Related Experiment Videos
Long-term linear growth of children with severe steroid-responsive nephrotic syndrome
Francesco Emma1, Antonella Sesto, Gianfranco Rizzoni
1Division of Nephrology, Children's Hospital and Research Institute Bambino Gesu', Piazza S. Onofrio 4, 00165 Rome, Italy. emma@opbg.net
Insights
Children with steroid-dependent nephrotic syndrome (SDNS) and frequently relapsing nephrotic syndrome (FRNS) face permanent growth impairment. Steroid therapy duration and cumulative dose are key predictors of reduced final height in these pediatric patients.
Area of Science:
- Pediatric Endocrinology
- Nephrology
- Growth and Development
Background:
- Steroid-dependent nephrotic syndrome (SDNS) and frequently relapsing nephrotic syndrome (FRNS) are chronic conditions in children.
- Long-term steroid treatment is often necessary but may impact growth.
- Understanding the risk of growth impairment is crucial for managing these conditions.
Purpose of the Study:
- To evaluate the risk of permanent linear growth impairment in children with SDNS and FRNS.
- To identify factors predicting growth retardation in these pediatric patients.
- To assess the impact of steroid therapy on final height.
Main Methods:
- Longitudinal height measurements were collected from disease onset in 42 children with SDNS and 14 with FRNS.
- Mean follow-up duration was 11.7 years.
- Growth was assessed using height standard deviation scores (HtSDS) from prepubertal and final height measurements.
Main Results:
- Patients experienced significant height loss during the prepubertal period (-0.49 HtSDS) and at final height (-0.92 HtSDS from onset).
- Steroid therapy (prednisone duration and cumulative dose) was the sole predictor of poor growth.
- Partial catch-up growth was observed after steroid withdrawal, with higher risk in early-onset cases and adolescents on prolonged treatment.
Conclusions:
- Children with severe steroid-responsive nephrotic syndrome are at risk of permanent growth retardation.
- Prolonged steroid treatment is a significant factor contributing to reduced final height.
- Close monitoring and management of steroid therapy are essential to mitigate growth impairment.
Abstract:
The present study was designed to evaluate the risk of permanent linear growth impairment in a selected group of 42 children with steroid-dependent nephrotic syndrome (SDNS) and 14 children with frequently relapsing nephrotic syndrome (FRNS). Longitudinal height measurements were available in all patients from the onset of the disease for a mean follow-up of 11.7+/-3.5 years. During the prepubertal period, patients lost 0.49+/-0.6 height SD score (HtSDS) ( P<0.001). Twenty-three patients have reached their final height with an average loss of 0.92+/-0.8 HtSDS from the onset of their disease ( P<0.001) and 0.68+/-0.7 from their target HtSDS ( P<0.001). The pubertal growth spurt was mildly delayed in male but not female patients. Steroid therapy, calculated as the mean duration of prednisone (PDN) treatment or as the average cumulative PDN dose, was the only predictor of poor growth evolution. Partial catch-up growth occurred after PDN withdrawal. Children with early onset NS and adolescent patients, who were still receiving PDN after the age of 9 years in girls and 11 years in boys, were at higher risk for HtSDS loss. In conclusion, children with severe steroid-responsive NS are at risk of permanent growth retardation secondary to prolonged courses of steroid treatment.