RE: Fibronectin glomerulopathy - post-kidney transplant 3 hours ago

FG is not a common renal disease. But it makes sense from what we know about it being genetic that it would recur. see attached --------...

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RE: Pla2r positive MN 6 hours ago

This very intriguing thread highlights one of the great knowns unknowns in MN- what is the natural untreated course of PLA2+ MN. We know that spo...

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RE: Fibronectin glomerulopathy - post-kidney transplant 6 hours ago

Fibronectin Glomerulopathy rather predictably recurs in kidney allografts. A biopsy would be required for accurate diagnosis , including immunocyto...

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RE: Pla2r positive MN 8 hours ago

Dr Aledan, the excellent paper (thanks!) you cited suggests that the Sink Phenomenon applies to recurrence of disease, not just initial presentatio...

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Fibronectin glomerulopathy - post-kidney transplant 9 hours ago

A 42-year-old man who has end stage kidney disease secondary to fibronectin nephropathy, has undergone a living unrelated kidney transplantation in...

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RE: IgA nephropathy with recurrent RPGN 10 hours ago

Good day all, managed to obtain urgent biopsy which reported as We have a total do 14 gloms in the LM, only 2/14 are patent. The rest are glob...

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RE: Pla2r positive MN 12 hours ago

I rarely encountered clinical relapse in MN with reappearance of serum PLA2R Ab so we trust serology or depend on hard outcome parameters such as e...

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RE: Crescentic GN, IgA, Pulm Hemorrhage and low C' 14 hours ago

Thanks Dr. Aledan for this interesting and informative post. The original case in this thread has a normal RF which pretty much excludes Rheumatoid...

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RE: Pla2r positive MN 15 hours ago

I had a couple of patients who had progressive decline in PLA2R antibodies levels and yet their proteinuria had a few spikes during the course. I n...

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RE: Pla2r positive MN 16 hours ago

Reasonable points. But the serum tests for anti-PLA2 R (ELISA and IFA) are 99% specific for PLA2R related MN. The declining level of anti-PLA2R ant...

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RE: Fibronectin Glomerulopathy 16 hours ago

Fibronectin Glomerulopathy is an Autosomal Dominant condition due to mutations in FN1 gene. Any FH of kidney disease.? You might try to obtain WES ...

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RE: Pla2r positive MN 16 hours ago

100% specific test is a perfect biomarker for confirming the diagnosis of MN but as serology for follow up may not be so perfect and we should depe...

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RE: Crescentic GN, IgA, Pulm Hemorrhage and low C' 16 hours ago

I have exactly similar case few days ago with an elderly woman who is a known case of RA with obvious deformities on biologic therapy who presented...

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Fibronectin Glomerulopathy 18 hours ago

A 33-years-old woman presented with edema. She have h/o recurrent abortions. PMH of hypothyroidism on thyroxine 100 mcg od. Negative FH. Her BMI is...

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RE: Crescentic GN, IgA, Pulm Hemorrhage and low C' 20 hours ago

I think the positive "auto-immune" serology in this case should be interpreted with great caution in light of her advanced age. -----------------...

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RE: Crescentic GN, IgA, Pulm Hemorrhage and low C' 22 hours ago

The kidney biopsy shows capillary wall breaks consistent with Vasculitis (glomerular capillaritis ) - ? IgA V or something else - the initial prese...

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RE: Crescentic GN, IgA, Pulm Hemorrhage and low C' 23 hours ago

All good points, but congenital C4 deficiency can occur with IgAV- pretty rare however. ------------------------------ Richard Glassock MD, FASN...

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RE: Crescentic GN, IgA, Pulm Hemorrhage and low C' 1 day ago

3 Possible differential for a unifying diagnosis Infection. 2. Drug Induced, 3 Paraneoplastic if cryo is out based on biopsy and IgAV less of ...

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RE: Hematuria in 14 years old 1 day ago

Agree with plan to biopsy. Can't have a diagnosis of IgA vasculitis without the rash (although it can evolve over time). Differential primarily IgA...

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RE: Crescentic GN, IgA, Pulm Hemorrhage and low C' 1 day ago

Here is my problem with that, it is not just a low C3, nor is it a mild low C at, C3 42, the C4 is undetectable, there also is a low titer ANA, ...

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RE: Appreciate Input in this case ESP 1 day ago

I would, if only out of desperation. If the gut is still working and you do not have constant adhesion obstruction etc, you may have chance to halt...

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RE: Appreciate Input in this case ESP 1 day ago

Would anyone treat this patient with Steroids and Tamoxifen? ------------------------------ Richard Glassock MD, FASN Laguna Woods CA (949) 388-...

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RE: Appreciate Input in this case ESP 1 day ago

Update: Surgeon tried to take a look but she has frozen abdomen. ------------------------------ Sachin ------------------------------

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RE: Crescentic GN, IgA, Pulm Hemorrhage and low C' 1 day ago

Yes cryoglobulins returned negative. Hepatitis studies negative. Cultures negative. Repeat C' pending. I sent genetics. Following up on the theo...

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RE: IgA nephropathy with recurrent RPGN 1 day ago

I think we need the original biopsy report to make any meaningful suggestions. The synopsis of the report posted here first biopsy for 2 glom...

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RE: IgA nephropathy with recurrent RPGN 1 day ago

Looks like IgA is a red herring. I will keep ANCA negative vasculitis in the differential given recurrence and treat. Of course patient deserves a...

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RE: Pla2r positive MN 1 day ago

Steady improvement in serum albumin level likely predicts a clinical remission, according to a MENTOR post-hoc analysis, but does this also apply t...

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RE: Pla2r positive MN 2 days ago

very odd course, Looks like spontaneous remission by PLA2R going from 380 to 180 to 38, this should predict eventual proteinuria reduction but ...

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RE: Pla2r positive MN 2 days ago

Many thanks Dr Leiba- The repeat anti-PLA2R antibody level will be very helpful to see if his spontaneous immunological remission is continuing- ...

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RE: Pla2r positive MN 2 days ago

Great questions...let me try and summarize This 74 year old man came to our attention on March 2024 with nephrotic syndrome, and what seemed to ...

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RE: IgA nephropathy with recurrent RPGN 2 days ago

Currently Patient is running microscopic hematuria sub nephrotic range proteinuria first biopsy for 2 gloms only reported as necrotising cresce...

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RE: Pla2r positive MN 2 days ago

We saw lag of proteinuria after serum PLA2 Ab reduction, but here there is increment in UPCR, is it sink phenomenon? What is the current serum PLA2...

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RE: Resistant Childhood Nephrotic Syndrome and Negative Genetic Testing 2 days ago

Very good point. The Combination of van Wyk-Grumbach Syndrome and Alport Syndrome have been reported on multiple occasions. The connection seems to...

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RE: Pla2r positive MN 2 days ago

Should be screened for RVT --------------------------------- Syed Mohkum Ud Din MBBS Balochistan Institute of Nephrology Urology Quetta 0333-78...

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RE: Crescentic GN, IgA, Pulm Hemorrhage and low C' 2 days ago

The kidney biopsy findings seem to make Cryoglobulinemia a rather unlikely possibility., it seems to me. ------------------------------ Richard G...

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RE: Crescentic GN, IgA, Pulm Hemorrhage and low C' 2 days ago

Believe endocarditis ruled out. Also test for sjogren SSA 60 is positive Cryo possibility but RF was normal --------------------------------- Sha...

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RE: IgA nephropathy with recurrent RPGN 3 days ago

Thanks. I agree. ------------------------------ Richard Glassock MD, FASN Laguna Woods CA (949) 388-8885 ------------------------------

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RE: IgA nephropathy with recurrent RPGN 3 days ago

Definitions are fudgy @ best. Previously it was defined as greater than 50% Crescents with rapidly worsening AKI. As you are aware in IgA crescents...

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RE: Pla2r positive MN 3 days ago

Was he completely normal in the near past without proteinuria? Does he have one or two kidneys? ------------------------------ Ashraf El-Meanaw...

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RE: Resistant Childhood Nephrotic Syndrome and Negative Genetic Testing 3 days ago

Because of the tandem gene duplication mutation, special PCR based methods are required. These mutations may escape detection by WGS methods. --...

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RE: Resistant Childhood Nephrotic Syndrome and Negative Genetic Testing 3 days ago

I totally agree with prof Richard I wonder if Repeat genetic testing by using Wide genome sequencing would be helpful any family history of C...

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RE: H. Influenza Vaccination before Eculizumab 3 days ago

Tried it but no response. I am even considering to enroll that patient in a clinical trial for pegcetacoplan but not sure if they are still enrolli...

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RE: H. Influenza Vaccination before Eculizumab 3 days ago

I think that current recommendation for vaccination before use of C inhibitors include one dose of H. influenza vaccine. The data on overall effica...

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RE: Resistant Childhood Nephrotic Syndrome and Negative Genetic Testing 3 days ago

I suspect that Dr Glassock is spot on. Two genetic testing strategies that may be employed would be to do a whole genome sequencing to look for int...

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H. Influenza Vaccination before Eculizumab 3 days ago

Hello Nephrology Community, I am planning on starting a patient of mine on Eculizumab for C3GN and was wondering if they need to be vaccinated...

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RE: IgA nephropathy with recurrent RPGN 3 days ago

How do you define 'True" Crescentic IgAN compared to IgAN with crescents? Just curious. ------------------------------ Richard Glassock MD, FASN...

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RE: IgA nephropathy with recurrent RPGN 3 days ago

Just one RCT showing adverse event rate increase in AZA treated patients with IgAN (Pozzi et al JASN 2010). Never duplicated as far as I know, but ...

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RE: IgA nephropathy with recurrent RPGN 3 days ago

This idea that aza works here and doesnt work here always bothered me for instance it (was) the preferred maintenance drug for vasculitis (before...

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RE: IgA nephropathy with recurrent RPGN 3 days ago

Addendum- it is worth remembering that Azathioprine is ineffective and possibly harmful in IgAN. See Pozzi C, et al. JASN 2010; 21: 1783-1790. It...

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RE: Anca vasculitis relapse without circulating antibodies 3 days ago

Thanks- interesting suggestion. No TMA in original biopsy. No comments about peripheral manifestation of TMA with relapse. A repeat biopsy would be...

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