Literature DB >> 33985857

Newborn screening programs for spinal muscular atrophy worldwide: Where we stand and where to go.

Tamara Dangouloff1, Eva Vrščaj2, Laurent Servais3, Damjan Osredkar4.   

Abstract

Spinal muscular atrophy (SMA) is a rare and devastating disease. New disease-modifying treatments have recently been approved and early treatment has been related to a better outcome. In this context, several newborn screening (NBS) programs have been implemented. The aim of the study was to obtain a global overview on the current situation and perspectives on SMA NBS. We conducted a survey and contacted experts from 152 countries, from which we gathered 87 responses. We identified 9 SMA NBS programs that have so far detected 288 newborns with SMA out of 3,674,277 newborns screened. Funding, screening methods, organisation, and consent process were variable between SMA NBS programs. Many respondents pointed the lack of cost/benefit data as a major obstacle to SMA NBS implementation. In the next four years, our data suggest a 24% coverage of newborns from countries where a disease-modifying drug is available and 8,5% coverage in countries with no diseases-modifying drugs. The annual proportion of newborns to be screened in the coming years is expected to increase steadily. The experts expressed a strong need for the implementation of SMA NBS as means to improve care for patients with SMA.
Copyright © 2021 Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Newborn screening; Nusinersen; Onasemnogene abeparvovec; Pre-symptomatic; Risdiplam; Spinal muscular atrophy

Mesh:

Year:  2021        PMID: 33985857     DOI: 10.1016/j.nmd.2021.03.007

Source DB:  PubMed          Journal:  Neuromuscul Disord        ISSN: 0960-8966            Impact factor:   4.296


  15 in total

Review 1.  Spinal muscular atrophy.

Authors:  Eugenio Mercuri; Charlotte J Sumner; Francesco Muntoni; Basil T Darras; Richard S Finkel
Journal:  Nat Rev Dis Primers       Date:  2022-08-04       Impact factor: 65.038

Review 2.  Onasemnogene Abeparvovec: A Review in Spinal Muscular Atrophy.

Authors:  Hannah A Blair
Journal:  CNS Drugs       Date:  2022-08-12       Impact factor: 6.497

3.  Onasemnogene abeparvovec for presymptomatic infants with two copies of SMN2 at risk for spinal muscular atrophy type 1: the Phase III SPR1NT trial.

Authors:  Kevin A Strauss; Michelle A Farrar; Francesco Muntoni; Kayoko Saito; Jerry R Mendell; Laurent Servais; Hugh J McMillan; Richard S Finkel; Kathryn J Swoboda; Jennifer M Kwon; Craig M Zaidman; Claudia A Chiriboga; Susan T Iannaccone; Jena M Krueger; Julie A Parsons; Perry B Shieh; Sarah Kavanagh; Sitra Tauscher-Wisniewski; Bryan E McGill; Thomas A Macek
Journal:  Nat Med       Date:  2022-06-17       Impact factor: 87.241

Review 4.  Disease Modifying Therapies for the Management of Children with Spinal Muscular Atrophy (5q SMA): An Update on the Emerging Evidence.

Authors:  Helgi Thor Hjartarson; Kristofer Nathorst-Böös; Thomas Sejersen
Journal:  Drug Des Devel Ther       Date:  2022-06-16       Impact factor: 4.319

5.  Integrating newborn screening for spinal muscular atrophy into health care systems: an Australian pilot programme.

Authors:  Arlene M D'Silva; Didu S T Kariyawasam; Stephanie Best; Veronica Wiley; Michelle A Farrar
Journal:  Dev Med Child Neurol       Date:  2021-11-28       Impact factor: 4.864

6.  The Birth Prevalence of Spinal Muscular Atrophy: A Population Specific Approach in Estonia.

Authors:  Siiri Sarv; Tiina Kahre; Eve Vaidla; Sander Pajusalu; Kai Muru; Haide Põder; Katrin Gross-Paju; Sandra Ütt; Riina Žordania; Inga Talvik; Eve Õiglane-Shlik; Kristina Muhu; Katrin Õunap
Journal:  Front Genet       Date:  2021-12-22       Impact factor: 4.599

7.  Three years pilot of spinal muscular atrophy newborn screening turned into official program in Southern Belgium.

Authors:  François Boemer; Jean-Hubert Caberg; Pablo Beckers; Vinciane Dideberg; Samantha di Fiore; Vincent Bours; Sandrine Marie; Joseph Dewulf; Lionel Marcelis; Nicolas Deconinck; Aurore Daron; Laura Blasco-Perez; Eduardo Tizzano; Mickaël Hiligsmann; Jacques Lombet; Tatiana Pereira; Lucia Lopez-Granados; Sarvnaz Shalchian-Tehran; Véronique van Assche; Arabelle Willems; Sofie Huybrechts; Bénédicte Mast; Rudolf van Olden; Tamara Dangouloff; Laurent Servais
Journal:  Sci Rep       Date:  2021-10-07       Impact factor: 4.379

8.  Serum neurofilament light chain in pediatric spinal muscular atrophy patients and healthy children.

Authors:  Elisa Nitz; Martin Smitka; Jens Schallner; Katja Akgün; Tjalf Ziemssen; Maja von der Hagen; Victoria Tüngler
Journal:  Ann Clin Transl Neurol       Date:  2021-09-04       Impact factor: 4.511

9.  Detection of Spinal Muscular Atrophy Patients Using Dried Saliva Spots.

Authors:  Yogik Onky Silvana Wijaya; Hisahide Nishio; Emma Tabe Eko Niba; Kentaro Okamoto; Haruo Shintaku; Yasuhiro Takeshima; Toshio Saito; Masakazu Shinohara; Hiroyuki Awano
Journal:  Genes (Basel)       Date:  2021-10-14       Impact factor: 4.096

10.  New-Born Screening for Spinal Muscular Atrophy: Results of a Latvian Pilot Study.

Authors:  Linda Gailite; Olga Sterna; Maija Konika; Aleksejs Isakovs; Jekaterina Isakova; Ieva Micule; Signe Setlere; Mikus Diriks; Madara Auzenbaha
Journal:  Int J Neonatal Screen       Date:  2022-02-14
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