Test your might!

[Depakote]

In the same vein as Allo's Name that Pathogen thread, let's get some good review going here. Anything on Step I is fair game.

A 37 year old male presents with a BP of 80/?. PT and PTT are elevated. Fibrinogen and Platelet count are decreased. D-Dimers are present. Peripheral blood smear reveals schistocytes as well as the following pathologic abnormality:

What is this patient's acute illness?
What is this patient's underlying illness?
Is there a specific abnormality associated with the underlying illness?
What must be done to treat this patient's underlying illness as not to aggravate the acute disease?

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[Richspiders07]

No clue how you treat it, but I'm going to say you need to find a new patient, this one is broken.

When all else fails, use charcoal??

 

[FutureInternist]

I tried answering this before so I'll bite with a real answer.

A) Long QT syndromes; either JLNS or Romano-Ward syndrome.
B) JLNS - AR; Romano-Ward - AD
C) JLNS - v-fib; RW - arrhythmia
D) Ask the parents if the kid has any problems hearing. If the kid's deaf, probably JLNS.

Awesome.

 

[FutureInternist]

what review book did you learn about these>?

UW Qs.

 

[Slide]

I just found this in my toxicology lecture.

Paraquat is a free radical that releases superoxide ions. Gets the lungs (had to look that up elsewhere).

No clue how you treat it, but I'm going to say you need to find a new patient, this one is broken.

Paraquat messes up the NADP --> NADPH process as well and generates tons of free radicals.

When all else fails, use charcoal??

If you're lucky and the paraquat is really dilute, you may be able to, but your patient will still be messed up.

What I found a little grotesquely humorous was that the answer to this on one of our questions was "Tell the patient to draw up his will; he is going to die." The only thing you could really do is palliative treatment. Kinda sucks.

 

[FutureInternist]

A 55 year old Caucasian male presents to the emergency room complaining of blurry vision and headache for the past 4 hours. He has not seen a physician in the past 25 years. He has moderate obesity and is employed as a Medical Malpractice Claims Attorney, despite this, he is calm and cooperative with your examination.

Fundoscopic examination reveals the following:

Blood pressure is found to be: 230/130
Auscultation of the lungs is clear.
Auscultation of the apex of the heart notes a S4 heart sound.
Auscultation of the abdomen reveals normal bowel sounds and no bruits.
No abnormalities are noted on auscultation of the chest or abdomen.


What is his current condition?
How should this be treated?
What is his underlying condition?
Does his underlying condition have a genetic predisposition? What is it's means of inheritance?
Explain the S4 heart sound.
If this dude checked out AMA b/c he planned to sue you, was standing on the corner minding his own business and was shot by "some dude"... What pathological observations (due to his current condition) would be visible at autopsy?

I will take a stab at this although not sure
Hypertensive crisis
IV labetalol
Polycystic kidney dz
AD
"Atrial Kick" i.e. blood being pushed into a stiff ventricle
Berry aneurysms

 

[FutureInternist]

"Tell the patient to draw up his will; he is going to die." The only thing you could really do is palliative treatment. Kinda sucks.

I like your teacher's sense of humor. We had one where it was ethylene glycol poisoning & one of the options was "Call the cops on his money hungry wife"

 

[Depakote]

I will take a stab at this although not sure
Hypertensive crisis
IV labetalol
Polycystic kidney dz
AD
"Atrial Kick" i.e. blood being pushed into a stiff ventricle
Berry aneurysms

Hypertensive crisis- check
IV labetalol - there's a better treatment for Malignant Hypertension... but it's risky, it can cause CN toxicity
Polycystic kidney dz- Probably should have worded this better, what is the most common cause of his condition? APKD isn't the most common cause of Malig HTN
AD- Right for APKD
"Atrial Kick" i.e. blood being pushed into a stiff ventricle- yup.
Berry aneurysms- Right for APKD, but what would you see that was related to the Malig HTN?

 

[Depakote]

BTW. The most common cause of Malig HTN was one they burned our class on... so figured I'd share it.

 

[Slide]

Hypertensive crisis- check
IV labetalol - there's a better treatment for Malignant Hypertension... but it's risky, it can cause CN toxicity
Polycystic kidney dz- Probably should have worded this better, what is the most common cause of his condition? APKD isn't the most common cause of Malig HTN
AD- Right for APKD
"Atrial Kick" i.e. blood being pushed into a stiff ventricle- yup.
Berry aneurysms- Right for APKD, but what would you see that was related to the Malig HTN?

My turn:
Drug - try nitroprusside and another drug that stimulates D receptors (can't remember drug name)
Most common cause - gonna go with renovascular stenosis/atherosclerosis, which could be ultimately due to diabetes type 2.
For pathological changes, I guess the obvious for malig HTN would be hyperplastic arterioscleroris (onion skinning) and fibrinoid necrosis. Atherosclerotic plaques would also be a possibility, esp since he has papilledema. Doubt you'd see lacunar infarcts though.

 

[Depakote]

My turn:
Drug - try nitroprusside and another drug that stimulates D receptors (can't remember drug name)
Most common cause - gonna go with renovascular stenosis/atherosclerosis, which could be ultimately due to diabetes type 2.
For pathological changes, I guess the obvious for malig HTN would be hyperplastic arterioscleroris (onion skinning) and fibrinoid necrosis. Atherosclerotic plaques would also be a possibility, esp since he has papilledema. Doubt you'd see lacunar infarcts though.

Nitroprusside
No bruits makes renovascular stenosis unlikely. This isn't the most common cause of Malig HTN.
Yup for the pathologic changes, you'd also see a "flea-bitten kidney"



Most common cause of malignant hypertension is essential hypertension.

(secondary causes of hypertension may be more likely to progress to malignant hypertension if you have identified a secondary cause of the hypertension, but overall they make up a significantly smaller number of the cases. 90% of hypertension is essential.)

 

[FutureInternist]

IV labetalol - there's a better treatment for Malignant Hypertension... but it's risky, it can cause CN toxicity

Would dantrolene be another option or is nitroprusside better?

 

[Slide]

Most common cause of malignant hypertension is essential hypertension.

(secondary causes of hypertension may be more likely to progress to malignant hypertension if you have identified a secondary cause of the hypertension, but overall they make up a significantly smaller number of the cases. 90% of hypertension is essential.)

forehead slap. Duh, ugh I can't believe I let that one through (my school is in the hypertensive capital in the entire world).

 

[Slide]

Would dantrolene be another option or is nitroprusside better?

I think dantrolene is mainly used for malig. hyperthermia or any symptoms caused by serotonin syndrome/neuroleptic malignant syndrome (which includes possible HTN).

 

[Depakote]

Oh, and essential HTN is multifactorial inheritance. Something they were pounding into us, too.

 

[Depakote]

I think dantrolene is mainly used for malig. hyperthermia or any symptoms caused by serotonin syndrome/neuroleptic malignant syndrome (which includes possible HTN).

sounds right.

 

[Depakote]

Per First Aid, you can give:

Nitroprusside (this was the only one I remember from lecture)
Fenoldopam (Dopamine receptor agonist)
Diazoxide (K+ channel opener)

 

[SomeDoc]

A male patient presents to the clinic with a diffuse rash on his arms, legs, and trunk. He complains of fatigue, and is pallid in appearance. He also has been constantly sick for as long as he can remember, has problems with recurrent respiratory infections, thick mucus, and often has greasy stool. Hematological analysis reveals a high PTT and PT, with a normal bleeding time. What anatomical structure in the gonadal region of this patient is usually absent in individuals of the same sex of the patient, who have this condition?

 

[Depakote]

A male patient presents to the clinic with a diffuse rash on his arms, legs, and trunk. He complains of fatigue, and is pallid in appearance. He also has been constantly sick for as long as he can remember, has problems with recurrent respiratory infections, thick mucus, and often has greasy stool. Hematological analysis reveals a high PTT and PT, with a normal bleeding time. What anatomical structure in the gonadal region of this patient is usually absent in individuals of the same sex of the patient, who have this condition?

I'm going to say he's got cystic fibrosis and bilateral absence of the vas deferens.

 

[bola]

A male patient presents to the clinic with a diffuse rash on his arms, legs, and trunk. He complains of fatigue, and is pallid in appearance. He also has been constantly sick for as long as he can remember, has problems with recurrent respiratory infections, thick mucus, and often has greasy stool. Hematological analysis reveals a high PTT and PT, with a normal bleeding time. What anatomical structure in the gonadal region of this patient is usually absent in individuals of the same sex of the patient, who have this condition?

yeah i agree with CBAVD secondary to CF too.
So
- the greasy stool is from fat malabsorption due to pancreatic exocrine insufficiency
- the prolonged PT and PTT are from Vit K deficiency and maybe low proteins?
- What is the pathophysiology of the rash? infection?

 

[SomeDoc]

I'm going to say he's got cystic fibrosis and bilateral absence of the vas deferens.

 

[SomeDoc]

yeah i agree with CBAVD secondary to CF too.
So
- the greasy stool is from fat malabsorption due to pancreatic exocrine insufficiency
- the prolonged PT and PTT are from Vit K deficiency and maybe low proteins?
- What is the pathophysiology of the rash? infection?

Rash= petechiae in this case. Due to malabsorption of fat soluble vitamins, erythrocytes have increased susceptibility to oxidative damage (vitamin E), leading to intravascular hemolysis, and lack of vitamin K leads to lack of vit K dependent coagulative factors (2, 7, 9, 10), leading to hemorrhagic symptoms.

 

[Depakote]

Rash= petechiae in this case. Due to malabsorption of fat soluble vitamins, erythrocytes have increased susceptibility to oxidative damage (vitamin E), leading to intravascular hemolysis, and lack of vitamin K leads to lack of vit K dependent coagulative factors (2, 7, 9, 10), leading to hemorrhagic symptoms.

nice. I hadn't put that together, but the rest was enough to get me to CF

 

[SomeDoc]

...If this dude checked out AMA b/c he planned to sue you, was standing on the corner minding his own business and was shot by "some dude"...

Just stumbled across this...

 

[SomeDoc]

A 20 year old caucasian male presents to the clinic with complaints of episodic bouts of heart palpitations, chest pains, inability to concentrate, agitations, and perspiration. These symptoms have been ongoing for "a long time," and they have gotten significant enough that he decides to seek medical help.

Which of the following conditions or signs, is most closely related, from a developmental pespective, to the cells involved in the etiology of the above patient's symptoms?


Seronegative Spondyloarthopathies
Rheumatoid Arthritis
Albinism
Metachromatic leukodystrophy
Angiokeratoma
Hemosiderin-laden macrophages

 

[BoneNibbler]

I am going with albinism.

A 20 year old caucasian male presents to the clinic with complaints of cyclical bouts of heart palpitations, chest pains, inability to concentrate, agitations, and perspiration. These symptoms have been ongoing for "a long time," and they have gotten significant enough that he decides to seek medical help.

Which of the following conditions or signs, is most closely related, from a developmental pespective, to the cells involved in the etiology of the above patient's symptoms?


Seronegative Spondyloarthopathies
Rheumatoid Arthritis
Albinism
Metachromatic leukodystrophy
Angiokeratoma
Hemosiderin-laden macrophages

 

[SomeDoc]

I am going with albinism.

The patient in the case is suffering from a pheochromocytoma. The cells involved in the production of catecholamines which produce the symptoms in the patient are chromaffin cells, which are embryologically derived from the neural crest.

Albinism, while a condition typically due to defective tyrosinase activity, or impaired absorption of tyrosine due to defective transporters, can also be caused by failure of neural crest cell migration.

 

[Depakote]

I'm making this one up off the top of my head here, bear with me:


A 24 year old male flight attendant is attacked by ninjas. One of the ninjas stabs the gentleman in the Left Lower Quadrant and perforates the large intestine. The ninjas vanish into the shadows and the patient is transported to the local ED. The patient is brought to surgery and the wound is repaired. He has a family history of gingivitis and admits to engaging in low-risk sexual behavior with his wife.

What is your post-operative concern with this patient?
What agents would be involved primarily?
How would you manage the above?

 

[SomeDoc]

I'm making this one up off the top of my head here, bear with me:


A 24 year old male flight attendant is attacked by ninjas. One of the ninjas stabs the gentleman in the Left Lower Quadrant and perforates the large intestine. The ninjas vanish into the shadows and the patient is transported to the local ED. The patient is brought to surgery and the wound is repaired. He has a family history of gingivitis and admits to engaging in low-risk sexual behavior with his wife.

What is your post-operative concern with this patient?
What agents would be involved primarily?
How would you manage the above?

Subacute bacterial endocarditis
Strep mutans, enterococcus faecalis/faecium
Ampicillin/Amoxicillin for both

 

[Depakote]

Subacute bacterial endocarditis
Strep mutans, enterococcus faecalis/faecium
Ampicillin/Amoxicillin for both

I was thinking more along the lines of a perforated bowel -> abscess w/ gut anaerobes when I wrote this and wanted someone to tell me what good drugs to cover that in this situation would be.

I guess I could see subacute bacterial endocarditis popping up, but I think that would be lower on the list.

 

[psychforme]

Took this question with a mainstream prep book. Which malignant neoplasm is associated with the correct paraneoplastic syndrome:

a) adrenal cortical carcinoma - hyperuricemia
b) glioblastome multiforme - hyperglycemia
c) hepatocellular carcinoma - polycythemia
d) renal cell carcinom - hypokalemia
e) squamous cell carcinoma of the lung - hypercalcemia

 

[SomeDoc]

I was thinking more along the lines of a perforated bowel -> abscess w/ gut anaerobes when I wrote this and wanted someone to tell me what good drugs to cover that in this situation would be.

I guess I could see subacute bacterial endocarditis popping up, but I think that would be lower on the list.

I see, perhaps you were going for a. nocardia of the mouth, and ecoli for the gut? Tx w/ metronidazole to cover both anaerobes, along w/ a sulfa Rx for a. nocardia (or alternatively, clindamycin).

 

[SomeDoc]

Took this question with a mainstream prep book. Which malignant neoplasm is associated with the correct paraneoplastic syndrome:

a) adrenal cortical carcinoma - hyperuricemia
b) glioblastome multiforme - hyperglycemia
c) hepatocellular carcinoma - polycythemia
d) renal cell carcinom - hypokalemia
e) squamous cell carcinoma of the lung - hypercalcemia

E. Squamous cell carcinoma's of the lung are associated w/ secretion of PTH-like peptide.

 

[psychforme]

E. Squamous cell carcinoma's of the lung are associated w/ secretion of PTH-like peptide.

Isn't C correct as well?

 

[SomeDoc]

Isn't C correct as well?

To my knowledge, renal carcinomas and hemangioblastomas of Von Hippel Lindau syndrome are associated with paraneoplastic EPO secretion. I am not aware of hepatocellular carcinoma as a paraneoplastic etiology of polycythemia.

 

[Redrox]

A pre-med body building, SDNer knows everything about medicine, including the gluconeogenesis, and decides to supplement his diet with a giant tub of pure alanine that he has bought on the interwebs.

He supplements his application by working in the projects of Chicago, promoting AIDS education in Africa, donating plasma and blood as often as possible, (which is ironic because he has required many transfusions after many rock climbing related traumas). And he writes an amazing application letter about the struggles he had with his racial identity as an Ashkenazi, African, Asian, Andalusian.

However, his dietary supplement in high doses over many years has given him an anemia due to an inhibition of a enzyme.
What is it?
What are the characteristic cells?
Name another compound that inhibits the same enzyme?
And one that stimulates it?

 

[Depakote]

I see, perhaps you were going for a. nocardia of the mouth, and ecoli for the gut? Tx w/ metronidazole to cover both anaerobes, along w/ a sulfa Rx for a. nocardia (or alternatively, clindamycin).

it's a perf'd bowel... the gingivitis is a distractor.

 

[future_dr_house]

I see, perhaps you were going for a. nocardia of the mouth, and ecoli for the gut? Tx w/ metronidazole to cover both anaerobes, along w/ a sulfa Rx for a. nocardia (or alternatively, clindamycin).

Those are possible, but if I remember from GI, I think bacteroides fragilis is the most common anaerobe in the gut and is involved in infx due to stasis or perforations. Tx is still what you mentioned, metronidazole.

 

[psychforme]

To my knowledge, renal carcinomas and hemangioblastomas of Von Hippel Lindau syndrome are associated with paraneoplastic EPO secretion. I am not aware of hepatocellular carcinoma as a paraneoplastic etiology of polycythemia.

Well, it must be a malfunct question in BRS path 2nd ed since both goljan and wikipedia lists hepatocellular carcinoma as a cause of polycythemia. At least I will never forget that.

 

[FutureInternist]

A pre-med body building, SDNer knows everything about medicine, including the gluconeogenesis, and decides to supplement his diet with a giant tub of pure alanine that he has bought on the interwebs.

He supplements his application by working in the projects of Chicago, promoting AIDS education in Africa, donating plasma and blood as often as possible, (which is ironic because he has required many transfusions after many rock climbing related traumas). And he writes an amazing application letter about the struggles he had with his racial identity as an Ashkenazi, African, Asian, Andalusian.

However, his dietary supplement in high doses over many years has given him an anemia due to an inhibition of a enzyme.
What is it?
What are the characteristic cells?
Name another compound that inhibits the same enzyme?
And one that stimulates it?

Alanine inhibits pyruvate kinase which does PEP --> pyruvate in glycolysis
"Characteristic cells" ?? Since RBCs will not be able to produce ATP their pumps won't work & they will swell up --> hemolytic anemia
ATP inhibits
AMP, insulin stimulates

 

[ENThopeful]

Rash= petechiae in this case. Due to malabsorption of fat soluble vitamins, erythrocytes have increased susceptibility to oxidative damage (vitamin E), leading to intravascular hemolysis, and lack of vitamin K leads to lack of vit K dependent coagulative factors (2, 7, 9, 10), leading to hemorrhagic symptoms.

does vitamin k deficiency lead to both an increase in PTT or just PT? Warfarin -> epoxide reductase inhibitor, is commonly monitored by PT..

 

[ENThopeful]

To my knowledge, renal carcinomas and hemangioblastomas of Von Hippel Lindau syndrome are associated with paraneoplastic EPO secretion. I am not aware of hepatocellular carcinoma as a paraneoplastic etiology of polycythemia.

it is, goljan rapid review pg 151

 

[ENThopeful]

"Characteristic cells" ?? Since RBCs will not be able to produce ATP their pumps

echinocytes!

 

[Redrox]

does vitamin k deficiency lead to both an increase in PTT or just PT? Warfarin -> epoxide reductase inhibitor, is commonly monitored by PT..

Both, anything that inhibits one of the factors of the final common pathway ( V, X, II, I ) will elevate both times.

 

[Redrox]

echinocytes!

Pyruvate Kinase Deficiency is an autosomal recessive anemia due to a defect in glycolosis (contrast this to G6PD which is sex recessive, pentose phosphate shunt) and echinocytes, RBC's with thorny projections, are seen on smear.

Also, ADP does stimulate the enzyme as does fructose 1-6 BP.

 

[Redrox]

Patient presents in the ER with voluminous watery diarrhea with numerous white flecks of mucous and severe vomiting. There is a language barrier and the patient is unable to answer what he has eaten recently, although Italian food is suspected (dude smells like garlic bread).

What is the diagnosis?
What is the enzyme inhibited or stimulated? Specifically what subunit?
Treatment?

 

[FutureInternist]

Patient presents in the ER with voluminous watery diarrhea with numerous white flecks of mucous and severe vomiting. There is a language barrier and the patient is unable to answer whether he has left the country or what he has eaten recently, although Italian food is suspected (dude smells like garlic bread).

What is the diagnosis?
What is the enzyme inhibited or stimulated? Specifically what subunit?
Treatment?

Vibrio parahemolyticus??? d/t raw seafood
Activation of alpha subunit of G-protein --> activation of AC --> increased cAMP --> e-lyte secretion w/ water following --> mucho diarrhea
Preventive

 

[Redrox]

The wife shows up, surprised and disappointed her husband is not dead yet, seems the guy is abusive and an all round dick.

 

[Depakote]

The wife shows up, surprised and disappointed her husband is not dead yet, seems the guy is abusive and an all round dick.

White Phosphorous poisoning? (I googled the crap out of this one)

 

[Redrox]

First Aid, yo.

 

[SomeDoc]

Patient presents in the ER with voluminous watery diarrhea with numerous white flecks of mucous and severe vomiting. There is a language barrier and the patient is unable to answer what he has eaten recently, although Italian food is suspected (dude smells like garlic bread).

What is the diagnosis?
What is the enzyme inhibited or stimulated? Specifically what subunit?
Treatment?

Vibrio Cholera w/ rice water diarrhea
Adenylate cyclase stimulation. Gs.
Fluid replacement