Every medical discipline can proudly list its triumphs. Nephrology is no different and outstanding accomplishments include improvements in dialysis therapy, hormonal therapy of chronic kidney disease, and the identification of the molecular genetic basis of a number of common conditions. However, each field also has glaring shortcomings in its knowledge base and obvious gaps in its therapeutic capacity. For nephrologists, idiopathic or primary focal segmental glomerulosclerosis (FSGS) is a prime example of this state-of-affairs. Practitioners from all around the globe have attested to the steadily increasing incidence and prevalence of this disorder [1, 2]. FSGS makes a prominent contribution to the list of children, adolescents and adults who are waiting for a kidney transplant [3–6]. Finally, this disease continues to rear its ugly head even after kidney transplantation because it can recur in 20–30% of allograft recipients [7]. If we are honest and evaluate the literature critically, then most everyone will agree that there is no good therapy for this disease. Thus, when our patients look to us for answers, we are forced to admit that there are none and that a great deal of work remains to be done to determine the cause and how to treat FSGS. All of our patients, in every country and in every clinic, are in the same predicament. Despite impressive research advances and enormous effort by clinical investigators, FSGS remains a pressing problem for pediatric nephrologists.

This Educational Feature is designed to update the readers of Pediatric Nephrology on the current status of knowledge about FSGS. The contributors to this series of teaching articles are all acknowledged experts in nephrology with extensive clinical experience in the diagnosis and treatment of pediatric patients with this disease. As a group, they represent the ideal people to author this collection of state-of-the-art articles on FSGS.

The first article written by Ronald Hogg, John Middleton, and Matti Vehaskari addresses the epidemiology and natural history of FSGS. Using data collected by established registries such as NAPRTCS, they highlight the dramatic rise in incidence of this disease and the substantial morbidity related to this glomerulopathy. The second article by Frederick Kaskel and Kimberly Reidy examines the current thinking about the pathophysiology of FSGS. They focus on new insights obtained from careful histopathological studies and the pivotal role of the podocyte in initiating proteinuria. They discuss the numerous factors that are instrumental in triggering and sustaining renal fibrosis that can progress to end stage renal disease. The third article by Robert Woroniecki and Jeff Kopp discusses the genetic forms of FSGS. For those of us who have been at this work for longer than we care to admit, the advances in the molecular biology of the podocyte and the delineation of mutations in structural proteins in this unique cell have been nothing short of revolutionary [8, 9]. The authors point out all of the key questions such as the likelihood of response to therapy and disease recurrence post-transplantation that have been raised by the new findings in genetics. The next article by Debbie Gipson, Sandra Watkins, and Marva Moxey-Mims admirably summarizes the state-of-the-art in the treatment of FSGS [10]. This is a large complex area that includes both non-specific antifibrotic agents and a vast array of immunosuppressive drugs. One could easily get discouraged reading this article but the authors point out new directions that may yield improved outcomes for future patients. Finally, Richard Fine describes the clinical features of recurrent FSGS in renal allografts and what can be done to alleviate this dreaded complication.

In closing, it is important to emphasize that each of the five teaching articles builds on a solid foundation of outstanding basic science and clinical research performed by colleagues in our international community from around the globe [11, 12]. The authors would like to dedicate their contributions to this Educational Feature on FSGS to the memory of Norman Siegel MD, our colleague and friend who died so unexpectedly in April 2006. There have been many well-deserved testimonials to Norm’s contributions to our field but there are few that I think are more fitting than this.