What is it about?
We propose a classification of porphyrias based on the pathophysiologic mechanism that causes each disease. Moreover, the mechanisms behind the signs and symptoms of the porphyrias are presented. An emphasis is made on the mechanisms of photosensitivity and on the role of iron and zinc in these diseases.
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Why is it important?
Porphyrias are presented and classified in manner easier to understand, remember and teach. This article provides an in depth review on the mechanisms of these diseases, in an effort to provide the reader with a simple and clear image on what to expect in each porphyria.
Perspectives
This article is focused on being didactic and can easily be transformed into a lesson. Our aim was to take this set of rare diseases, often overlooked by most curriculums (sometimes only mentioned as part of a lesson on heme metabolism) and present them in a memorable and easy to follow manner.
Loukas Kakoullis
University of Patras
Read the Original
This page is a summary of: Porphyrias and photosensitivity: pathophysiology for the clinician, Postgraduate Medicine, October 2018, Taylor & Francis,
DOI: 10.1080/00325481.2018.1533380.
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