Literature DB >> 641945

Recessively inherited costovertebral segmentation defect with mesomelia and peculiar facies (Covesdem syndrome): A new genetic entity?

R S Wadia, D B Shirole, M S Dikshit.   

Abstract

Two sibs of a consanguineous mating are described. Both have a gross costovertebral segmentation defect affecting nearly all the thoracic vertebrae, and mesomelia of the limbs, with the upper limbs being obviously more affected than the lower. The facial appearances of the two are identical, with hypertelorism, depressed nasal bridge, large bony upper lip, constantly open mouth, and peg-like teeth. We believe this combination has not been described before and represents the effect of a 'new' recessive gene. We would like to name this combination Covesdem syndrome (costovertebral segmentation defect with mesomelia).

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Mesh:

Year:  1978        PMID: 641945      PMCID: PMC1013660          DOI: 10.1136/jmg.15.2.123

Source DB:  PubMed          Journal:  J Med Genet        ISSN: 0022-2593            Impact factor:   6.318


  10 in total

1.  DWARFISM IN THE AMISH I. THE ELLIS-VAN CREVELD SYNDROME.

Authors:  V A MCKUSICK; J A EGELAND; R ELDRIDGE; D E KRUSEN
Journal:  Bull Johns Hopkins Hosp       Date:  1964-10

2.  A NEW TYPE OF DWARFISM WITH VARIOUS BONE APLASIAS AND HYPOPLASIAS OF THE EXTREMITIES.

Authors:  A QUELCE-SALGADO
Journal:  Acta Genet Stat Med       Date:  1964

3.  [Mandibulo-facial abnormalities and their differentiation from an oculo-vertebral syndrome].

Authors:  H WEYERS; C J THIER
Journal:  J Genet Hum       Date:  1958-10

4.  Multiple congenital dislocations associated with characteristic facial abnormality.

Authors:  L J LARSEN; E R SCHOTTSTAEDT; F C BOST
Journal:  J Pediatr       Date:  1950-10       Impact factor: 4.406

5.  Basal cell nevus syndrome. Combined clinical staff conference at the National Institutes of Health.

Authors:  N I Berlin; E J Van Scott; W E Clendenning; H O Archard; J B Block; C J Witkop; H A Haynes
Journal:  Ann Intern Med       Date:  1966-02       Impact factor: 25.391

6.  Spondylocostal dysplasia. A dominantly inherited form of short-trunked dwarfism.

Authors:  D L Rimoin; B D Fletcher; V A McKusick
Journal:  Am J Med       Date:  1968-12       Impact factor: 4.965

7.  A syndrome of bizarre vertebral anomalies.

Authors:  N W Lavy; C G Palmer; A D Merritt
Journal:  J Pediatr       Date:  1966-12       Impact factor: 4.406

8.  Dyschondrosteosis. The most common cause of Madelung's deformity.

Authors:  R C Herdman; L O Langer; R A Good
Journal:  J Pediatr       Date:  1966-03       Impact factor: 4.406

9.  Mesomelic dwarfism of the hypoplastic ulna, fibula, mandible type.

Authors:  L O Langer
Journal:  Radiology       Date:  1967-10       Impact factor: 11.105

10.  The Bloch-Sulzberger syndrome (incontinentia pigmenti).

Authors:  H HABER
Journal:  Br J Dermatol       Date:  1952-04       Impact factor: 9.302

  10 in total
  17 in total

1.  Robinow syndrome in two siblings from consanguineous parents.

Authors:  D F Schorderet; S Dahoun; I Defrance; D Nusslé; M A Morris
Journal:  Eur J Pediatr       Date:  1992-08       Impact factor: 3.183

2.  DVL1 frameshift mutations clustering in the penultimate exon cause autosomal-dominant Robinow syndrome.

Authors:  Janson White; Juliana F Mazzeu; Alexander Hoischen; Shalini N Jhangiani; Tomasz Gambin; Michele Calijorne Alcino; Samantha Penney; Jorge M Saraiva; Hanne Hove; Flemming Skovby; Hülya Kayserili; Elicia Estrella; Anneke T Vulto-van Silfhout; Marloes Steehouwer; Donna M Muzny; V Reid Sutton; Richard A Gibbs; James R Lupski; Han G Brunner; Bregje W M van Bon; Claudia M B Carvalho
Journal:  Am J Hum Genet       Date:  2015-03-26       Impact factor: 11.025

3.  Robinow syndrome with parental consanguinity.

Authors:  D Glaser; J Herbst; K Roggenkamp; W Tünte; W Lenz
Journal:  Eur J Pediatr       Date:  1989-06       Impact factor: 3.183

4.  Robinow syndrome without mesomelic 'brachymelia': a report of five cases.

Authors:  M D Bain; R M Winter; J Burn
Journal:  J Med Genet       Date:  1986-08       Impact factor: 6.318

5.  Metacarpophalangeal pattern profile analysis in Robinow syndrome.

Authors:  M G Butler; D D Gale; F J Meaney; W B Wadlington; M Robinow
Journal:  Am J Med Genet       Date:  1987-05

6.  Covesdem syndrome.

Authors:  R M Winter
Journal:  J Med Genet       Date:  1980-02       Impact factor: 6.318

7.  A gene for autosomal recessive spondylocostal dysostosis maps to 19q13.1-q13.3.

Authors:  P D Turnpenny; M P Bulman; T M Frayling; T K Abu-Nasra; C Garrett; A T Hattersley; S Ellard
Journal:  Am J Hum Genet       Date:  1999-07       Impact factor: 11.025

Review 8.  Robinow syndrome: report of two patients and review of literature.

Authors:  M G Butler; W B Wadlington
Journal:  Clin Genet       Date:  1987-02       Impact factor: 4.438

9.  Novel mutations in DLL3, a somitogenesis gene encoding a ligand for the Notch signalling pathway, cause a consistent pattern of abnormal vertebral segmentation in spondylocostal dysostosis.

Authors:  P D Turnpenny; N Whittock; J Duncan; S Dunwoodie; K Kusumi; S Ellard
Journal:  J Med Genet       Date:  2003-05       Impact factor: 6.318

10.  Novel Robinow syndrome causing mutations in the proximal region of the frizzled-like domain of ROR2 are retained in the endoplasmic reticulum.

Authors:  Bassam R Ali; Steve Jeffery; Neha Patel; Lorna E Tinworth; Nagwa Meguid; Michael A Patton; Ali R Afzal
Journal:  Hum Genet       Date:  2007-07-31       Impact factor: 4.132

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