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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
biochemistry, genetics and molecular biology
Key terms and definitions in acute porphyrias: Results of an international Delphi consensus led by the European porphyria network
Journal of Inherited Metabolic Disease, Volume 46, No. 4, Year 2023
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Description
Acute porphyrias are a group of rare inherited disorders causing acute neurovisceral attacks. Many terms used frequently in the literature and clinical practice are ambiguous, which can lead to confusion in the way patients are managed, studied, and reported in clinical studies. Agreed definitions are a necessary first step in developing management guidelines and will facilitate communication of results of future clinical research. The Delphi method was used to generate consensus on key terms and definitions in acute porphyria. The process started with a brainstorming phase offered to all members of the European Porphyria Network followed by two Delphi rounds among international experts in the field of porphyria (the Acute Porphyria Expert Panel). A consensus of 75% or more was defined as the agreement threshold. A total of 63 respondents from 26 countries participated in the brainstorming phase, leading to the choice of nine terms and definitions. A total of 34 experts were invited to take part in the Delphi rounds. Seven of the initial nine terms and definitions which entered the first Delphi round achieved the threshold for agreement. Following a second Delphi round, all nine definitions achieved agreement. Agreement on the definitions for nine important terms describing acute porphyrias represents a significant step forward for the porphyria community. It will facilitate more accurate comparison of outcomes among porphyria centres and in clinical trials and provide a strong framework for developing evidence-based clinical guidelines. © 2023 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.
Authors & Co-Authors
Mansour, Razan A.
United States, Kansas City
University of Kansas Health System
Mustafa, Reem A.
United States, Kansas City
University of Kansas Health System
Canada, Hamilton
Mcmaster University
Sandberg, Sverre
Norway, Bergen
Haukeland Universitetssjukehus
Norway, Bergen
Universitetet I Bergen
Badminton, Michael N.
Unknown Affiliation
Balwani, Manisha
Unknown Affiliation
Cappellini, Maria Teresa
Unknown Affiliation
Cassiman, David M.
Unknown Affiliation
Deybach, Jeancharles Charles P.
Unknown Affiliation
El Mikati, Ibrahim K.
Unknown Affiliation
Gouya, Laurent
Unknown Affiliation
Hift, Richard J.
Unknown Affiliation
Rees, David C.
Unknown Affiliation
Sonderup, Mark Wayne
Unknown Affiliation
Weiler-Normann, Christina
Unknown Affiliation
Whatley, Sharon D.
Unknown Affiliation
Statistics
Citations: 5
Authors: 15
Affiliations: 6
Identifiers
Doi:
10.1002/jimd.12612
ISSN:
01418955
Research Areas
Noncommunicable Diseases