Evaluating the Patient-Reported Outcomes Measurement Information System scales in acute intermittent porphyria.

ABSTRACT:

Purpose

Acute intermittent porphyria (AIP) is a rare inborn error of heme biosynthesis characterized by life-threatening acute attacks. Few studies have assessed quality of life (QoL) in AIP and those that have had small sample sizes and used tools that may not have captured important domains.

Methods

Baseline data from the Porphyrias Consortium's Longitudinal Study were obtained for 259 patients, including detailed disease and medical history data, and the following Patient-Reported Outcomes Measurement Information System (PROMIS) scales: anxiety, depression, pain interference, fatigue, sleep disturbance, physical function, and satisfaction with social roles. Relationships between PROMIS scores and clinical and biochemical AIP features were explored.

Results

PROMIS s

SUBMITTER: Naik H 

PROVIDER: S-EPMC7060090 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature