title: [Favorable course of collagenofibrotic glomerulopathy after kidney transplantation and questionnaire survey about the prognosis of collagenofibrotic glomerulopathy].
abstract: Collagenofibrotic glomerulopathy is a recently recognized entity that is characterized by massive accumulation of collagen fibrils in the mesangial and subendothelial areas, and an elevated serum level of procollagen III peptide (PIIIP). We report the first case of a collagenofibrotic glomerulopathy patient who received a kidney transplantation. She received the kidney transplantation at the age of 18 years and the post-operative course was uneventful with good renal function on immunosuppression, which consisted of methylprednisolone, tacrolimus and basiliximab. Although urinary protein was negative, the serum level of PIIIP gradually elevated which suggests new collagen production in the graft. These findings indicate that the recipient had a systemic factor that stimulated collagen production. To investigate the prognosis of collagenofibrotic glomerulopathy, we carried out a questionnaire survey on 14 patients at 9 hospitals. None of these patients had received a kidney transplantation. However, 7 already had end-stage renal failure. Ten years after diagnosis, the renal survival rate was 49%. This rate is lower than cited in previous reports.
authors.affiliation: Division of Pediatrics, Niigata University Graduate School of Medicine and Dental Sciences, Niigata, Japan.
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Suzuki
authors.forename: Soichiro
authors.initials: S
authors.lastname: Okubo
authors.forename: Yohei
authors.initials: Y
authors.lastname: Ikezumi
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Ueno
authors.forename: Shinichi
authors.initials: S
authors.lastname: Nishi
authors.forename: Kazuhide
authors.initials: K
authors.lastname: Saito
authors.forename: Kouta
authors.initials: K
authors.lastname: Takahashi
authors.forename: Makoto
authors.initials: M
authors.lastname: Uchiyama
mesh_terms: D000328:Adult; D002908:Chronic Disease; D003094:Collagen / Q000378:metabolism*; D005260:Female; D005355:Fibrosis; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D007678:Kidney Glomerulus / Q000378:metabolism / Q000473:pathology; D016030:Kidney Transplantation; D010446:Peptide Fragments / Q000097:blood; D011347:Procollagen / Q000097:blood; D011379:Prognosis; D011795:Surveys and Questionnaires
chemical_list: D010446:Peptide Fragments; D011347:Procollagen; C041876:procollagen Type III-N-terminal peptide; D003094:Collagen