title: Multidisciplinary Treatment of Severe Osteogenesis Imperfecta: Functional Outcomes at Skeletal Maturity.
abstract: OBJECTIVE
  To determine the functional outcomes associated with long-term multidisciplinary treatment, intravenous bisphosphonate treatment, orthopedic surgery, and rehabilitation in children with severe osteogenesis imperfecta (OI) (diagnosed clinically as OI types III or IV).
  
  
  METHODS
  Retrospective study where outcomes were measured prospectively.
  
  
  METHODS
  Pediatric orthopedic hospital.
  
  
  METHODS
  Adolescents (N=41; age range, 15-21y) with severe OI (OI type III: n=17; OI type IV: n=24) who had started therapy before the age of 6 years, had received treatment for at least 10 years, and had achieved final height.
  
  
  METHODS
  Intravenous bisphosphonate treatment, orthopedic surgery, and rehabilitation.
  
  
  METHODS
  Pediatric Evaluation of Disability Inventory.
  
  
  RESULTS
  At the time of the last available follow-up examination, none of the individuals diagnosed with OI type III (most severely affected group) was able to ambulate without ambulation aids, whereas 20 (83%) patients with OI type IV were able to ambulate without ambulation aids. Regarding self-care, we specifically assessed 8 skills that we deemed essential for living independently (grooming; dressing; toileting; bed, chair, toilet, tub, and car transfers). Only 6 (35%) of the youths with OI type III were able to complete all 8 items, whereas 23 (96%) individuals with OI type IV managed to perform all tasks. Teens with OI type III often needed assistance for the transfer to toilet, tub, and car and for personal hygiene and clothing management associated with toileting, usually because of limitations in upper-extremity function.
  
  
  CONCLUSIONS
  These observations suggest that further improvements in the functional status of the most severely affected children with OI are contingent on advances in the clinical management of upper-extremity issues.
authors.affiliation: Shriners Hospital for Children, Montreal, QC, Canada. Electronic address: kmontpetit@shrinenet.org.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Montpetit
authors.affiliation: Shriners Hospital for Children, Montreal, QC, Canada.
authors.forename: Telma
authors.initials: T
authors.lastname: Palomo
authors.affiliation: Shriners Hospital for Children, Montreal, QC, Canada; McGill University, Montreal, QC, Canada.
authors.forename: Francis H
authors.initials: FH
authors.lastname: Glorieux
authors.affiliation: Shriners Hospital for Children, Montreal, QC, Canada; McGill University, Montreal, QC, Canada.
authors.forename: François
authors.initials: F
authors.lastname: Fassier
authors.affiliation: Shriners Hospital for Children, Montreal, QC, Canada; McGill University, Montreal, QC, Canada.
authors.forename: Frank
authors.initials: F
authors.lastname: Rauch
mesh_terms: D000293:Adolescent; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D004164:Diphosphonates / Q000627:therapeutic use; D004185:Disability Evaluation; D005260:Female; D006801:Humans; D008297:Male; D019637:Orthopedic Procedures; D010013:Osteogenesis Imperfecta / Q000628:therapy*; D016059:Range of Motion, Articular; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D050071:Bone Density Conservation Agents; D004164:Diphosphonates
keywords: Mobility limitation; Osteogenesis imperfecta; Rehabilitation; Self care