Research progress of psycho-emotional abnormalities and quality of life indicators in patients with acute intermittent porphyria
10.3760/cma.j.cn115682-20210829-03871
- VernacularTitle:急性间歇性卟啉病患者的精神情绪异常和生活质量指标的研究进展
- Author:
Yanhong GE
1
;
Songyun ZHANG
;
Bing LI
;
Yue ZHANG
Author Information
1. 河北医科大学第二医院内分泌科，石家庄 050000
- Keywords:
Review;
Porphyria;
Anxiety;
Depression;
Quality of life;
Stigma
- From:
Chinese Journal of Modern Nursing
2022;28(11):1534-1540
- CountryChina
- Language:Chinese
-
Abstract:
Acute intermittent porphyria (AIP) is a rare disease caused by autosomal dominant inheritance caused by mutations in PDGD/ HMBS gene. Clinical manifestations include severe abdominal pain, intestinal paralysis, limb weakness, epilepsy and psycho-emotional abnormalities. Due to the rarity of the disease and insufficient awareness of medical staff, the rate of clinical misdiagnosis is extremely high. Psycho-emotional abnormality is an important clinical manifestation of AIP and a common cause of acute attacks, but the current clinical attention is far from enough. This article reviews the current situation of anxiety, depression and other psycho-emotional abnormalities, quality of life, pain, fatigue, sleep, fear of disease progression, disease stigma, psychological pain and other indicators of AIP patients, in order to provide directions for improving domestic medical staff's medical care for AIP patients and to provide a basis for preventing attacks, reducing complications and improving prognosis.
