title: Recent Advances in Treatments of Primary Focal Segmental Glomerulosclerosis in Children.
abstract: Focal segmental glomerulosclerosis (FSGS) is a nephrotic syndrome. Up to around 80% of cases of primary FSGS are resistant to steroid treatment. A large proportion of patients with steroid-resistant FSGS progress to end-stage renal disease. The purpose of treatment is to obtain a complete remission of proteinuria, a necessary step that precedes improved renal survival and reduces the risk of progression to chronic kidney disease. When this is not possible, the secondary goal is a partial remission of proteinuria. Reduction or remission of proteinuria is the most important factor predictive of renal survival. We will review the current updated strategies for treatment of primary FSGS in children, including traditional therapies consisting of corticosteroids and calcineurin inhibitors and novel therapies such as rituximab, abatacept, adalimumab, and fresolimumab.
authors.affiliation: Department of Pediatrics, Jeju National University School of Medicine, Aran 13gil 15, Jeju-si, Jeju Special Self-Governing Province 63241, Republic of Korea.
authors.forename: Kyoung Hee
authors.identifier: 0000-0002-6830-7311
authors.initials: KH
authors.lastname: Han
authors.affiliation: Department of Pediatrics, Pusan National University Children's Hospital, 20 Geumo-ro, Mulgeum-eup, Yangsan-si, Gyeongsangnam-do 50612, Republic of Korea; Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, 20 Geumo-ro, Mulgeum-eup, Yangsan-si, Gyeongsangnam-do 50612, Republic of Korea.
authors.forename: Seong Heon
authors.identifier: 0000-0001-8003-3010
authors.initials: SH
authors.lastname: Kim
mesh_terms: D002648:Child; D002675:Child, Preschool; D004351:Drug Resistance; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000503:physiopathology; D011507:Proteinuria / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D012074:Remission Induction; D013256:Steroids / Q000627:therapeutic use*
chemical_list: D013256:Steroids