Akademik Veri Yönetim Sistemi
Clinical nephrology. Case studies, cilt.11, sa.1, ss.50-54, 2021 (Scopus)
Kidney function may be im-paired during pregnancy due to various rea-sons, and the physiological changes of pregnancy may unmask or worsen pre-existing kidney disease. Herein, we report a pregnant patient presenting with nephrotic-range proteinuria. She later developed acute kidney injury and pre-eclampsia. However, he-molytic anemia and thrombocytopenia per-sisted after delivery, and she was diagnosed with atypical hemolytic uremic syndrome (aHUS). Although hematological abnormali-ties resolved with eculizumab treatment, her renal functions did not improve. Kidney biopsy showed crescentic glomerulonephri-tis without thrombotic microangiopathy features. Concurrently, she was evaluated for hearing impairment, and a diagnosis of Alport syndrome was confirmed with ge-netic testing. Kidney function may worsen in patients with Alport syndrome during pregnancy. However, crescentic glomeru-lonephritis (GN) is a rare finding in Alport disease. Pauci-immune crescentic GN has been shown to be related to dysregulated activation of the alternative complement pathway, which is also the underlying patho-physiological mechanism in aHUS.