RE: Treatment of progressing Fabrys 12 minutes ago

Really grateful for this erudite discussion! ------------------------------ Valerie Luyckx MD, MS, PhD Renal Division, Brigham and Women;s Hos...

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RE: Treatment of progressing Fabrys 13 minutes ago

BP is around 120 systolic but I have ordered a 24h ABPM, if any higher I was planning an ACEI…cardiac MRI is totally normal ---------------------...

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RE: Treatment of progressing Fabrys 15 minutes ago

Thank you! ------------------------------ Valerie Luyckx MD, MS, PhD Renal Division, Brigham and Women;s Hospital, Boston, MA; Department of Paed...

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RE: Treatment of progressing Fabrys 18 minutes ago

I am hoping this may be the case ;) ------------------------------ Valerie Luyckx MD, MS, PhD Renal Division, Brigham and Women;s Hospital, Bo...

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RE: C5 blocker in IgA and other controversial recommendations 1 hour ago

Dr. Dastoor- thanks for your detailed response- very helpful. Here are somexcomments. 1) you might find a better response to oral systemic ster...

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RE: Denovo Idiopathic MPGN of Renal Allograft 1 hour ago

And in this case there is dual pathology and if this is idiopathic Mpgn with rejection what to use to treat ? Tac and steroids and mmf ? -----...

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RE: C5 blocker in IgA and other controversial recommendations 1 hour ago

Dr. Dastoor- Your point #1 above, specifically stating that excluding all cost considerations all patients with IgAN (except these with an Oxford...

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RE: Denovo Idiopathic MPGN of Renal Allograft 1 hour ago

Thanks for case . Rejection and immune complex gn What induction agents was used in first and second transplant ? Please keep us updated as to out...

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RE: C5 blocker in IgA and other controversial recommendations 1 hour ago

I agree- the UAE is ahead of the USA. I suppose, that off-label use of Ravulizumab (like Eculizumab ) could ocurr, but not likely with insurance co...

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RE: Treatment of progressing Fabrys 2 hours ago

No elevated BP according to the original post. "Slippery slope" vs "Don't just stand there -do something". A common dilemma (or is it a conundrum! ...

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RE: Treatment of progressing Fabrys 3 hours ago

Thanks Dr Glassock, My thoughts on using RASi or SGLT2i are based solely on him being 20 and having progressive glomerular sclerosis already and ...

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RE: C5 blocker in IgA and other controversial recommendations 3 hours ago

I have not used ravulizumab in patients with IgA nephropathy. It is not yet FDA-approved for IgA nephropathy in the US. -------------------------...

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RE: C5 blocker in IgA and other controversial recommendations 3 hours ago

Thanks Dr. Abdeknour for your nice summary of how you use C inhibitors in your patients with IgAN. Just curious- have you accumulated any experienc...

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RE: C5 blocker in IgA and other controversial recommendations 4 hours ago

Dr. Dastoor, thank you for sharing your experience and reasoning so openly. This is a useful discussion as our options expand faster than the compa...

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RE: Treatment of progressing Fabrys 8 hours ago

Thanks Dr. Rodby (and Open Evidence) for adding to this already illuminating discussion of progression of renal functional decline and global Glome...

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RE: Treatment of progressing Fabrys 9 hours ago

Open evidence: "Does Enzyme replacement therapy prevent progression of CKD in Fabry?" Seems starting at age 10 should have helped more but it may...

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RE: Treatment of progressing Fabrys 16 hours ago

Thank you all. I had found the same info that ERT does not prevent ongoing podocyte injury. He has never had any microalbuminuria Sent from my iPh...

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RE: Treatment of progressing Fabrys 23 hours ago

Dr. Lerman, the following reference from the AI response is relevant to your question:: https://pmc.ncbi.nlm.nih.gov/articles/PMC10232004/ it a...

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RE: Treatment of progressing Fabrys 23 hours ago

Could absence of glomerular hypertrophy & proteinuria reflect converse of Brenner hypothesis (given ׳very tall’, ie height-correlated nephron over-...

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RE: Treatment of progressing Fabrys 23 hours ago

Dr. Venkat did your AI suggest any additional treatment? ------------------------------ [Mark] [Lerman] [MD,FASN,FACP,FAST] AST KPCOP EC [Past Me...

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RE: Treatment of progressing Fabrys 1 day ago

Dr. Venkat- many thanks. Hard to postulate progressive podocyte injury in absence of proteinuria (albuminuria ). Could this be a delayed response t...

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RE: Treatment of progressing Fabrys 1 day ago

Thanks for posting this case. I did a Google AI search with the following question: "Findings on serial kidney biopsies in Fabry's patients on cont...

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RE: Treatment of progressing Fabrys 1 day ago

Thanks for the quick reply. It might help to examine the sclerotic glomeruli more carefully. If they are of a solidified character they are not par...

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RE: C5 blocker in IgA and other controversial recommendations 1 day ago

Dr. Dastoor- thanks for your detailed response- very helpful. Here are somexcomments. 1) you might find a better response to oral systemic ster...

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RE: Treatment of progressing Fabrys 1 day ago

Antidrug antibodies have been considered, not yet done the sclerosed glomeruli are described as atrophic, no hypertrophy described IF: IgG and Ig...

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RE: Treatment of progressing Fabrys 1 day ago

What did the IF show? Does the patient have any anti-drug antibodies? At what age was therapy started? Please give the serum creatinine values ay ...

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Treatment of progressing Fabrys 1 day ago

I would be grateful for some suggestions. We have a patient with Fabrys who has been on Fabrizyme for around 10 years, on the maximal dose. We...

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RE: GLP -1 RA question 1 day ago

Thanks prof glsssock --------------------------------- Muhammad Soobadar MBChB UK ---------------------------------

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RE: nephrotic syndrome 1 day ago

Thanks for case Why is there increased bleeding risk ( related to biopsy ) with using VEGF inhibitor? I know there might be data that patients ...

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RE: C5 blocker in IgA and other controversial recommendations 1 day ago

thanks for the question.. we have 5 patients lined up for Ravulizumab . They all have had one or two cycles of oral steroids based on low dose TE...

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RE: C5 blocker in IgA and other controversial recommendations 1 day ago

Thanks- very clear. Why did you use Ravulizumab first in the case you posted.? Was there some clinical or morphological feature that drove you make...

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RE: salt losing nephropathy 1 day ago

Thanks dr. Mellas for the clarification. --------------------------------- Dr. Hormaz Dastoor. MD, MSc, FASN Divisional Head of Nephrology Sheik...

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RE: C5 blocker in IgA and other controversial recommendations 1 day ago

Thank u for your comments . I use the same criteria as recommended in Uptodate . We identify 4 high risk factors - 1. Hematuria 2. Proteinuria mor...

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RE: GLP -1 RA question 1 day ago

The easy answer to your Question is YES , in my opinion. But the severity of Diabetic Retinopathy should not be used as an argument against better ...

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RE: C5 blocker in IgA and other controversial recommendations 1 day ago

Dr. Dastoor- thank you for your comments and for opening a dialogue about when to use potent (and sometimes very expensive ) immunosupprressive/ an...

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RE: GLP -1 RA question 1 day ago

Thanks prof lerman and prof glassock Found interesting article https://pmc.ncbi.nlm.nih.gov/articles/PMC6587545/ If someone has proliferative d...

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C5 blocker in IgA and other controversial recommendations 2 days ago

3 days ago , the UAE , was the first country to officially approve Ravulizumab for IgA nephropathy , and we injected our first patient with the med...

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RE: Unexplained Renal Failure or Not 2 days ago

Very interesting theoretical question. Would repetitive episodes of low grade rhabdomyolysis and myoglobinuria predispose to heme-Fe -related tubul...

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RE: Unexplained Renal Failure or Not 2 days ago

No unlikely from AI • Persistent mild CK elevation and trivial myoglobinuria (26 vs [More]

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RE: Unexplained Renal Failure or Not 2 days ago

agree but he does have persistent mildly elevated CK without exercise and elevated uric acid so metabolic -muscle workup as I also suggested Number...

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RE: Unexplained Renal Failure or Not 2 days ago

Would this persistent low level rhabdomyolysis and myoglobinuria damage his kidney in the long run ?? --------------------------------- Dr. Horma...

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RE: Unexplained Renal Failure or Not 2 days ago

not an issue DR. GLASSOCK you are amazing -thanks for all you and Dr. Rodby do by creating this forum !!! ------------------------------ [Mark] [...

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RE: Unexplained Renal Failure or Not 2 days ago

We agree 100%. Sorry I missed your point in a prior post. ------------------------------ Richard Glassock MD, FASN Emeritus Professor Geffen Scho...

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RE: Unexplained Renal Failure or Not 2 days ago

"He does not have CKD- as he does not meet any of the criteria used for making this "diagnosis". -almost exactly what I said Number 5 above. Dr. ...

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RE: Unexplained Renal Failure or Not 3 days ago

He does not have CKD- as he does not meet any of the criteria used for making this "diagnosis". ------------------------------ Richard Glassock M...

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RE: Unexplained Renal Failure or Not 3 days ago

In the general population the eGFR Cystatin C is no more accurate the eGFR creatinine in estimating the level of measured GFR. The combination of t...

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RE: Unexplained Renal Failure or Not 3 days ago

I think one major issue is exercising at 50 degree C. You don't need a muscle disorder to produce AKI at that level. It may be better for him to ex...

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RE: Unexplained Renal Failure or Not 3 days ago

I dont think he has chronic kidney disease. His cystatin C is normal with normal eGFR of 125 ml/min. Despite the fact that he is on ARBs with diure...

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RE: Unexplained Renal Failure or Not 3 days ago

Or do genetic screening for the mutant PYGM allele. ------------------------------ Richard Glassock MD, FASN Emeritus Professor Geffen School of ...

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RE: Unexplained Renal Failure or Not 3 days ago

You might want to consider a muscle biopsy for glycogen deposits (McArdle disease) in light of exercise induced rhabdomyolysis. -----------------...

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