Brad is a fill in for Samir Parik
He has massive COI all over the place. Every drug. Every company. #NKFClinicals
Starts out with a case. Cr up from 0.6 to 1.2. 3500 mg of protein. Biopsy is WHO 4 and 5. She does not respond to MMF and steroids. Then does not respond to CYT. What to do?
Beware of getting on the roulette wheel of immunosuppression
An operational definition of refractory lupus nephritis.
Less than 20% of people are taking the drugs at 12 months. This is a huge problem. #NKFClinicals
HCQ levels are particularly useful. HCQ has along half live, 7-40 days so a low or zero level cannot be attributed to forgetting a dose. Similarly, just taking the drug before going to the doctor will not result in a therapeutic level. #NKFClinicals
Improve adherence. Good luck. Cost reduction with belimumab and voclosporin 🤣 #NKFClinicals
Be patient it takes several months to get response. Late response is possible. But should see some movement (25% reduction in proteinuria) in 2-3 months. #NKFClinicals
He just described the KDIGO guidelines that came out last fall as “In the old days” #NKFClinicals
The new therapies (voclosporin and belimumab) did not test on refractory lupus. But there are post-how analysis…one interpretation is best response to people with prior immunosuppression exposure. #NKFClinicals
The best data for rescue therapy is with rituximab. After that…welcome to the Wild West. All kinds of things have been tried from CASR-T to stem cell to leflunomide, anti-IL-2, etc. #NKFClinicals
Intensive B-Cell depletion. Impressive, but N=12 #NKFClinicals
Back to the case. Patient went for repeat biopsy and turned out immunocompromised were gone and she now had TMA from antiphospholipid antibody syndrome. #NKFClinicals
Summary slide
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Next session started by Rovin to talk about IGAN pathophysiology and the selection of therapeutics
4-hit model 1. formation of IGA galactose deficient 2. formation of autoantibodies against these IgA 3. Formation of circulating IgG-IgA1 immune complexes 4. Deposition of the immune complexes in the kidney
#RKDSummit
1st case
Hematuria on U/A
Gross hematuria after covid vaccine
10 RBC/HPF, no casts
Scar 0.9 mg/dl, 24-hr urine 750 mg of protein
#RKDSummit
A bit “Juicy”
Mesangial expansion (arrows, fig 1)
Mesangial hypercellularity (circle, fig 2)
Biopsy has no chronicity (fig 3)
Lights up with C3 and IgA (fig 4)
When we published our study <> of ODS and hyponatremia we were pummeled for including people at low risk of ODS because we included Na levels between 120 and 130. They said it is well known "that ODS is incredibly rare/non-existent at those levels." 1/4evidence.nejm.org/doi/10.1056/EV…
Of course one of the reasons it was thought to be incredibly rare was that no one looked for CPM in patients with Na from 120-130. We found a fair number (≤5 of 12). 2/4
Our findings are replicated in a study from Australia. The authors took a different approach to investigating ODS. Instead of starting w/ hyponatremia and working forward to ODS, they started with a dx of ODS and worked backwards
3/4ncbi.nlm.nih.gov/pubmed/35717664
Gadolinium in dialysis patients.
What's up with that?
#Tweetorial
1/11
Nephrogenic systemic fibrosis (NSF) is an iatrogenic disease that presents with hardening of the skin and other organs. It is often lethal. I treated 5 people with this condition (including one with AKI). Terrible.
2/11
The etiology of NSF was unknown and there were many theories. In 2006, Thomas Grobner published a small case series showing 5 patients developing NSF within weeks of receiving gadolinium contrast for MRI.
3/11pubmed.ncbi.nlm.nih.gov/16431890/
I just recently recommended the Renal Physiology book by Bruce Koeppen and Bruce Stanton. I thought it was a good medical student level text book: pbfluids.com/2023/08/ouwb-s…
But I came across this question in Chapter 8 Regulation of Acid Base. It is a straight forward question asking the learner to interpret simple acid-base cases.
But the question falls apart when you look at the answer...