Related Experiment Videos
Predicting first-year relapses in children with nephrotic syndrome
A R Constantinescu1, H B Shah, E F Foote
1Department of Pediatrics, Division of Pediatric Nephrology, University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School, New Brunswick, NJ, USA. constaar@umdnj.edu
Insights
Predicting nephrotic syndrome relapses in children is crucial. Rapid steroid remission and absence of hematuria at initial presentation indicate a lower likelihood of infrequent relapses (IRs).
Area of Science:
- Pediatric Nephrology
- Clinical Research
- Disease Prognosis
Background:
- Nephrotic syndrome frequently recurs in children, with relapse patterns categorized as infrequent (IR), frequent (FR), or steroid-dependent (SD).
- Understanding predictive factors for relapse patterns is essential for effective management and long-term patient outcomes.
- Initial histopathology is often deferred, necessitating prediction based on clinical presentation.
Purpose of the Study:
- To identify clinical factors at initial presentation that predict relapse patterns in the first year of childhood nephrotic syndrome.
- To differentiate between infrequent relapses (IR), frequent relapses (FR), and steroid-dependent (SD) courses without relying on renal biopsy findings.
Main Methods:
- Retrospective analysis of medical records of pediatric nephrotic syndrome patients followed for at least one year.
- Key variables analyzed included age, sex, race, presence of hematuria, and time to remission.
- Statistical analysis focused on identifying predictors of IR, FR, and SD relapse patterns.
Main Results:
- Of 56 analyzed patients, 41.1% had IR, 16.1% had FR, and 42.9% had SD.
- Independent variables like age, sex, race, and initial hematuria were not significant predictors of first-year relapse patterns.
- A significant finding emerged: children achieving remission within one week of steroid therapy and without hematuria were more likely to have an IR course (Sensitivity: 67%, Specificity: 89%, PPV: 94%).
Conclusions:
- The rapidity of initial steroid response, particularly achieving remission within a week, is a key predictor of relapse patterns.
- The absence of hematuria, when combined with a rapid initial response, strongly suggests an infrequent relapse course.
- Documenting these presenting features is vital for predicting future relapse frequency in pediatric nephrotic syndrome.
Objective:
More than half of the children diagnosed with nephrotic syndrome will have relapses. These can be infrequent relapses (IRs: <2 in 6 months or <3 in a year) or frequent relapses (FRs: >2 in 6 months or >3 in a year). Patients who relapse while on alternate day steroids or within 1 month of discontinuation of steroid therapy are considered steroid-dependent (SD; J Pediatr. 1982; 101:514-518). Patients with an IR course have a better long-term prognosis, and many of them have minimal-change disease without mesangial hypercellularity or sclerosis. The purpose of our study was to identify factors at initial presentation that could predict the relapse pattern in the first year after diagnosis, without taking into consideration the histopathology found on renal biopsy.
Design:
We analyzed the medical records of children who were seen by us before March 1997 and followed for at least 1 year. Variables selected in the study were age, sex, race, presence or absence of hematuria, and days to remission (defined as protein-free) at the initial presentation, because they could relate to the pattern of relapses (ie, IR, FR, and SD).
Results:
Of 70 patients, 14 were excluded because of insufficient data. There were 38 males (67.9%) and 18 females (32.1%), giving a male:female ratio of 1.8:1. Median age at presentation was 3.25 years (range: 1.5-13), and 76.9% were white, 8.9% black, 7.1% Hispanic, and 7.1% other. Of all the patients, 23 were IR (41.1%), 9 were FR (16.1%), and 24 were SD (42. 9%). Median days to remission were 10 (range: 2-60), on Prednisone 60 mg/M(2) daily. Hematuria was present initially in 26 patients (46. 4%), and absent in 30 (53.6%). Age, sex, race, and hematuria, as independent variables, were not predictors of relapses in the first year. However, using a stratified analysis based on the presence or absence of hematuria, we found that if the remission occurred within the first week of therapy, the patients without hematuria were more likely to be IR. The sensitivity and specificity of this finding were 67% and 89%, respectively, with a positive predictive value of 94%.
Conclusion:
We conclude that of all the presenting features, the rapidity of initial response to steroid therapy combined with the presence of hematuria, could predict future relapses and should be well documented.