Clinical Presentation and Management of Nephrotic Syndrome in the First Year of Life: A Report From the Pediatric Nephrology Research Consortium (PNRC)
Bibliographic record
Abstract
Background: Nephrotic syndrome (NS) in the first year of life is called congenital (CNS) if diagnosed between 0-3 months, or infantile (INS) if diagnosed between 3-12 months of age. The aim of this study was to determine if there were clinically meaningful differences between CNS vs. INS patients regarding clinical presentation, management, and outcomes. Methods: 11 PNRC sites participated in the study, using IRB-approved retrospective chart reviews of CNS and INS patients born between 1998-2019. Data were collected on patient characteristics, pertinent laboratory tests, need and frequency of albumin infusions, and type and timing of nephrectomy and renal replacement therapy (RRT). Results: The study included 69 patients, 49 with CNS and 20 with INS, median ages at diagnosis of 1 month and 6 months, respectively. Patients were similar with respect to nutrition, thyroxin supplementation, IVIG/SCIG prophylaxis, and thrombosis prophylaxis. Within the first 2 months after diagnosis, daily albumin infusions were more frequently needed in CNS vs. INS (79 vs. 30%; p=0.006), while weekly infusions were more frequently needed in INS vs. CNS (50 vs. 3%; p=0.001). Moreover, within the final 6 months preceding RRT, albumin infusions were more frequently required in CNS vs. INS (51 vs. 15%; p=0.007). Nephrectomy was also performed more frequently in CNS vs. INS (78 vs. 50%: p=0.024). Dialysis was similarly required in CNS vs. INS (73 vs. 55%; p=0.14). Pre-emptive kidney transplantations were similarly performed in CNS vs. INS patients (6% vs. 5%). Notably, management without either nephrectomy or RRT was more frequent in INS vs. CNS (40% vs. 16%; p=0.035). Sequences of interventions (i.e., Nephrectomy->RRT->Transplant [TXP]) were similar between the groups, although RRT->bilateral nephrectomy->TXP tended to occur more frequently in CNS vs. INS (61 vs. 36%; p=0.06). Conclusions: Compared to children with INS, those with CNS had more severe disease courses, requiring more frequent albumin infusions, and earlier onset of RRT. In addition, almost 25% of children with CNS or INS were able to be managed without need for either nephrectomy or RRT.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".