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myoclonictonicbionic
Thats the reason I put MBL, because the question mentioned that it got worse when she went to the beach so I was thinking some sort of contact with bacteria may have exacerbated her immune system.
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thotcandy
@myoclonictonicbionic i think that's just the typical SLE photosensitive malar/butterfly rash
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btl_nyc
So I thought this was Marfan's because the murmur from HOCM is at the left sternal border, but Marfan's is a defect in fibrillin, not in collagen.
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arcanumm
To help rule out Marfran's, it is stated that there are "no history of major medical illness," which I wouldn't expect them to put if there was a syndrome going on. (they also tend to give body habitus descriptors at least)
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dul071
This isn't HOCM, rather it's simply Mitral stenosis. He has a murmur that radiates at the apex which happens to be the Mitral area. Despite everything his BLOOD PRESSURE AND PULSE are normal. The heart is over working to keep the vitals normal and as a consequence, it is undergoing hypertrophy which dictates the answer
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dna_at
@dull071 I don't think this is MS. That would be 1) diastolic and not systolic, 2) less likely to cause LVH. I believe as others said it is just HOCM leading to MR, which is what we are hearing. MR secondary to HOCM would still increase in intensity with less preload as there would be more LVOT obstruction (thus more regurgitation)
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fatboyslim
@dul071 and @dna_et: HOCM causes a systolic murmur because the intraventricular septum hypertrophies to a point where it obstructs LV outfow, especially when there is less preload in the LV because the septum has less resistance against it to compress the LV. It causes a "sub-aortic narrowing", causing a systolic murmur. It is WORSENED with LESS preload (unlike most heart murmur) and SOFTENED with INCREASED preload or INCREASED afterload (increased afterload makes the LV have to pump harder and that pushes the septum away -> less subaortic narrowing).
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burningmoon
How about emphysema? airway volume changed but it's obstructive.
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almondbreeze
i think OP meant to say that something DECREASING airway volume = restrictive
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jgraham3
I think they mean if something is impacting LUNG volume (ie. expansion/compliance) = restrictive
Airway disorder --> obs. / Parenchymal disorder --> res.
With emphysema the airway collapses (obs.) before they are able to exhale fully thus the air is trapped
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dna_at
Just to be clear, this is not a classical obstructive lung disease affecting the small airways, as it is above the carina (trachea). This is better classified as a fixed upper airway obstruction. See the flow loop here for "fixed obstruction" - it came up in IMED UWorld so maybe familiarize yourself with the image since it is unique! https://www.grepmed.com/images/2948
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notpennysboat
@dna_at, can you explain the concept of the upper airway obstructions? I'm still pretty confused
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Complement is important for removing immune complexes, so patients with complement deficiencies *(c1-c4) are more likely to develop SLE. C1q is a better answer than than MBL (D) b/c the MBL pathway is triggered by bacteria.