Here we report the case of two brothers with collagenofibrotic glomerulopathy confirmed by histology. Patient 1 presented with proteinuria and hypertension and patient 2 presented with nephrotic-range proteinuria. Immunohistochemistry revealed strong staining PF-02341066 purchase for antibodies to type III collagen in the widened subendothelial spaces in both patients. Electron microscopy revealed numerous collagenous fibers in the mesangium and subendothelial space. P III P levels were elevated in both patients. Most reported cases of collagenofibrotic glomerulopathy, including the adult-onset type, have been
sporadic. Within the limits of our literature search, this is only the third report of adult siblings with collagenofibrotic glomerulopathy confirmed by histology, suggesting that adult-onset collagenofibrotic glomerulopathy may also be an inheritable disease. This report indicates that it may be beneficial to measure serum P III JAK inhibitor P levels in the siblings of patients diagnosed
with adult-onset collagenofibrotic glomerulopathy. PRASAD NARAYAN1,2,3,4,5, JAISWAL AKHILESH2, AGARWAL VIKAS3, YADAV BRIJESH4, RAI MOHIT5 1Department of Nephrology, Sgpgims, Lucknow, India; 2Department of Nephrology, Sgpgims, Lucknow, India; 3Department of Clinical Immunology, Sgpgims, Lucknow, India; 4Department of Nephrology, Sgpgims, Lucknow, India; 5Department of Clinical Immunology, Sgpgims, Lucknow, India Introduction: Approximately 60–80% of steroid responsive Nephrotic Syndrome (NS) patients experience relapses of proteinuria and it is one of the most challenging clinicial issues. NS is a disorder of T cells function. The ratio of different Endonuclease T cells subpopulation may affect steroid response in NS. P-glycoprotein (P-gp) on lymphocyte acts as efflux pump and may affect drug response. However,
there are a few such studies in NS. Methods: We recruited 26 NS patients at baseline, with steroid therapy 24 undergone complete remission, and after discontinuation of steroid 15 relapsed. Frequency of Treg, Th1 and Th2 lymphocytes and P-gp expression were analyzed using flowcytometry at baseline and followup at remission and relapse. The PBMC culture for cytokine Elisa were also done. Results: The percentage of Treg was significantly increased after achieving remission (6.82 ± 4.12) compared to that of baseline (1.83 ± 0.84, P = 0.001) and again decreased after relapse (3.03 ± 1.18, P = 0.016) Fig. A. The percentage of TH1 cells was significantly decreased in remission (9.9 ± 4.65) compared to that of baseline value (16.18 ± 7.19, P = 0.018) and again increased in relapse (19.83 ± 3.47, P = 0.001) Fig. B. The percentage of Th2 was significantly decreased in remission (4.81 ± 1.42) compared to that of baseline values (10.5 ± 4.66, P = 0.001) and again increased after relapse (9.89 ± 5.18, P = 0.008) Fig C. The absolute P-gp expression (P-gp positive cell × RFI) was significantly low during remission (35.11 ± 18.