Welcome to smc213’s page.
Contributor score: 169
Comments ...
drzed
And even if it was Wilson disease, it would have the exact same consequence leading to Fanconi syndrome.
+3
abhishek021196
Fanconi syndrome Generalized
reabsorption defect in
PCT = Increased excretion of
amino acids, glucose,
HCO 3 – , and PO 4 3– ,
and all substances
reabsorbed by the
PCT
May lead to
metabolic acidosis
(proximal RTA),
hypophosphatemia,
osteopenia
Hereditary defects
(eg, Wilson disease,
tyrosinemia,
glycogen storage
disease), ischemia,
multiple myeloma,
nephrotoxins/drugs
(eg, ifosfamide,
cisplatin), lead
poisoning.
Polyuria, renal tubular acidosis type II, growth failure,
electrolyte imbalances, hypophosphatemic rickets =
Fanconi syndrome (multiple combined dysfunction of the
proximal convoluted tubule).
+2
Subcomments ...
smc213
I googled the meaning of abnormal test results just to make sure.
A positive test is one in which the result of the test is abnormal; a negative test is one in which the test's result is normal.
+4
smc213
I googled the meaning of abnormal test results just to make sure.
A positive test is one in which the result of the test is abnormal; a negative test is one in which the test's result is normal.
+4
seagull
This has to do with Intention-to-treat analysis. Essentially, when participants are non-adherent but the data shouldn't be lost. They just undergo another statistical model to account for their changes.
Here is a nice video
https://www.youtube.com/watch?v=Kps3VzbykFQ&t=7s
+23
dr.xx
Where does the question mention "intention-to-treat"?
+
notadoctor
They seem to be pretty obsessed with "intention-to-treat" it's been asked in one way or another in all the new NBMEs that I've done. (Haven't done 24 as yet)
+8
wutuwantbruv
They don't, intention-to-treat is just the best way to go about it @dr.xx
+1
yex
I agree with @notadoctor !!
+
ergogenic22
i think if it were per protocol, both groups would be excluded, the ones that were inconsistent, the ones that dropped out, and the ones that switched. But answer choices only allow ITT or exclusion of one group.
+2
sympathetikey
& Galactokinase deficiency would be much milder.
+9
smc213
Big was soybean formula not giving any issues. Soy-milk can be used as a substitute formula in patients with Classic Galactosemia since it contains sucrose (->fructose and glucose).
+4
oslerweberenu
Why can't this be glucose 6 phosphatase deficiency
Confused me
+
almondbreeze
@oslerweberenu G6PD - increased RBC susceptibility to oxidant stress (eg, sulfa drugs, antimalarials, infections, fava beans) -> hemolysis; has nothing to do with presence of reducing sugar
+1
makinallkindzofgainz
@almondbreeze; Glucose-6-phosphatase deficiency is Von Gierke disease, they are not referring to G6PD deficiency (an entirely seperate disease)
+9
et-tu-bromocriptine
To add on to this, anterior* duodenal ulcers tend to perforate (makes sense because closest to the abdominal cavity) whereas posterior duodenal ulcers tend to bleed (due to proximity to the gastroduodenal artery).
+15
smc213
Acute pancreatitis can also occur with a posterior duodenal ulcer rupture. Source: Pathoma
+6
victor_abdullatif
Fun fact: anterior duodenal perforation are more common than posterior because of the physics of the flow of chyme from the stomach into the duodenum. It travels in a manner that hits the anterior portion of the duodenum, therefore leading to ulcer / perforation.
+2
d_holles
I thought this was medullary thyroid cancer but demographically SCC works better.
+
smc213
Medullary thyroid carcinoma increases calcitonin levels leading to decreased serum Ca2+ by increasing Ca2+ renal excretion. So high levels of calcitonin secreted by the tumor may lead to hypOcalcemia. Source: Pathoma
+24
brise
actually according to pathoma, you rarely see hypocalcemia even though calcitonin is high!
+3
smc213
@mguan1993 yes it does! With secondary hyperPTH due to CKD = incr. phosphate, dec. Ca2+ and incr. PTH. This can then progress to tertiary hyperPTH from longstanding secondary hyperPTH as a result of parathyroid HYPERPLASIA --> autonomously (refractory) functioning parathyroid. This will actually lead to INCREASED Ca2+, and significantly INCREASED PTH. Treatment would be surgical removal of the parathyroid glands. Sources: DIT and FA18 p340
+5
smc213
probably because the subscapularis m. is the only SITS muscle that does internal rotation & adduction along with the teres minor m. action being adduction & external rotation.
+1
smc213
Also, when Meningococcal meningitis is treated ... close contacts are also treated prophylactically whereas the others typically are not. There's also a subunit vaccine for n. meningitis due to high infectivity rate especially in crowded establishments.
+7
dentist
So, Cholera is also p2p but Mening is more likely?
+1
qball
Remember the fire sprinklers from Sketchy for M. Meningitis. as respiratory droplets are the easiest to transmit from person to person.
+
drschmoctor
but the poop water comes from people so....
+1
llamastep1
Respiratory dropplets is easier than fecal-oral tho
+2
lowyield
Can also reason that n. meningitidis is common in college students because they live in close quarters which suggests high rate of transmission even amongst immunocompetent individuals
+2
peridot
I can see why fecal-oral can seem like person-to-person transmission. What helped me reason it was that in countries with lots of cases of cholera, the primary reason is lack of water sanitation. Even when you google cholera, you get pictures of people collecting dirty water and how the WHO is aiming to reduce cases of the disease by improving water sources. Therefore it's more of a systemic/environmental problem rather than the fact that one person accidentally touched another person's poopy parts and then transmitted it to their own mouth, making this less of a person-to-person thing, especially when compared to another answer choice such as Meningococcal meningitis.
+
bbr
To add, think of the water in cholera as a reservoir. The bug is going to hang out there between infecting another person. In meningitis it seems we are going from 1 persons saliva to another. Without much of a reservoir inbetween. (might be using the word reservoir incorrectly).
+1
weirdmed51
Rocky Mountain spotted fever: dermacenter TICK
+
smc213
To clarify Methotrexate (inhibits dihydrofolate reductase) and 5-FU (inhibits thymidylate synthase) in the Pyrimidine synthesis pathway.
6-MP inhibits Glutamine PRPP amidotransferase in the PURINE synthesis pathway
+7
chaosawaits
ohhhhhh my dumb ass was thinking about integrins and so I went with disulfide bond formations.
+
kcyanide101
I have seen a Uworld Q, where there listed Amino Acids and asked to identify which of them is a part of the cell membrane component
+
yb_26
O-linked glycosylation of secreted and membrane bound proteins is a post-translational event that takes place in the cis-Golgi compartment after N-glycosylation and folding of the protein
+21
usmleuser007
in a per-protocol analysis,[6] only patients who complete the entire clinical trial according to the protocol are counted towards the final results
+1
sympathetikey
"In an ITT population, none of the patients are excluded and the patients are analyzed according to the randomization scheme."
+7
teepot123
the video explains it well, no need for fa
+1
smc213
Septic shock can also present with hypothermia <36C
+3
bethune
Why is it not gastrointestinal bleeding?
+4
beanie368
GI bleeding would present with increased SVR as a response to hypovolemia
+10
step1passfail
Pulmonary embolism would cause a decrease in cardiac output. There is increased pressure in the high compliant RV which can bulge and compress the LV, decreasing its preload. CO=Heart rate x stroke volume and stroke volume is partially determined by preload.
If the pulmonary embolism is large enough, it can also obstruct the pulmonary vessels and subsequently not have enough blood going to the LA and LV, ultimately making the cardiac output near 0.
+3
chj7
Out of all the different types of shock, cardiac output is increased only in distributive shock (ie. anaphylactic, septic).
+1
smc213
Increased PTHrP seen in squamous cell lung cancer leads to increased Ca2+ levels
+10
sherry
That's exactly what I was thinking when I was taking the test. But I was sidetracked by same HCO3 level. Can somebody explain this part to me??
+
charcot_bouchard
Because salivary duct removes Na & Cl while secrete K & Hco3 in lumen. In low flow rate HCO3 & K inc because duct is doing its thing for more time. At high flow rate K slightly dec (as cant be secrted as much) but HCO3 stays almost same. the reason is high flow indicates higher metabolism & higher bicarb production.
+
cienfuegos
Regarding the bicarb (via BRS Physiology, which explains flow rate as coming down to "contact time" where slow flow allows more reabsorption of NaCl): The only ion that does not “fit” this contact time explanation is HCO3−; HCO3− secretion is selectively stimulated when saliva secretion is stimulated.
+3
asapdoc
Im pretty sure so is strept pneumoniae
+4
usmleuser007
COPD is also exacerbated by Viral infection: Rhinovirus, influenza, parainfluenza; and Bacterial infection: Haemophilus influenzae, Moraxella catarrhalis, Streptococcus.
however, the questions gives a hint that it may be legionella = "weekend retreat" which may be associated with this infection
+5
loopers
From FA 2017 pg 139: Legionnaires’ disease—severe pneumonia (often unilateral and lobar A ), fever, GI and CNS symptoms. Common in smokers and in **chronic lung disease.**
+2
kentuckyfan
I also believe that the other attendees showed signs of pontiac fever, which is another hint they tried to get at.
+3
luke.10
i did it wrong and chose influenza virus since it is most common infection in COPD but the clue in the Question is that the other attendee didnt get sick since in legionella there is no person to person transmission
+2
endochondral
but in Uworld s. pneumo is one of the most common bacterial exacerbation of COPD legionella wasn't even mentioned. How do we rule out s. pneumo ?
+5
nala_ula
maybe because in children s.pneumo causes otitis media?
+1
smc213
Another hint made in the Q stem is the location being rural Pennsylvania.... Legionnaires disease was first discovered by the outbreak in 1976 at a convention held in Philadelphia, Pennsylvania. Not sure why I know this fact...
+10
hpsbwz
Biggest hint towards legionella to me was that they all were at a residence hall... i.e. where there'd be air conditioners and such.
+6
stresssweat
it says that other people developed pneumonia without needing to be hospitalized and that some people were asymptomatic. You would think walking pneumonia - Legionella
+
smc213
FA18 p.657 bird exposure--> HSN pneumonitis (restrictive lung disease) and FA18 p.214 granulomatous diseases: foreign material-->HSN pneumonitis. I had to make sense of it since I didn't know it was HSN pneumonitis at first.
+5
masonkingcobra
From Robbin's:
Fibromuscular dysplasia is a focal irregular thickening of the walls of medium-sized and large muscular arteries due to a combination of medial and intimal hyperplasia and fibrosis. It can manifest at any age but occurs most frequently in young women. The focal wall thickening results in luminal stenosis or can be associated with abnormal vessel spasm that reduces vascular flow; in the renal arteries, it can lead to renovascular hypertension. Between the focal segments of thickened wall, the artery often also exhibits medial attenuation; vascular outpouchings can develop in these portions of the vessel and sometimes rupture.
+1
asapdoc
I thought this was a weirdly worded answer. I immediately ( stupidly) crossed of fibromuscular dysplasia since it wasnt a younger women =/
+26
uslme123
I was thinking malignant nephrosclerosis ... but I guess you'd get hyperplastic arteries first -_-
+1
hello
The answer choice is fibromuscular HYPERplasia - I think this is different from fibromuscular DYSplasia (seen in young women);
+36
yotsubato
hello is right. Fibromuscular hyperplasia is thickening of the muscular layer of the arteriole in response to chronic hypertension (as the question stem implies)
+10
smc213
Fibromuscular Hyperplasia vs Dysplasia...... are supposedly the SAME thing with multiple names.
Fibromuscular dysplasia, also known as fibromuscular hyperplasia, medial hyperplasia, or arterial dysplasia, is a relatively uncommon multifocal arterial disease of unknown cause, characterized by nonatherosclerotic abnormalities involving the smooth muscle, fibrous and elastic tissue, of small- to medium-sized arterial walls.
http://www.medlink.com/article/fibromuscular_dysplasia
+3
smc213
*sorry I had to post this because it was confusing!!!*Fibromuscular dysplasia is most common in women between the ages of 40 of and 60, but the condition can also occur in children and the elderly. The majority (more than 90%) of patients with FMD are women. However, men can also have FMD, and those who do have a higher risk of complications such as aneurysms (bulging) or dissections (tears) in the arteries.
https://my.clevelandclinic.org/health/diseases/17001-fibromuscular-dysplasia-fmd
+2
momina_amjad
These questions are driving me crazy- fibromuscular dysplasia/hyperplasia is the same thing, and it is NOT this presentation and it doesn't refer to arteriolosclerosis seen in malignant HTN!
Is the HTN a cause, or a consequence? I read it as being the cause (uncontrolled HTN for many years)
If it was the consequence, the presentation is still not classical! -_-
+2
charcot_bouchard
Also guys if u take it as Fibromuscular dysplasia resulting in RAS none of the answer choice matches
+1
masonkingcobra
From Robbin's:
Fibromuscular dysplasia is a focal irregular thickening of the walls of medium-sized and large muscular arteries due to a combination of medial and intimal hyperplasia and fibrosis. It can manifest at any age but occurs most frequently in young women. The focal wall thickening results in luminal stenosis or can be associated with abnormal vessel spasm that reduces vascular flow; in the renal arteries, it can lead to renovascular hypertension. Between the focal segments of thickened wall, the artery often also exhibits medial attenuation; vascular outpouchings can develop in these portions of the vessel and sometimes rupture.
+1
asapdoc
I thought this was a weirdly worded answer. I immediately ( stupidly) crossed of fibromuscular dysplasia since it wasnt a younger women =/
+26
uslme123
I was thinking malignant nephrosclerosis ... but I guess you'd get hyperplastic arteries first -_-
+1
hello
The answer choice is fibromuscular HYPERplasia - I think this is different from fibromuscular DYSplasia (seen in young women);
+36
yotsubato
hello is right. Fibromuscular hyperplasia is thickening of the muscular layer of the arteriole in response to chronic hypertension (as the question stem implies)
+10
smc213
Fibromuscular Hyperplasia vs Dysplasia...... are supposedly the SAME thing with multiple names.
Fibromuscular dysplasia, also known as fibromuscular hyperplasia, medial hyperplasia, or arterial dysplasia, is a relatively uncommon multifocal arterial disease of unknown cause, characterized by nonatherosclerotic abnormalities involving the smooth muscle, fibrous and elastic tissue, of small- to medium-sized arterial walls.
http://www.medlink.com/article/fibromuscular_dysplasia
+3
smc213
*sorry I had to post this because it was confusing!!!*Fibromuscular dysplasia is most common in women between the ages of 40 of and 60, but the condition can also occur in children and the elderly. The majority (more than 90%) of patients with FMD are women. However, men can also have FMD, and those who do have a higher risk of complications such as aneurysms (bulging) or dissections (tears) in the arteries.
https://my.clevelandclinic.org/health/diseases/17001-fibromuscular-dysplasia-fmd
+2
momina_amjad
These questions are driving me crazy- fibromuscular dysplasia/hyperplasia is the same thing, and it is NOT this presentation and it doesn't refer to arteriolosclerosis seen in malignant HTN!
Is the HTN a cause, or a consequence? I read it as being the cause (uncontrolled HTN for many years)
If it was the consequence, the presentation is still not classical! -_-
+2
charcot_bouchard
Also guys if u take it as Fibromuscular dysplasia resulting in RAS none of the answer choice matches
+1
sympathetikey
I would say this is Lynch Syndrome (APC is usually thousands of polyps) but lynch syndrome would generally have a family history of other cancers as well, so you might be right. Either way, both autosomal dominant so win win.
+3
smc213
uptodate states: Classic FAP is characterized by the presence of 100 or more adenomatous colorectal polyps
+
dickass
@sympathetikey Lynch Syndrome is literally called "Hereditary NON-POLYPOSIS colorectal cancer"
+14
fatboyslim
I think this actually is Lynch syndrome. Lynch syndrome can also develop colonic polyps but not nearly as bad as FAP. FAP has so many polyps you can't even see the normal mucosa. If you Google Lynch colonoscopy you can see that they develop a few polyps.
+
rockodude
I forgot it was AD inheritance but regardless at the time I was confused because APC is a tumor suppressor so it needs two hits. I guess AD inheritance and then you need another hit to develop CRC kind of like familial retinoblastoma or li fraumeni syndrome
+
johnthurtjr
Have you mixed Chlamydia in with Mycoplasma?
+4
smc213
I mean the Q stem is not about Chlamydiae, but Chlamydiae does lack the classic PTG cell wall d/t decreased muramic acid = beta-lactam abx ineffective. FA 2018 p.148
+2
missi199
Could I ask why it is not Lysosomal protease
+8
smc213
"Certain viruses have evolved to recruit the cellular E3 ligases to induce the degradation of cellular proteins that might have harmful effects on the viral life cycle. For instance, the protein E6 of Human papillomavirus (HPV) recruits the cellular E3 ubiquitin ligase E6-AP to induce ubiquitination and degradation of p53, thereby allowing viral replication."
from: https://www.mdpi.com/1999-4915/9/11/322/htm
+5
smc213
USMLE Kaplan: A majority of cellular
proteins are degraded via the ubiquitin proteasome pathway, including many
proteins that play a role in maintaining cellular homeostasis. These include
proteins that regulate the cell cycle, apoptosis, etc.
+4
missi199
Could I ask why it is not Lysosomal protease
+8
smc213
"Certain viruses have evolved to recruit the cellular E3 ligases to induce the degradation of cellular proteins that might have harmful effects on the viral life cycle. For instance, the protein E6 of Human papillomavirus (HPV) recruits the cellular E3 ubiquitin ligase E6-AP to induce ubiquitination and degradation of p53, thereby allowing viral replication."
from: https://www.mdpi.com/1999-4915/9/11/322/htm
+5
smc213
USMLE Kaplan: A majority of cellular
proteins are degraded via the ubiquitin proteasome pathway, including many
proteins that play a role in maintaining cellular homeostasis. These include
proteins that regulate the cell cycle, apoptosis, etc.
+4
merpaperple
It's not necessarily late dumping syndrome, this is the dietary guideline for early dumping syndrome too. Based on UpToDate and ScienceDirect this is how it works:
Absent or dysfunctional pyloric sphincter
-> food is rapidly emptied from the stomach into the small bowel
-> hypertonic solution forms in the jejunum
-> rapid fluid shifts from the plasma into the bowel
-> hypotension and SNS response (eg. colicky abdominal pain, diarrhea, nausea, tachycardia)
Simple carbohydrates are more hypertonic, I think.
https://www.sciencedirect.com/topics/medicine-and-dentistry/dumping-syndrome
+5
j44n
starches are complex carbs= more than 2-3 sugar molecules, if they have dumping syndrome they have decreased gastric transit time= more undigested carbs are delivered to the intestines and that gives you more carbs for bacteria to break down (flatulence and osmotic diarrhea)
+
To be completely clear!
This patient has Cystinosis a rare autosomal recessive lysosomal storage disorder and most common cause of Fanconi syndrome in children. Cystinosis is systemic and leads to cystine crystal deposits in cells and tissues throughout the body.
Although Wilsons disease can lead to FS, the crystals in the corneas does not correlate with Wilsons disease.
More info: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4841061/