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Resolution versus persistence of childhood idiopathic nephrotic syndrome-A population-based study
Evgenia Gurevich1,2,3, Odeyah Abeles4, Daniel Landau5,6
1Southern District Clalit Health Services, Pediatric Nephrology, Beer Sheva, Israel.
Insights
Most children with idiopathic nephrotic syndrome (INS) achieve remission by age 11.2. However, about a fifth of relapsing patients experience continued disease into adulthood, necessitating careful transition to adult care.
Area of Science:
- Pediatrics
- Nephrology
- Immunology
Background:
- Idiopathic nephrotic syndrome (INS) is a significant kidney disorder in children.
- Understanding the long-term course of relapsing INS is crucial for patient management and prognosis.
Purpose of the Study:
- To determine the duration and long-term outcomes of relapsing childhood idiopathic nephrotic syndrome.
- To identify factors associated with disease persistence into adulthood.
Main Methods:
- Retrospective analysis of a large, population-based health maintenance organization database.
- Inclusion of children aged 2-10 years with a new INS diagnosis and corticosteroid prescription between 2000-2010.
- Classification of NS category based on corticosteroid and/or steroid-sparing agent purchases.
Main Results:
- Out of 608 eligible children, 113 (21.6%) had persistent disease into adulthood, with a mean age of 19.3 years.
- The majority of patients (411) entered long-lasting treatment-free remission by age 11.2 ± 5.7 years.
- Patients with more frequent medication purchases showed an older age at last relapse and higher steroid-sparing agent use.
Conclusions:
- Childhood idiopathic nephrotic syndrome typically enters remission by age 11.2 years.
- A significant proportion of patients with relapsing INS continue to have active disease into adulthood.
- Transition of care from pediatric to adult services is essential for patients with persistent INS into adulthood.
Aim:
To determine the duration of relapsing childhood idiopathic nephrotic syndrome (INS).
Methods:
In this population-based study, we retrospectively analysed the computerised database of Israel's largest health maintenance organisation. Children (age 2-10 years) with a new INS diagnosis and a corticosteroid (CS) prescription between 2000 and 2010 were included. NS category was determined, according to CS and/or steroid-sparing agents (SSA) purchases.
Results:
Out of 1 669 977 eligible children, 608 fulfilled inclusion criteria. Patients in the fourth quartile of purchases (n = 132) had an older age at last relapse (17.9 ± 6.3 vs. 11.3 ± 5.9 years, p < 0.001) and more SSA use (78.8% vs. 20%, p < 0.001) compared to the remaining three quartiles. A single episode occurred in 84 patients. Of the remaining 524 patients (males 66%, diagnosis age: 4.8 ± 2.2 years, SSA prescribed: 35%) who were followed for 15.5 ± 5.1 years, 113 (21.6%) had a continuing disease at an age of 19.3 ± 6.3 years. The leftover 411 entered long-lasting treatment-free remission at age 11.2 ± 5.7 years.
Conclusion:
In this multicentre study, we identified INS disease course by medication delivery. NS long-standing remission occurs at age 11.2 ± 5.7 years in most cases. However, the disease continues into adulthood in a fifth of the relapsing patients, implicating the need for proper transition to adult care.
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