Idiopathic membranous nephropathy in children.
Idiopathic membranous nephropathy (MN) is a rare cause of asymptomatic proteinuria (AP) or nephrotic syndrome (NS) in childhood. To improve our understanding of its clinical course, we retrospectively reviewed 19 cases of idiopathic MN seen in our hospital over a period of 28.5 years, i.e., from January 1977 to July 2005. Eight patients (39%) had AP and 11 (61%) presented with NS. All eight AP patients achieved remission, regardless of treatment modality. Oral corticosteroid was given to all 11 NS patients, but only three of them responded to corticosteroid. Of the eight steroid non-responders, three achieved remissions with the addition of cyclosporine, and the five who were not administered additional immunosuppressive drugs had persistent NS. At the latest evaluation, all six NS patients that achieved remission remained free of proteinuria and had a normal renal function. Moreover, two of the 5 steroid non-responders showed persistent nephrotic-range proteinuria but a stable renal function. The remaining three steroid non-responders progressed into chronic renal insufficiency, and this progression was preceded by renal vein thrombosis (RVT) in two of the three patients. Presentation with NS (P=0.045) and the development of RVT (P=0.010) were identified as poor prognostic factors.
Lee
Beom Hee
BH
Department of Pediatrics, Seoul National University Children's Hospital, 28 Yongon-Dong, Chongno-Gu, Seoul, 110-744, South Korea.
Cho
Hee Yeon
HY
Kang
Hee Gyung
HG
Ha
Il Soo
IS
Cheong
Hae Il
HI
Moon
Kyung Chul
KC
Lim
In Seok
IS
Choi
Yong
Y
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004341:Drug Evaluation; D005260:Female; D005500:Follow-Up Studies; D015433:Glomerulonephritis, Membranous / Q000145:classification / Q000188:drug therapy* / Q000453:epidemiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D009404:Nephrotic Syndrome / Q000145:classification / Q000188:drug therapy* / Q000453:epidemiology; D011507:Proteinuria / Q000145:classification / Q000188:drug therapy* / Q000453:epidemiology; D012189:Retrospective Studies; D016896:Treatment Outcome
D007166:Immunosuppressive Agents