Literature DB >> 9018420

Antley-Bixler syndrome: case report and review of the literature.

G Crisponi1, C Porcu, M E Piu.   

Abstract

The purpose of this report is to describe a patient with a pattern of malformations very similar to those described by Antley and Bixler. They include craniosynostosis, midface hypoplasia with proptosis, ear anomalies, choanal stenosis, long tapered fingers with a bulbous tip, elbow joint contracture due to radio-ulnar synostosis and talipes equinovarus. Necropsy revealed cardiac and renal malformations. The infant died a few days after birth, of respiratory failure. Unlike previously described cases the elbow joint contracture was due to radio-ulnar synostosis rather than radio-humeral synostosis, she did not have long bone fractures and the femora were not markedly bowed. Since the first report in 1975, at least 23 cases have been reported.

Entities:  

Mesh:

Year:  1997        PMID: 9018420

Source DB:  PubMed          Journal:  Clin Dysmorphol        ISSN: 0962-8827            Impact factor:   0.816


  9 in total

Review 1.  Malformation syndromes caused by disorders of cholesterol synthesis.

Authors:  Forbes D Porter; Gail E Herman
Journal:  J Lipid Res       Date:  2010-10-07       Impact factor: 5.922

2.  Evidence for digenic inheritance in some cases of Antley-Bixler syndrome?

Authors:  W Reardon; A Smith; J W Honour; P Hindmarsh; D Das; G Rumsby; I Nelson; S Malcolm; L Adès; D Sillence; D Kumar; C DeLozier-Blanchet; S McKee; T Kelly; W L McKeehan; M Baraitser; R M Winter
Journal:  J Med Genet       Date:  2000-01       Impact factor: 6.318

Review 3.  Defects of steroidogenesis.

Authors:  A Biason-Lauber; M Boscaro; F Mantero; G Balercia
Journal:  J Endocrinol Invest       Date:  2010-02-24       Impact factor: 4.256

4.  Diversity and function of mutations in p450 oxidoreductase in patients with Antley-Bixler syndrome and disordered steroidogenesis.

Authors:  Ningwu Huang; Amit V Pandey; Vishal Agrawal; William Reardon; Pablo D Lapunzina; David Mowat; Ethylin Wang Jabs; Guy Van Vliet; Joseph Sack; Christa E Flück; Walter L Miller
Journal:  Am J Hum Genet       Date:  2005-03-25       Impact factor: 11.025

5.  Antley-Bixler syndrome with radioulnar synostosis.

Authors:  Maja E Hurley; Martin J White; Andrew J Green; Jerry Kelleher
Journal:  Pediatr Radiol       Date:  2003-09-26

6.  A case of Antley-Bixler syndrome caused by compound heterozygous mutations of the cytochrome P450 oxidoreductase gene.

Authors:  Jung Min Ko; Chong-Kun Cheon; Gu-Hwan Kim; Han-Wook Yoo
Journal:  Eur J Pediatr       Date:  2008-10-14       Impact factor: 3.183

7.  NADPH-cytochrome P450 oxidoreductase: roles in physiology, pharmacology, and toxicology.

Authors:  David S Riddick; Xinxin Ding; C Roland Wolf; Todd D Porter; Amit V Pandey; Qing-Yu Zhang; Jun Gu; Robert D Finn; Sebastien Ronseaux; Lesley A McLaughlin; Colin J Henderson; Ling Zou; Christa E Flück
Journal:  Drug Metab Dispos       Date:  2012-10-19       Impact factor: 3.922

8.  A case of antley-bixler syndrome.

Authors:  Subhrajit Lahiri; Bhaswati Ghoshal; Debabrata Nandi
Journal:  J Clin Neonatol       Date:  2012-01

9.  B3GAT3-related disorder with craniosynostosis and bone fragility due to a unique mutation.

Authors:  Kevin Yauy; Frederic Tran Mau-Them; Marjolaine Willems; Christine Coubes; Patricia Blanchet; Christian Herlin; Ikram Taleb Arrada; Elodie Sanchez; Jean-Michel Faure; Marie-Pascale Le Gac; Olivier Prodhomme; Anne Boland; Vincent Meyer; Jean-Baptiste Rivière; Yannis Duffourd; Jean-François Deleuze; Thomas Guignard; Guillaume Captier; Mouna Barat-Houari; David Genevieve
Journal:  Genet Med       Date:  2017-08-03       Impact factor: 8.822

  9 in total

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