title: Low-density lipoprotein apheresis in a pediatric patient with refractory nephrotic syndrome due to focal segmental glomerulosclerosis.
abstract: Focal segmental glomerulosclerosis (FSGS) often leads to refractory nephrotic syndrome (NS). A high level of low-density lipoprotein (LDL) is a risk factor for the progression of NS. An 8-year-old girl presented with severe proteinuria refractory to steroid therapy. She was diagnosed with non-IgA diffuse mesangial proliferative glomerulonephritis. Oral prednisolone, methylprednisolone (mPL) pulse therapy, and cyclosporine and cyclophosphamide therapy failed to achieve remission. Follow-up renal biopsy revealed FSGS. Her serum level of LDL was high, and LDL-apheresis (LDL-A) was performed five times, followed by mPL pulse therapy. Urinary protein decreased from 2-4 g x day(-) to 0.5-1.0 g x day(-). LDL-A may be beneficial in the treatment of multidrug-resistant FSGS.
authors.affiliation: Department of Emergency and Critical Care Medicine, Tokushima University Hospital, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Oto
authors.forename: Kenichi
authors.initials: K
authors.lastname: Suga
authors.forename: Sato
authors.initials: S
authors.lastname: Matsuura
authors.forename: Shuji
authors.initials: S
authors.lastname: Kondo
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Ohnishi
authors.forename: Daisuke
authors.initials: D
authors.lastname: Inui
authors.forename: Hideaki
authors.initials: H
authors.lastname: Imanaka
authors.forename: Shoji
authors.initials: S
authors.lastname: Kagami
authors.forename: Masaji
authors.initials: M
authors.lastname: Nishimura
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001781:Blood Component Removal / Q000379:methods*; D002648:Child; D004351:Drug Resistance; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology; D008077:Lipoproteins, LDL / Q000097:blood*; D009404:Nephrotic Syndrome / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D011507:Proteinuria / Q000209:etiology; D020551:Pulse Therapy, Drug
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D008077:Lipoproteins, LDL