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Patients' Perceptions of Nusinersen Effects According to Their Responder Status

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F24%3A10481842" target="_blank" >RIV/00064203:_____/24:10481842 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216208:11130/24:10481842

  • Result on the web

    <a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=I_2MqIFC7d" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=I_2MqIFC7d</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.3390/jcm13123418" target="_blank" >10.3390/jcm13123418</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Patients' Perceptions of Nusinersen Effects According to Their Responder Status

  • Original language description

    Background and Objective: Patients with spinal muscular atrophy (SMA) treated with a disease-modifying therapy (DMT) are often classified as responders or non-responders based on the attainment of a specific improvement threshold on validated functional scales. This categorization may significantly impact treatment reimbursement in some countries. The aim of this research is to evaluate the perception of treatments and their benefit by patients considered as responders or non-responders. Methods: In this non-commercial multicenter study, 99 post-symptomatically treated SMA type I-III patients with a median age of 11.2 (0.39-57.4) years at treatment initiation were stratified into three groups based on their treatment outcomes, i.e., those exhibiting clinically significant improvement (N = 41), those with non-clinically significant improvement (N = 18), or those showing no improvement (N = 40). Fifteen months after treatment, the initiation patients or patients&apos; caregivers were assessed using a patient-rated scoring system based on the Patient Global Impression of Change (PGIC) scale, comprising 22 questions targeting important aspects and tasks in the daily life of patients with SMA. Results: We found no statistical difference in the patient perception of treatment benefits in 17 out of 22 domains across patient groups. Conclusions: Our results suggest that functional motor scales do not recapitulate patients&apos; and patients&apos; caregivers&apos; experience of the effect of nusinersen treatment in SMA.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30103 - Neurosciences (including psychophysiology)

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2024

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Journal of Clinical Medicine

  • ISSN

    2077-0383

  • e-ISSN

    2077-0383

  • Volume of the periodical

    13

  • Issue of the periodical within the volume

    12

  • Country of publishing house

    CH - SWITZERLAND

  • Number of pages

    14

  • Pages from-to

    3418

  • UT code for WoS article

    001256562500001

  • EID of the result in the Scopus database

    2-s2.0-85197131917