engleski

Xanthogranulomatous pyelonephritis in a 6 month old infant presented with nephrocalcinosis and afunctional left kidney

Introduction: Xanthogranulomatous pyelonephritis (XPN) is a rare presentation of severe chronic pyelonephritis characterised by renal parenchymal destruction by granulomatous tissue, inflammatory infiltration and fibrosis. The possible underlying mechanism involves urinary tract obstruction due to congenital anomalies, nephrolithiasis and recurrent urinary infection, resulting in unspecific symptoms such as abdominal pain, fever, abdominal mass, growth and weight retardation with persistent anemia, leucocytosis, elevated erythrocyte sedimentation rate (ESR), bacteriuria and pyuria. Therefore, the range of possible differential diagnosis is wide. Material and methods: Case report Results: The presented patient was born from an uneventful pregnancy and delivery. He first came to our attention at the age of 6 months due to acute cystopyelonefritis. Subsequently, left-sided hydroureteronephrosis (without vesicoureteral reflux), lymphadenopathy, severe anemia, elevated ESR and liver enzymes, as well as hypergammaglobulinemia and hypertriglyceridemia were noted. Other immune mediated, infectious, metabolic and malignant conditions were excluded by thorough diagnostic workup, including bone marrow analysis and whole exome sequencing. Abdominal ultrasound and computed tomography revealed unilateral nephrocalcinosis, and dynamic renal scintigraphy afunction of the left enlarged kidney. Despite antibiotic therapy, recurrent sterile pyuria with ongoing inflammation was present, necessitating a short course of glucocorticoids which led to gradual improvement of laboratory findings. Finally, left nephrectomy was performed with pathohistological finding suggestive of XPN. Conclusions: Regardless of diligent diagnostic workup, the precise mechanisms leading to XPN and intense immune reaction in our patient remains inconclusive. The literature data suggest that it might be a result of a macrophage defect in microbial processing, although no other signs of this process were present in the presented patient. Since this condition is very rarely present in children, especially infants, increased reporting of such challenging cases might increase the awareness, alleviate diagnostic dilemmas and steer the most appropriate treatment options.

xanthogranulomatous pyelonephritis ; differential diagnosis ; children ;

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