Alport's syndrome with focal segmental glomerulosclerosis lesion - Pattern to recognize.

We report a case of a 42-year-old male presenting with proteinuria, microscopic hematuria, elevated serum creatinine and hypertension with unremarkable physical examination apart from obesity. The renal biopsy showed well-established FSGS pattern of injury with mild interstitial fibrosis and tubular atrophy, while the electron microscopic examination demonstrated glomerular basement membranes (GBM) changes compatible with AS. AS can be complicated by segmental glomerular scarring, which can mimic primary FSGS, while familial FSGS can result from mutations in collagen IV network of the GBM. This overlap can complicate histopathological interpretation of renal biopsy, which should be accompanied by mutational analysis for accurate diagnosis and proper therapeutic intervention. PMID: 29456224 [PubMed - in process]

https://www.ncbi.nlm.nih.gov/pubmed/29456224?dopt=Abstract

Source: Saudi Journal of Kidney Diseases and Transplantation - January 1, 2018 Category: Urology & Nephrology Authors: Alsahli AA, Alshahwan SI, Alotaibi AO, Alsaad KO, Aloudah N, Farooqui M, Al Sayyari AA Tags: Saudi J Kidney Dis Transpl Source Type: research