title: Rapidly progressive, pauci-immune diffuse crescentic glomerulonephritis in an infant.
abstract: A Japanese male infant presented with nephrotic syndrome at 41 days. His renal function progressively deteriorated, and he died at 4 months of the age. An open renal biopsy revealed diffuse crescentic glomerulonephritis (CrGN) without immune complex deposition, which is not characteristic of the congenital nephrotic syndrome (CNS). Examination for nephrin antigen using rabbit anti-nephrin extra- and intracellular site antibodies was positive. These clinical observations suggest that the patient had a unique histological variant of CNS. This is the first report of rapidly progressive, pauci-immune diffuse CrGN in infancy.
authors.affiliation: Department of Pediatrics, Hirosaki University, School of Medicine, 5 Zaifu-cho, Hirosaki, 036-8562 Japan. hirotana@cc.hirosaki-u.ac.jp
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Tanaka
authors.forename: Shinobu
authors.initials: S
authors.lastname: Waga
authors.forename: Koichi
authors.initials: K
authors.lastname: Suzuki
authors.forename: Tohru
authors.initials: T
authors.lastname: Nakahata
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kawachi
authors.forename: Fujio
authors.initials: F
authors.lastname: Shimizu
authors.forename: Etsuro
authors.initials: E
authors.lastname: Ito
mesh_terms: D018450:Disease Progression; D005921:Glomerulonephritis / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009404:Nephrotic Syndrome / Q000151:congenital