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Introduction: There is a paucity of data and therapeutic options nationally and internationally on calcineurin inhibitor (CNI)-resistant forms of focal segmental glomerulosclerosis (FSGS) in children. CNI (tacrolimus or cyclosporine) are proven monotherapy in children with FSGS with a steroid-dependent (SD) or steroid-resistant (SR) course. We analyzed a novel therapeutic option in CNI-resistant FSGS by using the dual therapy of rituximab and mycophenolate to maintain remission. Methods: This is a retrospective analysis of clinical, therapeutic profile, and treatment outcomes (sustained remission versus no remission) in subjects with CNI-resistant FSGS who received dual rituximab therapy along with mycophenolate as maintenance therapy for a minimum of 1 year. Results: The median age of presentation of 13 recruited children was 7.8 years (range: 2.4-17.6 years); nine (69.2%) were males. Ten (76.9%) of them had an SD course and three (23.1%) had an SR course. Four (30.7%) had evidence of acute/chronic CNI toxicity, and the remaining nine (69.3%) showed no response to CNI therapy despite adequate trough levels. Post dual therapy, 11 (84.6%) had sustained remission for at 1 year and two (15.4%) children did not show remission. None reported adverse reactions or infections, and all had preserved renal functions. Conclusion: Dual combination therapy with rituximab and mycophenolate among children with CNI-resistant FSGS can emerge as a promising and efficacious treatment strategy to ensure sustained remission in this subset of patients.
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Introduction: There is a paucity of clinical data on C1q nephropathy (C1qN) in children in India and Southeast Asia. This is the first detailed analysis conducted to elucidate the prevalence, clinicopathological profile, and response to different immunosuppressives in children with C1qN in India. Materials and Methods: Detailed demographic profile, clinical features, urine and blood chemistries, kidney biopsy, and response to different immunosuppressives of the study participants were analyzed between August 2015 and October 2020 for steroid-dependent/-resistant nephrotic syndrome (NS). Results: C1qN was diagnosed in 16 (14.13%) of 113 children who underwent biopsy for steroid-dependent/-resistant NS. The mean age was 44 months (range 18-99 months) and male and female number was 12 (75%) and four (25%), respectively, and mean follow-up was 3.5 years. Eight (50%) had coexistent minimal-change nephrotic syndrome (MCNS) pattern, seven (43.7%) had focal segmental glomerulosclerosis (FSGS), and one (6.2%) had diffuse mesangial hypercellularity. Thirteen children had complete follow-up, of which eight (61.5%) and four (30.7%) cases presented as steroid-dependent and primary steroid-resistant NS, respectively, whereas one (7.6%) had joint pain with rashes. At presentation, seven (53.8%) had hypertension, 12 (92.3%) had nephrotic range proteinuria, and six cases (46.1%) had hematuria. Nine (75%) of 12 cases achieved complete remission with calcineurin inhibitor (CNI) therapy, and two were non responders, one was a partial responder, and one responded to mycophenolate. Of six FSGS cases, four had complete remission, one had partial remission, and one was in non-remission. Of six cases with MCNS, five had complete remission and one was in non-remission. Renal functions remained normal in all except one case who had progression to chronic kidney disease Stage 3. Conclusion: One out of seven children with difficult NS can have underlying C1qN. CNIs are most beneficial to attain and maintain remission. Renal functions remain normal in the majority. Along with C1q deposits, MCNS and FSGS patterns are seen equally and respond almost similarly to CNIs.
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BACKGROUND: Immunoadsorption (IA) plasmapheresis is standard modality for pretransplant desensitization in ABO-incompatible solid organ transplants though technically challenging when considered for an infant or a child less than 10 kg due to non-availability of pediatric immunoadsorption (IA) columns. The major challenge is to maintain hemodynamic stability considering the large extracorporeal circuit volume meant for adults. To our best knowledge after extensive search in acclaimed global medical journals, this is the first successful attempt in an underweight (6 kg) infant of less than 1 year of age using adult size IA Column thus making it a reality. CASE CHARACTERISTICS: We report an 8-month-old male infant (A positive) of 6 kg with decompensated liver disease secondary to extrahepatic biliary atresia requiring urgent live donor liver transplantation with AB positive donor with significantly elevated pretransplant anti-B IgG/ IgM antibody titers >1:1024. Baby underwent multiple sessions of anti-B immunoadsorption plasmapheresis to lower anti-B IgM / IgG titers using available adult anti-B immunoadsorption column. Postprocedure, the antibody titers reduced to 1:8 (anti-IgG) 1:16 (anti-IgM) followed by successful ABO-incompatible live donor liver transplant (LDLT). OUTCOME: Anti-B titers remained in normal range in the immediate and post-transplant period with satisfactory liver functions and no rejection. CONCLUSION: Immunoadsorption plasmapheresis for ABO-incompatible solid organ transplantation in infants gives desirable results and can be offered to small sized infants using currently available adult sized IA columns when conducted with adequate technical expertise.
