[Clinical, radiographic and laboratory evaluation of patients with osteogenesis imperfecta].
BACKGROUND
Osteogenesis imperfecta is a genetic disorder characterized by defects in type I collagen. The main symptom is bone fragility and susceptibility to fractures. Other clinical findings are dentinogenesis imperfecta, blue sclera, early deafness and joint laxity. The purpose of this paper is to establish a practical relationship of the clinical differences between the Sillence's groups.


METHODS
22 patients were classified according to Sillence et al criteria and submitted to laboratory tests including blood calcium level and bone densitometry.


RESULTS
All clinical and laboratory differences were discussed in the text.


CONCLUSIONS
Differences such as results that were found in walking ability, height and bone densitometry were significant and may help to classify patients and to establish prognosis.
Santili
Cláudio
C
Grupo de Ortopedia Pediátrica, Departamento de Ortopedia e Traumatologia, Hospital Central da Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo (SP), Brazil. c.santili@terra.com.br
Akkari
Miguel
M
Waisberg
Gilberto
G
Bastos Júnior
José Olympio Catão
JO
Ferreira
William Martins
WM
D015502:Absorptiometry, Photon; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001827:Body Height; D002118:Calcium / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003811:Dentinogenesis Imperfecta / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010013:Osteogenesis Imperfecta / Q000145:classification / Q000175:diagnosis* / Q000000981:diagnostic imaging; D011379:Prognosis; D012590:Sclera / Q000002:abnormalities; D012737:Sex Factors; D018709:Statistics, Nonparametric
D002118:Calcium