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Gunther Bernert

Explore the profile of Gunther Bernert including associated specialties, affiliations and a list of published articles. Areas
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Articles 34
Citations 713
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Recent Articles
1.
Weiss C, Becker L, Friese J, Blaschek A, Hahn A, Illsinger S, et al.
Lancet Reg Health Eur . 2024 Oct; 47:101092. PMID: 39434961
Background: Real-world data on gene addition therapy (GAT) with onasemnogene abeparvovec (OA), including all age groups and with or without symptoms of the disease before treatment are needed to provide...
2.
Kirschner J, Bernert G, Butoianu N, Waele L, Fattal-Valevski A, Haberlova J, et al.
Eur J Paediatr Neurol . 2024 Jun; 51:73-78. PMID: 38878702
Spinal muscular atrophy (SMA) is one of the most common genetic diseases and was, until recently, a leading genetic cause of infant mortality. Three disease-modifying treatments have dramatically changed the...
3.
Schwartz O, Vill K, Pfaffenlehner M, Behrens M, Weiss C, Johannsen J, et al.
JAMA Pediatr . 2024 Apr; 178(6):540-547. PMID: 38587854
Importance: There is increasing evidence that early diagnosis and treatment are key for outcomes in infants with spinal muscular atrophy (SMA), and newborn screening programs have been implemented to detect...
4.
GuNTHER R, Wurster C, Brakemeier S, Osmanovic A, Schreiber-Katz O, Petri S, et al.
Lancet Reg Health Eur . 2024 Feb; 39:100862. PMID: 38361750
Background: Evidence for the efficacy of nusinersen in adults with 5q-associated spinal muscular atrophy (SMA) has been demonstrated up to a period of 16 months in relatively large cohorts but...
5.
Hiebeler M, Thiele S, Reilich P, Bernert G, Walter M
Sci Rep . 2023 Jan; 13(1):179. PMID: 36604563
Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder manifesting in early childhood with progressive muscular weakness and atrophy, and resulting in early loss of ambulation. The collection and evaluation...
6.
Pechmann A, Behrens M, Dornbrack K, Tassoni A, Wenzel F, Stein S, et al.
J Neuromuscul Dis . 2022 Dec; 10(1):29-40. PMID: 36565133
Background And Objectives: Disease progression in patients with spinal muscular atrophy (SMA) has changed dramatically within the past years due to the approval of three different disease-modifying treatments. Nusinersen was...
7.
Krenn M, Sener M, Rath J, Zulehner G, Keritam O, Wagner M, et al.
J Neurol . 2022 Oct; 270(2):909-916. PMID: 36308527
Background: Congenital myasthenic syndromes (CMS) are a heterogeneous group of disorders caused by genetic defects resulting in impaired neuromuscular transmission. Although effective treatments are available, CMS is probably underdiagnosed, and...
8.
Pechmann A, Behrens M, Dornbrack K, Tassoni A, Wenzel F, Stein S, et al.
Orphanet J Rare Dis . 2022 Oct; 17(1):384. PMID: 36274155
Background: The development and approval of disease modifying treatments have dramatically changed disease progression in patients with spinal muscular atrophy (SMA). Nusinersen was approved in Europe in 2017 for the...
9.
Pechmann A, Behrens M, Dornbrack K, Tassoni A, Stein S, Vogt S, et al.
Brain . 2022 Jul; 146(2):668-677. PMID: 35857854
5q-associated spinal muscular atrophy is a rare neuromuscular disorder with the leading symptom of a proximal muscle weakness. Three different drugs have been approved by the European Medicines Agency and...
10.
Weiss C, Ziegler A, Becker L, Johannsen J, Brennenstuhl H, Schreiber G, et al.
Lancet Child Adolesc Health . 2021 Nov; 6(1):17-27. PMID: 34756190
Background: Given the novelty of gene replacement therapy with onasemnogene abeparvovec in spinal muscular atrophy, efficacy and safety data are limited, especially for children older than 24 months, those weighing...