title: Chronic tubulo-interstitial nephropathy in children with cranioectodermal dysplasia.
abstract: We report two pairs of siblings with the syndrome cranioectodermal dysplasia who have subsequently developed chronic renal failure secondary to tubulo-interstitial nephropathy. Renal disease has not been described before in this rare syndrome but has now occurred in four of the seven reported cases. Children with cranioectodermal dysplasia appear to be at significant risk of developing chronic renal failure. This is important information for clinicians looking after such children, and their families, when considering the long-term prognosis and management.
authors.affiliation: Paediatric Renal Unit, Nottingham City Hospital, UK.
authors.forename: G A
authors.initials: GA
authors.lastname: Savill
authors.forename: I D
authors.initials: ID
authors.lastname: Young
authors.forename: R J
authors.initials: RJ
authors.lastname: Cunningham
authors.forename: I D
authors.initials: ID
authors.lastname: Ansell
authors.forename: J H
authors.initials: JH
authors.lastname: Evans
mesh_terms: D002675:Child, Preschool; D002908:Chronic Disease; D004476:Ectodermal Dysplasia / Q000150:complications / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000473:pathology; D008297:Male; D009395:Nephritis, Interstitial / Q000150:complications / Q000473:pathology*