Experience of a 2-year spinal muscular atrophy NBS pilot study in Italy: towards specific guidelines and standard operating procedures for the molecular diagnosis.


Journal

Journal of medical genetics
ISSN: 1468-6244
Titre abrégé: J Med Genet
Pays: England
ID NLM: 2985087R

Informations de publication

Date de publication:
07 2023
Historique:
received: 12 08 2022
accepted: 06 11 2022
medline: 23 6 2023
pubmed: 23 11 2022
entrez: 22 11 2022
Statut: ppublish

Résumé

Spinal muscular atrophy (SMA) is due to the homozygous absence of The screening test was performed by an in-house-developed qPCR assay, amplifying We found 15 patients/90885 newborns (incidence 1:6059) having the following The molecular diagnosis of SMA needs to adapt to the new era of the disease with specific guidelines and standard operating procedures. In detail, SMA diagnosis should be felt as a true medical urgency due to therapeutic implications;

Sections du résumé

BACKGROUND
Spinal muscular atrophy (SMA) is due to the homozygous absence of
METHODS
The screening test was performed by an in-house-developed qPCR assay, amplifying
RESULTS
We found 15 patients/90885 newborns (incidence 1:6059) having the following
CONCLUSION
The molecular diagnosis of SMA needs to adapt to the new era of the disease with specific guidelines and standard operating procedures. In detail, SMA diagnosis should be felt as a true medical urgency due to therapeutic implications;

Identifiants

pubmed: 36414255
pii: jmg-2022-108873
doi: 10.1136/jmg-2022-108873
doi:

Types de publication

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

Langues

eng

Sous-ensembles de citation

IM

Pagination

697-705

Investigateurs

Maria Accardo (M)
Beatrice Gambi (B)
Simonetta Giampaoli (S)
Ubah Farah Ahmed (UF)
Carlo Giannini (C)
Concetta Alecci (C)
Valerio Giardini (V)
Antonella Amendolea (A)
Monica Guarguagli (M)
Monica Bartolucci (M)
Cristina Haass (C)
Ivana Belisario (I)
Isabella Innocenti (I)
Paola Belluomini (P)
Maria Cristina Innocenti Berti (MC)
Claudia Bernardini (C)
Claudia Maggi (C)
Roberto Bernardini (R)
Letizia Magi (L)
Luisa Bertò (L)
Roccuccia Malorgio (R)
Rita Bini (R)
Giovanna Maragliano (G)
Leonardo Bolgia (L)
Vita Maraglino (V)
Mario Boscioni (M)
Anna Marotta (A)
Giorgio Bracaglia (G)
Cristina Martini (C)
Elisa Brachini (E)
Palmina Martufi (P)
Elisa Bruschi (E)
Stefania Mastropasqua (S)
Monica Campa (M)
Maria Rosaria Matera (MR)
Monica Caprilli (M)
Graziano Memmini (G)
Simona Carcione (S)
Angela Mendicino (A)
Angelo Cardiello (A)
Sabrina Mercurio (S)
Francesco Cartolano (F)
Alessandra Meucci (A)
Alessandra Casati (A)
Osvaldo Milita (O)
Sandra Ceccarelli (S)
Zeudi Morano (Z)
Marzia Chiellini (M)
Eleonora Nardi (E)
Rossella Ciccotti (R)
Sandra Novelli (S)
Elena Cioli (E)
Luigi Padovano (L)
Flavio Civitelli (F)
Maria Gabriella Palermo (MG)
Maria Giovanna Colella (MG)
Serena Pascucci (S)
Luigina Coppotelli (L)
Caterini Patrizia (C)
Francesco Crescenzi (F)
Assunta Percoco (A)
Stefania Petrolati (S)
Roberto Danieli (R)
Rosanna Pitaro (R)
Tamara De Angelis (T)
Marilena Raponi (M)
Mauro De Martinis (M)
Francesco Riccobene (F)
Elena degl'Innocenti (E)
Giovanni Rosa (G)
Laura Del Sette (LD)
Amanda Roversi (A)
Ambrogio Di Paolo (AD)
Sabrina Sadocco (S)
Luigi Di Ruzza (LD)
Emanuela Santarelli (E)
Rosalia Di Silvio (RD)
Luca Tafi (L)
Dituri Francesco (D)
Barbara Tomasini (B)
Gwenna Egho (G)
Martha Traupe (M)
Daniela Emili (D)
Angelina Vaccaro (A)
Luca Filippo (L)
Pierluigi Vasarri (P)
Claudia Foglia (C)
Valentina Ventura (V)
Francesco Gabriellini (F)
Stefano Vitale (S)
Luigi Gagliardi (L)
Anna Maria Zingoni (AM)
Annamaria Gallo (A)

Informations de copyright

© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

Auteurs

Emanuela Abiusi (E)

Section of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.

Alessandro Vaisfeld (A)

Section of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.

Stefania Fiori (S)

Section of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.

Agnese Novelli (A)

Section of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.

Serena Spartano (S)

Section of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.

Maria Vittoria Faggiano (MV)

Section of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.

Teresa Giovanniello (T)

Department of Experimental Medicine, Newborn Screening Center-Clinical Pathology Unit, Sapienza University of Rome, University Hospital Policlinico Umberto I, Roma, Italy.

Antonio Angeloni (A)

Department of Experimental Medicine, Newborn Screening Center-Clinical Pathology Unit, Sapienza University of Rome, University Hospital Policlinico Umberto I, Roma, Italy.

Giovanni Vento (G)

Section of Pediatrics, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.
Neonatology Unit, Fondazione Policlinico Universitario IRCCS "A. Gemelli", Roma, Italy.

Roberta Santoloci (R)

Obstetrics and Gynecology operating Unit, Fondazione Policlinico Universitario IRCCS "A. Gemelli, Roma, Italy.

Francesca Gigli (F)

Neonatology Unit, Fondazione Policlinico Universitario IRCCS "A. Gemelli", Roma, Italy.

Adele D'Amico (A)

Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital IRCCS, Roma, Italy.

Simonetta Costa (S)

Section of Pediatrics, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.

Alessia Porzi (A)

Section of Pediatrics, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.

Mara Panella (M)

Obstetrics and Gynecology operating Unit, Fondazione Policlinico Universitario IRCCS "A. Gemelli, Roma, Italy.

Chiara Ticci (C)

Unit of hereditary metabolic and muscular disorders, Meyer Children's University Hospital, Firenze, Italy.

Marta Daniotti (M)

Unit of hereditary metabolic and muscular disorders, Meyer Children's University Hospital, Firenze, Italy.

Michele Sacchini (M)

Unit of hereditary metabolic and muscular disorders, Meyer Children's University Hospital, Firenze, Italy.

Ilaria Boschi (I)

Forensic Medicine operating Unit, Fondazione Policlinico Universitario IRCCS "A. Gemelli", Roma, Italy.

Carlo Dani (C)

Division of Neonatology, Careggi University Hospital of Florence, Florence, Italy.
Department of Neurosciences, University of Florence, Florence, Italy.

Rino Agostiniani (R)

Department of Pediatrics and Neonatology, ASL Toscana Centro, Florence, Italy.

Enrico Bertini (E)

Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital IRCCS, Roma, Italy.

Antonio Lanzone (A)

Obstetrics and Gynecology operating Unit, Fondazione Policlinico Universitario IRCCS "A. Gemelli, Roma, Italy.
Section of Obstetrics and Gynecology, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.

Giancarlo Lamarca (G)

Newborn Screening, Clinical Chemistry and Pharmacology Laboratory, Meyer Children's University Hospital, Firenze, Italy.

Maurizio Genuardi (M)

Section of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.
Medical Genetics operating Unit, Fondazione Policlinico Universitario IRCCS "A. Gemelli", Roma, Italy.

Marika Pane (M)

Section of Child Psychiatry, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.
Child Psychiatry operating Unit, Fondazione Policlinico Universitario IRCCS "A. Gemelli", Roma, Italy.

Maria Alice Donati (MA)

Unit of hereditary metabolic and muscular disorders, Meyer Children's University Hospital, Firenze, Italy.

Eugenio Mercuri (E)

Section of Child Psychiatry, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy.
Child Psychiatry operating Unit, Fondazione Policlinico Universitario IRCCS "A. Gemelli", Roma, Italy.

Francesco Danilo Tiziano (FD)

Section of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Roma, Italy francescodanilo.tiziano@unicatt.it.
Medical Genetics operating Unit, Fondazione Policlinico Universitario IRCCS "A. Gemelli", Roma, Italy.

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