Literature DB >> 18388777

Familial multiple ventricular extrasystoles, short stature, craniofacial abnormalities and digital hypoplasia: a further case of Stoll syndrome?

Catherine L Mercer1, Barry Keeton, Nicolas R Dennis.   

Abstract

We report two brothers, their mother and a maternal cousin who had a distinctive facial phenotype, mild brachydactyly and prominence of the interphalangeal joints. One brother and the mother also had multiple ventricular extrasystoles. Six other relatives in four generations were probably affected on the basis of history and family photographs. We also report a further individual from a different family with a similar facial phenotype, Pierre-Robin sequence, tapering fingers and multiple ventricular extrasystoles. These families have some similarities to those reported by Stoll et al. in a single family, showing dominant inheritance. Our patients would seem to have the same or a related condition.

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Year:  2008        PMID: 18388777     DOI: 10.1097/MCD.0b013e3282efefc9

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


  2 in total

1.  Mutations specific to the Rac-GEF domain of TRIO cause intellectual disability and microcephaly.

Authors:  Reuben J Pengelly; Stephanie Greville-Heygate; Susanne Schmidt; Eleanor G Seaby; M Reza Jabalameli; Sarju G Mehta; Michael J Parker; David Goudie; Christine Fagotto-Kaufmann; Catherine Mercer; Anne Debant; Sarah Ennis; Diana Baralle
Journal:  J Med Genet       Date:  2016-07-14       Impact factor: 6.318

Review 2.  Pathophysiological Mechanisms in Neurodevelopmental Disorders Caused by Rac GTPases Dysregulation: What's behind Neuro-RACopathies.

Authors:  Marcello Scala; Masashi Nishikawa; Koh-Ichi Nagata; Pasquale Striano
Journal:  Cells       Date:  2021-12-02       Impact factor: 6.600

  2 in total

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