Literature DB >> 8599362

Short rib-dysplasia group (with/without polydactyly): report of a patient suggesting the existence of a continuous spectrum.

P Franceschini1, A Guala, M P Vardeu, F Signorile, D Franceschini, M P Bolgiani.   

Abstract

We report on a patient with manifestations typical of Mohr syndrome and of the short rib (polydactyly) syndromes (SR(P)S) Majewski, Verma-Naumoff, Beemer, and Jeune. It seems possible that the different types of SR(P) syndromes, rather than being distinct conditions, are part of a large disease spectrum. The frequent overlap between orofaciodigital syndromes and SR(P) syndromes may be interpreted as the outcome of deletions of different size within the same chromosome region.

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Year:  1995        PMID: 8599362     DOI: 10.1002/ajmg.1320590316

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


  4 in total

1.  Ciliary abnormalities due to defects in the retrograde transport protein DYNC2H1 in short-rib polydactyly syndrome.

Authors:  Amy E Merrill; Barry Merriman; Claire Farrington-Rock; Natalia Camacho; Eiman T Sebald; Vincent A Funari; Matthew J Schibler; Marc H Firestein; Zachary A Cohn; Mary Ann Priore; Alicia K Thompson; David L Rimoin; Stanley F Nelson; Daniel H Cohn; Deborah Krakow
Journal:  Am J Hum Genet       Date:  2009-04       Impact factor: 11.025

2.  Compound heterozygous NEK1 variants in two siblings with oral-facial-digital syndrome type II (Mohr syndrome).

Authors:  Glen R Monroe; Isabelle Fpm Kappen; Marijn F Stokman; Paulien A Terhal; Marie-José H van den Boogaard; Sanne Mc Savelberg; Lars T van der Veken; Robert Jj van Es; Susanne M Lens; Rutger C Hengeveld; Marijn A Creton; Nard G Janssen; Aebele B Mink van der Molen; Michelle B Ebbeling; Rachel H Giles; Nine V Knoers; Gijs van Haaften
Journal:  Eur J Hum Genet       Date:  2016-08-17       Impact factor: 4.246

3.  The many faces of oral-facial-digital syndrome.

Authors:  E Sukarova-Angelovska; N Angelkova; S Palcevska-Kocevska; M Kocova
Journal:  Balkan J Med Genet       Date:  2012-06       Impact factor: 0.519

4.  Antenatal Diagnosis of Jeune Syndrome (Asphyxiating Thoracic Dysplasia) with Micromelia and Facial Dysmorphism on Second-Trimester Ultrasound.

Authors:  Kewal A Mistry; Pokhraj P Suthar; Siddharth R Bhesania; Ankitkumar Patel
Journal:  Pol J Radiol       Date:  2015-06-07
  4 in total

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