End-stage renal disease due to retroperitoneal fibrosis in neurofibromatosis type I.

Autor:	Ramanzini LG; Federal University of Santa Maria (UFSM), Santa Maria, Rio Grande do Sul, Brazil., Camargo LFM; Federal University of Santa Maria (UFSM), Santa Maria, Rio Grande do Sul, Brazil., Caixeta TLO; Federal University of Santa Maria (UFSM), Santa Maria, Rio Grande do Sul, Brazil., Louzada RC; Federal University of Santa Maria (UFSM), Santa Maria, Rio Grande do Sul, Brazil., Frare JM; Federal University of Santa Maria (UFSM), Santa Maria, Rio Grande do Sul, Brazil.
Jazyk:	angličtina
Zdroj:	Intractable & rare diseases research [Intractable Rare Dis Res] 2023 May; Vol. 12 (2), pp. 129-131.
DOI:	10.5582/irdr.2023.01018
Abstrakt:	Retroperitoneal fibrosis (RF) commonly leads to renal impairment due to compression of ureters, and around 8% of patients eventually progress to end-stage renal disease (ESRD). We present a case of RF in a 61-year-old female patient with neurofibromatosis type 1 (NF1) who developed ESRD. She presented with a postrenal acute kidney injury, being initially treated with an ureteral catheter. A magnetic resonance imaging of the abdomen showed parietal thickening of the right ureter, and she underwent right ureter reimplantation through bladder flap and psoas hitch. There was an extensive area of fibrosis and inflammation over the right ureter. Biopsy disclosed nonspecific fibrosis, which was consistent with RF. Although the procedure was successful, she developed ESRD. We review atypical presentations of RF and causes of renal injury in NF1. RF should be considered a possible cause of chronic kidney disease in patients with NF1, perhaps due to an unknown underlying mechanism. Competing Interests: The authors have no conflicts of interest to disclose. (2023, International Research and Cooperation Association for Bio & Socio - Sciences Advancement.)
Databáze:	MEDLINE
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