Literature DB >> 25253244

The characteristics of scoliosis in Prader-Willi syndrome (PWS): analysis of 58 scoliosis patients with PWS.

Yutaka Nakamura1, Nobuyuki Murakami, Takahiro Iida, Satoru Ozeki, Satoshi Asano, Yutaka Nohara, Toshiro Nagai.   

Abstract

BACKGROUND: The purpose of this study was to compare the characteristics of scoliosis in Prader-Willi syndrome (PWS) patients versus idiopathic scoliosis (IS).
METHODS: We identified 193 PWS patients. Scoliosis was found in 58 PWS patients, 39 of whom were treated with GH. Fifty-five IS patients were consecutively selected from an outpatient clinic. We investigated 113 patients (58 PWS group, 55 IS group) followed for a minimum of 2 years. The mean age was 17.9 and 16.1 years, respectively. Deformity was measured using Lenke classification, Cobb angle, thoracic kyphosis at T2-5 and T5-12, lumbar lordosis at T12-S1, and sagittal alignment at the C7 plumb line. BMI was also recorded.
RESULTS: According to the Lenke system, patients were classified as (PWS group/IS group): Type 1 (8/26), Type 2 (1/4), Type 3 (3/15), Type 4 (1/0), Type 5 (32/8), and Type 6 (13/2). The average Cobb angles were 32.6° in the PWS and 35.4° in the IS. No significant differences were found for the thoracic kyphosis (T2-5, T5-12), lumbar lordosis (T12-S1) or C7 plumb line between the two groups. BMI was increased in the PWS group not treated previously with GH as compared with the IS group and the PWS group with GH.
CONCLUSIONS: Most PWS patients presented with lumbar or thoracolumbar curves (Type 5, 6), whereas IS patients typically had thoracic scoliosis (Type 1, 2, 3).

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Year:  2014        PMID: 25253244     DOI: 10.1007/s00776-014-0651-y

Source DB:  PubMed          Journal:  J Orthop Sci        ISSN: 0949-2658            Impact factor:   1.601


  7 in total

1.  Growth hormone receptor (GHR) gene polymorphism and scoliosis in Prader-Willi syndrome.

Authors:  Merlin G Butler; Waheeda Hossain; Maaz Hassan; Ann M Manzardo
Journal:  Growth Horm IGF Res       Date:  2017-12-06       Impact factor: 2.372

Review 2.  Cellular and disease functions of the Prader-Willi Syndrome gene MAGEL2.

Authors:  Klementina Fon Tacer; Patrick Ryan Potts
Journal:  Biochem J       Date:  2017-06-16       Impact factor: 3.857

3.  Spinal Gorham-Stout syndrome: radiological changes and spinal deformities.

Authors:  Chang-Zhi Du; Song Li; Liang Xu; Qing-Shuang Zhou; Ze-Zhang Zhu; Xu Sun; Yong Qiu
Journal:  Quant Imaging Med Surg       Date:  2019-04

4.  Respiratory Failure due to Severe Obesity and Kyphoscoliosis in a 24-Year-Old Male with Molecularly Confirmed Prader-Willi Syndrome in Tertiary Hospital in Northern Tanzania.

Authors:  Elichilia R Shao; Lucas F Kiyegi; Amos O Mwasamwaja; Kajiru Kilonzo; Ben C J Hamel
Journal:  Case Rep Genet       Date:  2017-04-09

Review 5.  Clinical Observations and Treatment Approaches for Scoliosis in Prader-Willi Syndrome.

Authors:  Harold J P van Bosse; Merlin G Butler
Journal:  Genes (Basel)       Date:  2020-02-28       Impact factor: 4.096

6.  Influence of molecular classes and growth hormone treatment on growth and dysmorphology in Prader-Willi syndrome: A multicenter study.

Authors:  Ranim Mahmoud; Anna Leonenko; Merlin G Butler; Pamela Flodman; June-Anne Gold; Jennifer L Miller; Elizabeth Roof; Elisabeth Dykens; Daniel J Driscoll; Virginia Kimonis
Journal:  Clin Genet       Date:  2021-03-13       Impact factor: 4.296

7.  High Prevalence of Scoliosis in a Large Cohort of Patients with Prader-Willi Syndrome.

Authors:  Antonino Crinò; Michela Armando; Marco Crostelli; Osvaldo Mazza; Dario Bruzzese; Alessio Convertino; Danilo Fintini; Sarah Bocchini; Sara Ciccone; Alessandro Sartorio; Graziano Grugni
Journal:  J Clin Med       Date:  2022-03-13       Impact factor: 4.241

  7 in total

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