05 October 2017
: Case report
Early Progression of Xanthogranulomatous Pyelonephritis in Children Might Be Dependent on Vimentin Expression
Unusual clinical course, Challenging differential diagnosis, Educational Purpose (only if useful for a systematic review or synthesis)
Danuta Ostalska-Nowicka1A*, Katarzyna Mackowiak-Lewandowicz1B, Aneta Konwerska2C, Jacek Zachwieja1ADDOI: 10.12659/AJCR.904376
Am J Case Rep 2017; 18:1066-1072
Abstract
BACKGROUND: Xanthogranulomatous pyelonephritis (XP) is an extremely rare, severe, atypical form of chronic renal parenchymal inflammation accompanied by hydronephrosis and/or urolithiasis. The pathomechanism of XP is not yet fully understood. Microscopically, XP is indicated by the presence of multinucleated giant cells and lipid-laden macrophages, as well as inflammatory infiltration and intensive renal fibrosis. The lipid accumulation in kidney parenchyma may be secondary to the altered flow of low-density lipoprotein (LDL)-derived cholesterol particles inside the affected cells. Physiologically, the process of LDL-derived cholesterol transport from lysosomes to the sites of its esterification is dependent on vimentin, which is a molecule comprising the cytoskeleton in mesenchymal cells.
CASE REPORT: A 7-year old girl was hospitalized because of the finding of unexplained kidney lesions on an abdominal ultrasound examination (an enlarged and deformed collecting system of the right kidney with hyperechogenic, solid, staghorn lesions in the calyces). Three months earlier, the patient had experienced recurrent urinary tract infection. Based on the subsequent laboratory and imaging diagnostics, the final diagnosis of XP was established and the girl was qualified for right-sided nephrectomy Microscopic examination revealed numerous foci of granuloma formations with no evident exponents of dysplastic or neoplastic abnormalities. Significant CD68-positive cell infiltrations and scattered foam cells arranging the numerous foci of granuloma inflammation were noticed. Renal parenchyma, adjacent to granuloma lesions, presented a vimentin expression.
CONCLUSIONS: Vimentin expression in XP may confirm a focal character of chronic granuloma formation and may suggest the complexity of XP pathogenesis involving not only macrophage and fibroblast activation but also local lipid deregulation and fibrosis.
Keywords: Nephritis, Interstitial, Pyelonephritis, Xanthogranulomatous
In Press
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946074
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946165
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946414
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.944665
Most Viewed Current Articles
21 Jun 2024 : Case report
89,141
DOI :10.12659/AJCR.944371
Am J Case Rep 2024; 25:e944371
07 Mar 2024 : Case report
50,239
DOI :10.12659/AJCR.943133
Am J Case Rep 2024; 25:e943133
20 Nov 2023 : Case report
25,805
DOI :10.12659/AJCR.941424
Am J Case Rep 2023; 24:e941424
18 Feb 2024 : Case report
22,697
DOI :10.12659/AJCR.943030
Am J Case Rep 2024; 25:e943030