1.Case studyMahmoud said

2. A 30 years old lady referred to the renal clinic complaining of L.L is free except for soft pitting oedema, morning puffiness , and prot.2+ in urine . Chest ,heart ,abdomen and LND examination all are free she is non-diabetic but hypertensive since 5 years No history of NSAID On examination :B.P:150/100Pulse:90L.L edema :2+ 3. Which investigations would you ask for in this case ????? 4. 24hr urinary protein:4g/day Urea: 18 Creat:0.6 cholest: 350mg/dl T.G: 200mg/dl S.albumin :2.7 ESR: 80/120 ???? ANA ,AntiDsDNA,C3 all are of normal range HCVAb , HBSAg negative. PT: 100%INR: 1 5. ESR in nephrotic syndromeThe erythrocyte sedimentation rate (ESR) is elevated (greater than 25 mm/h by the Westergren method) in almost all patients with the nephrotic syndrome.(T/F)This finding alone is not an indication to evaluate a patient for an underlying systemic disorder (T/F) 6. ESR in nephrotic syndromeA direct relation between the degree of proteinuria and the ESR has been noted in patients with glomerular disease in which the ESR was approximately 10 times the daily rate of protein excretion 7. To biopsy or not ?Biopsy was done 8. Microscopic picture:with H&E,PAS,trichrome,and congo-red revealedWidening of the mesangial area-glomerular cellularity +Thickening of GBM, podocytes were hypertrophied.Renal tubules-intersetium and the included arterioles were unremarkable 9. Immunohistochemical for IgG,IgM,IgAIgG: moderate (++) positive staining insubendothelial areaIgA: mild (+) positive staining in subendothelial areaIgM:(+/-) positive staining 10. What is your histopathological diagnosis??A) membranous GNB) Mesangioproliferative GNC) Membranoproliferative Type Id) Membranoproliferative typeIIIe) non of the above 11. A) Membranous GNB) Mesangioproliferative GNC) Membranoproliferative Type Id) Membranoproliferative typeIIIe) non of the above 12. Welldone 13. Microscopic picture:with H&E,PAS,trichrome,and congo-red revealedWidening of the mesangial area-glomerular cellularity +Thickening of GBM, podocytes werehypertrophied.Renal tubules-intersetium and the included arterioles wereunremarkable 14. So Why is type I not III? Immunohistochemical for IgG,IgM,IgAIgG: moderate (++) positive staining insubendothelial areaIgA: mild (+) positive staining in subendothelialareaIgM:(+/-) positive staining 15. EM in Renal Biopsy (KDIGOguidelines) sufficient tissue is needed to perform not only anexamination by light microscopy, but alsoimmunohistochemical staining to detect immunereactants (including immunoglobulins andcomplement components),and electron microscopy to define precisely the location,extent and, potentially, the specific characteristics of theimmune deposits. We recognize that electronmicroscopy isnot routinely available in many parts of the world, buttheadditional information defined by this technique maymodifyand even change the histologic diagnosis, and mayinfluencetherapeutic decisions; hence, it is recommended 16. So you now Know this is a case of MPGN type IWhat will you do ? Based on Evidence !!!!!! 17. She was put onARBs & ACEI Omega 3 plus ???? Cyclosporin 50 mg 1*2 ??????????? Steroids 40 mg/day with tapering the dose 18. 24 hr.urinarys.creatinine Date protein g/day s.albumin g/dlmg/dl C0 1.4.201142.7 0.61.6.2011 5 188 1.7.20116 30.7 1421.8.2011140.61.9.2011 30.71.10.20115.23.51.11.20111.3 41391.12.20111.21.1.2012 0.7701-02-12 1.54.4 1.4 2381.3.2012 1.81.328.3.20124 1.4.20112.61.225.4.20110.84.5 1.11.6.2011 1.8 1 1.7.20111.31.8.2011 40.91.9.2011 1.31.10.2011 2.7 3.9 19. During the period of treatment the patient sufferedfrom severe headache that was not responsiveto any line of treatment she was refereed to aneurologist . .A CT scan was done but was unremarkableMRI was also unremarkableBilateral papilledeoma was noted on FUNDUSexamination and was diagnosed as pseudo tumorcerebriAcetazolamide and thiazide diuretics was addedalong with increasing the dose of the steroids.She had maevellous response and know she is 20. Prognosis in Idiopathic MPGNIdiopathic MPGN in adults also carriesan unfavorable prognosis. Five years after biopsy,50% of patients either die or need renalreplacement therapy (dialysis or transplantation).This proportion increases to 64% after 10 years. Risk of progression increases with elevated creatinine, nephrotic proteinuria, and severe hypertension or if a biopsy specimen shows more than 50% crescents or markedinterstitial fibrosis 21. So ,after this what do you think the prognosis of this patient is? 22. Do you consider shifting cyclosporin to otherlines ofimmunosuppressive drugs is useful or not?and Why? 23. Thank you