Low-density lipoprotein apheresis in a pediatric patient with refractory nephrotic syndrome due to focal segmental glomerulosclerosis

• Published in: Journal of anesthesia 2009, Vol.23 (2), p.284-287
• Main Authors: Oto, Jun, Suga, Kenichi, Matsuura, Sato, Kondo, Shuji, Ohnishi, Yoshiaki, Inui, Daisuke, Imanaka, Hideaki, Kagami, Shoji, Nishimura, Masaji
• Format: Article
• Language: English
• Subjects: Anesthesiology; Anti-Inflammatory Agents - therapeutic use; Blood Component Removal - methods; Child; Clinical Report; Critical Care Medicine; Drug Resistance; Emergency Medicine; Female; Glomerulosclerosis, Focal Segmental - complications; Glomerulosclerosis, Focal Segmental - pathology; Humans; Immunosuppressive Agents - therapeutic use; Intensive; Kidney - pathology; Lipoproteins, LDL - blood; Medicine; Medicine & Public Health; Nephrotic Syndrome - etiology; Nephrotic Syndrome - pathology; Nephrotic Syndrome - therapy; Pain Medicine; Proteinuria - etiology; Pulse Therapy, Drug
• ISSN: 0913-8668; 1438-8359
• DOI: 10.1007/s00540-008-0726-z

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·day − to 0.5–1.0 g·day − . LDL-A may be beneficial in the treatment of multidrug-resistant FSGS.