Nephrology Crosswords: Genetic GNs

From Susan Quaggin's team on Genetics and GNs
http://www.nature.com/ki/journal/v87/n4/pdf/ki201488a.pdf

Clinical Case 87: Answers and Summary

34 y old Indian Male with IgA nephropathy, crt is 2.4mg/dl and 2.5gm of proteinuria: treatment?

RAAS blockade only

  6 (12%)

 

RAAS blockade and Fish oil

  9 (19%)

 

Steroids with RAAS blockade

  19 (40%)

 

Treatment depends on Biopsy Oxford Classification of IgA Nephropathy

  13 (27%)

 

The KDIGO recommends no specific guidance for treatment with steroids in IgA nephropathy patients when they present with GFR between 30-50cc/min. A recent study published in JASN in 2015 looked at steroid use in IgA Nephropathy patients. It is called the VALIGA study.  A retrospective study that studied over 140 patients with IgA nephropathy from European registry and classified based on Oxford classification MEST score.   46% received immunosuppresive agents and of them 985 were steroids.  The ones who were treated had all the features of clinical progression( rising crt, or proteinuria).  All also received RAAS blockade.  The patients who got steroids had a significant reduction in proteinuria, a slower rate of renal function decline and greater chance of not being on dialysis.  While, initially we had thought that the benefit of such treatment was only in patients with mild- moderate AKI, this study found benefit even in the GFR<50cc/min cohort with levels of proteinuria.  

So in the above patient, the best answer would be Steroids with RAAS blockade. If the biopsy did show crescentic GN, the treatment ofcourse would be with cytotoxic agents in addition. 

ASN- KSAP First installment

Nephrology Self-Assessment Program (NephSAP) has served for twelve years as a strategy for nephrologists to keep knowledge up-to-date and earn both CME credits.

ASN has recognized a need for material that covers basic principles of clinical nephrology that would be appropriate for individuals who are studying for the In-Training/In-Service examination or preparing for the American Board of Internal Medicine (ABIM) examination in the subspecialty of Nephrology. To this end, the concept of the Kidney Self-Assessment Program (KSAP) was developed.

KSAP is similar to having a Q bank for board preparation and re certification exams.
Please visit and see what you all think

This was must needed and well intended

http://asn-online.org/education/ksap/

Topic Discussion: Renal Vein Thrombosis in the Allograft

Incidence:

0.5%-4% and frequently results in graft loss

Risk factors:

Technique error

Hypovolaemia Atherosclerosis OKT3 (plus high-dose methylprednisolone) Antiphospholipid antibodies High dose steroids Long cold ischaemia time Delayed graft function recovery

Certain renal diseases( membranous GN, SLE)

Antiphospholipid antibody syndrome

Use of oral contraception, 

Hereditary thrombophilia secondary to protein C or factor V deficiency

Clinical presentation:

Acute anuria, hematuria, graft tenderness, primary non function of the graft

Best test:

Renal sonogram with dopplers waveform evaluation( most important part)
A normal renal arterial waveform in either an allograft or native kidney shows antegrade flow throughout the entire cardiac cycle. Reversal of diastolic flow in the allograft renal artery, although not specific, is considered abnormal. This finding is caused by a significant increase in resistance in small intrarenal or large extrarenal vessels. This sign is not pathognomonic for renal vein thrombosis ( as can be seen with ATN or rejection as well).  Renal vein thrombosis was more likely to occur in the acute (24 hours) and perioperative (30 days) periods. It is important to recognize the abnormal duplex Doppler waveform pattern, reversal of diastolic flow, which is associated with renal vein thrombosis.

Treatment: 
Timely returning back to the OR for thrombectomy and or reconstruction of venous component
Streptokinase or urokinase
Percutaneous mechanical thrombectomy and
Localized catheter-directed thrombolysis

But overall, prognosis is poor with many times leading to nephrectomy.

Two great reviews:

http://www.ajkd.org/article/S0272-6386(09)00981-0/pdf

http://ndt.oxfordjournals.org/content/24/5/1388.full