Key terms and definitions in acute porphyrias

Dear colleagues and friends,

We are very happy to inform you that the paper “Key terms and definitions in acute porphyrias: Results of an international Delphi consensus led by the European porphyria network” is now published in JIMD.

The original article can be completely downloaded as a pdf file by clicking here.

A podcast produced by JIMD dealing with this paper can be found at Soundcloud, Spotify or Apple.

We thank the whole international porphyria community and especially the Expert Panel for their contribution to this paper. We are now working to develop Evidence-based Guidelines for the treatment and monitoring of acute porphyrias.

Best regards from Sverre Sandberg (Ipnet president)


"Acute porphyrias are a group of rare inherited disorders causing acute neuro-visceral 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."