Abstract
| Original language | English |
|---|---|
| Pages (from-to) | 373-377 |
| Number of pages | 5 |
| Journal | Journal of Orthopaedics |
| Volume | 16 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - 1 Sep 2019 |
Cite this
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Disturbed remodeling and delayed fracture healing in pediatric pycnodysostosis patients. / Grewal, Simran; Kilic, Özgür; Savci-Heijink, C. Dilara; Kloen, Peter.
In: Journal of Orthopaedics, Vol. 16, No. 5, 01.09.2019, p. 373-377.Research output: Contribution to journal › Article › Academic › peer-review
TY - JOUR
T1 - Disturbed remodeling and delayed fracture healing in pediatric pycnodysostosis patients
AU - Grewal, Simran
AU - Kilic, Özgür
AU - Savci-Heijink, C. Dilara
AU - Kloen, Peter
PY - 2019/9/1
Y1 - 2019/9/1
N2 - 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.
AB - 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.
KW - Endochondral
KW - Fracture
KW - Intramembranous
KW - Osteoclasts
KW - Pycnodysostosis
KW - Remodeling
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85064545973&origin=inward
UR - https://www.ncbi.nlm.nih.gov/pubmed/31048950
U2 - 10.1016/j.jor.2019.03.022
DO - 10.1016/j.jor.2019.03.022
M3 - Article
VL - 16
SP - 373
EP - 377
JO - Journal of Orthopaedics
JF - Journal of Orthopaedics
SN - 0972-978X
IS - 5
ER -