Still going... Flashcards
(121 cards)
1
Q
anterograde and retrograde microtubular transport?
A
anterograde - kinesin retrograde - dynein
Both are ATP dependent microtubular proteins
2
Q
renal agenesis vs duodenal/esophageal atresia
A
renal agenesis –> no urine production –> oligohydramnios –> potter’s sequence atresia –> no swallow amniotic fluid –> polyhydramnios
3
Q
elevated ACE and calcium in an African American woman with dyspnea?
A
sarcoidosis –> sarcoid granulomas produce ACE and active vitamin D
4
Q
predominant lymphocyte found in sarcoid granulomas?
A
CD4+ T helper cells
5
Q
bronchial veins drain where? O2 effect?
A
mostly to the left heart via the pulmonary veins –> decreases O2 sat of newly oxygenated blood going to systemic circulation
6
Q
carotid sinus baroreceptor signal carried by?
A
glossopharyngeal nerve (Hering nerve, a branch of CN IX)
7
Q
aortic arch baroreceptor signal carried by?
A
efferent limb of vagus nerve (CN X)
8
Q
atrial natriuretic peptide and brain natriuretic peptide both act via? What else does this? Effect prolonged by?
A
ANP receptor –> activate guanylyl cyclase –> cGMP Nitric oxide also activates cGMP cGMP effect is prolonged by phosphodiesterase inhibitors (like sildenafil)
9
Q
MEN 2B associations?
A
Medullary thyroid cancer Mucosal neuromas/marfanoid habitus Pheochromocytoma (remember the triangle)
10
Q
MEN 2A associations?
A
Medullary thyroid cancer Parathyroid hyperplasia Pheochromocytoma (remember the square)
11
Q
Distinguish MEN 2A from MEN 2B?
A
Both have medullary thyroid cancer and pheochromocytoma 2A –> parathyroid hyperplasia 2B –> mucosal neuromas
12
Q
MEN 1 associations?
A
Pituitary tumors (prolacinomas) Primary hyperparathyroid Pancreatic tumors (gastrinomas) (remember the diamond)
13
Q
only MEN with head involvement?
A
MEN 1 –> pituitary tumors
14
Q
only MEN with pancreatic involvement?
A
MEN 1 –> gastrinomas
15
Q
Patient with history of thyroidectomy now has an endocrine tumor?
A
MEN 2A or MEN 2B – thyroidectomy was probably for medullary thyroid cancer
16
Q
only blood supply to femoral head? Problem?
A
medial circumflex artery –> osteonecrosis in hip fracture
17
Q
brown stones in gall bladder means?
A
biliary tract infection (E. coli, liver fluke – beta glucoronidases)
18
Q
black stones in gall bladder means?
A
Chronic hemolysis (sickle cell, spherocytosis, beta thalassemia) –> increased enterohepatic cycling of bilirubin (ileal disease)
19
Q
human epidermal growth factor receptor 2 (HER2) is what kind of receptor?
A
tyrosine kinase
20
Q
Trastuzumab MoA?
A
binds HER2 receptor (tyrosine kinase receptor) –> downregulates cellular proliferation and promotes apoptosis.
21
Q
swelling and/or erythema on the palms and soles of a young child of Asian descent? Also see?
A
Kawasaki disease (they won’t come out and say rash on hands and feet). Also see conjunctival injection, red/cracking tongue and lips, fever, centripetally-spreading urticarial rash, cervical lymphadenopathy
22
Q
Streptomycin is a member of which family of antibiotics?
A
Aminoglycosides (not macrolides) –> binds 30S ribosome
23
Q
Cause of virion genetic instability?
A
No proofreading, probably in the 3’ -> 5’ virion-encoded RNA polymerase
24
Q
Negative feedback on Thyrotropin Releasing Hormone?
A
T3
25
Negative feedback on Thyroid Stimulating Hormone?
T3
26
Descrobe T4/T3?
T4 (thyroxine) released from thyroid gland in response to TSH, converted to T3 peripherally. T3 (triiodothyronine) causes most physiological effects
27
Recognized hematologic complication in renal transplant?
Distal ureteral ischemia, shows up as leaking urine 5-10 days after transplant. Renal artery supplies new kidney, but blood flow to distal ureter is sparse.
28
Proton pump inhibitors can lead to osteoporosis how?
Decreased acidity, decreased absorption of calcium. Watch out for this in an older female with PUD.
29
Corneal reflex is carried by?
V (sensory, afferent) and VII (motor, efferent)
30
Very first step in atherosclerosis? Next step?
Pathogenesis begins with damage to endothelial cells (smoking, diabetes, hyperlipidemia, homocysteine, etc). Next step is monocyte and lymphocyte adhesion and intimal migration.
31
Which is more important for tumor staging and prognosis? Depth of invasion or absolute tumor size?
Invasion. A small tumor that invades deeper layers has a poorer prognosis than a large tumor that is confined to the epithelial layer (carcinoma in situ). So the "T" in TNM staging must consider depth of invasion, not just absolute size.
32
Sickle cell treatment and MoA?
Hydroxyurea --\> promotes production of hemoglobin F (i.e., 2 alpha, 2 gamma)
33
Respectable gardener-type person suddenly seems to suffer sympathetic overstimulation? Remedy?
Jimson weed poisoning --\> belladonna alkaloids possess strong anticholinergic properties. Use physostigmine, a cholinesterase inhibitor
34
Describe NF-kappaB? Chron's tie-in?
proinflammatory transcription factor that increases cytokine production. NOD2 mutation results in decreased activity of NF-kappaB protein with reduced cytokine production, impairs innate barrier function of the intestinal mucosa and allows intestinal microbes to induce an exaggerated response by the adaptive immune system.
35
Congenital bilateral absence of the vas deferens?
Cystic fibrosis - CFTR mutations are likely responsible for abnormal development of Wolffian structures. Note that sperm production is normal, but sperm cannot be secreted (azoospermia).
36
Right gastric artery comes from?
Celiac trunk --\> common hepatic --\> hepatic --\> RG
37
Left gastric artery comes from?
Celiac trunk (anastomoses with right gastric along lesser cuvature of stomach)
38
electron micrograph of a sarcomere - identify the Z line?
The darkest line on the micrograph - this is where actin filaments (thin filaments) bind via structural proteins
39
surgeon notices cyanosis and mottling of an organ during a procedure?
Hyperacute rejection due to preformed IgG antibodies withing the recipient that are directed against donor antigens --\> suggests anti-ABO blood group and anti-HLA antibodies
40
"Dry tap" suggestive of?
Marrow fibrosis, maybe hairy cell leukemia (also see lymphocytes with cytoplasmic projections.
41
Aspiring professional athlete has some sort of rash in skin-exposed areas?
Consider anabolic steroid use (i.e., methyltestosterone); androgens promote both follicular epidermal hyperproliferation and excessive sebum production (comedonal and inflammatory nodular eruptions)
42
Acute onset heart failure in the setting of recent respiratory or viral infection?
Raise suspicion for dilated cardiomyopathy (viral myocarditis, Coxsackie B)
43
Patient has a specific phobia (heights, spiders, etc.). Treatment?
Treat with behavioral therapy, maybe add a short-acting benzo acutely
44
infant on formula doesn't tolerate galactose? missing enzyme?
galactosemia - caused by impaired galactose-1-phosphate metabolism; could be any of three enzymes missing, but classically it's galactose 1-phosphate uridyl transferase
45
lont-term problem of unmanaged galactosemia?
galactitol accumulation --\> irreversible eye and liver damage.
46
Benzo effect on chloride channels?
Increase frequency of opening
47
What drug increases the duration of time a chloride channel is open?
Barbiturates
48
Name three treatments for C diff infection?
1) Metronidazole 2) Oral vancomycin 3) Fidaxomicin -\> new (2011) macrocyclic abx that inhibitis the sigma subunit of RNA polymerase, used for recurrent CDI
49
Someone has an extra rib?
Cervical rib - pokes out through the scalene muscles - can contribute to problems with brachial plexus, thoracic outlet syndrome, etc.
50
First and second line treatments for status epilepticus? If that doesn't work?
Benzodiazepine (Lorazepam), then phenytoin, often simultaneous administration; If seizure does not stop, phenobarbital is indicated, or general anesthesia.
51
Etanercept indication and MoA?
Can use as adjunctive therapy with methotrexate for RA. Reduces the biological activity of TNF-alpha by acting as a decoy receptor.
52
-nib suffix means?
kinase inhibitor
53
-cept suffix means?
receptor molecule
54
-mab suffix means?
monoclonal antibody
55
What lies between the head of the caudate nucleus and the globus pallidus and putamen? Other nearby structure you should identify in a cross-section?
Internal capsule. Caudate nucleus is superior. Also look for the thalamus, which is inferior to the caudate nucleus and medial to the putamen and globus palladus, immediately adjacent to the third ventricle. Third ventricle is in the middle, lateral ventricles above, the whole thing looks like the face of a goat.
56
Treatment when you notice Warfarin skin necrosis?
Discontinue Warfarin (duh) and administer fresh frozen plasma (vitamin K injection too slow)
57
Newborn stops nursing after a couple days, vomits green stuff? What else will you find?
Cystic fibrosis. Bowel obstruction with a green inspisated mass in the distal ileum points to meconium ileus --\> very specific finding for CF. X-ray will show air-fluid levels, small bowel dilation consistent with obstruction.
58
Gardener's knee? Housemaid's knee?
Inflammation of the prepatellar bursa from repetitive pressure
59
Sodium channel blocker effect on QT interval?
None - this is all Calcium and Potassium channel territory.
60
light microscopy of liver biopsy specimen shows Easter eggs in the glomerulus?
Those are Kimmerlstiel-Wilson nodules, diagnostic for nodular glomerulosclerosis. This is likely a function of diabetic nephropathy --\> glomerular basement membrane thickening and increased mesangial matrix deposition.
61
Teratogenic effect of valproate?
Neural tube defects, such as myelomeningocele
62
Teratogenic effect of phenytoin?
Neural tube defects, orofacial clefts, microcephaly, nail or digit hypoplasia
63
Teratogenic effect of Warfarin?
Nasal hypoplasia, stippled epiphysis
64
Teratogenic effect of methotrexate?
Neural tube defects, limb and craniofacial abnormalities, abortion
65
What medications might contribute to neural tube defect in a developing fetus?
Methotrexate, phenytoin, valproate
66
Teratogenic effect of lithium?
Ebstein anomaly (tricuspid valve malformation), nephrogenic DI (just like in mom, maybe), and hypothyroidism
67
Antibodies sensitive to RA? Specific?
Autoantibodies against the Fc portion of IgG (Rheumatoid Factor) are sensitive, but not specific to RA (about 80%) Autoantibodies against cyclic citrullinated peptide (anti-CCP antibodies) are specific to RA (95%-98%)
68
Laboratory finding in menopause? Why?
Elevated FSH - ovaries are no longer responding, no inhibin feedback
69
Normal language at 2 years?
50+ words, 2-word phrases
70
Age to identify colors? Speech intelligibility?
4 years - 100% intelligible speech
71
Most commonly delayed milestone?
Language - 10%-15% prevalence at age 2. Most catch up in preschool (prevalence falls to 5% after age 3)
72
Drug-induced Parkinsonianism caused by? Treated by? Definitely not a treatment?
Dopamine (D2) receptor blockade in the nigrostriatal pathway. Reduce or discontinue drug, or coadminister a centrally acting antimuscarinic agent (trihexyphenidyl or benztropine). Do not use levodopa --\> precipitates psychosis in these patients.
73
Brain gross histology finding in Wilson's Disease?
Cystic degeneration of the putaman, as well as damage to other basal ganglia structures. On cross-section, the damaged area is lateral to the GP, and inferiolateral to the caudate.
74
Bilateral periorbital edema and dark urine in a high school athlete?
Psych! Poststreptococcal glomerulonephritis. Athlete has nothing to do with it, except to make you think about rhabdomyolysis. Check the CK, prolly normal (60-174)
75
Normal CK levels?
Creatine Kinase 60-174
76
Tricky medication to prevent gynecomastia in a gentleman being treated for prostate cancer?
The treatment for prostate cancer is androgen deprivation therapy (GnRH agonist, androgen receptor inhibitors). Blocking this enzyme results in excess estrogen production by aromatase. Block the effect of estrogen on the breast with an estrogen receptor blocker, like Tamoxifen!
77
What happens to left ventricular diastolic pressure if both the mitral and aortic valves are involved with Rheumatic Fever?
Increased pressure. Diastolic pressure is normal or even decreased if only the mitral valve is involved.
78
TNF-alpha inhibitors are great for some things, except...
TNF-alpha is necessary for effective sequestration of mycobacteria within granulomas. TNF-alpha inhibitors promote reactivation of latent TB and can incrase the risk of disseminated disease. Get a TB test (PPD/IGRA) prior to starting treatment with these agents.
79
Cell types derived from the neural crests?
MOTEL PASS: melanocytes, odontoblasts, tracheal cartilage, enterochromaffin cells, laryngeal cartilage, parafollicular cells of the thyroid, adrenal medulla, and all ganglia, Schwann cells, and spiral membrane. Yeah, I thought that, too.
80
What signalling molecule is the most important mediator of sepsis? Two more that are involved?
tumor necrosis factor alpha (TNF-alpha), an acute phase cytokine produced by activated macrophages. IL-1 and IL-6 are also involved in systemic inflammatory response.
81
Long-term risk in patients with psoriasis?
Deforming joint disease, polyarthritis
82
Mechanism to reduce ICP in a comatose patient when you hyperventilate them?
The idea is that decreasing pCO2 causes vasoconstriction, effectively increasing vascular resistance and reducing cerebral blood flow. This is how ICP will drop. Watch out for trick answer of decreased cerebral perfusion pressure - lowering systemic blood pressure would actually do the trick, but hyperventilation will not reduce systemic pressure. Pressure is the same, it's the increase in vascular resistance due to decreased pCO2 that exerts effect.
83
Patient presents with bad headache, hyperdensity on non-contrast CT? Etiology?
Sub-arachnoid hemorrhage, most likely caused by ruptured saccular (berry) aneurysm in the circle of Willis. Associated with Ehlers-Danlos syndrome and ADPKD.
84
Where do the bronchial and pulmonary circulations merge?
Bronchial arteries join the post-alveolar capillary beds that subsequently drain into the pulmonary vein.
85
Mechanism of oxalate kidney stones in Crohn's Disease?
Calcium binds to lipids instead of insuluble oxalate salts (which are excreted), resulting in increased oxalate absorption --\> oxalate kidney stones.
86
key consequence of hyaline arteriolosclerosis progression?
glomerular scarring - kidney change in diabetes, hypertension --\> renal failure
87
gross pathology of atrophic kidney?
small, bumpy surface of cortex
88
small blood vessel histology shows thickening with layers of smooth muscle? Kidney effect?
"onion-skinning" - due to malignant hypertension --\> end organ ischemia --\> acute renal failure, pinpoint hemorrhage --\> "flea-bitten" appearance of kidney on gross examination
89
aortic dissection happens where? why?
proximal 10cm of aorta, high pressure, pre-existing weakness of media
90
hypertension does what to vaso vasorum?
hyaline arteriolosclerosis --\> predispose to aortic dissection via aortic vessel weakening/atrophy of the media
91
chief complication of thoracic aneurysm?
Dilation of aortic valve root --\> aortic insufficiency; compression of mediastinal structures; thrombosis or embolism
92
location of abdominal aortic aneurysm? Due to? Presentation?
Below renal arteries, above bifurcation/ Due to atherosclerosis/smoker's problem, especially males \> 60 with HTN; presents as a pulsatile abdominal mass, ruptures when \> 5cm --\> hypotension, flank pain
93
most common location of hemangiomas?
skin and liver
94
angiosarcoma triggers?
PVC, arsenic, Thorotrast (VAT)
95
tumor of endothelial cells?
Kaposi Sarcoma - a blood vessel-ish disease (HHV-8, Eastern European males, immunocompromised)
96
location of infantile coarctation? Presentation? Association?
Distal to aortic arch & vessels, prosimal to PDA; See lower extremity cyanosis; Turner's Syndrome
97
Hypertension in upper extremities, hypotension in lower extremities? Association? Finding on X-ray?
Adult form coarctation, associated with bicuspid aortic valve --\> on X-ray, notice notched ribs due to development of collateral circulation involving the intracostal arteries.
98
Distinguish AML from myeloproliferative disorder
AML --\> proliferation of blasts myeloproliferative disorder --\> proliferation of mature myeloid cells
99
Name the myeloproliferative disorders
RBC --\> polycythemia vera Granulocytes --\> CML (neutrophils, basophils, eosinophils) Megakaryocytes --\> Essential thrombocytopenia Monocytes --\> myelomonocytic leukemia (CMML)
100
Is WBC increased in myeloproliferative disorders?
Yes, as long as not polycythemia Vera - all other myeloid cells are WBC (leukocytes)
101
Significance of BCR-ABL fusion? Treatment?
t(9;22) --\> increased tyrosine kinase activity --\> CML Treat with imatinib ("-nib" = tyrosine kinase inhibitor)
102
Describe transformation in CML?
mutation is in hematopoietic stem cell, so can transform to either AML or ALL as disease progresses
103
Significance of LAO +/-
normal leukomoid reaction is inflammatory; therefore, granulocytes will be leukocyte alkaline phosphatase (LAP) positive. In CML, there is no infection to fight, so proliferative cells will be LAP negative; also, t(9,22)
104
Polycythemia Vera lab findings?
Decreased SaO2, increased EPA --\> PV (say, COPD and associated hypoxia) Normal SaO2, decrased EPO --\> PV (leukemia)
105
Source of ectopic EPO?
Renal Cell Carcinoma
106
Why is there no risk for hyperuricemia/gout in essential thrombocytosis?
No cell turnover, megakaryocytes just pinched off to make platelets (i.e., no purine metabolism)
107
Teardrop cells suggestive of?
myelofibrosis
108
Sinus histiocytes are found where?
live in medullary region of lymph nodes
109
Where is the SA node?
In the myocardium, just internal to the epicardium, lateral to the sinus venarum, at the junction of the SVC and RA
110
lymph node histology shows lots of "sub" areas, maybe demarcated circles? Etiology? Treatment?
Follicular lymphoma --\> lots of follicles, t(14;18) --\> overexpression of BCL2, stabilizes mitochondrial membrane, no apoptosis = immortal cells. Treat with rituximab, antibody against CD20+ receptor.
111
Signs and symptoms of acute leukemia?
Anemia, thrombocytopenia, neutropenia - blasts crowd out normal hematopoiesis
112
Key histological marker for ALL? Key histological marker for AML?
ALL --\> TdT+ -- This is a marker specific for DNA polymerase, which is only in the nucleus of lymphoblasts, not in mature cells. AML --\> mpo+ -- This is myeloperoxidase, the enzyme that crystalizes into Auer rods
113
Distinguish B-ALL from T-ALL
In addition to TdT+: - B cells --\> CD10+, CD19+, CD20+ - T cells --\> CD2+ through CD8+, but NOT CD10+
114
Cytogenetic markers for ALL?
t(12;21) --\> kids, good prognosis t(9;22) --\> adults, poor prognosis
115
Thymic mass in a teenager?
"T"-cell lymphoma, Acute Lymphoblastic Lymphoma (Teenager, Thymic, T-cell)
116
Down Syndrome leukemias?
Acute magakaryoblastic leukemia 5 years old
117
Distinguish acute from chronic leukemia?
acute --\> proliferation of blasts, decreased everything else chronic --\> proliferation of mature, circulating lymphocytes with insidious onset, usually in older adults
118
Chronic lymphocytic leukemia markers?
Neoplastic proliferation of naive B-cells - express CD5 and CD20 - Note that expression of CD5 is abberant, usually only T cells express CD5 - You may see "smudge" cells on histology, which are delicate cells that ruptured in the slide preparation
119
TRAP stain detects? Clinical features? Treatment?
Tartrate-resistant Acid Phosphatase stain detects hairy cell leukemia. Clinically, see splenomegaly (expanded red pulp) and a dry tap on bone marrow aspiration, no lymphadenopathy. Treat with 2-CDA, an adenosine deaminase inhibitor --\> adenosine accumulates in affected cells to toxic levels.
120
Newborn presents with floppy legs, absent ankle reflexes, lumbar spine/sacral anamoly?
Caudal regression syndrome (1:60,000 live births); associated with maternal diabetes
121
