Mason AE, Sen ES, Bierzynska A, et al. - This review of comprehensively genetically screened patients with steroid-resistant nephrotic syndrome was performed to determine whether response to initial intensified immunosuppression in these patients may be related to disease progression and/or post-transplant recurrence. The ultimate goal was to improve disease stratification and that is why patients from the United Kingdom National Study of Nephrotic Syndrome cohort were analyzed. This study included 271 pediatric patients, of these, 178 received intensified immunosuppression with response available. Findings revealed a poor therapeutic response as well as no post-transplant recurrence in patients experiencing monogenic steroid-resistant nephrotic syndrome. Response to first intensified immunosuppression was shown to be correlated to long-term outcome in genetic-testing-negative patients. Progression to kidney failure rarely occurred in patients with complete response, whereas poor kidney survival as well as a high post-transplant recurrence rate was evident in nonresponders. A response, especially to rituximab, was more likely to be seen in patients with secondary steroid resistance.