1.A severe acute intermittent porphyria patient: Successful treatment and management
Pei LI ; Songyun ZHANG ; Ninglin KANG ; Lijing JIAO ; Jie LI ; Guizhi LI ; Fenglin MENG ; Lihua WANG ; Fei TONG ; Lin YANG
Chinese Journal of Endocrinology and Metabolism 2022;38(4):335-338
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.
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