Literature DB >> 12647202

A new case of an interstitial deletion (4)(q25q27) characterised by molecular cytogenetic techniques and review of the literature.

Sandra Anna Becker1, Susanne Popp, Klaus Rager, Anna Jauch.   

Abstract

UNLABELLED: Interstitial deletions of the long arm of chromosome 4 involving the region 4q25-q27 are rare. Clinical features of patients carrying such a deletion include craniofacial and skeletal anomalies, malformations of the eye, cardiac abnormalities, congenital hypotonia, and developmental retardation. Here we report a new case of a de novo interstitial deletion 4(q25q27) in a girl with congenital malformations and findings of Rieger syndrome. The abnormal chromosome 4 was characterised by G-banding and molecular cytogenetic methods including comparative genomic hybridisation and two-colour fluorescent in situ hybridisation with band-specific probes.
CONCLUSION: this report highlights the importance of high quality banding and fluorescent in situ hybridisation analyses to screen for subtle chromosome 4q25-q27 aberrations in patients with clinical features of Rieger syndrome.

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Year:  2003        PMID: 12647202     DOI: 10.1007/s00431-003-1161-7

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  15 in total

1.  High resolution comparative genomic hybridisation analysis reveals imbalances in dyschromosomal patients with normal or apparently balanced conventional karyotypes.

Authors:  M Kirchhoff; H Rose; J Maahr; T Gerdes; M Bugge; N Tommerup; Z Tümer; J Lespinasse; P K Jensen; J Wirth; C Lundsteen
Journal:  Eur J Hum Genet       Date:  2000-09       Impact factor: 4.246

2.  Rieger syndrome and interstitial 4q26 deletion.

Authors:  J P Fryns; H Van Den Berghe
Journal:  Genet Couns       Date:  1992

3.  Multiple congenital anomalies including the Rieger eye malformation in a boy with interstitial deletion of (4) (q25-->q27) secondary to a balanced insertion in his normal father: evidence for haplotype insufficiency causing the Rieger malformation.

Authors:  A Schinzel; L Brecevic; F Dutly; A Baumer; F Binkert; R H Largo
Journal:  J Med Genet       Date:  1997-12       Impact factor: 6.318

4.  Interstitial deletion of the long arm of chromosome 4.

Authors:  A E Chudley; M R Verna; M Ray; D Riordan
Journal:  Am J Med Genet       Date:  1988-11

5.  Interstitial deletion 4q and Rieger syndrome.

Authors:  I Ligutić; L Brecević; I Petković; T Kalogjera; Z Rajić
Journal:  Clin Genet       Date:  1981-11       Impact factor: 4.438

6.  Cloning and characterization of a novel bicoid-related homeobox transcription factor gene, RIEG, involved in Rieger syndrome.

Authors:  E V Semina; R Reiter; N J Leysens; W L Alward; K W Small; N A Datson; J Siegel-Bartelt; D Bierke-Nelson; P Bitoun; B U Zabel; J C Carey; J C Murray
Journal:  Nat Genet       Date:  1996-12       Impact factor: 38.330

Review 7.  Rieger syndrome with de novo reciprocal translocation t(1;4) (q23.1;q25).

Authors:  Y Makita; M Masuno; K Imaizumi; S Yamashita; S Ohba; D Ito; Y Kuroki
Journal:  Am J Med Genet       Date:  1995-05-22

8.  Deletion of a single chromosome band 4q26 in a malformed girl: exclusion of Rieger syndrome associated gene(s) from the 4q26 segment.

Authors:  T Motegi; K Nakamura; T Terakawa; A Oohira; K Minoda; K Kishi; Y Yanagawa; H Hayakawa
Journal:  J Med Genet       Date:  1988-09       Impact factor: 6.318

Review 9.  Second case report of del(4) (q25q27) and review of the literature.

Authors:  C Raczenbek; N Krassikoff; P Cosper
Journal:  Clin Genet       Date:  1991-06       Impact factor: 4.438

10.  Detection of complete and partial chromosome gains and losses by comparative genomic in situ hybridization.

Authors:  S du Manoir; M R Speicher; S Joos; E Schröck; S Popp; H Döhner; G Kovacs; M Robert-Nicoud; P Lichter; T Cremer
Journal:  Hum Genet       Date:  1993-02       Impact factor: 4.132

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  5 in total

Review 1.  Axenfeld-Rieger syndrome and spectrum of PITX2 and FOXC1 mutations.

Authors:  Zeynep Tümer; Daniella Bach-Holm
Journal:  Eur J Hum Genet       Date:  2009-06-10       Impact factor: 4.246

2.  A Rare Recurrent 4q25 Proximal Deletion Not Involving the PITX2 Gene: A Genomic Disorder Distinct from Axenfeld-Rieger Syndrome.

Authors:  Jennifer L Heithaus; Kimberly A Twyman; Jacqueline R Batanian
Journal:  Mol Syndromol       Date:  2016-06-23

3.  Potential novel mechanism for Axenfeld-Rieger syndrome: deletion of a distant region containing regulatory elements of PITX2.

Authors:  Bethany A Volkmann; Natalya S Zinkevich; Aki Mustonen; Kala F Schilter; Dmitry V Bosenko; Linda M Reis; Ulrich Broeckel; Brian A Link; Elena V Semina
Journal:  Invest Ophthalmol Vis Sci       Date:  2011-03-18       Impact factor: 4.799

4.  Renal Dysplasia and Progressive Renal Failure in a Newborn with Interstitial Chromosome 4 Deletion 4q25-28.3: A New Phenotype?

Authors:  Cláudia Teles-Silva; Francisca Martins; Sandra Costa; Paulo Soares; Gustavo Rocha; Filipa Flor-de-Lima; Helena Pinto; Carla Ramalho; Renata Oliveira; Otília Brandão; Hercília Guimarães
Journal:  J Pediatr Genet       Date:  2020-01-22

Review 5.  Chromosome abnormalities and the genetics of congenital corneal opacification.

Authors:  A Mataftsi; L Islam; D Kelberman; J C Sowden; K K Nischal
Journal:  Mol Vis       Date:  2011-06-17       Impact factor: 2.367

  5 in total

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