A severe acute intermittent porphyria patient: Successful treatment and management
10.3760/cma.j.cn311282-20210913-00594
- VernacularTitle:一例重症急性间歇性卟啉病患者的成功救治与管理
- Author:
Pei LI
1
;
Songyun ZHANG
;
Ninglin KANG
;
Lijing JIAO
;
Jie LI
;
Guizhi LI
;
Fenglin MENG
;
Lihua WANG
;
Fei TONG
;
Lin YANG
Author Information
1. 河北医科大学第二医院肾内科，石家庄 050000
- Keywords:
Acute intermittent porphyria;
Emotional;
Renal biopsy;
Crystal
- From:
Chinese Journal of Endocrinology and Metabolism
2022;38(4):335-338
- CountryChina
- Language:Chinese
-
Abstract:
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.
