RE: C3 or IgA 3 hours ago

IgAN looks like it is splitting into multiple entities in the footsteps of FSGS. The O-glycosylation deficiency seemed to be giving IgA some cohere...

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RE: treatment for hypophosphatemia 5 hours ago

I also agree with Dr Rodby, although I would check a 24 hour urine for Phos, Ca, Na and Creatinine to make she has inappropriate hyperphosphaturia ...

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RE: C3 or IgA 18 hours ago

Thanks Dr. Hughson for your comment. I may have been too enthusiastic about the potential utility of KN 55 staining to help sort out the conundrums...

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RE: treatment for hypophosphatemia 19 hours ago

Sorry for my typing errors. Yes her age and ethnicity are also importan; ferritins and TSATs run much lower in China, Korea, and Japan and in most ...

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RE: treatment for hypophosphatemia 20 hours ago

Could you please specify what do you mean by iron exposure and why was the patient "exposed" to iron. If the patient received IV iron, was this fe...

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RE: treatment for hypophosphatemia 21 hours ago

I presume IV iron has been dicontinued and which IV iron as used? What are ger current iron parameters and Hb? ------------------------------ Ana...

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RE: treatment for hypophosphatemia 1 day ago

I agree 100% with Dr. Rodby's assessment and recommendations. ------------------------------ Richard Glassock MD, FASN Laguna Woods CA (949) ...

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RE: treatment for hypophosphatemia 1 day ago

This seems like a board question , almost too good, so depending on the IV iron it may stimulate FGF 23 (set attached) I would get a FGF 23 l...

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treatment for hypophosphatemia 1 day ago

Good afternoon, A pt with h/o IV iron exposure has developed hypophosphatemia. Is currently on 200 mg elemental phosphorus/day PO and iv kphos...

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RE: C3 or IgA 1 day ago

This exercise is generating a lot of misinformation. At least it has been decided that CFHR variants have a positive association with IgAN, not the...

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RE: C3 or IgA 2 days ago

KN 55 staining has high specificity but lower sensitivity for identifying Primary IgAN (or IgAV) so only positive staining would have pathogenic si...

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RE: C3 or IgA 2 days ago

I think it may be available at Arkana by request,. Both of these cases were outside the USA. ------------------------------ Richard Glassock MD, ...

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RE: ANCA vasculitis with Pulmonary Hemorrhage - To Rituximab or MMF 2 days ago

I discussed with hematologist, he is of the view to err on the side of caution in view of the HBV serology and closely watch HBV serology gor 6 mon...

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RE: C3 or IgA 2 days ago

How available is KM55 in the US? Most reports are from China, Japan and India. Thanks. Mario ------------------------------ Mario Rubin, M.D. Hou...

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RE: C3 or IgA 2 days ago

Thanks for allowing me to get a little more sleep. One way to better understand the two cases would be to stain the kidney biopsy with IgA deposits...

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RE: C3 or IgA 2 days ago

With a CFHR5 mutation, the patient could have presented with C3 or IgA . I would have expected Pegcetacoplan to have the pathways leading to expres...

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RE: C3 or IgA 2 days ago

I don't think we can have a proper answer to that. There are case reports of CFH mutations with C3GN becoming HUS after kidney transplant. These ar...

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RE: C3 or IgA 2 days ago

Thank you, but am still waiting for an explanation on how he went from C3 deposits , to IgA deposits after treatment with C3 inhibitors .. no point...

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RE: C3 or IgA 2 days ago

Centogene has reported it as a VUS. I think you should get this published to add to the literature on this variant. ClinVar has reported this as be...

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RE: C3 or IgA 2 days ago

Centrogene- from Germany has reported on it .. please see attachment --------------------------------- Dr. Hormaz Dastoor. MD, FASN Nephrology Co...

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RE: C3 or IgA 3 days ago

Thanks for the genetic update. CFHR5 486dup is described as a variant of uncertain significance. There are also reports stating that it is benign. ...

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RE: C3 or IgA 3 days ago

Thanks .. let's wait for the Professor to wake up and give a response ... I bet he is thinking over the possibilities while he lays in bed . ----...

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RE: C3 or IgA 3 days ago

Both hypothyses i.e, complement mutations confering susceptibility to IgA as well as C3GN and IgA-Antidrug antibodies make intuitive sense. My case...

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RE: C3 or IgA 3 days ago

Ok, great ... So to summarize this case , with his CFHR5 mutation he is at risk for C3G and IgAN. Prior to Pegcetacoplan he manifested as C3G, and ...

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RE: C3 or IgA 3 days ago

It is very clear that CFHR5 mutations are associated with an INCREASED risk of both C3GN and IgAN. ------------------------------ Richard Glassoc...

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RE: C3 or IgA 3 days ago

He had a biopsy at 3 months and 6 months post transplant . He also got 1 dose Eculizumab, 1 week prior to the transplant and a second dose 2 weeks ...

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RE: C3 or IgA 3 days ago

Thanks for the follow-up. How long following kidney transplantation is follow up available. I would judge that there is a high risk of recurrence o...

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RE: C3 or IgA 3 days ago

I was under the same assumption as Dr. Balan. That is the generic mutations causing aHUS/C3 , were protective against IgA . These mutations are ver...

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RE: C3 or IgA 3 days ago

This patient had a successful kidney transplant . His post transplant biopsy is pristine , no IgA or C3 . On a regimen on Tac and MMF ------------...

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RE: C3 or IgA 3 days ago

Addendum- I Would probably stop the Pegacetocoplan and start MMF and moderate does of steroids or even Iptacopan. But who really knows how to treat...

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RE: C3 or IgA 3 days ago

This provides the Answer to your question. This mutation (CFHR5) is associated with an increased risk of BOTH C3GN and IgAN!!! Why the Pegacetocopl...

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RE: C3 or IgA 3 days ago

See attachments --------------------------------- Dr. Hormaz Dastoor. MD, FASN Nephrology Consultant - Seha Kidney Care. Nephrology Consulta...

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RE: Renal phosphate wasting 3 days ago

Another suggestion. If you are thinking of Albers Schonberg disease, (and which seems likely here), then please screen other family members as this...

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RE: C3 or IgA 3 days ago

What was the genetic variant in this patient? To add to the complexity, CHFR variants are known to protect against IgA nephropathy -------------...

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RE: C3 or IgA 3 days ago

I also suppose that in such counter-intuitive outcomes, a technical error needs to be given serious consideration. Time will tell. -------------...

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RE: C3 or IgA 3 days ago

Btw... The first 2 biopsies were done in the Kingdom of Bahrain, I did try to contact them but was unsuccessful . I could make another push though ...

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RE: C3 or IgA 3 days ago

Agree this is a strange and counter-intuitive outcome. All the more reason to pursue the cause with greater intensity and depth. Nature may be tryi...

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RE: C3 or IgA 3 days ago

This thread only has 2 cases , my patient with C3G had 3 biopsies --------------------------------- Dr. Hormaz Dastoor. MD, FASN Nephrology Consul...

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RE: C3 or IgA 3 days ago

Come to think of it , C3 inhibitors such as Pegcetacoplan are being trialled in IgA nephroapthy... So for them to cause igA deposits would be stran...

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RE: Initiating Statin In Dialysis Patient 3 days ago

For thosw who do not use the international units for various blood components, would be helpful to provide the values in amount/dL or L, or also pr...

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RE: C3 or IgA 3 days ago

Thinking out loud. How would the intial insult that led to C3 disease be followed by IgA qhich requires a first hit by the lymphoid tissue in the g...

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RE: Renal phosphate wasting 3 days ago

This is a very interesting case and I agree with Dr Glassock that the combination of what sounds like Osteopetrosis and phosphaturia suggests an Os...

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RE: C3 or IgA 3 days ago

The first approach would be to be to stain the post therapy biopsy with KN55 (anti-gdIgA) and the second would be to do Pronase digestion and Paraf...

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RE: C3 or IgA Friday, June 20 @ 7:11 AM

I really don't have an answer ... was hoping for the forum to assist , as this case has been on my mind for a few months . ----------------------...

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RE: C3 or IgA Friday, June 20 @ 6:59 AM

So are these cases examples of drug induced (Secondary) IgA deposit disease (not true de novo Primary IgAN)? I think this is very possible. Further...

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RE: C3 or IgA Friday, June 20 @ 6:35 AM

The patient also had significant Generic mutations consistent with C3G. So am inclined to believe it's not Pseudo C3 deposits -------------------...

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RE: ANCA vasculitis with Pulmonary Hemorrhage - To Rituximab or MMF Friday, June 20 @ 6:31 AM

Are you worried about RTX reactivating HBV with + anti-HBc antibody.? I am. ------------------------------ Richard Glassock MD, FASN Laguna Woo...

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RE: ANCA vasculitis with Pulmonary Hemorrhage - To Rituximab or MMF Friday, June 20 @ 3:24 AM

Cr now normal after ritux steroids and plex for Pauci immune GN with pulmonary hemorrhage in setting of MULTIPLE infections. what is urinalysis n...

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RE: C3 or IgA Friday, June 20 @ 2:21 AM

These are "ominous" findings. Is it possible that the apparent "de novo" IgAN represents an IgA antibody response to "planted"Pegacetocoplan or Ecu...

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RE: C3 or IgA Friday, June 20 @ 1:55 AM

Unable to explain the reason but have observed the same in one of my patient, initial biopsy C3GN, second biopsy after treatment with Eculizumab, s...

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