Storjord Elin, Wahlin Staffan, Karlsen Bård Ove, Hardersen Randolf I, Dickey Amy K, Ludviksen Judith K, Brekke Ole-Lars
Department of Laboratory Medicine, Nordland Hospital Trust, 8092 Bodø, Norway.
Hepatology Division, Department of Upper GI Diseases, Porphyria Centre Sweden, Karolinska Institute and Karolinska University Hospital, 14186 Stockholm, Sweden.
Life (Basel). 2023 Dec 21;14(1):19. doi: 10.3390/life14010019.
Acute intermittent porphyria (AIP) is an inherited metabolic disorder associated with complications including kidney failure and hepatocellular carcinoma, probably caused by elevations in the porphyrin precursors porphobilinogen (PBG) and delta-aminolevulinic acid (ALA). This study explored differences in modern biomarkers for renal and hepatic damage between AIP patients and controls. Urine PBG testing, kidney injury panels, and liver injury panels, including both routine and modern biomarkers, were performed on plasma and urine samples from AIP cases and matched controls (50 and 48 matched pairs, respectively). Regarding the participants' plasma, the AIP cases had elevated kidney injury marker-1 (KIM-1, = 0.0002), fatty acid-binding protein-1 (FABP-1, = 0.04), and α-glutathione S-transferase (α-GST, = 0.001) compared to the matched controls. The AIP cases with high PBG had increased FABP-1 levels in their plasma and urine compared to those with low PBG. In the AIP cases, KIM-1 correlated positively with PBG, CXCL10, CCL2, and TCC, and the liver marker α-GST correlated positively with IL-13, CCL2, and CCL4 (all < 0.05). In conclusion, KIM-1, FABP-1, and α-GST could represent potential early indicators of renal and hepatic damage in AIP, demonstrating associations with porphyrin precursors and inflammatory markers.
急性间歇性卟啉病(AIP)是一种遗传性代谢紊乱疾病,与包括肾衰竭和肝细胞癌在内的并发症相关,可能由卟啉前体胆色素原(PBG)和δ-氨基-γ-酮戊酸(ALA)水平升高引起。本研究探讨了AIP患者与对照组之间肾脏和肝脏损伤现代生物标志物的差异。对AIP病例和匹配对照组(分别为50对和48对匹配组)的血浆和尿液样本进行了尿液PBG检测、肾脏损伤指标检测以及肝脏损伤指标检测,包括常规和现代生物标志物。关于参与者的血浆,与匹配对照组相比,AIP病例的肾脏损伤标志物-1(KIM-1,P = 0.0002)、脂肪酸结合蛋白-1(FABP-1,P = 0.04)和α-谷胱甘肽S-转移酶(α-GST,P = 0.001)升高。高PBG的AIP病例与低PBG的病例相比,其血浆和尿液中的FABP-1水平升高。在AIP病例中,KIM-1与PBG、CXCL10、CCL2和TCC呈正相关,肝脏标志物α-GST与IL-13、CCL2和CCL4呈正相关(均P < 0.05)。总之,KIM-1、FABP-1和α-GST可能代表AIP中肾脏和肝脏损伤的潜在早期指标,表明它们与卟啉前体和炎症标志物有关。