title: Glomerular epithelial cell phenotype in diffuse mesangial sclerosis: a report of 2 cases with markedly increased urinary podocyte excretion.
abstract: We report 2 cases of diffuse mesangial sclerosis (DMS) accompanied by severe podocyte excretion in urine. Patient 1 was a 9-day-old girl with a WT1 mutation who developed Wilms tumor at 6 months of age and was subsequently diagnosed with Denys-Drash syndrome. Patient 2 was a 1-year-old boy without a WT1 abnormality but presenting with heavy proteinuria. In both patients, histological examination showed findings of DMS. Immunohistochemical staining for synaptopodin (a podocyte marker) revealed a reduced number of podocytes in the glomeruli with severe sclerosis; however, podocytes persisted in the relatively intact glomeruli. Some glomeruli were accompanied by sclerotic lesions surrounded by proliferating cells; immunofluorescence staining revealed a majority of these proliferating cells to be positive for claudin-1 (a parietal cell marker) but negative for synaptopodin. These findings suggest that podocyte loss and the consequent proliferation of parietal cells are common processes in the pathogenesis of DMS.
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, 1-784 Asahimachi-dori, Cyuo-ku, Niigata, 951-8520, Japan. Electronic address: ikezumi@med.niigata-u.ac.jp.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Ikezumi
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, 1-784 Asahimachi-dori, Cyuo-ku, Niigata, 951-8520, Japan.
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Suzuki
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, 1-784 Asahimachi-dori, Cyuo-ku, Niigata, 951-8520, Japan.
authors.forename: Tamaki
authors.initials: T
authors.lastname: Karasawa
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, 1-784 Asahimachi-dori, Cyuo-ku, Niigata, 951-8520, Japan.
authors.forename: Utako
authors.initials: U
authors.lastname: Kaneko
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, 1-784 Asahimachi-dori, Cyuo-ku, Niigata, 951-8520, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Yamada
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, 1-784 Asahimachi-dori, Cyuo-ku, Niigata, 951-8520, Japan.
authors.forename: Hiroya
authors.initials: H
authors.lastname: Hasegawa
authors.affiliation: Department of Kidney and Vascular Pathology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, 305-8575, Japan.
authors.forename: Michio
authors.initials: M
authors.lastname: Nagata
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, 1-784 Asahimachi-dori, Cyuo-ku, Niigata, 951-8520, Japan.
authors.forename: Akihiko
authors.initials: A
authors.lastname: Saitoh
mesh_terms: D030321:Denys-Drash Syndrome / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007678:Kidney Glomerulus / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D009404:Nephrotic Syndrome / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D010641:Phenotype; D050199:Podocytes / Q000473:pathology* / Q000502:physiology; D012598:Sclerosis / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D025721:WT1 Proteins / Q000235:genetics; D009396:Wilms Tumor / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology
chemical_list: D025721:WT1 Proteins
keywords: Denys-Drash syndrome; Diffuse mesangial sclerosis; Epithelial hyperplasia; Parietal epithelial cell; Podocyte; Urinary podocyte