NBME 16 Flashcards
Cystic fibrosis is due to a defect in what
protein structure of the ATP gated Cl- channel
What is the cellular process for muscle atrophy
polyubiquitination
Our body goes through arteriolar vasoconstriction to do what?
MAMMALS IN A COLD ENVIRONMENT
Vasoconstriction also occurs in superficial blood vessels of warm-blooded animals when their ambient environment is cold; this process diverts the flow of heated blood to the center of the animal, preventing the loss of heat.
What does central venous pressure represent?
pressure in the thoracic vena cava near the right atrium (therefore CVP and right atrial pressure are essentially the same)
- it is a major determinant of the filling pressure and therefore the preload of the right ventricle, which regulates stroke volume
What causes hemochromatosis?
- recessive mutation HFE gene C282Y which is a point missense mutation changing cysteine to tyrosine
- this results in abnormal iron sensing and increase intestinal absorption
- can also be secondary to chronic transfusion therapy like in beta-thalassemia major
A 45 year old man is admitted because of CHF. 2 relatives died of dilated cardiopathy and cirrhosis. PE shows hyperpigmentation and elevated glucose. What is the cause?
hemochromatosis
Presents in patients after age 40 when total body iron >20g. Women show signs later because they lose iron through menstruation. Classic triad is: DIABETES, CIRRHOSIS, SKIN PIGMENTATION (BRONZE DIABETES)
In addition to cirrhosis, diabetes, skin pigmentation, what can patients with hemochromatosis present with?
- classically restrictive cardiomyopathy but also dilated cardiomyopathy (reversible)
- hypogonadism
- arthropathy (calcium pyrophosphate deposition especially in MCP joints)
- HCC is common cause of death
How is hemochromatosis treated?
- repeated phlebotomy
- chelation with deferasirox, deferoxamine, oral deferiprone
What are neuroendocrine tumors?
Group of neoplasms originating from Kulchitsky and enterochromaffin-like cells.
Occur in various organs: 1. thyroid: medullary carcinoma 2. Lungs: small cell carcinoma 3. pancreas: islet cell tumor 4. adrenals: pheochromocytoma
Cells contain amine precursor uptake decarboxylase (APUD) and
secrete different hormones (eg, 5-HIAA, neuron-specific enolase [NSE], chromogranin A
What is the MOA of hydralazine?
increased cGMP to cuase smooth muscle relaxation
Vasodilates arterioles > veins; afterload reduction
What is hydralazine used for?
- Severe hypertension (particularly acute), 2. HF (with organic nitrate).
Safe to use during pregnancy.
Frequently coadministered with a β-blocker to prevent reflex tachycardia
What are the SE of hydralazine?
Compensatory tachycardia (contraindicated in angina/CAD)
fluid retention, headache, angina.
Lupus-like syndrome.
Non-selective α-blockers
- phenoxybenzamine
2. Phentolamine
α-1-selective-blockers
prazosin
terazosin
doxazosin
tamsulosin
- zosin ending
α-2-selective-blockers
mirtazapine
β1-selective blockers [β1>β2]
acebutolol (partial agonist) atenolol betaxolol bisoprolol esmolol metoprolol
- SELECTIVE ANTAGONIST MOSTLY GO FROM A to M
nonselective β blockers [β1=β2]
nadolol
pindolol (partial agonist)
propranolol
timolol
Nonselective α1 and β-blocker
carvedilol
labetalol
MOA of Nebivolol
cardioselective β1-blocking with stimulating β3- receptors (activate NOS synthase in vasculature and decreases SVR)
What is cystic hygroma associated with?
Turner syndrome.
What is tracheomalacia?
Tracheal cartilage is soft so that the trachea partially collapses when the person breathes out
can be congenital, compression from vascular rings or acquired during infection
What does the SMA supply
Distal duodenum to proximal 2/3 of transverse
colon
[midgut]
What does the celiac artery supply
Pharynx (vagus nerve only) and lower esophagus
(celiac artery only) to proximal duodenum;
liver, gallbladder, pancreas, spleen (mesoderm)
What does the IMA supply
Distal 1/3 of transverse colon to upper portion of
rectum
The greater curvature of the stomach is supplied by which two arteries?
The right gastro-omental artery supplies the inferior part of the greater curvature.
The left gastro-omental artery supplies the superior part of the Greater Curvature.
What supplies the lesser curvature of the stomach?
The inferior part is supplied by the right gastric artery, which comes from the Hepatic artery, a branch of the Common Hepatic artery.
The superior part, and the cardia, are supplied by the left gastric artery, which is a direct branch from the Celiac Trunk
Mechanisms of C. Diff colitis
Toxin A, an enterotoxin,
binds to brush border of gut and alters fluid
secretion
Toxin B, a cytotoxin, disrupts cytoskeleton via actin depolymerization. Both
toxins lead to diarrhea- watery but occasionally bloody
A 4-month old boy is diagnosed with a rare autosomal recessive skeletal dysplasia involving abnormal endochondral bone formation. Genetic analysis shows null mutations in a gene for a protein that controls the traffic of vesicles into the golgi complex. EM of this patient’s cells will most likely show which of the following findings?
dilated rough endoplasmic reticulum
assuming since things can’t get moved from the RER to golgi so it gets stuck in the Golgi
What is Cyclin D1 associated with?
mantle cell lymphoma
Which drug reversibly inhibits dihydroorotate dehydrogenase, preventing pyrimidine synthesis. Suppresses
T-cell proliferation
Leflunomide
- used for Rheumatoid arthritis, psoriatic arthritis.
How is NAPQI, the toxic metabolite of Tynelol normally inactivated?
inactivated by conjugation with glutathione
in general the majority of Tynelol is excreted in bile after glucuronidation
Which class of chemotherapy drugs causes peripheral neuropathy?
microtubule inhibitors
When would you use clomiphene?
Used to treat infertility due
to anovulation (eg, PCOS). May cause hot flashes, ovarian enlargement, multiple simultaneous
pregnancies, visual disturbances.
What is the cause of urticaria?
Mast cell degranulation. Macrophages produce IL-1, IL-6, TNF-alpha
Which drug is a derivative of Jimsonweed and causes anti-cholinergic symptoms?
atropine
How would you treat atropine poisoning?
physostigmine [indirect agonist- anticholinesterase]
What is carbachol used for?
Copies Ach- Constricts pupil and relieves intraocular
pressure in open-angle glaucoma
Uses of pilocarpine
- muscarinic receptor (M3) found on the iris sphincter muscle, causing the muscle to contract -resulting in pupil constriction (miosis)
- Pilocarpine also acts on the ciliary muscle and causes it to contract. When the ciliary muscle contracts, it opens the trabecular meshwork through increased tension on the scleral spur. This action facilitates the rate that aqueous humor leaves the eye to decrease intraocular pressure
What is Peutz-Jeghers
syndrome?
PATHGNOMIC for hyperpigmented lip
- Autosomal dominant syndrome featuring numerous hamartomas throughout GI tract, along with
hyperpigmented mouth, lips, hands, genitalia. - Associated with increased risk of breast and GI cancers (eg,
colorectal, stomach, small bowel, pancreatic)
What are the characteristics of Gardner syndrome?
FAP + osseous and soft tissue tumors, congenital hypertrophy of retinal pigment epithelium,
impacted/supernumerary teeth.
Common culprits of acute prostatis
Common bacteria are Escherichia coli, Klebsiella, Proteus, Pseudomonas, Enterobacter, Enterococcus, Serratia, and Staphylococcus aureus.
What is special about Ureaplasma Urealyticum
like mycoplasma it has no cell wall
What are the antibodies for celiac disease?
- IgA anti-tissue
transglutaminase (IgA tTG) - anti-endomysial
- anti-deamidated gliadin peptide antibodies
Histology shows loss of villi and increased number of intraepithelial lymphocytes
celiac disease