BIPNET Guidelines
BIPNET have developed a Best Practice Guideline on the 'Clinical management of Acute Attacks of Porphyria and their Complications'.
These were published in the May 2013 edition of Annals of Clinical Biochemistry.
A full copy of this published guideline is available now without charge from the Annals of Biochemistry electronic publication. See below for a copy of the abstract.
Best practice guidelines on clinical management of acute attacks of porphyria and their complications
Authors: Penelope Stein1, Mike Badminton2, Julian Barth3, David Rees4, and M Felicity Stewart5.
1. Department of Medicine, Addenbrooke's Hospital, Cambridge CB2 0QQ
2. Department of Medical Biochemistry and Immunology, University Hospital of Wales, Cardiff CF14 4XW
3. Department of Clinical Chemistry, Leeds General Infirmary, Leeds LS1 3EX
4. Department of Haematology, King's College Hospital, London SE5 9RS
5. Department of Clinical Biochemistry, Salford Royal NHS Foundation Trust, Salford M6 8HD, UK
Abstract:
The British and Irish Porphyria Network guidelines describe best practice in the clinical assessment, investigation and management of acute porphyria attacks and their complications, including severe attacks with neuropathy. Acute attacks of porphyria may occur in acute intermittent porphyria (AIP), variegate porphyria (VP) and hereditary coproporphyria (HCP). Aminolaevulinic acid dehydratase deficiency porphyria (ADP) is a very rare autosomal recessive porphyria; only six cases substantiated by mutation analysis have yet been described in the literature.
Urinary porphobilinogen (PBG) is always raised in an acute attack due to AIP, VP or HCP and this analysis is essential to confirm the diagnosis. A positive result in a qualitative or semi-quantitative screening test must be followed by PBG quantitation at the earliest opportunity. However in a severely ill patient, treatment should not be delayed.
Removal of precipitating factors, effective analgesia and control of symptoms with safe medication, attention to nutrition and fluid balance are essential. The indications for use of intravenous haem arginate are set out, together with advice on its administration. A small proportion of acute porphyria patients develop recurrent attacks and management options that may be considered include gonadotrophin-releasing hormone analogues, ‘prophylactic’ regular haem arginate infusion or ultimately, liver transplantation.
EPNET Guidelines
EPNET (our European sister organisation) is currently developing guidelines for the management of patients with acute hepatic porphyrias based on best available evidence and consensus expert opinion. A link to these will be added when available.
BIMDG Guidelines
The BIMDG management guidelines for acute porphyria
These are practical guidelines developed by the National Acute Porphyria Service to help clinicians treating patients with acute attacks of porphyria in the UK.
UK Porphyria Medicines Information Service
2021 Safe drug list for use in acute porphyrias.
The safe list (copies available below) have been produced jointly by the UK Porphyria Medicines Information Service (UKPMIS) and Cardiff Porphyria Service and is supported by the National Acute Porphyria Service (NAPS).
These lists compliment the 'Drug Database for Acute Porphyria', located at http://www.drugs-porphyria.org/.
Safe Drug List - alphabetical drug name version
Safe Drug list - drug categories version