Literature DB >> 3236370

Is geroderma osteodysplastica underdiagnosed?

A G Hunter1.   

Abstract

A girl with mild geroderma osteodysplastica is reported in order to raise the profile of this autosomal recessive condition which may be underdiagnosed. The important signs of this syndrome include a droopy, jowly face with a degree of malar hypoplasia and mandibular prognathism, lax, but non-hyperelastic skin, most marked over the extremities, and osteoporosis which may be associated with fractures and vertebral collapse.

Entities:  

Mesh:

Year:  1988        PMID: 3236370      PMCID: PMC1051618          DOI: 10.1136/jmg.25.12.854

Source DB:  PubMed          Journal:  J Med Genet        ISSN: 0022-2593            Impact factor:   6.318


  5 in total

1.  [Osteodysplasic geroderma of sex-linked heredity, a new clinical and genetic entity].

Authors:  G Boreux
Journal:  J Genet Hum       Date:  1969-05

2.  Congenital cutis laxa and osteoporosis.

Authors:  N O Sakati; W L Nyhan
Journal:  Am J Dis Child       Date:  1983-05

3.  Congenital cutis laxa with retardation of growth and development.

Authors:  M A Patton; J Tolmie; P Ruthnum; S Bamforth; M Baraitser; M Pembrey
Journal:  J Med Genet       Date:  1987-09       Impact factor: 6.318

4.  Gerodermia osteodysplastica hereditaria: report of three affected brothers and literature review.

Authors:  R Lisker; A Hernández; M Martínez-Lavin; O Mutchinick; C Armas; P Reyes; J Robles-Gil
Journal:  Am J Med Genet       Date:  1979

5.  Geroderma osteodysplastica. A report of two affected families.

Authors:  A G Hunter; J T Martsolf; C G Baker; M H Reed
Journal:  Hum Genet       Date:  1978-02-16       Impact factor: 4.132

  5 in total
  5 in total

Review 1.  Membrane trafficking in osteoblasts and osteoclasts: new avenues for understanding and treating skeletal diseases.

Authors:  Haibo Zhao
Journal:  Traffic       Date:  2012-07-24       Impact factor: 6.215

Review 2.  Clinical and Molecular Delineation of Cutis Laxa Syndromes: Paradigms for Homeostasis.

Authors:  Aude Beyens; Lore Pottie; Patrick Sips; Bert Callewaert
Journal:  Adv Exp Med Biol       Date:  2021       Impact factor: 2.622

3.  Examining tissue composition, whole-bone morphology and mechanical behavior of GorabPrx1 mice tibiae: A mouse model of premature aging.

Authors:  Haisheng Yang; Laia Albiol; Wing-Lee Chan; Dag Wulsten; Anne Seliger; Michael Thelen; Tobias Thiele; Lyudmila Spevak; Adele Boskey; Uwe Kornak; Sara Checa; Bettina M Willie
Journal:  J Biomech       Date:  2017-10-25       Impact factor: 2.712

Review 4.  The Close Relationship between the Golgi Trafficking Machinery and Protein Glycosylation.

Authors:  Anna Frappaolo; Angela Karimpour-Ghahnavieh; Stefano Sechi; Maria Grazia Giansanti
Journal:  Cells       Date:  2020-12-10       Impact factor: 6.600

5.  Gerodermia osteodysplastica is caused by mutations in SCYL1BP1, a Rab-6 interacting golgin.

Authors:  Hans Christian Hennies; Uwe Kornak; Haikuo Zhang; Johannes Egerer; Xin Zhang; Wenke Seifert; Jirko Kühnisch; Birgit Budde; Marc Nätebus; Francesco Brancati; William R Wilcox; Dietmar Müller; Paige B Kaplan; Anna Rajab; Giuseppe Zampino; Valentina Fodale; Bruno Dallapiccola; William Newman; Kay Metcalfe; Jill Clayton-Smith; May Tassabehji; Beat Steinmann; Francis A Barr; Peter Nürnberg; Peter Wieacker; Stefan Mundlos
Journal:  Nat Genet       Date:  2008-11-09       Impact factor: 38.330

  5 in total

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