Tommy’s High Yield Concepts 1332 - 1362

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Tommy’s High Yield Concepts 1332 - 1362



1332



Aspartate, in the urea cycle, feeds in with what compound to form Arginosuccinate?



A) Citruline...arginosuccinate synthase catalyzes. This is the rate limiting rxn in liver.





1333



In pyrimidine synthesis,



1) what is the first compound needed?



2) what is the first enzyme used?



3) what compound inhibits the first step?



4) What is the second compound formed?





A1) Glutamine



a2) CAP synthetase II



a3) The later product, OROTIC ACID inhibits (PRPP activates)



a4) CAP





1334



1) What is the initial compound for PURINE synthesis?



2) What compound feeds in with PRPP to form Ribosylamine5phosphate?







a1) PRPP



a2) Glutamine, which leaves as Glutamate





1335



Baby boy comes to your office with a cut that does not stop bleeding one week after birth, who IS being breast fed by Mommy. What could be the problem?



a) Breast milk is known to be LOW in vitamin K. (VERY IMPORTANT)





1336



AD inheritance is VERY important cuz offspring have a 50/50 chance of getting disease. So...



Dwarfism is common and when presented by mom, you tell her the cause is due to what and what are chances her next baby will be short as such?



a) Cause is mutated FGF (fibroblast growth factor) receptor and her unborn child will have a 50/50 chance of shortness too!!!





1337



Patient comes to you with "choreiform movements" and sadness and loss of mental status. She is about 45 years old...



1) What is the disease?



2) What brain structure is lesioned?



3) Which neurotransmitters are low in da brain?



4) Which chromosome is the problem?



a1) Huntington's chorea



a2) Caudate nucleus



a3) ACh and GABA are low



a4) Defect is on chromosome 4







1338



This again is an Autosomal Dominant disease.

Patient comes in distraught cause he has these nodules all over his face and back and torso. On a chest x ***, you see more nodules. What is the disease?



a) This is von Recklinhausen's disease/Neurofibromatosis type1...the most common benign tumor of the lung.





1339



Patient presents who is TALL, long arms n legs, joints are hyperextensible (see pics on Google).

What is this horrible AD disease?

What heart problem does he have?

What gene mutation is here?



a1) This is MARFAN's syndrome. Famous!!!

a2) His heart may have a "floppy" valve (mitral and aortic). AND, he may suffer an aortic dissection.

a3) FIBRILLIN gene is broke.







1340





Q) Patient presents with lesions(or nodules) of the skin and central nervous system, and seizures. He or she has experienced developmental delay, mental retardation and autism. His mom is crying, because she has...?

(Remember: This is autosomal dominant)



1) This is the FAMOUS TUBEROUS SCLEROSIS. On top of all this, you can geta retinal hamartoma and cyts in the kidneys!







1341





Patient presents with some complaints of vision, rashes on the knees among many other places. A CT scan shows lesion/significance around the cerebellum.



This AD disorder involving blood vessels is what?

What # chromosome is involved?

a) This is Von Hippel-Lindau disease, a genetic condition involving the abnormal growth of blood vessels in some parts of the body which are particularly rich in blood vessels. These little knots are called angiomas, or hemangioblastomas. These angiomas may cause problems (esp. in the retinas), and problems can develop around them. The disease can be different in every patient. As the angiomas grows, the walls of the blood vessels may weaken and some blood leakage may occur, causing damage to surrounding tissues. Blood leakage from angiomas in the retina can interfere with vision.

a2) Chromosome is 3





1342



KNOW that a common AD disease the boards LOVE is APKD or Adult Polycystic Kidney disease.

On chromosome 16, the broken APKD1 gene results in Hypertension, hematuria, and berry aneurysms, and mitral valve prolapse.





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1344



Patient presents with hemolytic anemia. A peripheral blood smear shows round/sphere RBCs. What is dx and treatment?



a) This is also AD! Tis Hereditary Spherocytosis and you can remove the spleen as a cure.





1345



Young Female patient comes in with facial rash, POSITIVE VDRL but neg. FTA-ABS, and joint pain.



a) Yes, it IS SLE systemic lupus erythematosus. The lab was false positive from anti cardiolipin antibodies.





1346



OK, patient comes in and his face looks like a monster's! Gargoylelike. What is the missing enzyme and dx? (Hint, Disorder of GAG, Glycosaminoglycans and it is a lysosomal storage disease). So sad...........



a) This is one of the Mucopolysaccharidoses (MPSs), a group of 7 inherited lysosomal storage diseases caused by a deficiency in the lysosomal enzymes that degrade glycosaminoglycans (GAGs).

a2) Missing enzyme is alpha l iduronidase.

Patient likely will die in youth...







1347



Another patient comes in with similar features as the previous dx, but this disease name is sex linked RECESSIVE and has less terrible effects. BUT, what is the NAME of the missing enzyme here?



a) You have to be perfect...L iduronosulfate sulfatase is missing in HUNTER's disease.





1348



All were asked to calculate half life at least once during their exam. Do you know the equation?

t 1/2 = (.7 x (Volume of distribution)) / Clearance





1349



What is the equation for clearance if asked?

CL= drug elimination rate/plasma concentration





1350



Q) What is the mech of action of vancomycin?

a) Peptidoglycan synthesis blockade





1351



Pfizer makes Zithromax, a macrolide, like erythromycin. What is MOA?

a) The 50S ribosomal subunit is shut down.





1352



Everyone had tons of Mech of action so...



What is MOA of Edrophonium?



a) This is a short action anticholinesterase to diagnose myasthenia gravis.





1353



So...what is the Mech of action of Atropine?

a) This is a muscarinic antagonist soooo, you cannot salivate urinate or poop. Know that in the eye, it DILATES the pupil as well as doing a bunch of other "sympathetic" stuff.





1354



What is the MOA of Benztropine?

a) Again, this is another muscarinic antagonist used in Parkinson's Disease.





1355



Why should you use Dopamine instead of norepinephrine for decreased renal perfusion?

a) Norepinephrine decreases renal perfusion!



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1356



Q) Phenytoin is used ALL da time. So what happens if you overdose someone with it?

a) This drug will cause diplopia and nystagmus and malaise on overdose. The MOA is blocking Na channels.





1357



What do you give to a woman who took a whole bottle of Acetaminophen?

A) N-acetylcysteine, via glutathione rejuvination





1358



A small child patient overdoses on a large bottle of cherry cough syrup containing dextramethorphan. What are the effects?

a) He is likely to get CNS and respiratory depression and constipation and miosis.





1359



Quick! What is the overdose effect of Acetazolamide, a diuretic? And the MOA?

a) the overdose effect is acidosis as HCO3- is not reabsorbed in the kidney. The MOA is that it blocks carbonic anhydrase in the proximal convoluted tubule.





1360



Many told me that the test emphasized many popular CT scans of thd lungs, eyes, bone fractures, and brain stem cuts and popular CROSS sections (like around the abdomen). You need to know the major structures like which is the sympathetic ganglion, point to the pineal gland on a brain cross section, etc.





1361



Everyone uses Lasix, known as Furosemide. What is the MOA? What are the overdose features? Can you point to a picture of where it acts?



The MOA of this SULFA drug is it blocks Na, Cl, and K at the ascending loop of Henle. The URINE becomes less concentrated as the ions leave the tubule.

The overdose rxns are Allergy to sulfa drugs, OTOTOXICITY, GOUT, and HYPOkalemia. KNOW this COLD. (Everyone loves Lasix...)





1362



The next favorite drug is HTZ Hydroclorothiazide. This actually is used to PREVENT gout as well as reabsorbing K+. So...tell me the MOA, overdose effects? You already know this is used as a "water pill".



a) The MOA is it blocks Na+ and Cl- reabsorption in early distal tubule. It lowers Ca excretion. Many of the diuretics are used to manage hypertension and congestive heart failure.

on OVERDOSE, it causes hyperlipidemia, hyperuricemia, hyperglycemia, hypercalcemia.

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These were already done through 1362