Low-density lipoprotein apheresis in a pediatric patient with refractory nephrotic syndrome due to focal segmental glomerulosclerosis.
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.
Oto
Jun
J
Department of Emergency and Critical Care Medicine, Tokushima University Hospital, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, Japan.
Suga
Kenichi
K
Matsuura
Sato
S
Kondo
Shuji
S
Ohnishi
Yoshiaki
Y
Inui
Daisuke
D
Imanaka
Hideaki
H
Kagami
Shoji
S
Nishimura
Masaji
M
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
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D008077:Lipoproteins, LDL