Sanjay Agarwal : Expert in Hepatitis/ Tuberculosis and RRT, CKD, magnitude, epidemiology, GN – The ISN Website

I do not know answer.

However, mu guess is that it has not been studied adequately in adult nephrotic syndrome.

I think I would like to be little more specific in relation to statement “HBsAg positive status”. In hepatotropic viral infection, one has to be sure of activity of viral infection. It means that one should do AST/ALT, HBeAg and HBV-DNA to assess the replicative stage of virus as well as liver injury. In patient with associated glomerulonephritis (MPGN in this case), the obvious question will be to assess cause and effect relationship. It is easier said than done. I will assess HBV markers in renal biopsy to presume cause and effect relationship by immunohistochemistry. In MPGN, usually HBsAg is antigen seen in histology.

If that is demonstrated, I will take it as cause of MPGN and will treat with anti-viral drugs unless, the clinical course is of rapidly progressive glomerulonephritis, in which case I will use immunosuppressive agents. Our own experience of treating HBV infection in maintenance dialysis has shown good response with Entecavir and I will use that.

Between 2006-2012 immunosuppressive treatment should be mentioned in detail say in what doses and in what sequence. That will help in interpratation of use of further immunosuppression at this stage. I understand that it was primary FSGS. One of the option may be to repeat a biopsy and then decide. I will assess for re-biopsy after knowing details of treatment between 2006-2012.

rgds

Firstly, thank you for using in the future pharmacological name of drugs instead of brands. At least some of these I could not understand. I will ask what is his weight to interpret whether steroid doses were adequate or not and whether he is complaint for medication.

After Cyclosporin, was the drug level adequate? I will use Tacrolimus before thinking of Rituximab. Obviously the case is little resistant to many drug. I do not see any advantage of using cyclophosphamide once he is not responding to CNI. Together I will continue symptomatic treatment along with immunosuppressive treatment.

In a primary glomerular disease (Not a systemic disease), if the proteinuria is near 2 gm/day or so, then probably best will be to use non-specific proteinuria reduction like ACEI/ARB. In this situation, risk benefit for steroid use is not in favour of benefit and I will suggest to avoid.

In a FSGS, I usually continue Prednsiolone 1mg/Kg for 4-6 months unless there is some complication in patient who is not responding. Further, 1 month of cyclosporin in my opinion is nothing. At least few months of treatment is required. So, overall drugs look to be changed frequently without giving adequate time for response. This way we land up in issue whether to go back to previous medicine and give adequate time for response or to change to new drug. Now what has been done is done. I will start a fresh with Tacrolimus 0.1 mg/Kg + Steroid (0.5 mg/Kg) combination and give for at least 4-6 months before saying that he is not responding and go from there.

What is important in this case is to know details of Prednisolone, Cyclophosphamide and CsA doses and duration before I would like to comment on further treatment.

Secondly, I do not know whether the other comment relates to this patient. Milky urine in Indian context we have often seen in cases of Chyluria, often due to tuberculosis and filariasis. These patients also have significant fluctuating urinary protein and significant number of RBCs in urine sediment. Often they have gross hematuria also, which is usually intermittent. Urine color chnages with diet content and becomes more milky once patient takes fatty meal. These patients mostly do not have very heavy proteinuria. Associated heavy proteinuria requires kidney biopsy to assess specific glomerular pathology. If this is associated with filariasis, we have seen associated glomerular disease concomitant with heavy proteinuria. I do not know whether if associated with filariasis, immunosuppressive treatment will help or not. Just a information to be known for others.

In my opinion, drug therapy in a case of recurrent calculus disease are not effective alone. It should be added on top of high fluid therapy, which is corner stone of prevention of recurrent stone disease. Yes, diet should be decreased in Calcium and Thiazide is good addition in drug therapy. I think unless there is hyperuricemia, allopurinol may not be useful. You should also decreas salt intake in diet as well as decrease high purine diet to decrease hyperuricosuria by non-pharmacological means. In addition, existing stones should be surgically removed.

Firstly, I will say that in this forum better to use pharmacological name of drugs. At least some of these I could not understand. I will ask what is his weight to interpret whether steroid doses were adequate or not and whether he is complaint for medication.

After Cyclosporin, was the drug level adequate? I will use Tacrolimus before thinking of Rituximab. Obviously the case is little resistant to many drug. I do not see any advantage of using cyclophosphamide once he is not responding to CNI. Together I will continue symptomatic treatment along with immunosuppressive treatment.

First of all I will suggest that personal questions like this may not be asked in this form. It will always be better to ask from the treating doctor, who is treating you. However, without further information, I will answer that most of such type of patients need a graft biopsy to approach the patient treatment. Needless to say that much more information is required even after the biopsy to decide treatment approach rather than just hematuria and 3+ proteinuria.

Good luck

To clarify: There are more than 90 types of pneumococcal bacteria. PCV13 protects against only 13 of them. However, these 13 strains are the one which cause most severe infections in children and about half of infections in adults.

So, PCV 13 may not cover a large number of pneumococcal infection in adult, though they not be so serious. PCV13 is routinely given to children at 2, 4, 6, and 12–15 months of age as children in this age range are at greatest risk for serious diseases caused by pneumococcal infection.

PCV13 vaccine may also be recommended for some older children or adults who are at risk of serious pneumococcal infection. So, a person should be vaccinated in childhood with PCV13 already once he is in adulthood and then if he has NS and CKD and at risk for Pneumococcal infection, he should get PPSV. If a person is not vaccinated with PCV13 in childhhod, there is suggestion to give BOTH PCV13 as well as PPSV.

In my opinion gross hematuria with clots is very very unlikely in glomerular diseases and I will keep possibilities of non-glomerular hematuria unless proved otherwise. In my opinion, coagulation disorder is also non-glomerular situation. Left kidney being small and creatinine already 1.6 mg% at baseline suggest that patient already had probably chronic kidney disease and this deterioration is acute on chronic. Cystoscopy showing blood coming from right ureter further confirm that it is non-glomerular hematuria as all glomerular hematuria will cause blood from both orifices. Now there are two issues. What is cause of hematuria and what is cause of further deterioration of kidney function on pre-existing kidney disease. Further, there are two possibilities. Cause of both the problem may be same or causes may be different. Another important issue is why left kidney is small? The cause may be responsible for the current situation on right side also. The very fact that right kidney is large suggest that left kidney is small for a longer time to give time for right kidney to get enlarge. Flank pain with gross hematuria can occur with stone/ tumuour tissue causing pain/ clot itself / papillary necrosis/ Severe UTI. So, we need to look out for pre-renal cause for renal deterioration and post renal cause of deterioration and sepsis as cause for acute or chronic. Another condition/physiology which can cause unilateral pain in kidney and deterioration in renal function is rflux anuria (Though this case does not have anuria).

I think that it is combination of Lupus nephritis and amyloidosis. In absence of AA positivity, non-AA amyloidosis is labeled but bone marrow is normal. Frackly speaking I can not suggets now how to proceed. I will treat her for Lupus nephritis and wait for cause of amyloidosis. May be with time she develop other abnormalities to label a cause of non-AA amyloidosis.

It will always be better to taper steroid in adult MCD over few months or so rather than abruptly stopping. Also, be sure that it is MCD as hypertension is not common in MCD though 10-20% adult MCD do have mild hypertension.