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Ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: A case report

RATIONALE: Progressive pseudorheumatoid dysplasia (PPD) is a rare autosomal recessive inherited disease that causes severe systemic joint deformity and articular dysfunction in young patients. PATIENT CONCERNS: Ilizarov technique treatment in PPD patients has never been reported before. DIAGNOSES: A...

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Autores principales: Xiao, Ke, Li, Tao, Jiang, Yaping, Li, Zheng, Zhu, Qiankun, Wu, Zhihong, Weng, Xisheng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6081158/
https://www.ncbi.nlm.nih.gov/pubmed/30075503
http://dx.doi.org/10.1097/MD.0000000000011375
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author Xiao, Ke
Li, Tao
Jiang, Yaping
Li, Zheng
Zhu, Qiankun
Wu, Zhihong
Weng, Xisheng
author_facet Xiao, Ke
Li, Tao
Jiang, Yaping
Li, Zheng
Zhu, Qiankun
Wu, Zhihong
Weng, Xisheng
author_sort Xiao, Ke
collection PubMed
description RATIONALE: Progressive pseudorheumatoid dysplasia (PPD) is a rare autosomal recessive inherited disease that causes severe systemic joint deformity and articular dysfunction in young patients. PATIENT CONCERNS: Ilizarov technique treatment in PPD patients has never been reported before. DIAGNOSES: A 17-year-old male patient presented with a 10-year history of polyarthritis and 4-year history of progressive hip and knee pain and stiffness. Genetic testing for the WISP3 gene was done and showed compound heterozygous mutations: NM_198239.1 (WISP3):c.1064_1065dupGT (p.Gln356ValfsTer33) and NM_198239.1 (WISP3):c.643+2T > C. INTERVENTIONS: Taking his young age into consideration, the Ilizarov external fixation technique was adopted for the treatment of the deformity in knees. OUTCOMES: One year after the operation, the improvement of joint deformity was satisfactory. LESSONS: The Ilizarov technique is economical and less invasive, and most importantly, it can delay the possible arthroplasty. It gives young PPD patients with arthropathy an alternative treatment.
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spelling pubmed-60811582018-08-17 Ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: A case report Xiao, Ke Li, Tao Jiang, Yaping Li, Zheng Zhu, Qiankun Wu, Zhihong Weng, Xisheng Medicine (Baltimore) Research Article RATIONALE: Progressive pseudorheumatoid dysplasia (PPD) is a rare autosomal recessive inherited disease that causes severe systemic joint deformity and articular dysfunction in young patients. PATIENT CONCERNS: Ilizarov technique treatment in PPD patients has never been reported before. DIAGNOSES: A 17-year-old male patient presented with a 10-year history of polyarthritis and 4-year history of progressive hip and knee pain and stiffness. Genetic testing for the WISP3 gene was done and showed compound heterozygous mutations: NM_198239.1 (WISP3):c.1064_1065dupGT (p.Gln356ValfsTer33) and NM_198239.1 (WISP3):c.643+2T > C. INTERVENTIONS: Taking his young age into consideration, the Ilizarov external fixation technique was adopted for the treatment of the deformity in knees. OUTCOMES: One year after the operation, the improvement of joint deformity was satisfactory. LESSONS: The Ilizarov technique is economical and less invasive, and most importantly, it can delay the possible arthroplasty. It gives young PPD patients with arthropathy an alternative treatment. Wolters Kluwer Health 2018-08-03 /pmc/articles/PMC6081158/ /pubmed/30075503 http://dx.doi.org/10.1097/MD.0000000000011375 Text en Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0
spellingShingle Research Article
Xiao, Ke
Li, Tao
Jiang, Yaping
Li, Zheng
Zhu, Qiankun
Wu, Zhihong
Weng, Xisheng
Ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: A case report
title Ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: A case report
title_full Ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: A case report
title_fullStr Ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: A case report
title_full_unstemmed Ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: A case report
title_short Ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: A case report
title_sort ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: a case report
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6081158/
https://www.ncbi.nlm.nih.gov/pubmed/30075503
http://dx.doi.org/10.1097/MD.0000000000011375
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