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Bilateral preaxial polydactyly in a WAGR syndrome patient

Bilateral preaxial polydactyly in a WAGR syndrome patient
Bilateral preaxial polydactyly in a WAGR syndrome patient
We report on a 30-month-old baby girl with typical clinical features of WAGR syndrome. In addition, the patient showed bilateral preaxial polydactyly of the feet. Cytogenetic and fluorescent in situ hybridization (FISH) analyses identified a deletion, del(11)(p13p14.1), extending from 6.1 to 21.7 Mb in size. Although the simultaneous appearance of WAGR and polydactyly has been already described, to our knowledge this is the first case in which the characterization at the cytogenetic molecular level of a patient with these phenotypes is reported. These observations indicate that preaxial polydactyly may be another feature of the WAGR syndrome and suggest the existence of a related gene in the WAGR critical region or in its proximity
polymerase chain reaction, fluorescence, report, phenotype, wt1 proteins, observation, preschool, syndrome, female, research support, restriction fragment length, child, pair 11, in situ hybridization, karyotyping, hallux, dna, humans, abnormalities, polymorphism, chromosome banding, chromosome deletion, polydactyly, proteins, non-us government, human, genetics, chromosomes, microsatellite repeats, pathology, wagr syndrome, multiple, protein
1552-4825
426-429
Manoukian, Siranoush
87f7b60d-b65f-4655-b1d8-3b7c5dcf37d2
Crolla, John A.
c5f23751-8de9-4a55-9cc5-ca2fb635769c
Mammoliti, Palma M.A.
4c6cf6fd-a043-4ac2-bc6a-6c478b60bea8
Testi, Maria Adele
3b0d7db0-8321-40e4-ad41-5bb7cfb96acc
Zanini, Rinaldo
1d1515d3-4784-4d91-8836-63ef8ec8ed20
Carpanelli, Maria Luisa
8fd342df-61b1-4875-a164-f74569842ae5
Piozzi, Elena
d360ad97-0aae-45f2-9653-bdc178d90658
Sozzi, Gabriella
84afed20-0afe-439a-8e66-acd6d310d93c
De Vecchi, Giovanna
6a0384c8-5ef1-4d2c-85e9-ed027010aa1c
Terenziani, Monica
ecdc4139-37f6-4983-b4b0-27c3dfac31d8
Spreafico, Filippo
b2202034-2a47-4fbe-b53b-788edaab71da
Collini, Paola
0f7cace0-a895-42a7-85c2-8dfa03b1cf7a
Radice, Paolo
d5bff2d9-ed03-4a8e-a9ca-f896a1666e2c
Perotti, Daniela
c7db6330-361b-453a-a69c-9db3ee0ab136
Manoukian, Siranoush
87f7b60d-b65f-4655-b1d8-3b7c5dcf37d2
Crolla, John A.
c5f23751-8de9-4a55-9cc5-ca2fb635769c
Mammoliti, Palma M.A.
4c6cf6fd-a043-4ac2-bc6a-6c478b60bea8
Testi, Maria Adele
3b0d7db0-8321-40e4-ad41-5bb7cfb96acc
Zanini, Rinaldo
1d1515d3-4784-4d91-8836-63ef8ec8ed20
Carpanelli, Maria Luisa
8fd342df-61b1-4875-a164-f74569842ae5
Piozzi, Elena
d360ad97-0aae-45f2-9653-bdc178d90658
Sozzi, Gabriella
84afed20-0afe-439a-8e66-acd6d310d93c
De Vecchi, Giovanna
6a0384c8-5ef1-4d2c-85e9-ed027010aa1c
Terenziani, Monica
ecdc4139-37f6-4983-b4b0-27c3dfac31d8
Spreafico, Filippo
b2202034-2a47-4fbe-b53b-788edaab71da
Collini, Paola
0f7cace0-a895-42a7-85c2-8dfa03b1cf7a
Radice, Paolo
d5bff2d9-ed03-4a8e-a9ca-f896a1666e2c
Perotti, Daniela
c7db6330-361b-453a-a69c-9db3ee0ab136

Manoukian, Siranoush, Crolla, John A., Mammoliti, Palma M.A., Testi, Maria Adele, Zanini, Rinaldo, Carpanelli, Maria Luisa, Piozzi, Elena, Sozzi, Gabriella, De Vecchi, Giovanna, Terenziani, Monica, Spreafico, Filippo, Collini, Paola, Radice, Paolo and Perotti, Daniela (2005) Bilateral preaxial polydactyly in a WAGR syndrome patient. American Journal of Medical Genetics part A, 134A (4), 426-429. (doi:10.1002/ajmg.a.30647).

Record type: Article

Abstract

We report on a 30-month-old baby girl with typical clinical features of WAGR syndrome. In addition, the patient showed bilateral preaxial polydactyly of the feet. Cytogenetic and fluorescent in situ hybridization (FISH) analyses identified a deletion, del(11)(p13p14.1), extending from 6.1 to 21.7 Mb in size. Although the simultaneous appearance of WAGR and polydactyly has been already described, to our knowledge this is the first case in which the characterization at the cytogenetic molecular level of a patient with these phenotypes is reported. These observations indicate that preaxial polydactyly may be another feature of the WAGR syndrome and suggest the existence of a related gene in the WAGR critical region or in its proximity

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More information

Published date: 1 May 2005
Keywords: polymerase chain reaction, fluorescence, report, phenotype, wt1 proteins, observation, preschool, syndrome, female, research support, restriction fragment length, child, pair 11, in situ hybridization, karyotyping, hallux, dna, humans, abnormalities, polymorphism, chromosome banding, chromosome deletion, polydactyly, proteins, non-us government, human, genetics, chromosomes, microsatellite repeats, pathology, wagr syndrome, multiple, protein

Identifiers

Local EPrints ID: 60023
URI: http://eprints.soton.ac.uk/id/eprint/60023
ISSN: 1552-4825
PURE UUID: 483ec524-ea24-4108-a89b-02ce1fb68734

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Date deposited: 03 Sep 2008
Last modified: 15 Mar 2024 11:18

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Contributors

Author: Siranoush Manoukian
Author: John A. Crolla
Author: Palma M.A. Mammoliti
Author: Maria Adele Testi
Author: Rinaldo Zanini
Author: Maria Luisa Carpanelli
Author: Elena Piozzi
Author: Gabriella Sozzi
Author: Giovanna De Vecchi
Author: Monica Terenziani
Author: Filippo Spreafico
Author: Paola Collini
Author: Paolo Radice
Author: Daniela Perotti

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