Patients and methods
Sixty-five children seen in a Paediatric Hospital during six months in 2007 were evaluated. Results
Thirty-five were type I OI, and thirty were types III–IV. Median age was 7.8 years (range 1.9–19.2); mean length of follow up was 4.7 years. The majority of children attended regular school for their corresponding age. Mean height was −1.4 sDS and −5.64 sDS in types I and III–IV respectively.
Osteogenesis imperfecta. Clinical, functional and multidisciplinary evaluation of 65 patients
 
  Keywords
 
 
  
   Osteogenesis Imperfecta
  
 
 
  
   Disability evaluation
  
 
 
  
   Pain
  
 
 
  
   Scoliosis
  
 
 
  
   Multidisciplinary treatment
  
 
 
  
   Anthropometry
  
 
 
  
   Children
  
 
Abstract
 Introduction
Osteogenesis Imperfecta (OI) is a genetic disease, in which the main clinical features are increased bone fragility, pathological fractures, blue sclera, dentinogenesis imperfecta and <dis>conductive or mixed hearing loss</dis>.
Clinical variability is wide.
Although there is no curative treatment, there are several therapeutic tools capable of improving the course of the condition and patient quality of life.
Patients and methods
Sixty-five children seen in a Paediatric Hospital during six months in 2007 were evaluated.
Results
Thirty-five were type I OI, and thirty were types III–IV.
Median age was 7.8 years (range 1.9–19.2); mean length of follow up was 4.7 years.
The majority of children attended regular school for their corresponding age.
Mean height was −1.4 sDS and −5.64 sDS in types I and III–IV respectively.
Nineteen percent of patients were overweight and 11% were obese.
Mean age at first orthopaedic surgery inserting telescopic rods was 6.5 years.
Scoliosis was present in 44.6% of patients and was directly related to severity.
Bleck′s motor scale showed that 93% of patients with mild forms and 29% of severe forms had a sustainable walking ability.
A wheelchair was used by 25% of patients.
Family inheritance was confirmed in 65% of cases.
Conclusions
Integral care using a multidisciplinary approach is required due to the complexity and clinical variability of the condition.
Abstract
 Introduction
Osteogenesis Imperfecta (OI) is a genetic disease, in which the main clinical features are increased bone fragility, pathological fractures, blue sclera, dentinogenesis imperfecta and conductive or mixed hearing loss.
Clinical variability is wide.
Although there is no curative treatment, there are several therapeutic tools capable of improving the course of the condition and patient quality of life.
Patients and methods
Sixty-five children seen in a Paediatric Hospital during six months in 2007 were evaluated.
Results
Thirty-five were type I OI, and thirty were types III–IV.
Median age was 7.8 years (range 1.9–19.2); mean length of follow up was 4.7 years.
The majority of children attended regular school for their corresponding age.
Mean height was −1.4 sDS and −5.64 sDS in types I and III–IV respectively.
Nineteen percent of patients were overweight and 11% were obese.
Mean age at first orthopaedic surgery inserting telescopic rods was 6.5 years.
Scoliosis was present in 44.6% of patients and was directly related to severity.
Bleck′s motor scale showed that 93% of patients with mild forms and 29% of severe forms had a sustainable walking ability.
A wheelchair was used by 25% of patients.
Family inheritance was confirmed in 65% of cases.
Conclusions
Integral care using a multidisciplinary approach is required due to the complexity and clinical variability of the condition.
Patients and methods
Sixty-five children seen in a Paediatric Hospital during six months in 2007 were evaluated. Results
Thirty-five were type I OI, and thirty were types III–IV. Median age was 7.8 years (range 1.9–19.2); mean length of follow up was 4.7 years. The majority of children attended regular school for their corresponding age. Mean height was −1.4 sDS and −5.64 sDS in types I and III–IV respectively.