Literature DB >> 6182156

A recurrent pattern syndrome of craniosynostosis associated with arachnodactyly and abdominal hernias.

R J Shprintzen, R B Goldberg.   

Abstract

A new syndrome of craniosynostosis is described in two unrelated male children. Associated anomalies include severe exophthalmus; maxillary and mandibular hypoplasia; soft tissue hypertrophy of the palatal shelves; low-set ears with soft, pliable auricles; thoracic anomalies; multiple abdominal hernias; arachnodactyly; and camptodactyly. Functional disorders include infantile hypotonia, developmental delay, mental retardation, and obstructive apnea. Karyotypes were normal. An etiology for this recurrent pattern syndrome has not yet been established in the absence of a family history of similar anomalies in both cases.

Entities:  

Mesh:

Year:  1982        PMID: 6182156

Source DB:  PubMed          Journal:  J Craniofac Genet Dev Biol        ISSN: 0270-4145


  18 in total

Review 1.  Marfan's syndrome.

Authors:  Daniel P Judge; Harry C Dietz
Journal:  Lancet       Date:  2005-12-03       Impact factor: 79.321

2.  Radiographic findings in Shprintzen-Goldberg syndrome.

Authors:  G Nishimura; T Nagai
Journal:  Pediatr Radiol       Date:  1996-11

3.  Shprintzen-Goldberg syndrome presenting as umbilical hernia in an Indian child.

Authors:  Bhushan Shah; Suman Sahu; Piyush Kalakoti; Sankalp Yadav; M M Aarif Syed; Venugopal Brijmohan Bhattad; Meena Shaikh
Journal:  Australas Med J       Date:  2014-02-28

4.  The SMAD-binding domain of SKI: a hotspot for de novo mutations causing Shprintzen-Goldberg syndrome.

Authors:  Dorien Schepers; Alexander J Doyle; Gretchen Oswald; Elizabeth Sparks; Loretha Myers; Patrick J Willems; Sahar Mansour; Michael A Simpson; Helena Frysira; Anneke Maat-Kievit; Rick Van Minkelen; Jeanette M Hoogeboom; Geert R Mortier; Hannah Titheradge; Louise Brueton; Lois Starr; Zornitza Stark; Charlotte Ockeloen; Charles Marques Lourenco; Ed Blair; Emma Hobson; Jane Hurst; Isabelle Maystadt; Anne Destrée; Katta M Girisha; Michelle Miller; Harry C Dietz; Bart Loeys; Lut Van Laer
Journal:  Eur J Hum Genet       Date:  2014-04-16       Impact factor: 4.246

Review 5.  Connective tissue disorders and cardiovascular complications: the indomitable role of transforming growth factor-beta signaling.

Authors:  Jason B Wheeler; John S Ikonomidis; Jeffrey A Jones
Journal:  Adv Exp Med Biol       Date:  2014       Impact factor: 2.622

6.  Recurrence of Marfan syndrome as a result of parental germ-line mosaicism for an FBN1 mutation.

Authors:  T Rantamäki; I Kaitila; A C Syvänen; M Lukka; L Peltonen
Journal:  Am J Hum Genet       Date:  1999-04       Impact factor: 11.025

Review 7.  [Modern aortic surgery in Marfan syndrome--2011].

Authors:  K Kallenbach; S Schwill; M Karck
Journal:  Herz       Date:  2011-09       Impact factor: 1.443

8.  Connective Tissue Disorders and Cardiovascular Complications: The Indomitable Role of Transforming Growth Factor-β Signaling.

Authors:  Jason B Wheeler; John S Ikonomidis; Jeffrey A Jones
Journal:  Adv Exp Med Biol       Date:  2021       Impact factor: 2.622

9.  In-frame mutations in exon 1 of SKI cause dominant Shprintzen-Goldberg syndrome.

Authors:  Virginie Carmignac; Julien Thevenon; Lesley Adès; Bert Callewaert; Sophie Julia; Christel Thauvin-Robinet; Lucie Gueneau; Jean-Benoit Courcet; Estelle Lopez; Katherine Holman; Marjolijn Renard; Henri Plauchu; Ghislaine Plessis; Julie De Backer; Anne Child; Gavin Arno; Laurence Duplomb; Patrick Callier; Bernard Aral; Pierre Vabres; Nadège Gigot; Eloisa Arbustini; Maurizia Grasso; Peter N Robinson; Cyril Goizet; Clarisse Baumann; Maja Di Rocco; Jaime Sanchez Del Pozo; Frédéric Huet; Guillaume Jondeau; Gwenaëlle Collod-Beroud; Christophe Beroud; Jeanne Amiel; Valérie Cormier-Daire; Jean-Baptiste Rivière; Catherine Boileau; Anne De Paepe; Laurence Faivre
Journal:  Am J Hum Genet       Date:  2012-10-25       Impact factor: 11.025

10.  Early-Onset Scoliosis Associated with Shprintzen-Goldberg Syndrome Treated with Growing Rods and Required Multiple Unplanned Surgeries: A Case Report.

Authors:  Yoshiyuki Takahashi; Kota Watanabe; Mitsuru Yagi; Satoshi Suzuki; Satoshi Nori; Osahiko Tsuji; Narihito Nagoshi; Eijiro Okada; Nobuyuki Fujita; Masaya Nakamura; Morio Matsumoto
Journal:  Spine Surg Relat Res       Date:  2020-08-20
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