title: Tubular functions in familial Mediterranean fever.
abstract: In this study, we aimed to evaluate renal tubular function in familial Mediterranean fever (FMF). Urinary N-acetyl-beta-D glucosaminidase (U-NAG, beta2-microglobulin (U-beta2M) and microalbumin (Ua) levels were measured in children with different clinical stages of FMF (58 patients with FMF, 9 patients with amyloidosis secondary to (FMF). Control groups were healthy children (n=21), children with upper respiratory tract infection (URTI) (n=21) and with steroid sensitive nephrotic syndrome (SSNS) (n=18). U-NAG was significantly increased in patients with a recent diagnosis of FMF compared to patients with FMF on colchicine and to healthy controls. In patients with recently diagnosed FMF, a marked decrease in U-NAG, U-beta2M and Ua were determined after three months on colchicine therapy. On the other hand, U-NAG and Ubeta2M levels were increased in patients with FMF during attacks and then decreased in the post-attack period. U-beta2M in patients with FMF during attacks was significantly different from patients with URTI. Finally, U-NAG and U-beta2M were increased significantly in patients with FMF-amyloidosis and SSNS when compared with other FMF groups and healthy controls, respectively. In conclusion, the high U-NAG value in newly diagnosed patients compared to that of patients taking colchicine and the decline of U-NAG and U-beta2M levels after attack to the levels observed in colchicine users (without a significant change in Ua value) suggest that the renal injury early in the course of FMF might be dominantly at the level of the tubuli.
authors.affiliation: Department of Pediatrics, Istanbul University Cerrahpaşa Faculty of Medicine, Istanbul, Turkey.
authors.forename: Semra
authors.initials: S
authors.lastname: Akkuş
authors.forename: Salim
authors.initials: S
authors.lastname: Calişkan
authors.forename: Ozgür
authors.initials: O
authors.lastname: Kasapçopur
mesh_terms: D000118:Acetylglucosaminidase / Q000652:urine*; D000293:Adolescent; D000686:Amyloidosis / Q000209:etiology / Q000473:pathology; D015415:Biomarkers / Q000652:urine; D002648:Child; D002675:Child, Preschool; D003078:Colchicine / Q000627:therapeutic use; D010505:Familial Mediterranean Fever / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology* / Q000652:urine; D005260:Female; D006801:Humans; D007223:Infant; D007674:Kidney Diseases / Q000209:etiology; D007684:Kidney Tubules / Q000503:physiopathology*; D008297:Male; D011446:Prospective Studies; D012016:Reference Values; D001613:beta 2-Microglobulin / Q000652:urine*
chemical_list: D015415:Biomarkers; D001613:beta 2-Microglobulin; D000118:Acetylglucosaminidase; D003078:Colchicine