RESUMO
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.
Assuntos
Rim/metabolismo , Pielonefrite Xantogranulomatosa/metabolismo , Vimentina/metabolismo , Criança , Progressão da Doença , Feminino , Humanos , Rim/patologia , Nefrectomia , Pielonefrite Xantogranulomatosa/cirurgiaRESUMO
La pielonefritis xantogranulomatosa es una enfermedad inflamatoria muy poco frecuente, que se caracteriza por la sustitución del parénquima renal por tejido granulomatoso. Su presentación se caracteriza por dolor abdominal y síntomas constitucionales asociados a infecciones de orina de repetición. Los gérmenes más frecuentemente implicados son Escherichia coli y Proteus mirabilis. El diagnóstico definitivo es anatomopatológico y el único tratamiento curativo es la nefrectomía total o parcial. Presentamos una actualización de los conocimientos de esta enfermedad a raíz de un caso recientemente diagnosticado en nuestro servicio
Xanthogranulomatous pyelonephritis is a rare inflammatory disease, characterized by replacement of renal parenchyma with granulomatous tissue. Initial clinical presentation includes abdominal pain and constitutional symptoms related to recurrent urinary infections. The microorganisms most commonly involved are Escherichia coli and Proteus mirabilis. Final diagnosis is made by histopathology, and the only curative treatment is total or partial nephrectomy. A recently diagnosed case in our unit is presented, as well as an update on the knowledge of this disease
Assuntos
Humanos , Feminino , Criança , Pielonefrite Xantogranulomatosa/congênito , Pielonefrite Xantogranulomatosa/complicações , Pielonefrite Xantogranulomatosa/diagnóstico , Pielonefrite Xantogranulomatosa/metabolismo , Anorexia/complicações , Anorexia/dietoterapia , Infecções Urinárias/diagnóstico , Pielonefrite Xantogranulomatosa/induzido quimicamente , Pielonefrite Xantogranulomatosa/mortalidade , Pielonefrite Xantogranulomatosa/patologia , Anorexia/metabolismo , Anorexia/prevenção & controle , Infecções Urinárias/complicações , Infecções Urinárias/urina , Abscesso/complicaçõesRESUMO
TOPIC: Xanthogranulomatous pyelonephritis (XGP) is a chronic inflammation of the kidney characterized by destruction and replacement of its parenchyma with granulomatous tissue. It is associated with both chronic urinary obstruction and urinary tract infection (UTI). METHODS: We studied two children with chronic ureteropelvic junction obstruction (UPJO) and recurrent UTI nephrectomized for poor kidney function. An intraoperative renal biopsy was taken to relate the presence of infiltrating monocytes plus tubular atrophy to tissue expression of monocyte chemotactic protein-1 (MCP-1) and epidermal growth factor (EGF). XGP was diagnosed by a pathologist in both cases. RESULTS: MCP-1 expression was significantly higher in the two patients compared with the controls or patients with uncomplicated UPJO. It also correlated with the extent of monocyte infiltration, whereas EGF was only significantly downregulated when compared with the controls. CONCLUSIONS: MCP-1 would seem to play a key role in the pathogenesis of XGP by mediating the recruitment of circulating monocytes or by cells resident in the interstitial space.
Assuntos
Quimiocina CCL2/genética , Expressão Gênica , Pielonefrite Xantogranulomatosa/genética , RNA Mensageiro/genética , Biomarcadores/metabolismo , Biópsia , Quimiocina CCL2/biossíntese , Pré-Escolar , Fator de Crescimento Epidérmico/biossíntese , Fator de Crescimento Epidérmico/genética , Feminino , Humanos , Imuno-Histoquímica , Hibridização In Situ , Lactente , Pielonefrite Xantogranulomatosa/metabolismo , Pielonefrite Xantogranulomatosa/patologia , RNA Mensageiro/biossíntese , Reação em Cadeia da Polimerase Via Transcriptase ReversaAssuntos
Rim/patologia , Pielonefrite Xantogranulomatosa/patologia , Antígenos CD/metabolismo , Antígenos de Diferenciação Mielomonocítica/metabolismo , Feminino , Células Gigantes/patologia , Humanos , Imuno-Histoquímica , Rim/fisiopatologia , Rim/cirurgia , Pessoa de Meia-Idade , Nefrite/patologia , Pielonefrite Xantogranulomatosa/metabolismo , Pielonefrite Xantogranulomatosa/cirurgia , Vimentina/metabolismoRESUMO
In an attempt to identify a tumour marker, we investigated peripheral-type benzodiazepine binding sites (PBS) in kidney specimens obtained from patients who underwent nephrectomy due to a renal mass. [3H]PK 11195, an isoquinoline carboxamide derivative, was used as a ligand. Binding assays were conducted on samples of membrane homogenate taken from both the healthy portion and the tumour site of the kidney. It was found that binding characteristics of benign tumours and normal kidney tissues were not significantly different, i.e. equilibrium dissociation constants of 2.20 +/- 0.73 and 2.38 +/- 0.98 nM, respectively, and maximal number of binding sites of 3190 +/- 1081 and 4189 +/- 998 fmol/mg protein, respectively. In contrast, no PBS were detectable in renal carcinoma. The absence of PBS in malignant renal tissues may serve as a biochemical marker for tumours of the kidney.