title: IgA nephropathy with presentation of nephrotic syndrome at onset in children.
abstract: Despite a low incidence, nephrotic syndrome (NS) can present with IgA nephropathy (IgAN). The clinical characteristics and long-term outcomes of pediatric patients with IgAN presenting with NS (NS-IgAN) at onset have not been fully elucidated.
  
  
  
  We retrospectively analyzed 426 patients, and compared clinical and pathological (Oxford) findings between those with NS-IgAN and those with non-NS-IgAN.
  
  
  
  Among 426 patients, 30 (7.0 %) had NS-IgAN. Logistic analyses showed that male sex (OR: 7.6, p = 0.0002), M1 (OR: 10.3, p = 0.002), and E1 (OR: 15.2, p = 0.0001) were significantly related to NS. The mean observation period was 6.2 ± 3.2 years. Although NS-IgAN was associated with significantly lower renal survival than non-NS-IgAN according to Kaplan-Meier analysis (p = 0.02), renal survival of NS-IgAN was good (92.4 % at 10 years). The most significant prognostic factor for renal survival was remission of proteinuria after treatment, and NS at onset is also a significant prognostic factor for renal survival after adjusting for remission of proteinuria. Twenty children with NS-IgAN were treated with prednisolone alone, or prednisolone and immunosuppressant. Remission of proteinuria occurred in 21 patients. Three cases of NS-IgAN progressed to stage III-V chronic kidney disease at the most recent observation. They all demonstrated heavy proteinuria after the 2-year initial treatment. The significant factor for persistent proteinuria at 5 years was S1 in NS-IgAN.
  
  
  
  The most significant factor for renal survival was responsiveness to treatment, not NS itself. As modifiable acute lesions are the dominant pathological findings in NS-IgAN, histological improvements achieved by appropriate treatments can result in a favorable prognosis.
authors.affiliation: Department of Pediatrics, Wakayama Medical University, 811-1 Kimiidera, Wakayama City, Wakayama, 641-8509, Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Shima
authors.affiliation: Department of Pediatrics, Wakayama Medical University, 811-1 Kimiidera, Wakayama City, Wakayama, 641-8509, Japan. knakanis@wakayama-med.ac.jp.
authors.forename: Koichi
authors.identifier: 0000-0001-8681-9129
authors.initials: K
authors.lastname: Nakanishi
authors.affiliation: Department of Pediatrics, Wakayama Medical University, 811-1 Kimiidera, Wakayama City, Wakayama, 641-8509, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Sato
authors.affiliation: Department of Pediatrics, Wakayama Medical University, 811-1 Kimiidera, Wakayama City, Wakayama, 641-8509, Japan.
authors.forename: Taketsugu
authors.initials: T
authors.lastname: Hama
authors.affiliation: Department of Pediatrics, Wakayama Medical University, 811-1 Kimiidera, Wakayama City, Wakayama, 641-8509, Japan.
authors.forename: Hironobu
authors.initials: H
authors.lastname: Mukaiyama
authors.affiliation: Department of Pediatrics, Wakayama Medical University, 811-1 Kimiidera, Wakayama City, Wakayama, 641-8509, Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Togawa
authors.affiliation: Department of Nephrology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyogo, Japan.
authors.forename: Ryojiro
authors.initials: R
authors.lastname: Tanaka
authors.affiliation: Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan.
authors.forename: Kandai
authors.initials: K
authors.lastname: Nozu
authors.affiliation: Division for Clinical Trials, Center for Clinical Research and Development, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Mayumi
authors.initials: M
authors.lastname: Sako
authors.affiliation: Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan.
authors.forename: Kazumoto
authors.initials: K
authors.lastname: Iijima
authors.affiliation: Department of Pediatrics, Wakayama Medical University, 811-1 Kimiidera, Wakayama City, Wakayama, 641-8509, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Suzuki
authors.affiliation: Clinical Research Center, Wakayama Medical University, Wakayama City, Wakayama, Japan.
authors.forename: Norishige
authors.initials: N
authors.lastname: Yoshikawa
mesh_terms: D017668:Age of Onset; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D005922:Glomerulonephritis, IGA / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D053208:Kaplan-Meier Estimate; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D009404:Nephrotic Syndrome / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D011507:Proteinuria / Q000188:drug therapy / Q000209:etiology; D051436:Renal Insufficiency, Chronic / Q000188:drug therapy / Q000209:etiology; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D011239:Prednisolone
keywords: End-stage renal disease; IgA nephropathy; Nephrotic syndrome; Remission of proteinuria