Transition of Kidney Care at 18: Challenges and Practical Solutions for India.

Health-care transition (HCT) from pediatric-centered to adult-oriented health-care setting is more than a simple transfer of care. It is a carefully planned movement specially tailored for the needs of adolescents and young adults (AYAs). Similar to other chronic diseases, the need for HCT for AYAs with kidney disease has been well established by the International Society of Nephrology (ISN) and the International Pediatric Nephrology Association (IPNA) consensus statements since 2011. However, successful HCT in India and other low- and middle-income countries (LMICs) has been limited. Undertaking the HCT program in India requires involvement of many stakeholders, that is, AYAs, parents/caregivers, health-care providers, and the health-care system. In this article, we discuss the need for HCT, the challenges faced during the transition, and the recommended models for HCT in kidney care. We focus on the unique challenges faced in India and conclude with practical suggestions to implement HCT in our setting.

Prevalence and outcome of abdominal wall hernia in patients with end-stage renal disease on peritoneal dialysis.

INTRODUCTION: We aimed to study the prevalence, risk factors, management, and outcome of hernias in end-stage renal disease (ESRD) patients on peritoneal dialysis (PD) from India. METHODS: This was a retrospective study of ESRD-PD patients who developed hernias over 11 years. RESULTS: Of 470 PD patients, 21 developed hernias (4.2%). Mean age of patients was 49.9 ± 15.36 years; 15 (66.66%) were males; 18 (85.71%) patients had umbilical hernia, 3 (14.28%) had inguinal hernia. Continuous ambulatory PD (CAPD) versus automated PD (APD) (OR: 11.623, 95% CI: 2.060-65.581, p = 0.005) was the independent risk factor identified. Incarcerated umbilical/inguinal hernia was managed surgically (6 [28.57%]); uncomplicated umbilical hernia (15 [71.42%]) managed conservatively (shift to (APD) [33.33%]; switch to low-volume APD [20%], switch to low-volume CAPD [46.66%]). None had postoperative hernia recurrences; 4 (19%) had PD technique failure; median PD survival was 36 (IQR 17-55) months. CONCLUSION: Although complicated hernias in PD require surgical repair, uncomplicated umbilical hernias can be managed conservatively by switching to APD/low-volume CAPD, with good long-term PD technique survival.

Complete Remission of Lupus Nephritis Following Chemoradiotherapy of Carcinoma Cervix: An Association.

Systemic lupus erythematosus (SLE) is associated with a higher incidence of solid organ malignancies, including cervical carcinoma, creating a paradox in their management in the context of autoimmunity. We present a case of 45-year-old female presented with mucocutaneous, musculoskeletal symptoms of SLE. Renal biopsy showed class IV lupus nephritis (LN); modified NIH activity score: 8/24, chronicity score: 6/12. Post NIH regimen induction, she achieved partial remission; further developed proteinuric relapse which was re-induced with mycophenolate mofetil (MMF) to which she failed to respond. Subsequently diagnosed with carcinoma cervix stage IIB, she received four cycles of concurrent cisplatin-based chemoradiotherapy. MMF was stopped; low dose steroids continued. Following this, the patient achieved complete remission (CR) of LN and is in remission for 5 years. This is an unexpected association between chemoradiotherapy of cervical carcinoma and CR of class IV LN, allowing long-term discontinuation of immunosuppression.

Rituximab in treatment of collapsing FSGS-A case series.

BACKGROUND: Collapsing focal segmental glomerulosclerosis (cFSGS) is an aggressive glomerular disease presenting as a nephrotic syndrome that has lower rates of remission with conventional immunosuppressive therapy and rapidly progresses to end-stage-renal-disease (ESRD). We report eight cases of HIV-negative cFSGS treated with rituximab. METHODS: The current report is a retrospective case series of cFSGS treated with rituximab from January 2011 to March 2020, at varying phases of the disease. RESULTS: Eight out of the 70 cFSGS patients received rituximab. The median age of patients was 30 years (IQR 24.25-37.5); five patients were males. The median serum creatinine, mean serum albumin and median 24 hours urinary protein at presentation was 0.9 (IQR 0.66-1.27) mg/dL, 2.95 ± 1.15 g/dL, 4.87 (IQR 1.64-5.75) g/day, respectively. Two patients were steroid-resistant, one steroid and tacrolimus dependent, one steroid and cyclosporine dependent, two steroids and tacrolimus resistant, one steroid, tacrolimus, cyclophosphamide, mycophenolate mofetil resistant and one steroid-resistant and tacrolimus dependent before rituximab therapy. Rituximab was given either as targeted therapy (after an initial dose of 375 mg/m2 ; patients having CD-19 levels >5/µL or >1% at 1 month received additional low-dose [100 mg] of rituximab), or weekly regimen. Five patients received CD-19 targeted rituximab; three received weekly doses of 375 mg/m2 , cumulative doses being 820 ± 228.03 mg, and 1800 ± 721.11 mg, respectively. At the end of median follow-up of 15 months, five (62.5%) patients were in remission (three partial, two complete remissions), two (25%) were resistant to therapy; one (12.5%) progressed to ESRD. CONCLUSION: Rituximab is reasonably safe and achieves/maintains remission in 60% of cFSGS cases.

Regulatory B Cells Are Reduced and Correlate With Disease Activity in Primary Membranous Nephropathy.

INTRODUCTION: Primary membranous nephropathy (PMN) is an autoimmune disease. Both T-regulatory cells (TREGs) and B-regulatory cells (BREGs) are decreased in patients with autoimmune disease. We evaluated the TREG and BREG population in patients of PMN treated with cyclical cyclophosphamide and steroid therapy (cCYC/GC). METHODS: Twenty-four patients with PMN resistant to a restrictive strategy and treated with cCYC/GC therapy and 10 healthy controls were enrolled. The proteinuria, serum creatinine, and serum albumin were tested at monthly intervals and blood samples were collected before starting cCYC/GC and at 6 and 8 (2 months wash out) months of therapy. The peripheral blood mononuclear cells (PBMCs) after staining with fluorochrome-conjugated antibodies were then subjected to flow cytometric analysis for detection of TREGs (CD3+CD4+CD25hiCD127loFoxP3+) and BREGs (CD19+CD5+CD1dhiIL10+). TREGs and BREGs are presented as the percentage of T and B cells, respectively. Cases with remission at month 18 were classified as responders, and those without any remission as nonresponders. RESULTS: Patients with PMN had a lower percentage of TREGs (P = 0.07) and BREGs compared with healthy controls (P = 0.0007). As compared with baseline, there was a significant increase in both BREGs (P = 0.001) and TREGs (P = 0.02) with the treatment (8 months). Patients who responded to therapy by 18 months had an increase in TREG (P = 0.05) and BREG (P = 0.0001) at month 8 compared with baseline. CONCLUSION: As compared with healthy controls, patients with PMN displayed a lower percentage of BREGs. Both TREGs and BREGs significantly improved with disease-specific therapy. BREGs had an association with clinical activity.

Successful renal transplantation in a family with a novel mutation in COL4A3 gene and autosomal recessive Alport syndrome.

Alport syndrome (AS) is an inherited disorder of basement membranes caused by mutations affecting specific proteins of the type IV collagen family, presenting with nephropathy and extrarenal manifestations such as sensorineural deafness and ocular anomalies. Ten percentage to 15% of the patients with AS have autosomal recessive (ARAS) due to mutation in either COL4A3 or COL4A4 gene. We report a novel mutation in the COL4A3 gene in an Indian family with ARAS. The above-mentioned genetic anomaly was a missense variation in exon 26 of the COL4A3 gene (chr2:228137797G>A; c.1891G>A) that resulted in the amino acid substitution of Arginine for Glycine at codon 631 (p.Gly631Arg) that was present in the heterozygous state in the asymptomatic parents and homozygous state in the male offspring who presented with early-onset end-stage renal disease, lenticonus and hearing loss. The patient (male offspring) underwent successful renal transplantation with his mother as a donor.