Xanthogranulomatous pyelonephritis in a pediatric patient

Area of Science:

• Pediatric Nephrology
• Pediatric Urology
• Infectious Diseases

Background:

• Xanthogranulomatous pyelonephritis (XGP) is a rare, chronic inflammatory kidney disease.
• It is a variant of chronic obstructive pyelonephritis, more common in adults but infrequently seen in children.
• Less than 300 pediatric cases of XGP have been documented globally.

Observation:

• A 2-year-old boy presented with abdominal distension, fever, pallor, and refractory microcytic anemia.
• Imaging revealed a significantly enlarged left kidney with parenchymal destruction, calyceal dilation forming a "bear's paw" sign, and a staghorn calculus.
• Clinical presentation included nonspecific symptoms like abdominal distension and asthenia.

Findings:

• XGP in children is more common in males and frequently involves the left kidney.
• Associated findings include kidney stones, microcytic anemia, leukocytosis, thrombocytosis, and signs of inflammation in urine.
• The "bear's paw" sign on contrasted CT scans can suggest the diagnosis.

Implications:

• Prompt diagnosis of XGP is essential, often requiring pathological examination.
• Treatment strategies may involve broad-spectrum antibiotics and surgical intervention, such as nephrectomy.
• Understanding the clinical and imaging characteristics aids in managing this rare pediatric condition.