FOCAL SEGMENTAL GLOMERULOSCLEROSIS IN YOUNG PATIENTS

这个子项目是众多研究子项目之一

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Focal segmental glomerulosclerosis causes about 10 to 20% of all cases of idiopathic nephrotic syndrome, which is an inflammation of the internal structures of the kidney, in children and 35% in adults. In FSGS, protein is persistently released in the urine, especially in the form of urine albumin, a large protein molecule. Most patients with FSGS will progress to long term, chronic renal failure which is gradual and results in a progressive loss of the ability of the kidneys to excrete wastes, concentrate urine, and conserve electrolytes. In the majority of patients, FSGS is refractory to therapeutic interventions. The morbidity and mortality for patients with FSGS is compounded by the relatively high recurrence rate for this lesion following renal transplantation. The outcomes for patients with recurrent FSGS are as poor if not worse than those for patients with primary FSGS. Over the past two decades there has been an increased incidence of FSGS in both urban and rural populations. Although the disorder seems to be immune system related, response to corticosteroid or immunosuppressive medications, which modify our immune response, is inconsistent. This research study is being sponsored by the National Institute of Health to develop evidence based treatment guidelines to intervene with FSGS and its progression to chronic renal failure. It is a multi-center randomized trial that will be conducted throughout the United States in 500 children and young adults.