Literature DB >> 10441826

[Scoliosis in patients with osteogenesis imperfecta: a federal nation-wide cross-sectional study].

A Karbowski1, M Schwitalle, A Eckardt.   

Abstract

OBJECTIVE: Quite different prevalence of scoliosis in patients with Osteogenesis imperfecta is reported, not at least owing to the difficult recruitment of patients. The present study aims to investigate Osteogenesis imperfecta-patients with scoliosis by means of a cross-section study. PATIENTS AND METHODS: A total of 102 patients (46 males, 56 females) with an average age of 24.6 years (3-71 yrs.) were investigated in 1995 supported by the "Gesellschaft für Osteogenesis imperfecta Betroffene e.V.". Physical examination included pain and range of motion. Localisation and expanse of the curvature as well as attendant deformities of the spine were assessed by radiograph.
RESULTS: Scoliosis was observed in 76 patients (74.5%). Most of them showed thoracal or thoracolumbar localisation with 36 cases of right and 40 cases of left convexity. The curvature was mainly mild (56 pat. < 40 degrees), while 8 patients showed moderate (< 60 degrees), 7 patients severe (< 80 degrees) and 5 patients very severe deformity (> 80 degrees). Average expansion amounted to 6.7 vertebrae (3-12) with an average rotation of 2 (1-4). Attendant deformities were distributed as follows: codfish (59 pat.) or wedge-shaped vertebrae (42 pat.), platyspondylia (16 pat.), thoracal cyphosis (5 pat.), flat back (1 pat.) or lordosis (2 pat.) and lumbar lordosis (10 pat.) or vertical position (14 pat.). 69 scoliosis patients complained about pain with an average VAS-value of 4.3. They also showed moderate to severe impairment of the range of motion.
CONCLUSIONS: Our cross-section study with 102 Osteogenesis imperfecta-patients showed scoliotic deformity in 74.5% of cases. 69 patients suffered from remarkable pain and impaired range of motion, which was not only attributable to scoliosis alone, but also to concomitant spinal deformities.

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Mesh:

Year:  1999        PMID: 10441826     DOI: 10.1055/s-2008-1037397

Source DB:  PubMed          Journal:  Z Orthop Ihre Grenzgeb        ISSN: 0044-3220


  7 in total

Review 1.  Osteogenesis imperfecta: pathophysiology and treatment.

Authors:  Heike Hoyer-Kuhn; Christian Netzer; Oliver Semler
Journal:  Wien Med Wochenschr       Date:  2015-06-09

Review 2.  The genetic implication of scoliosis in osteogenesis imperfecta: a review.

Authors:  Gang Liu; Jia Chen; Yangzhong Zhou; Yuzhi Zuo; Sen Liu; Weisheng Chen; Zhihong Wu; Nan Wu
Journal:  J Spine Surg       Date:  2017-12

3.  The clinical features of osteogenesis imperfecta in Vietnam.

Authors:  Ho Duy Binh; Katre Maasalu; Vu Chi Dung; Can T Bich Ngoc; Ton That Hung; Tran V Nam; Le N Thanh Nhan; Ele Prans; Ene Reimann; Lidiia Zhytnik; Sulev Kõks; Aare Märtson
Journal:  Int Orthop       Date:  2016-11-02       Impact factor: 3.075

4.  Rare causes of scoliosis and spine deformity: experience and particular features.

Authors:  Konstantinos C Soultanis; Alexandros H Payatakes; Vasilios T Chouliaras; Georgios C Mandellos; Nikolaos E Pyrovolou; Fani M Pliarchopoulou; Panayotis N Soucacos
Journal:  Scoliosis       Date:  2007-10-23

5.  Genotype and phenotype analysis of Taiwanese patients with osteogenesis imperfecta.

Authors:  Hsiang-Yu Lin; Chih-Kuang Chuang; Yi-Ning Su; Ming-Ren Chen; Hui-Chin Chiu; Dau-Ming Niu; Shuan-Pei Lin
Journal:  Orphanet J Rare Dis       Date:  2015-12-01       Impact factor: 4.123

Review 6.  Managing the patient with osteogenesis imperfecta: a multidisciplinary approach.

Authors:  Caroline Marr; Alison Seasman; Nick Bishop
Journal:  J Multidiscip Healthc       Date:  2017-04-04

Review 7.  Complex spine deformities in young patients with severe osteogenesis imperfecta: current concepts review.

Authors:  R M Castelein; C Hasler; I Helenius; D Ovadia; M Yazici
Journal:  J Child Orthop       Date:  2019-02-01       Impact factor: 1.548

  7 in total

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