Disturbed remodeling and delayed fracture healing in pediatric pycnodysostosis patients.
J Orthop
; 16(5): 373-377, 2019.
Article
em En
| MEDLINE
| ID: mdl-31048950
ABSTRACT
Pycnodysostosis is an autosomal recessive disease caused by a gene mutation leading cathepsin K deficiency. Pathological fractures of the long bones are common, but guidelines on fracture treatment in these patients are still lacking. We have treated 5 fractures in 2 pediatric pycnodysostosis patients. We hypothesize that pycnodysostosis patients have an incomplete remodeling process in fracture healing because of cathepsin K deficiency. Therefore, to minimize the role of endochondral bone formation (indirect) after a fracture, it seems prudent to strive for direct bone healing (intramembranous) instead of indirect bone healing. Open reduction with internal fixation should be the goal.
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1
Coleções:
01-internacional
Base de dados:
MEDLINE
Tipo de estudo:
Guideline
Idioma:
En
Ano de publicação:
2019
Tipo de documento:
Article