Monitoring and integrated care coordination of patients with alpha-mannosidosis: A global Delphi consensus study
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064165%3A_____%2F24%3A10483347" target="_blank" >RIV/00064165:_____/24:10483347 - isvavai.cz</a>
Alternative codes found
RIV/00216208:11110/24:10483347
Result on the web
<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=xsp.WVsXFd" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=xsp.WVsXFd</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1016/j.ymgme.2024.108519" target="_blank" >10.1016/j.ymgme.2024.108519</a>
Alternative languages
Result language
angličtina
Original language name
Monitoring and integrated care coordination of patients with alpha-mannosidosis: A global Delphi consensus study
Original language description
Introduction: Current literature lacks consensus on initial assessments and routine follow-up care of patients with alpha-mannosidosis (AM). A Delphi panel was conducted to generate and validate recommendations on best practices for initial assessment, routine follow-up care, and integrated care coordination of patients with AM. Methods: A modified Delphi method involving 3 rounds of online surveys was used. An independent administrator and 2 nonvoting physician co-chairs managed survey development, anonymous data collection, and analysis. A multidisciplinary panel comprising 20 physicians from 12 countries responded to 57 open-ended questions in the first survey. Round 2 consisted of 11 ranking questions and 44 voting statements. In round 3, panelists voted to validate 60 consensus statements. The panel response rate was >= 95% in all 3 rounds. Panelists used 5-point Likert scales to indicate importance (score of >3) or agreement (score of >4). Consensus was defined a priori as >= 75% agreement with >= 75% of panelists voting. Results: Consensus was reached on 60 statements, encompassing 3 key areas: initial assessments, routine followup care, and treatment-related follow-up. The panel agreed on the type and frequency of assessments related to genetic testing, baseline evaluations, quality of life, biochemical measures, affected body systems, treatment received, and integrated care coordination in patients with AM. Forty-nine statements reached 90% to 100% consensus, 8 statements reached 80% to 85% consensus, and 1 statement reached 75% consensus. Two statements each reached consensus on 15 baseline assessments to be conducted at the initial follow-up visit after diagnosis in pediatric and adult patients. Conclusion: This is the first Delphi study providing internationally applicable, best-practice recommendations for monitoring patients with AM that may improve their care and well-being.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30209 - Paediatrics
Result continuities
Project
—
Continuities
N - Vyzkumna aktivita podporovana z neverejnych zdroju
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
Molecular Genetics and Metabolism
ISSN
1096-7192
e-ISSN
1096-7206
Volume of the periodical
142
Issue of the periodical within the volume
4
Country of publishing house
US - UNITED STATES
Number of pages
14
Pages from-to
108519
UT code for WoS article
001278795500001
EID of the result in the Scopus database
2-s2.0-85198607494