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- Title
Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation.
- Authors
Dharnidharka, Vikas R.; Scobell, Rebecca R.; Kallash, Mahmoud; Davies, Amy J. Goodwin; Marchesani, Nicole; Maltenfort, Mitchell G.; Walther, Leslie; Kelton, Megan; Bock, Margret; Blanchette, Eliza; Stone, Hillarey K.; Gluck, Caroline; Hullekes, Frank; Riella, Leonardo V.; Smoyer, William E.; Mitsnefes, Mark; Dixon, Bradley P.; Flynn, Joseph T.; Somers, Michael J. G.; Forrest, Christopher B.
- Abstract
Background: Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods: We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results: From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions: Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents.
- Subjects
UNITED States; STEROID drugs; KIDNEY transplantation; RISK assessment; IMMUNOSUPPRESSIVE agents; GRAFT survival; RESEARCH funding; FOCAL segmental glomerulosclerosis; SYMPTOMS; TREATMENT effectiveness; HOMOGRAFTS; FUNCTIONAL status; PLASMAPHERESIS; MULTIVARIATE analysis; DESCRIPTIVE statistics; NEPHROTIC syndrome; SURGICAL complications; REMISSION induction; LOW density lipoproteins; RESEARCH; DISEASE relapse; ALBUMINS; DATA analysis software; DRUG resistance; DISEASE risk factors; CHILDREN
- Publication
Pediatric Nephrology, 2024, Vol 39, Issue 11, p3317
- ISSN
0931-041X
- Publication type
Article
- DOI
10.1007/s00467-024-06452-z