PICO questions and DELPHI methodology for improving the management of patients with acute hepatic porphyria.
Rev Clin Esp (Barc)
; 224(5): 272-280, 2024 May.
Article
in En
| MEDLINE
| ID: mdl-38642893
ABSTRACT
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 ANDMETHODS:
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. 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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Delphi Technique
/
Porphyrias, Hepatic
/
Porphobilinogen Synthase
Limits:
Humans
Language:
En
Journal:
Rev Clin Esp (Barc)
Year:
2024
Document type:
Article
Country of publication: