Ortopedická protetika Praha sro - Společnost pro pojivové tkáně

-bone deformities, and/or children
with OI type III or IV.
3. In severe cases, treatment can be started
during infancy, but these children need
to be monitored very closely, especially
during the first infusion cycle.
4. Treatment continues to be effective
in older teenagers and the upper age
limit of responsiveness still remains to
be defined.
5. Several dosage regimens appear to be
effective.
6. All children treated with a bisphosphonate
should be part of a well run
clinical trial. This will enable the safety
and efficacy of these compounds can
be adequately evaluated.
7. Further study is required before bisphosphonates
can be administered to
all patients with OI on the basis of low
BMD alone.
TEAMWORK
Optimal care of children with osteogenesis
imperfecta necessities a multi-disciplinary
approach.
It is imperative that there are open lines
of communication between team members.
This is especially important around critical
events such as fracture and surgery. With
this in mind, The Children’s Hospital at
Westmead has established an Osteogenesis
Imperfecta Clinic, which includes a paediatric
endocrinologist, two orthopaedic
surgeons, a geneticist, two clinical nurse
practitioners, a physiotherapist, a rehabilitation
physician, an orthotist, a social worker
and a musculoskeletal radiologist. At
one clinic visit the patient can be assessed
by all the therapists involved in his/her
care. The physiotherapist and rehabilita-
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tion physician play an important role in
assessing gross motor development and,
maintaining patient mobility and planning
rehabilitation following orthopaedic surgery.
Bisphosphonate therapy is prescribed by
the endocrinologist according to an internationally
accepted regime but varied in each
patient based on the results of XRays and
bone density studies. Bisphosphonate therapy
can be titrated around surgical intervention
to decrease the incidence of delayed
union and non-union of osteotomies.
Maintaining weight bearing activities
and early postoperative mobilisation is
aided by fitting orthoses.
The team approach is also an excellent
moral booster for families of OI patients
and it means less disruption to family life
with fewer visits to the hospital.
REFERENCES
1. SILLENCE D.O., SENN A., DANKS D.M.
Genetic heterogenicity in osteogenesis imperfecta.
J Med Genet 1979; 16: 101–116.
2. KUIVANIEMI H., TROMP G., PROCKOP
D.J. Mutations in fibrillar collagens (types I, II,
III, and XI), fibril- associated collagen (type IX),
and network-forming collagen (type X) cause
a spectrum of diseases of bone, cartilage, and
blood vessels. Hum Mutat 1997; 9: 300–315.
3. GLORIEUX F.H., RAUCH F., PLOTKIN H.,
WARD L., TRAVERS R., ROUGHLEY P., et al.
Type V osteogenesis imperfecta: a new form of
brittle bone disease. J Bone Miner Res 2000; 15:
1650–1658.
4. GLORIEUX F.H., WARD L.M., RAUCH F.,
LALIC L., TRAVERS R. Osteogenesis imperfecta
type VI: a form of brittle bone disease with
a mineralization defect. J Bone Miner Res 2002;
17: 30–38.
LOCOMOTOR SYSTEM vol. 14, 2007, No. 3+4