Population-Based Prevalence of Myotonic Dystrophy Type 1 Using Genetic Analysis of Statewide Blood Screening Program.


Journal

Neurology
ISSN: 1526-632X
Titre abrégé: Neurology
Pays: United States
ID NLM: 0401060

Informations de publication

Date de publication:
16 02 2021
Historique:
received: 31 07 2020
accepted: 12 10 2020
pubmed: 22 1 2021
medline: 3 3 2021
entrez: 21 1 2021
Statut: ppublish

Résumé

To determine whether the genetic prevalence of the CTG expansion in the This study used a cross-sectional cohort of deidentified dried blood spots from the newborn screening program in the state of New York, taken from consecutive births from 2013 to 2014. Blood spots were screened for the CTG repeat expansion in the Of 50,382 consecutive births, there were 24 with a CTG repeat expansion ≥50, consistent with a diagnosis of DM1. This represents a significantly higher DM1 prevalence of 4.76 per 10,000 births (95% confidence interval 2.86-6.67) or 1 in every 2,100 births. There were an additional 96 samples (19.1 per 10,000 or 1 in 525 births) with a CTG expansion in the The prevalence of individuals with CTG repeat expansions in

Identifiants

pubmed: 33472919
pii: WNL.0000000000011425
doi: 10.1212/WNL.0000000000011425
pmc: PMC8055332
doi:

Substances chimiques

DMPK protein, human 0
Myotonin-Protein Kinase EC 2.7.11.1

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

e1045-e1053

Subventions

Organisme : NINDS NIH HHS
ID : K08 NS097631
Pays : United States
Organisme : NINDS NIH HHS
ID : K23 NS091511
Pays : United States
Organisme : NINDS NIH HHS
ID : R21 NS100040
Pays : United States

Informations de copyright

© 2021 American Academy of Neurology.

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Auteurs

Nicholas E Johnson (NE)

From the Department of Neurology (N.E.J.), Virginia Commonwealth University, Richmond; and Departments of Pediatrics (R.J.B., K.M., T.N., C.I., M.L.F.) and Nicholas.johnson@vcuhealth.org.
Human Genetics (D.D., B.D., R.B.W.), University of Utah, Salt Lake City. Nicholas.johnson@vcuhealth.org.

Russell J Butterfield (RJ)

From the Department of Neurology (N.E.J.), Virginia Commonwealth University, Richmond; and Departments of Pediatrics (R.J.B., K.M., T.N., C.I., M.L.F.) and.
Human Genetics (D.D., B.D., R.B.W.), University of Utah, Salt Lake City.

Katie Mayne (K)

From the Department of Neurology (N.E.J.), Virginia Commonwealth University, Richmond; and Departments of Pediatrics (R.J.B., K.M., T.N., C.I., M.L.F.) and.
Human Genetics (D.D., B.D., R.B.W.), University of Utah, Salt Lake City.

Tara Newcomb (T)

From the Department of Neurology (N.E.J.), Virginia Commonwealth University, Richmond; and Departments of Pediatrics (R.J.B., K.M., T.N., C.I., M.L.F.) and.
Human Genetics (D.D., B.D., R.B.W.), University of Utah, Salt Lake City.

Carina Imburgia (C)

From the Department of Neurology (N.E.J.), Virginia Commonwealth University, Richmond; and Departments of Pediatrics (R.J.B., K.M., T.N., C.I., M.L.F.) and.
Human Genetics (D.D., B.D., R.B.W.), University of Utah, Salt Lake City.

Diane Dunn (D)

From the Department of Neurology (N.E.J.), Virginia Commonwealth University, Richmond; and Departments of Pediatrics (R.J.B., K.M., T.N., C.I., M.L.F.) and.
Human Genetics (D.D., B.D., R.B.W.), University of Utah, Salt Lake City.

Brett Duval (B)

From the Department of Neurology (N.E.J.), Virginia Commonwealth University, Richmond; and Departments of Pediatrics (R.J.B., K.M., T.N., C.I., M.L.F.) and.
Human Genetics (D.D., B.D., R.B.W.), University of Utah, Salt Lake City.

Marcia L Feldkamp (ML)

From the Department of Neurology (N.E.J.), Virginia Commonwealth University, Richmond; and Departments of Pediatrics (R.J.B., K.M., T.N., C.I., M.L.F.) and.
Human Genetics (D.D., B.D., R.B.W.), University of Utah, Salt Lake City.

Robert B Weiss (RB)

From the Department of Neurology (N.E.J.), Virginia Commonwealth University, Richmond; and Departments of Pediatrics (R.J.B., K.M., T.N., C.I., M.L.F.) and.
Human Genetics (D.D., B.D., R.B.W.), University of Utah, Salt Lake City.

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