一例重症急性间歇性卟啉病患者的成功救治与管理  

A severe acute intermittent porphyria patient: Successful treatment and management

作　　者：李佩 张松筠[2] 康宁林 焦丽静 李杰[2] 李贵芝[2] 孟凤苓[2] 王莉华[4] 佟飞[5] 杨林[1] Li Pei;Zhang Songyun;Kang Ninglin;Jiao Lijing;Li Jie;Li Guizhi;Meng Fenglin;Wang Lihua;Tong Fei;Yang Lin(Department of Nephrology,the Second Hospital of Hebei Medical University,Shijiazhuang 050000,China;Department of Endocrinology,the Second Hospital of Hebei Medical University,Shijiazhuang 050000,China;Department of Endocrinology,Hebei Hospital of Traditional Chinese Medicine,Shijiazhuang 050000,China;Department of Hemodialysis,the Second Hospital of Hebei Medical University,Shijiazhuang 050000,China;Department of Critical Care Medicine,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肾内科,石家庄050000 [2]河北医科大学第二医院内分泌科,石家庄050000 [3]河北省中医院内分泌科,石家庄050000 [4]河北医科大学第二医院血液透析科,石家庄050000 [5]河北医科大学第二医院重症医学科,石家庄050000

出　　处：《中华内分泌代谢杂志》2022年第4期335-338,共4页Chinese Journal of Endocrinology and Metabolism

摘　　要：急性间歇性卟啉病(acute intermittent porphyria,AIP)是罕见的遗传性代谢疾病,可发生严重致死性急性发作。本文分享1例重症AIP患者的救治与管理经验。针对急性发作,提出:(1)规避诱因至关重要;(2)关注易被忽略的情绪问题;(3)长期规范随访与患者教育可改善预后。针对慢性肾衰竭,于急性发作缓解期进行了肾穿刺活检,发现了既往文献未曾报道的偏振光显微镜下的红棕黄白色折光结晶,推测其为卟啉结晶。Acute intermittent porphyria(AIP)is a rare inherited metabolic disease that can cause severe and fatal acute attacks.This article shares the treatment and management of a severe AIP patient.It is proposed that(1)avoiding incentives is essential;(2)emotional problems easily overlooked should be paid attention;(3)long-term follow-up and patient education can improve the prognosis.The patient underwent renal biopsy during the remission period.We found a red-brown-yellow-white refractive index crystal under a polarized light microscope that had not been reported in the previous literature,which was speculated to be a porphyrin crystal.

关 键 词：急性间歇性卟啉病 情绪 肾活检 结晶 

分 类 号：R589.8[医药卫生—内分泌]