Gene therapy for spinal muscular atrophy: the Qatari experience.


Journal

Gene therapy
ISSN: 1476-5462
Titre abrégé: Gene Ther
Pays: England
ID NLM: 9421525

Informations de publication

Date de publication:
11 2021
Historique:
received: 08 12 2020
accepted: 16 06 2021
revised: 02 06 2021
pubmed: 20 7 2021
medline: 3 3 2022
entrez: 19 7 2021
Statut: ppublish

Résumé

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder characterized by hypotonia, progressive muscle weakness, and wasting. Onasemnogene abeparvovec (Zolgensma

Identifiants

pubmed: 34276047
doi: 10.1038/s41434-021-00273-7
pii: 10.1038/s41434-021-00273-7
pmc: PMC8599021
doi:

Substances chimiques

Bilirubin RFM9X3LJ49

Types de publication

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

Langues

eng

Sous-ensembles de citation

IM

Pagination

676-680

Informations de copyright

© 2021. The Author(s).

Références

Lefebvre S, Burglen L, Reboullet S, Clermont O, Burlet P, Viollet L, et al. Identification and characterization of a spinal muscular atrophy-determining gene. Cell. 1995;80:155–65.
doi: 10.1016/0092-8674(95)90460-3
Prior TW, Leach ME, Finanger E. Spinal Muscular Atrophy. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, et al., editors. GeneReviews
Verhaart IEC, Robertson A, Wilson IJ, Aartsma-Rus A, Cameron S, Jones CC, et al. Prevalence, incidence and carrier frequency of 5q-linked spinal muscular atrophy - a literature review. Orphanet J Rare Dis. 2017;12:124.
doi: 10.1186/s13023-017-0671-8
Al-Rajeh S, Bademosi O, Gascon GG, Stumpf D. Werdnig Hoffman’s disease (spinal muscular atrophy type I): A clinical study of 25 Saudi nationals in Al-Khobar. Ann Saudi Med. 1992;12:67–71.
doi: 10.5144/0256-4947.1992.67
al Rajeh S, Bademosi O, Ismail H, Awada A, Dawodu A, al-Freihi H, et al. A community survey of neurological disorders in Saudi Arabia: the Thugbah study. Neuroepidemiology. 1993;12:164–78.
doi: 10.1159/000110316
Rajab A, Bappal B, Al-Shaikh H, Al-Khusaibi S, Mohammed AJ. Common autosomal recessive diseases in Oman derived from a hospital-based registry. Community Genet. 2005;8:27–30.
doi: 10.1159/000083334
al-Gazali LI, Dawodu AH, Sabarinathan K, Varghese M. The profile of major congenital abnormalities in the United Arab Emirates (UAE) population. J Med Genet. 1995;32:7–13.
doi: 10.1136/jmg.32.1.7
Shawkya RM, El-Sayedb NS. Clinico-epidemiologic characteristics of spinal muscular atrophy among Egyptians. Egyptian J Med Hum Genet. 2011;12:25–30.
doi: 10.1016/j.ejmhg.2011.02.015
Al Jumah M, Majumdar R, Rehana Z, Al Rajeh S, Eyaid W. A pilot study of spinal muscular atrophy carrier screening in Saudi Arabia. J Pediatric Neurol. 2007;5:221–4.
Albrechtsen SS, Born AP, Boesen MS. Nusinersen treatment of spinal muscular atrophy—a systematic review. Dan Med J. 2020;67:A02200100.
pubmed: 32800069
Mendell JR, Al-Zaidy S, Shell R, Arnold WD, Rodino-Klapac LR, Prior TW, et al. Single-dose gene-replacement therapy for spinal muscular atrophy. N Engl J Med. 2017;377:1713–22.
doi: 10.1056/NEJMoa1706198
Lowes LP, Alfano LN, Arnold WD, Shell R, Prior TW, McColly M, et al. Impact of age and motor function in a phase 1/2A study of infants with SMA type 1 receiving single-dose gene replacement therapy. Pediatr Neurol. 2019;98:39–45.
doi: 10.1016/j.pediatrneurol.2019.05.005
Al-Zaidy SA, Kolb SJ, Lowes L, Alfano LN, Shell R, Church KR, et al. AVXS-101 (Onasemnogene Abeparvovec) for SMA1: Comparative Study with a Prospective Natural History Cohort. J Neuromuscul Dis. 2019;6:307–17.
doi: 10.3233/JND-190403
Waldrop MA, Karingada C, Storey MA, Powers B, Iammarino MA, Miller NF, et al. Gene therapy for spinal muscular atrophy: safety and early outcomes. Pediatrics. 2020;146:e20200729.
doi: 10.1542/peds.2020-0729
Friese J, Geitmann S, Holzwarth D, Muller N, Sassen R, Baur U, et al. Safety monitoring of gene therapy for spinal muscular atrophy with onasemnogene abeparvovec—a single centre experience. J Neuromuscul Dis. 2021;8:209–16.
doi: 10.3233/JND-200593
Glanzman AM, Mazzone E, Main M, Pelliccioni M, Wood J, Swoboda KJ, et al. The Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND): test development and reliability. Neuromuscul Disord. 2010;20:155–61.
doi: 10.1016/j.nmd.2009.11.014
Chand D, Mohr F, McMillan H, Tukov FF, Montgomery K, Kleyn A, et al. Hepatotoxicity following administration of onasemnogene abeparvovec (AVXS-101) for the treatment of spinal muscular atrophy. J Hepatol. 2020;74:560–6.
doi: 10.1016/j.jhep.2020.11.001
Feldman AG, Parsons JA, Dutmer CM, Veerapandiyan A, Hafberg E, Maloney N, et al. Subacute liver failure following gene replacement therapy for spinal muscular atrophy type 1. J Pediatr. 2020;225:252–8.e251.
doi: 10.1016/j.jpeds.2020.05.044
Chand DH, Zaidman C, Arya K, Millner R, Farrar MA, Mackie FE, et al. Thrombotic microangiopathy following onasemnogene abeparvovec for spinal muscular atrophy: a case series. J Pediatr. 2020;231:265–8.
doi: 10.1016/j.jpeds.2020.11.054
Kirschner J, Butoianu N, Goemans N, Haberlova J, Kostera-Pruszczyk A, Mercuri E, et al. European ad-hoc consensus statement on gene replacement therapy for spinal muscular atrophy. Eur J Paediatr Neurol. 2020;28:38–43.
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doi: 10.1002/ana.24864

Auteurs

Hossamaldein Gaber Ali (HG)

Department of Pharmacy, Hamad Medical Corporation, Doha, Qatar.

Khalid Ibrahim (K)

Department of Pediatrics, Sidra Medicine, Doha, Qatar.
Weill Cornell Medical College, Doha, Qatar.

Mahmoud Fawzi Elsaid (MF)

Department of Pediatrics, Sidra Medicine, Doha, Qatar.
Weill Cornell Medical College, Doha, Qatar.

Reem Babiker Mohamed (RB)

Qatar Rehabilitation Institute, Hamad Medical Corporation, Doha, Qatar.
Department of Pediatrics, Hamad Medical Corporation, Doha, Qatar.

Mahmoud I A Abeidah (MIA)

Qatar Rehabilitation Institute, Hamad Medical Corporation, Doha, Qatar.
Department of Pediatrics, Hamad Medical Corporation, Doha, Qatar.

Azhar Othman Al Rawwas (AO)

Qatar Rehabilitation Institute, Hamad Medical Corporation, Doha, Qatar.
Department of Pediatrics, Hamad Medical Corporation, Doha, Qatar.

Khaled Elshafey (K)

Department of Pediatrics, Hamad Medical Corporation, Doha, Qatar.

Hajer Almulla (H)

Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar.

Karen El-Akouri (K)

Department of Pediatrics, Sidra Medicine, Doha, Qatar.
Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar.

Mariam Almulla (M)

Department of Pediatrics, Sidra Medicine, Doha, Qatar.
Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar.

Amna Othman (A)

Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar.

Sara Musa (S)

Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar.

Fatma Al-Mesaifri (F)

Department of Pediatrics, Sidra Medicine, Doha, Qatar.
Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar.

Rehab Ali (R)

Department of Pediatrics, Sidra Medicine, Doha, Qatar.
Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar.
Center of Rare Disease, Hamad Medical Corporation, Doha, Qatar.

Noora Shahbeck (N)

Department of Pediatrics, Sidra Medicine, Doha, Qatar.
Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar.

Mariam Al-Mureikhi (M)

Department of Pediatrics, Sidra Medicine, Doha, Qatar.
Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar.

Reem Alsulaiman (R)

Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar.
Center of Rare Disease, Hamad Medical Corporation, Doha, Qatar.

Saad Alkaabi (S)

Center of Rare Disease, Hamad Medical Corporation, Doha, Qatar.

Tawfeg Ben-Omran (T)

Department of Pediatrics, Sidra Medicine, Doha, Qatar. tawben11@hotmail.com.
Weill Cornell Medical College, Doha, Qatar. tawben11@hotmail.com.
Department of Medical Genetics, Hamad Medical Corporation, Doha, Qatar. tawben11@hotmail.com.
Center of Rare Disease, Hamad Medical Corporation, Doha, Qatar. tawben11@hotmail.com.

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