[Abstract] Porphyria is a genetic metabolic disease related to the lack of enzyme activity in heme biosynthesis pathway, among which, porphyria characterized by liver function damage are rare, and therefore prone to misdiagnosis and missed diagnosis. From November 2014 to December 2019, 6 cases of porphyria with liver function impairment were admitted to our center. To better understand porphyria with liver function impairment, the clinical presentations, liver injury indices, liver pathological features, gene detection results, treatment and prognosis were summarized and analyzed. |