Literature DB >> 1956057

Velocardiofacial (Shprintzen) syndrome: an important syndrome for the dysmorphologist to recognise.

A H Lipson1, D Yuille, M Angel, P G Thompson, J G Vandervoord, E J Beckenham.   

Abstract

We report the dysmorphological, genetic, and speech therapy aspects of 38 cases of velocardiofacial syndrome presenting to a craniofacial clinic and a specialised children's hospital, to indicate a relatively low incidence of clefting, good response to pharyngoplasty, considerable variability of the syndrome, and two further familial cases. We emphasise the low index of suspicion by paediatricians and paediatric subspecialists which resulted in delayed diagnosis and delayed treatment for the hypernasal speech and velopharyngeal insufficiency for periods of four months to seven years.

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Mesh:

Year:  1991        PMID: 1956057      PMCID: PMC1015789          DOI: 10.1136/jmg.28.9.596

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


  34 in total

1.  Di George anomaly and velocardiofacial syndrome.

Authors:  C A Stevens; J C Carey; A O Shigeoka
Journal:  Pediatrics       Date:  1990-04       Impact factor: 7.124

2.  Familial syndrome of right-sided aortic arch, mental deficiency, and facial dysmorphism.

Authors:  W B Strong
Journal:  J Pediatr       Date:  1968-12       Impact factor: 4.406

3.  The velo-cardio-facial syndrome: a clinical and genetic analysis.

Authors:  R J Shprintzen; R B Goldberg; D Young; L Wolford
Journal:  Pediatrics       Date:  1981-02       Impact factor: 7.124

4.  Cardiac malformations in the velocardiofacial syndrome.

Authors:  D Young; R J Shprintzen; R B Goldberg
Journal:  Am J Cardiol       Date:  1980-10       Impact factor: 2.778

5.  A deletion in chromosome 22 can cause DiGeorge syndrome.

Authors:  A de la Chapelle; R Herva; M Koivisto; P Aula
Journal:  Hum Genet       Date:  1981       Impact factor: 4.132

6.  The association of the DiGeorge anomalad with partial monosomy of chromosome 22.

Authors:  R I Kelley; E H Zackai; B S Emanuel; M Kistenmacher; F Greenberg; H H Punnett
Journal:  J Pediatr       Date:  1982-08       Impact factor: 4.406

7.  Linear growth patterns in patients with cleft lip or palate or both.

Authors:  P A Duncan; L R Shapiro; R L Soley; S E Turet
Journal:  Am J Dis Child       Date:  1983-02

8.  The DiGeorge syndrome and the fetal alcohol syndrome.

Authors:  A J Ammann; D W Wara; M J Cowan; D J Barrett; E R Stiehm
Journal:  Am J Dis Child       Date:  1982-10

9.  A new syndrome involving cleft palate, cardiac anomalies, typical facies, and learning disabilities: velo-cardio-facial syndrome.

Authors:  R J Shprintzen; R B Goldberg; M L Lewin; E J Sidoti; M D Berkman; R V Argamaso; D Young
Journal:  Cleft Palate J       Date:  1978-01

10.  Congenital heart anomalies in patients with clefts of the lip and/or palate.

Authors:  R K Wyse; M Mars; S al-Mahdawi; I M Russell-Eggitt; K D Blake
Journal:  Cleft Palate J       Date:  1990-07
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  31 in total

1.  Velocardiofacial syndrome presenting as distal arthrogryposis.

Authors:  Koen Devriendt; Ann Swillen; Marc Gewillig; Jean-Pierre Fryns; Pierre Moens; Luc De Smet
Journal:  Eur J Pediatr       Date:  2004-06       Impact factor: 3.183

2.  [Genetic causes in mild speech acquisition delay with/without mild developmental delay: significance for ENT physicians].

Authors:  C Schwemmle; U Schwemmle; M Ptok
Journal:  HNO       Date:  2010-04       Impact factor: 1.284

3.  Lower prepulse inhibition in children with the 22q11 deletion syndrome.

Authors:  Christina Sobin; Karen Kiley-Brabeck; Maria Karayiorgou
Journal:  Am J Psychiatry       Date:  2005-06       Impact factor: 18.112

4.  Pinch1 is required for normal development of cranial and cardiac neural crest-derived structures.

Authors:  Xingqun Liang; Yunfu Sun; Jurgen Schneider; Jian-Hua Ding; Hongqiang Cheng; Maoqing Ye; Shoumo Bhattacharya; Ann Rearden; Sylvia Evans; Ju Chen
Journal:  Circ Res       Date:  2007-02-01       Impact factor: 17.367

Review 5.  Velocardiofacial syndrome.

Authors:  A C Pike; M Super
Journal:  Postgrad Med J       Date:  1997-12       Impact factor: 2.401

6.  Chromosome 22q11 deletion presenting as the Potter sequence.

Authors:  K Devriendt; P Moerman; D Van Schoubroeck; K Vandenberghe; J P Fryns
Journal:  J Med Genet       Date:  1997-05       Impact factor: 6.318

Review 7.  22q11 deletion syndrome: a genetic subtype of schizophrenia.

Authors:  A S Bassett; E W Chow
Journal:  Biol Psychiatry       Date:  1999-10-01       Impact factor: 13.382

Review 8.  Particularly interesting cysteine- and histidine-rich protein in cardiac development and remodeling.

Authors:  Xingqun Liang; Yunfu Sun; Ju Chen
Journal:  J Investig Med       Date:  2009-12       Impact factor: 2.895

9.  22q11 deletions in isolated and syndromic patients with tetralogy of Fallot.

Authors:  F Amati; A Mari; M C Digilio; R Mingarelli; B Marino; A Giannotti; G Novelli; B Dallapiccola
Journal:  Hum Genet       Date:  1995-05       Impact factor: 4.132

10.  [Small deletion--large effect].

Authors:  K Lüerssen; M Pruggmayer; M Ptok
Journal:  HNO       Date:  2004-03       Impact factor: 1.284

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