PICO questions and DELPHI methodology for improving the management of patients with acute hepatic porphyria

Background Acute hepatic porphyrias (AHPs) are a group of rare diseases that encompasses acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, and 5-aminolaevulinic acid dehydratase deficiency porphyria. Symptoms of AHP are nonspecific which, together with its low prevalence, difficult the diagnosis and follow-up of these patients. Material and methods This project used DELPHI methodology to answer PICO questions related to management of patients with AHPs. The objective was to reach a consensus among multidisciplinary porhyria experts providing answers to those PICO questions for improving diagnosis and follow-up of patients with AHP. Results Ten PICO questions were defined and grouped in four domains: 1.- Biochemical diagnosis of patients with AHP, 2.- Molecular tests for patients with AHP, 3.- Follow-up of patients with AHP and 4.- Screening for long-term complications of patients with AHP. Conclusions PICO questions and DELPHI methodology have provided a consensus on relevant and controversial issues for improving the management of patients with AHP.