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Published on: July 18, 2017
[Abdominal mass revealing xanthogranulomatous pyelonephritis in an infant]
Anass Ayad1, Badr Ettouhami1, Benouachane Thami1
1Service de Pédiatrie 4, Hôpital d'Enfants de Rabat, Maroc.
Insights
Xanthogranulomatous pyelonephritis (XGP) is a rare kidney inflammation. Early diagnosis and treatment are crucial for infants presenting with vague symptoms and renal masses.
Area of Science:
- Pediatric Nephrology
- Pediatric Urology
- Pathology
Background:
- Xanthogranulomatous pyelonephritis (XGP) is a rare, chronic inflammatory kidney disease.
- It predominantly affects adults but can occur in children, often presenting with vague symptoms that delay diagnosis.
- Management typically involves medical therapy, but surgical intervention is frequently required, often leading to poor renal outcomes.
Observation:
- A 15-month-old infant presented with an isolated left flank mass.
- The infant lacked typical signs of infection such as fever and had sterile urine cultures.
- Radiological imaging revealed a non-functioning left kidney with features suggestive of hydronephrosis, raising suspicion for XGP.
Findings:
- Diagnostic imaging included renal ultrasound, uroscan, and renal scintigraphy.
- These investigations indicated a non-functioning left kidney, initially presumed to be hydronephrosis.
- Surgical nephrectomy confirmed diffuse Xanthogranulomatous pyelonephritis upon pathological examination.
Implications:
- This case highlights the importance of considering XGP in infants with renal masses or malformations.
- Vague symptomatology and sterile urine cultures can mask this serious condition.
- Prompt and accurate diagnosis followed by rigorous, codified treatment is essential for improved patient prognosis.
Abstract:
Xanthogranulomatous pyelonephritis (XGP) is a chronic pyelonephritis observed in children and exceptionally in infants. Symptomatology is vague and may delay diagnosis and patient's management. Treatment is based on medical therapy but most often on surgery with poor renal prognosis. We report the case of a 15-month old infant with isolated mass in the left flank. He had no fever or alteration of general state and urine cultures were sterile. Radiological evaluation (renal ultrasound, uroscan and renal scintigraphy) highlighted left non-functioning kidney with "hydropyonephrosis" evoking the diagnosis of XGP. The indication for total nephrectomy by lombotomy was posed and definitive anatomo-pathological examination confirmed the diagnosis of diffuse XGP. This observation emphasizes the importance of suspect PXG in patients with renal mass or malformative uropathy with recurrent urinary tract infections whose treatment should be rigorous and codified.
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