title: Clinicopathologic features, outcome, and therapeutic interventions in four children with isolated C3 mesangial proliferative glomerulonephritis.
abstract: Since isolated C3 mesangial proliferative glomerulonephritis in the absence of systemic disease (i-C3-GN) is an uncommon chronic glomerular disease, long-term prognosis and optimal therapeutic intervention for it are not yet fully defined, especially in children. We report clinical features, outcome, and interventions in 4 patients, ranging from 6 to 18 years old, with i-C3-GN. Microscopic or macroscopic hematuria with or without proteinuria was first noted between 3 and 8 years. When present, proteinuria ranged from 0.2 to 1.0 g/24 h. Persistent hypocomplementemia and circulating immune complexes were found in 1 patient. None of the patients had nephrotic syndrome or hypertension. Percutaneous renal biopsy specimens showed varying degrees of mesangial proliferative glomerulonephritis; 2 patients showed mild mesangial proliferation, while others exhibited moderate histologic severity. In 1 patient with a mild mesangial increase, tubulointerstitial changes were associated. Both patients exhibiting mild mesangial changes followed a benign clinical course with normal renal function over 10 years of follow-up. Patients with moderately severe mesangial alteration manifested slight renal function loss and moderate proteinuria at the time of biopsy, but these largely resolved after a six-month course of prednisolone combined with cyclophosphamide, warfarin, and an angiotensin-converting enzyme inhibitor. Thus, clinical manifestations and the need for aggressive treatment appear to vary among pediatric patients with i-C3-GN. Therapy combining prednisolone with immunosuppression seemed to reduce proteinuria and improve glomerular function in patients with moderately severe mesangial proliferation.
authors.affiliation: Department of Pediatrics, Kinki University School of Medicine, 377-2 Ohno-higashi, 589-8511 Osaka-Sayama, Japan.
authors.forename: Kazuro
authors.initials: K
authors.lastname: Yagi
authors.forename: Hidehiko
authors.initials: H
authors.lastname: Yanagida
authors.forename: Keisuke
authors.initials: K
authors.lastname: Sugimoto
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Kuwajima
authors.forename: Nobutada
authors.initials: N
authors.lastname: Tabata
authors.forename: Kosuke
authors.initials: K
authors.lastname: Morita
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Okada
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Takemura
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003176:Complement C3 / Q000276:immunology*; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology*; D006801:Humans; D008297:Male; D016896:Treatment Outcome
chemical_list: D003176:Complement C3