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What are your recommendations for adult sickle cell patients who end up being admitted several times a year for pain crises despite hydroxyurea, crizanlizumab, voxelotor, etc?

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Hematology · Boston University School of Medicine

This is the problem that vexes all people treating sickle cell disease. First, is to be sure that drug dosing is optimized. Both voxelotor and crizanlizumab can be added to hydroxyurea. All three drugs could be used together. Perhaps equally important as drug therapy is to cope as best as possible w...

What is your approach to abbreviated DAPT in post-PCI patients (ACS and non-ACS) with high bleeding risk?

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Cardiology · Brigham Health Inc

Current guidelines suggest dual antiplatelet therapy (DAPT) for a minimum of 6 months after PCI for patients undergoing PCI for stable CAD (non-ACS) and 1 year of DAPT after PCI for patients undergoing PCI for ACS. Patients at higher bleeding risk (HBR) after PCI, however, may not be able to tolerat...

When is a kidney biopsy warranted in a patient with possible scleroderma renal crisis?

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Rheumatology · Mayo Clinic

Scleroderma renal crisis (SRC) is a relatively early complication of Systemic sclerosis that almost invariably occurs within the first five years after the onset of the disease and may even be the initial manifestation of SSc. Abrupt onset of moderate to marked hypertension and acute kidney injury w...

Would you recommend mycophenolate mofetil for patients with progressive IgA nephropathy who do not tolerate corticosteroids?

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Nephrology · King Abdul-Aziz Medical City, Ministry of National Guards Health Affairs

Multiple studies showed no benefit of immunosuppressive agents (MMF, CYC), so this study is standing alone with relatively small number and limited centers. Better to role those IgAN patients in current ongoing studies, yet, I won't blame trying MMF if appropriate conservative management is optimize...

What is your PTH threshold for referring an ESKD patient with secondary hyperparathyroidism on maximum medical therapy for parathyroidectomy?

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Nephrology · Robert Wood Johnson University Hospital

I think PTX should be done pretty much never with PTH <800 and most of the time with values >2000. Why such a large range? The biggest consideration is symptoms; if present, my threshold approaches 800. However, ascribing symptoms to hPTH is problematic. Hypercalcemia is the most specific finding wi...

Would you be comfortable using a JAK inhibitor in a patient with baseline thrombocytosis?

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Rheumatology · Harvard Medical School

Knowing the etiology of this patient's thrombocytosis is a critical piece of information required before determining whether a JAK- inhibitor drug could be safely prescribed. Mutations in JAK-2 are responsible for several myelodysplastic disorders, some of which present with thrombocytosis. Therefor...

What is your approach to initial work up for a young patient with bronchiectasis?

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Pulmonology · Virginia Commonwealth University

I assume we are talking about patients in their 20s, 30s-50s who have had a HRCT with radiological bronchiectasis. It will be good to know if they have had clinical implications and symptoms of cough, infections, and mucus production. It will be good to know about multiorgan involvement like sinuses...

What is the best approach to manage iron overload secondary to both heterozygous HFE gene mutation and two heterozygous aceruloplasminemia gene mutations?

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Hematology · Georgetown University School of Medicine

The question is good as both heterozygotes for HFE and double heterozygotes (I suspect the same applies for aceruloplasminemia) for HFE are usually invisible. That being said, not always. What I do, if the increased iron is not urgent (normal LFTs, ferritins <1,500), is get them to become blood dono...

What is your approach to management of elderly patients with cardiopulmonary comorbidities and severe pulmonary hypertension?

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Pulmonology · Hospital of the University of Pennsylvania

I generally follow the current ERS/ESC guidelines with regard to the treatment of patients with WHO group 1 PAH. I often will often start with a low dose of PDE5i and see them back within 4 weeks prior to increasing the dose. Similarly, I will see back within 4 weeks of adding an ERA to look for sid...

What is your approach to using nintedanib in patients on baseline immunosuppression?

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Pulmonology · University of North Carolina @ Chapel Hill

Typically I start antifibrotic therapy in a few situations: The most common reason is ILD progression despite adequate immune suppression, defined as no extra-pulmonary disease activity (usually joint disease, but can tailor according to the patient's disease/situation, such as by presence of rash, ...