cells-->Red Pulp hyperplasia

Question 40

When does passive splenic congestion occur?

Answer 40

In the setting of portal hypertention or anything that backs up blood.

Question 41

Track the descent of the testes through the abdominal wall to the scrotum. Name the structures it passes and their origins:

Answer 41

Deep Inguinal Ring (transversealis fascia) Inguinal Canal: many things Superficial inguinal ring (external oblique apo) Scrotum

Question 42

What CD cell markers are present on reed sternburg cells?

Answer 42

CD15 and 30. 2x15=30 Hodgkins lymphoma (owl eye cells)

Question 43

What cells have CD 16?

Answer 43

NK, neutrophils, and macrophages 16 year olds are innate killers.

Question 44

Describe the Hawthorne effect with reguards to studies.

Answer 44

Children playing together during an experiment begin to share more once they learn they are being observed.

Question 45

What is Berkson's Bias?

Answer 45

Selection bias created by selecting hospital patients.

Question 46

What is the Pygmalion effect?

Answer 46

Researchers belief in the efficacy of intervention can affect outcome. ie Randomly selected Gifted and Talented students perform better on exams because teachers help them more unconsciously.

Question 47

How does Mumps present?

Answer 47

Fever Malaise, headaches, myalgias--> *Parotid gland swelling* within 48 hours.

Question 48

What is an L/S ratio and when would you expect it to be 2:1 or higher?

Answer 48

L/S is the Lecithin (aka phosphatidylcholine) to sphingomyelin ratio. When lung is maturing the utero (at around 35 weeks) the ratio is 2:1 or higher.

Question 49

What is the presentation of a Krukenburg tumor? What other ovarian tumor may be bilateral?

Answer 49

Early satiety, gastric thickening on CT, adenexal masses (most often bilateral!!!)

Question 50

The following histological findings are indicative of which kinds of cancer? A. Bilobed nuclei and inclusion-like nucleoli B. Intercellular Bridges and Keratin pearls C. Mucin secreting signet cells D. Epithelial Cells with abundant clear cytoplasm E. Sebaceous glands and keratinaceous debris

Answer 50

A. Hodgkin Lymphoma (these are reed-sternburg cells) B. Squamous Cell Cancer C. Primary gastric Cancer (signet cells are full of mucus with nucleus pushed to the side. D. Renal Cell Carcinoma, (Clear cell type) E. Teratoma (hair and skin glands)

Question 51

Which type of hypersensitivity reaction is Poststreptococcal GN classified as? Why? How is it different from Goodpastures?

Answer 51

It is a Type III HS reaction. M-protein complexes with IgG antibodies and DEPOSITS in basement membrane. Goodpastures (TII rxn) has autoantibodies against the BM.

Question 52

A patient is given a card full of different potential allergens. It is taped to his skin and he is asked to return to the office in 2 days. After his return he has some rashes under certain samples. What is happening? What type of hypersensitivity is this?

Answer 52

Contact Dermatitis Type 4 HS. Foreign molecules detected by Antigen Presenting cells which stimulate T cells.

Question 53

How do you think of Type II vs III hypersensitivity reactions in general?

Answer 53

Type II is 2-issue specific | Type III is general.

Question 54

Which sex chromosome disorder is associated with Macroorchidism, large jaw, and intellectual disability? Inheritance pattern?

Answer 54

Pts with Fragile X. "Big Balls mean not enough X chromosome" X linked Dominant

Question 55

Which genetic disorder is characterized by short stature, hypotonia, intellectual disability, hyperphagia and obesity?

Answer 55

Prader Willi Matt Prader is the fattest kid on the football team. Defect in Paternal gene 15q 11-13.

Question 56

How can you tell the difference between a clue cell and a koilocyte? Which infections do each represent?

Answer 56

Clue Cells have Rod shaped bacteria adhering to their cell membrane. -Gardnella Vag. Koilocyte:Dense irregular staining cytoplasm. Raisin shaped nuclei. HPV

Question 57

What is the action of interferon gamma?

Answer 57

Inc MCH I and II on antigen presenting cells.

Question 58

What are the signs of organophosphate poisioning?

Answer 58

``` Inc Parasympathetic Activity DUMBELSS Diarrhea Urination Miosis Bronchospasms/Bradycardia Excitation of skeletal muscle Lacrimation Sweating/Salivation ```

Question 59

What is the difference between suppression and repression in psychology?

Answer 59

Supression is a MATURE defense, where someone decides to not let something bother them. Repression is when thoughts/actions are suppressed subconsciously (ie woman doesn't want sex bc she was violently raped as a kid)

Question 60

Give an example of reaction formation.

Answer 60

A person worried about failing step 1 is overly confident in his interactions with others.

Question 61

What does a 99MTc-pechnetate test look for?

Answer 61

Gastric Parietal Cells. Used to ID atopic gastric mucosa such as a meckel diverticulum.

Question 62

What are the 3 main causes of hypoglycemia in Diabetic type I patients?

Answer 62

Insulin OD, Dec food intake, Exercise.

Question 63

What are somethings that can increase blood sugar levels other than food intake?

Answer 63

Infection, Sleep depravaiton, pain/stress All trigger cortisol/catecholamine release.