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Orthopedic Manifestations of Bruck Syndrome: A Case Series with Intermediate to Long-term Follow-Up
Joint Authors
Santana, Adolfredo
Oleas-Santillán, Geovanny
Franzone, Jeanne M.
Nichols, L. Reid
Bowen, J. Richard
Kruse, Richard W.
Source
Issue
Vol. 2019, Issue 2019 (31 Dec. 2019), pp.1-7, 7 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2019-03-13
Country of Publication
Egypt
No. of Pages
7
Main Subjects
Abstract EN
The aim of this study was to evaluate the association of contractures, fractures, and deformities in four patients with Bruck syndrome treated in our facility.
Data were collected from medical records, radiographs, dual-energy X-ray absorptiometry (DEXA) scans, genetic tests, and gait analysis.
All had contractures at birth and genotypic findings including mutations in PLOD2 or FPKB10.
Three cases were treated with bisphosphonates with improvement in bone density verified by DEXA.
In Bruck syndrome, orthopedic deformities include the following sequential aspects: contractures, characterized by upper and lower extremity contractures such as clubfeet; fractures, characterized by multiple diaphyseal fractures in the long bones of the extremities; and deformities, characterized by malalignment of extremities and the spine.
Physical therapy and bracing proved helpful for the contractures to try to stop progression.
Bone fragility needs to be considered when deciding to attempt cast correction.
Surgeries in the soft tissues can be performed to retain joint movement.
In fractures with angulation, intramedullary nail fixation was useful, and in cases without deformity, casting alone was successful.
We suggest monitoring the bone density with DEXA, nutrition support with vitamin D and calcium, and treatment with bisphosphonates.
Spine deformities were successfully treated by spinal fusion and instrumentation.
American Psychological Association (APA)
Santana, Adolfredo& Oleas-Santillán, Geovanny& Franzone, Jeanne M.& Nichols, L. Reid& Bowen, J. Richard& Kruse, Richard W.. 2019. Orthopedic Manifestations of Bruck Syndrome: A Case Series with Intermediate to Long-term Follow-Up. Case Reports in Orthopedics،Vol. 2019, no. 2019, pp.1-7.
https://search.emarefa.net/detail/BIM-1142980
Modern Language Association (MLA)
Santana, Adolfredo…[et al.]. Orthopedic Manifestations of Bruck Syndrome: A Case Series with Intermediate to Long-term Follow-Up. Case Reports in Orthopedics No. 2019 (2019), pp.1-7.
https://search.emarefa.net/detail/BIM-1142980
American Medical Association (AMA)
Santana, Adolfredo& Oleas-Santillán, Geovanny& Franzone, Jeanne M.& Nichols, L. Reid& Bowen, J. Richard& Kruse, Richard W.. Orthopedic Manifestations of Bruck Syndrome: A Case Series with Intermediate to Long-term Follow-Up. Case Reports in Orthopedics. 2019. Vol. 2019, no. 2019, pp.1-7.
https://search.emarefa.net/detail/BIM-1142980
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1142980