Prognostic Factors in Childhood Nephrotic Syndrome
Prognostic Factors in Childhood Nephrotic Syndrome
Vivarelli M, Moscaritolo E, Tsalkidis A, Massella L, Emma F
J Pediatr. 2010;156:965-971
Vivarelli and coworkers assert that idiopathic nephrotic syndrome (INS) is the most common cause of nephrosis in childhood, and all children with this diagnosis initially receive systemic steroids. Biopsy is not indicated initially for most children. However, the ideal dose and duration of steroid therapy have not been well established. If children fail to enter remission after an initial trial of steroids, then biopsy may be indicated. This study sought to identify characteristics that predict successful remission in children with INS who are treated with steroids.
The data for this study were evaluated retrospectively, from patients seen between 1992 and 2006 on a single pediatric nephrology service. The patients were all children who were followed at the site without interruption, from diagnosis onward; who were treated according to a standard, international protocol; who achieved remission without the use of medications other than steroids; and who were followed for at least 2 years in the clinic after diagnosis. During the study period, 103 children met these criteria and were included.
The steroid treatment regimen was 60 mg/m/day for 4 weeks, followed by 4 weeks at two thirds of that dose. Remission was defined as 3 consecutive days of no protein on urine dipstick testing. Finally, outcomes were labeled as steroid-dependent, frequent relapsing, infrequent relapsing, and nonrelapsing (relapse-free for 2 years). After the initial attempt at remission, further therapy was tailored to each patient, including whether immunosuppressive medications (beyond steroids) were given.
The children were 72% boys, with an average age of onset of 3.4 years. The median length of follow-up for the children was > 40 months. Only 8.7% of children had nonrelapsing INS; 24% were infrequent relapsers; 24% were frequent relapsers; and 43% had steroid-dependent INS. The median time to reaching a response to steroids was 7 days. In several analytic approaches, the time to reach admission was strongly associated with risk of developing frequently relapsing or steroid-dependent INS, even in multivariable analyses. For example, 50% of the children who took more than 14 days to achieve remission actually relapsed while on steroids. Almost all of the patients who entered remission in fewer than 7 days remained in remission during the initial treatment, and 50% were relapse free at 3 months after completing the initial steroid course. The investigators concluded that the time to remission with initial steroid therapy is predictive of later prognosis, and children who respond within 7 days have the best prognosis.
Vivarelli and colleagues noted that owing to subsequent data from other centers and other studies, they have extended the time of the initial duration of steroid induction therapy compared with these patients seen from 1992 to 2006. The greatest value of these data for primary care providers is that they can help a provider to manage parental expectations when their child is diagnosed with INS. It is worth keeping in mind that 70% of these children required maintenance steroids of some sort, and 60% required a suppressive drug other than steroids. The investigators postulated that future evaluations of longer initial steroid courses can improve the proportion who become relapse-free after the initial induction.
Abstract
Time for Initial Response to Steroids Is a Major Prognostic Factor in Idiopathic Nephrotic Syndrome
Vivarelli M, Moscaritolo E, Tsalkidis A, Massella L, Emma F
J Pediatr. 2010;156:965-971
Study Summary
Vivarelli and coworkers assert that idiopathic nephrotic syndrome (INS) is the most common cause of nephrosis in childhood, and all children with this diagnosis initially receive systemic steroids. Biopsy is not indicated initially for most children. However, the ideal dose and duration of steroid therapy have not been well established. If children fail to enter remission after an initial trial of steroids, then biopsy may be indicated. This study sought to identify characteristics that predict successful remission in children with INS who are treated with steroids.
The data for this study were evaluated retrospectively, from patients seen between 1992 and 2006 on a single pediatric nephrology service. The patients were all children who were followed at the site without interruption, from diagnosis onward; who were treated according to a standard, international protocol; who achieved remission without the use of medications other than steroids; and who were followed for at least 2 years in the clinic after diagnosis. During the study period, 103 children met these criteria and were included.
The steroid treatment regimen was 60 mg/m/day for 4 weeks, followed by 4 weeks at two thirds of that dose. Remission was defined as 3 consecutive days of no protein on urine dipstick testing. Finally, outcomes were labeled as steroid-dependent, frequent relapsing, infrequent relapsing, and nonrelapsing (relapse-free for 2 years). After the initial attempt at remission, further therapy was tailored to each patient, including whether immunosuppressive medications (beyond steroids) were given.
The children were 72% boys, with an average age of onset of 3.4 years. The median length of follow-up for the children was > 40 months. Only 8.7% of children had nonrelapsing INS; 24% were infrequent relapsers; 24% were frequent relapsers; and 43% had steroid-dependent INS. The median time to reaching a response to steroids was 7 days. In several analytic approaches, the time to reach admission was strongly associated with risk of developing frequently relapsing or steroid-dependent INS, even in multivariable analyses. For example, 50% of the children who took more than 14 days to achieve remission actually relapsed while on steroids. Almost all of the patients who entered remission in fewer than 7 days remained in remission during the initial treatment, and 50% were relapse free at 3 months after completing the initial steroid course. The investigators concluded that the time to remission with initial steroid therapy is predictive of later prognosis, and children who respond within 7 days have the best prognosis.
Viewpoint
Vivarelli and colleagues noted that owing to subsequent data from other centers and other studies, they have extended the time of the initial duration of steroid induction therapy compared with these patients seen from 1992 to 2006. The greatest value of these data for primary care providers is that they can help a provider to manage parental expectations when their child is diagnosed with INS. It is worth keeping in mind that 70% of these children required maintenance steroids of some sort, and 60% required a suppressive drug other than steroids. The investigators postulated that future evaluations of longer initial steroid courses can improve the proportion who become relapse-free after the initial induction.
Abstract
