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  

Research progress of psycho-emotional abnormalities and quality of life indicators in patients with acute intermittent porphyria

作　　者：葛艳红[1] 张松筠[1] 李冰[1] 张玥[1] Ge Yanhong;Zhang Songyun;Li Bing;Zhang Yue(Department of Endocrinology,the Second Hospital of Hebei Medical University,Shijiazhuang 050000,China)

机构地区：[1]河北医科大学第二医院内分泌科,石家庄050000

出　　处：《中华现代护理杂志》2022年第11期1534-1540,共7页Chinese Journal of Modern Nursing

基　　金：河北省科技计划部分专项(202130821010045)。

摘　　要：急性间歇性卟啉病(AIP)是一种罕见病,是由PDGD/HMBS基因突变所致的常染色体显性遗传而引起。临床表现包括剧烈腹痛、肠麻痹、肢体无力、癫痫、精神情绪异常等。因疾病罕见,医务人员认知不足,临床误诊率极高。精神情绪异常是AIP重要临床表现,也是急性发作的常见诱因,但目前临床关注度远远不够。本文综述AIP患者焦虑、抑郁等精神情绪异常,生活质量、疼痛、疲劳、睡眠、疾病进展恐惧感、疾病病耻感、心理痛苦等指标的现状,旨为提高国内医护人员对AIP患者医疗照护提供方向,为预防发作、减少并发症、改善预后提供依据。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.

关 键 词：综述 卟啉病 焦虑 抑郁 生活质量 病耻感 

分 类 号：R589.8[医药卫生—内分泌]