Progression of proliferative glomerulonephritis with monoclonal IgG deposits in pediatric patients.

BACKGROUND: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a glomerular disease defined by non-organized glomerular deposits of heavy and light chain-restricted immunoglobulin and is rarely reported in children. METHODS: We characterized a series of nine pediatric patients from two academic centers with biopsy-proven PGNMID and additionally describe two patients with monotypic IgG in the setting of IgM deposition. RESULTS: Each patient presented with hematuria and/or proteinuria; however, only five had elevated serum creatinine. Prodromal or concurrent infection was identified in six patients, low C3 in five, and alternate complement pathway gene variants in two. No monoclonal serum proteins were identified in five tested patients. Seven patients had monotypic deposits composed of IgG3-λ, two showed IgG3-κ, and one each IgG1 and IgG3 with lambda dominance in the setting of IgM deposition. The glomerular pattern was predominantly mesangial proliferative or membranoproliferative glomerulonephritis (MPGN). Treatment and outcomes were variable; four patients have recent PGNMID diagnoses and therefore minimal follow up, one had relatively stable kidney function for over a decade, and six experienced kidney failure, with four receiving transplants. Recurrent deposits of the same isotype were identified in five of six transplanted kidneys, corresponding to three of four transplanted patients. One of these patients developed PGNMID recurrences in three separate kidney allografts over a 20-year disease course. CONCLUSIONS: Our study emphasizes the need for upfront IgG subclass investigation in pediatric mesangial or MPGN with IgG deposition and monotypic or biased light-chain staining. Furthermore, this pediatric experience suggests expanded pathogenic considerations in PGNMID. Graphical abstract.

Sparking a Movement, Not a Moment: Framework and Outcomes From a Pediatrics Department-Wide Coalition to Advance Anti-Racism.

OBJECTIVE: The Stanford Pediatrics Advancing Anti-Racism Coalition (SPAARC) was created to promote a culture of anti-racism through immediate action, development of nimble systems, and longitudinal commitment toward equity. Evaluate gaps in the Stanford Department of Pediatrics (DoP) efforts to advance anti-racism and form a coalition of faculty, staff, and trainees to prioritize, design, and implement targeted activities with immediate and long-term measurable outcomes. METHODS: A needs assessment was conducted across all DoP members in July to August 2020 to identify gaps in anti-racism efforts. Listening sessions were recorded and transcribed to extrapolate key themes and 2 rounds of consensus surveys were done to identify and prioritize actions. Actions teams were created and co-led by faculty-staff dyads with trainee representation. A final activity survey was conducted in January 2021 to determine the specific activities (ie, interventions) each team would design and implement. RESULTS: Ten small group listening sessions (70 participants) and 3 surveys (1005 responses) led to the creation of 7 action teams with associated activities 1) training, 2) community engagement and research, 3) communication, 4) faculty and staff recruitment and advancement, 5) leadership representation, 6) human resources, and 7) staff engagement. Four hundred forty-three (41%) DoP members were directly involved in SPAARC through participation in the needs assessment, action teams, and/or implementation of activities. CONCLUSION: SPAARC can serve as an adaptable framework for how a DoP can create a coalition to identify gaps in anti-racism efforts and create and implement targeted activities with associated outcomes.