Article

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). DESIGN: Retrospective study where
outcomes were measured prospectively. SETTING:
Pediatric orthopedic hospital.
PARTICIPANTS: 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.
INTERVENTIONS: Intravenous bisphosphonate treatment, orthopedic surgery, and
rehabilitation. MAIN OUTCOME MEASURE: 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.
CI - Copyright (c) 2015 American Congress of Rehabilitation Medicine. Published by
Elsevier Inc. All rights reserved.

Langue : ANGLAIS