Clinical and molecular characterization of patients affected by Beckwith-Wiedemann spectrum conceived through assisted reproduction techniques.


Journal

Clinical genetics
ISSN: 1399-0004
Titre abrégé: Clin Genet
Pays: Denmark
ID NLM: 0253664

Informations de publication

Date de publication:
10 2022
Historique:
revised: 13 07 2022
received: 21 06 2022
accepted: 14 07 2022
pubmed: 18 7 2022
medline: 9 9 2022
entrez: 17 7 2022
Statut: ppublish

Résumé

The prevalence of Beckwith-Wiedemann spectrum (BWSp) is tenfold increased in children conceived through assisted reproductive techniques (ART). More than 90% of ART-BWSp patients reported so far display imprinting center 2 loss-of-methylations (IC2-LoM), versus 50% of naturally conceived BWSp patients. We describe a cohort of 74 ART-BWSp patients comparing their features with a cohort of naturally conceived BWSp patients, with the ART-BWSp patients previously described in literature, and with the general population of children born from ART. We found that the distribution of UPD(11)pat was not significantly different in ART and naturally conceived patients. We observed 68.9% of IC2-LoM and 16.2% of mosaic UPD(11)pat in our ART cohort, that strongly differ from the figure reported in other cohorts so far. Since UPD(11)pat likely results from post-fertilization recombination events, our findings allows to hypothesize that more complex molecular mechanisms, besides methylation disturbances, may underlie BWSp increased risk in ART pregnancies. Moreover, comparing the clinical features of ART and non-ART BWSp patients, we found that ART-BWSp patients might have a milder phenotype. Finally, our data show a progressive increase in the prevalence of BWSp over time, paralleling that of ART usage in the last decades.

Identifiants

pubmed: 35842840
doi: 10.1111/cge.14193
pmc: PMC9545072
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

314-323

Informations de copyright

© 2022 The Authors. Clinical Genetics published by John Wiley & Sons Ltd.

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Auteurs

Diana Carli (D)

Department of Public Health and Pediatric Sciences, University of Torino, Torino, Italy.

Matteo Operti (M)

Department of Public Health and Pediatric Sciences, University of Torino, Torino, Italy.

Silvia Russo (S)

Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy.

Guido Cocchi (G)

Neonatology Unit, St. Orsola-Malpighi Polyclinic, Department of Medical and Surgical Sciences (DIMEC), University of Bologna, Bologna, Italy.

Donatella Milani (D)

Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.

Chiara Leoni (C)

Center for Rare Diseases and Birth Defects, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Elisabetta Prada (E)

Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.

Daniela Melis (D)

Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Fisciano, Italy.

Mariateresa Falco (M)

Pediatric Unit, San Giovanni di Dio e Ruggi D'Aragona University Hospital, Salerno, Italy.

Jennifer Spina (J)

Department of Public Health and Pediatric Sciences, University of Torino, Torino, Italy.

Vera Uliana (V)

Medical Genetics Unit, University Hospital of Parma, Parma, Italy.

Osimani Sara (O)

Department of Pediatrics, Scientific Institute San Raffaele, Milan, Italy.

Fabio Sirchia (F)

Unit of Medical Genetics, Department of Diagnostic Medicine, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Luigi Tarani (L)

Department of Pediatrics, Medical Faculty, "Sapienza" University of Rome, Rome, Italy.

Marina Macchiaiolo (M)

Rare Diseases and Medical Genetics, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Flavia Cerrato (F)

Department of Environmental Biological and Pharmaceutical Sciences and Technologies (DiSTABiF), Università degli Studi della Campania "Luigi Vanvitelli", Caserta, Italy.

Angela Sparago (A)

Department of Environmental Biological and Pharmaceutical Sciences and Technologies (DiSTABiF), Università degli Studi della Campania "Luigi Vanvitelli", Caserta, Italy.

Laura Pignata (L)

Department of Environmental Biological and Pharmaceutical Sciences and Technologies (DiSTABiF), Università degli Studi della Campania "Luigi Vanvitelli", Caserta, Italy.

Pierpaola Tannorella (P)

Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy.

Simona Cardaropoli (S)

Department of Public Health and Pediatric Sciences, University of Torino, Torino, Italy.

Andrea Bartuli (A)

Rare Diseases and Medical Genetics, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Andrea Riccio (A)

Department of Environmental Biological and Pharmaceutical Sciences and Technologies (DiSTABiF), Università degli Studi della Campania "Luigi Vanvitelli", Caserta, Italy.
Institute of Genetics and Biophysics A. Buzzati-Traverso, Consiglio Nazionale delle Ricerche, Naples, Italy.

Giovanni Battista Ferrero (GB)

Department of Clinical and Biological Sciences, University of Torino, Torino, Italy.

Alessandro Mussa (A)

Department of Public Health and Pediatric Sciences, University of Torino, Torino, Italy.
Pediatric Clinical Genetics Unit, Regina Margherita Childrens Hospital, Città della Salute e della Scienza di Torino, Torino, Italy.

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