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How do you decide when to obtain genetic testing for suspected autoinflammatory conditions in adults?

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Rheumatology · Arthritis Associates

We have identified an autoinflammatory disorder that occurs in people of the Southern Appalachian mountains known as "Melungeons." Several have been evaluated at NIH, and while they do not have any of the known markers the diagnosis of familial Mediterranean fever has been confirmed. Dr. Kastner tol...

How long after surgical repair of a pleuroperitoneal leak do you resume peritoneal dialysis?

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Nephrology · UCHealth University of Colorado Hospital (UCH)

This is a bit of a challenging question as I'm not aware of any data to help guide my response. It seems to me however that, since failure of the surgical repair would inevitably culminate in permanent shift to HD, one should allow ample time for healing to occur and thereby to achieve the best poss...

What factors guide you in adjusting the dose of fludrocortisone in a patient with primary adrenal insufficiency?

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Endocrinology

Adjustment of the dose is generally based on clinical factors. Orthostatic blood pressure should be measured on each visit with normalization of blood pressure as a goal. Also electrolytes should be measured each visit to make sure potassium levels are normal and a supplement added if need it. Lower...

Do you recommend Moonstone supplements for patients with recurrent calcium oxalate nephrolithiasis who have hypocitraturia and mild hyperkalemia for which potassium citrate is contraindicated?

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Nephrology · NYU Grossman School of Medicine

First, my conflict of interest: I am one of the inventors of Moonstone so I do have a financial interest in sales of the product. But 30 mEq of Moonstone product has only 7 mEq of K, so I consider it a useful way of alkalinization if serum K is an issue. And only 10 mEq of Na, the rest Mg. By spread...

If a patient who has previously been taking lamotrigine stops taking it for between two weeks and a month, do you have to uptitrate it all over again or do you have another way to resume it?

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Neurology · University of Rochester Medical Center

I'm not aware of good data to answer this question. To be safest, I would probably titrate again at the usual schedule as though starting from scratch. In the epilepsy monitoring unit, we're typically comfortable with restarting at-home doses without retitrating up to 14 days off meds (the lower lim...

How would you approach the workup and management of a young patient with recurrent biannual non-scarring oral ulcers and new onset neurologic symptoms with associated CNS white matter lesions concerning for Behcet’s?

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Rheumatology · Massachusetts General Hospital

This is a challenging case. It should be noted that oral ulcers in Behcet's typically occur more than twice per year (by ISG criteria should occur at least 3x per year) and without other symptoms of BD it can be very challenging to make a probable diagnosis of BD in this scenario. A careful history ...

Do you recommend SGLT2i use for patients with chronic kidney disease, diabetes mellitus, and polycystic kidney disease? 

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Nephrology · UCSF

While we cannot conscientiously recommend this YET, I do not think it is necessarily contraindicated. I would not use it in conjunction with tolvaptan, but I think the results of the UC Denver trial will be informative. It may indeed be as useful for PKD patients as in other CKD patients, including ...

Are there patients with granulomatosis with polyangiitis on maintenance rituximab therapy for whom you do not co-administer glucocorticoid therapy?

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Nephrology · Ohio State Department Of Nephrology

I think this is a great question. @Dr. First Last et al (NCT01933724) have conducted a study to answer that question (conveniently called TAPIR). In their analysis that was presented 1 week ago at the International Vasculitis Workshop, they found that patients on low-dose prednisone along with other...

Do you extend the duration of maintenance therapy past 24 months for patients with ANCA glomerulonephritis who have multiple organ involvement?

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Nephrology · Ohio State Department Of Nephrology

The duration of maintenance therapy in patients with AAV depends on many factors and should be individualized. Some factors that are associated with a higher risk of relapse include PR3 positivity, seroconversion from negative to positive, ENT disease, use of a tailored approach to RTX dosing, and u...

How do you risk stratify patients with different WHO groups of pulmonary hypertension prior to non-cardiac surgeries?

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Pulmonology · Hospital of the University of Pennsylvania

First, I would direct the audience to recent AHA guidelines on the perioperative management of PH in non-cardiac surgery. Rajagopal et al., PMID 36924225In general, the severity of pulmonary hypertension and relevant comorbidities are likely more important than the WHO group. In patients with CTEPH,...