Literature DB >> 8882401

Craniosynostosis, Philadelphia type: a new autosomal dominant syndrome with sagittal craniosynostosis and syndactyly of the fingers and toes.

N H Robin1, B Segel, G Carpenter, M Muenke.   

Abstract

The acrocephalosyndactyly syndromes (ACS) are a group of clinically similar disorders that share the manifestations of craniosynostosis and a variety of hand and foot anomalies. Here we report on a 5-generation kindred segregating sagittal craniosynostosis and syndactyly of the fingers and the toes in an autosomal dominant manner. The anomalies seen in this kindred comprise a syndrome distinct from other craniosynostosis syndromes. For this novel syndrome, we propose the name craniosynostosis, Philadelphia type.

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Year:  1996        PMID: 8882401     DOI: 10.1002/(SICI)1096-8628(19960315)62:2<184::AID-AJMG13>3.0.CO;2-K

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  4 in total

1.  Copy-number variations involving the IHH locus are associated with syndactyly and craniosynostosis.

Authors:  Eva Klopocki; Silke Lohan; Francesco Brancati; Randi Koll; Anja Brehm; Petra Seemann; Katarina Dathe; Sigmar Stricker; Jochen Hecht; Kristin Bosse; Regina C Betz; Francesco Giuseppe Garaci; Bruno Dallapiccola; Mahim Jain; Maximilian Muenke; Vivian C W Ng; Wilson Chan; Danny Chan; Stefan Mundlos
Journal:  Am J Hum Genet       Date:  2010-12-17       Impact factor: 11.025

2.  The epidemiology, genetics and future management of syndactyly.

Authors:  D Jordan; S Hindocha; M Dhital; M Saleh; W Khan
Journal:  Open Orthop J       Date:  2012-03-23

3.  Locus homogeneity between syndactyly type 1A and craniosynostosis Philadelphia type?

Authors:  Mahim Jain; Deeann Wallis; Nathaniel H Robin; Fabienne Wavrant De Vrieze; John A Hardy; Mohsen Ghadami; Kristin Bosse; Regina C Betz; Markus M Nöthen; Mauricio Arcos-Burgos; Maximilian Muenke
Journal:  Am J Med Genet A       Date:  2008-09-01       Impact factor: 2.802

Review 4.  Ciliary Signalling and Mechanotransduction in the Pathophysiology of Craniosynostosis.

Authors:  Federica Tiberio; Ornella Parolini; Wanda Lattanzi
Journal:  Genes (Basel)       Date:  2021-07-14       Impact factor: 4.096

  4 in total

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