Literature DB >> 28601949

Autosomal dominant brachyolmia: transient metaphyseal striations.

Yun-Jung Lim1, Hye-Ran Lee2, Ok-Hwa Kim3, Tae-Joon Cho2, Kun-Bo Park4.   

Abstract

We report transient proximal and distal femoral metaphyseal striations that have not previously been described in autosomal dominant brachyolmia. The pelvis/hip radiograph of a 13-year-old boy demonstrated bilaterally symmetrical proximal femoral metaphyseal vertical striations. Additional vertical striations were also observed at the distal femur and proximal tibia metaphysis. Radiography of the thoracolumbar spine demonstrated platyspondyly with irregular endplates and overfaced pedicles. TRPV4 mutations were confirmed in this patient. Similar proximal femoral metaphyseal vertical striations were noted in the patient's sibling. Those streaks disappeared on the follow-up radiographs, and we considered it a unique radiologic finding transiently observed in autosomal dominant brachyolmia.

Entities:  

Keywords:  Brachyolmia; Metaphyseal striations; TRPV4

Mesh:

Year:  2017        PMID: 28601949     DOI: 10.1007/s00256-017-2684-8

Source DB:  PubMed          Journal:  Skeletal Radiol        ISSN: 0364-2348            Impact factor:   2.199


  8 in total

1.  Novel and recurrent TRPV4 mutations and their association with distinct phenotypes within the TRPV4 dysplasia family.

Authors:  J Dai; O-H Kim; T-J Cho; M Schmidt-Rimpler; H Tonoki; K Takikawa; N Haga; K Miyoshi; H Kitoh; W-J Yoo; I-H Choi; H-R Song; D-K Jin; H-T Kim; H Kamasaki; P Bianchi; G Grigelioniene; S Nampoothiri; M Minagawa; S-i Miyagawa; T Fukao; C Marcelis; M C E Jansweijer; R C M Hennekam; F Bedeschi; A Mustonen; Q Jiang; H Ohashi; T Furuichi; S Unger; B Zabel; E Lausch; A Superti-Furga; G Nishimura; S Ikegawa
Journal:  J Med Genet       Date:  2010-06-24       Impact factor: 6.318

2.  Spondylo-epiphyseal dysplasia, Maroteaux type (pseudo-Morquio syndrome type 2), and parastremmatic dysplasia are caused by TRPV4 mutations.

Authors:  Gen Nishimura; Jin Dai; Ekkehart Lausch; Sheila Unger; André Megarbané; Hiroshi Kitoh; Ok Hwa Kim; Tae-Joon Cho; Francesca Bedeschi; Francesco Benedicenti; Roberto Mendoza-Londono; Margherita Silengo; Maren Schmidt-Rimpler; Jurgen Spranger; Bernhard Zabel; Shiro Ikegawa; Andrea Superti-Furga
Journal:  Am J Med Genet A       Date:  2010-06       Impact factor: 2.802

Review 3.  The importance of conventional radiography in the mutational analysis of skeletal dysplasias (the TRPV4 mutational family).

Authors:  Stefan F Nemec; Daniel H Cohn; Deborah Krakow; Vincent A Funari; David L Rimoin; Ralph S Lachman
Journal:  Pediatr Radiol       Date:  2011-08-24

Review 4.  Autosomal dominant brachyolmia in a large Swedish family: phenotypic spectrum and natural course.

Authors:  Giedre Grigelioniene; Stefan Geiberger; Eva Horemuzova; Eva Moström; Nina Jäntti; Lo Neumeyer; Eva Åström; Magnus Nordenskjöld; Ann Nordgren; Outi Mäkitie
Journal:  Am J Med Genet A       Date:  2014-03-26       Impact factor: 2.802

Review 5.  Brachyolmia: radiographic and genetic evidence of heterogeneity.

Authors:  M Shohat; R Lachman; H E Gruber; D L Rimoin
Journal:  Am J Med Genet       Date:  1989-06

6.  Gain-of-function mutations in TRPV4 cause autosomal dominant brachyolmia.

Authors:  Matthew J Rock; Jean Prenen; Vincent A Funari; Tara L Funari; Barry Merriman; Stanley F Nelson; Ralph S Lachman; William R Wilcox; Soraya Reyno; Roberto Quadrelli; Alicia Vaglio; Grzegorz Owsianik; Annelies Janssens; Thomas Voets; Shiro Ikegawa; Toshiro Nagai; David L Rimoin; Bernd Nilius; Daniel H Cohn
Journal:  Nat Genet       Date:  2008-06-29       Impact factor: 38.330

7.  Autosomal recessive brachyolmia: early radiological findings.

Authors:  Atsuhiko Handa; Emma Tham; Zheng Wang; Eva Horemuzova; Giedre Grigelioniene
Journal:  Skeletal Radiol       Date:  2016-08-21       Impact factor: 2.199

Review 8.  TRPV4-associated skeletal dysplasias.

Authors:  Gen Nishimura; Ekkehart Lausch; Ravi Savarirayan; Masahiro Shiba; Juergen Spranger; Bernhard Zabel; Shiro Ikegawa; Andrea Superti-Furga; Sheila Unger
Journal:  Am J Med Genet C Semin Med Genet       Date:  2012-07-12       Impact factor: 3.908

  8 in total

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