Jiang Yeping, Chang Menglin, Fu Qian, Wang Hui
Department of Nephrology, Beijing Children's Hospital, Capital Medical University, Beijing, China.
Department of Nephrology, Baoding Hospital of Beijing Children's Hospital, Capital Medical University, Regional Center for Children's Health, Baoding, China.
Front Pediatr. 2025 Jul 10;13:1625781. doi: 10.3389/fped.2025.1625781. eCollection 2025.
Xanthogranulomatous pyelonephritis (XGP), a rare granulomatous renal disease linked to bacterial infection (e.g., ), presents challenges in pediatric diagnosis, especially in infants, due to overlap with neoplastic renal masses like Wilms tumor.
A 47-day-old male infant with fever, elevated inflammatory markers (WBC 13.94 × 10/L, CRP 110.43 mg/L), and urinary leukocytes/hematuria showed a left renal mass (1.7 × 1.8 × 2.1 cm) on imaging. Biopsy revealed histiocytic-neutrophilic infiltration with focal necrosis, and metagenomic sequencing identified dominant E. coli. Antibiotic therapy (cefoperazone-sulbactam followed by cefdinir) induced regression (1.1 × 0.8 × 1.1 cm at 2 weeks). Elevated AFP (888.27 ng/ml) normalized, excluding malignancy.
This case highlights XGP as a critical differential diagnosis for febrile infants with renal masses. Integration of histopathology, metagenomic sequencing, and prolonged follow-up confirms that focal XGP can be managed successfully with targeted antibiotics, avoiding nephrectomy.
黄色肉芽肿性肾盂肾炎（XGP）是一种与细菌感染相关的罕见肉芽肿性肾脏疾病（例如），由于与肾母细胞瘤等肿瘤性肾肿块存在重叠，在儿科诊断中面临挑战，尤其是在婴儿中。
一名47日龄男婴，有发热、炎症指标升高（白细胞13.94×10/L，C反应蛋白110.43mg/L）以及尿白细胞/血尿，影像学检查显示左肾有一肿块（1.7×1.8×2.1cm）。活检显示组织细胞-中性粒细胞浸润伴局灶性坏死，宏基因组测序鉴定出优势菌为大肠杆菌。抗生素治疗（先使用头孢哌酮-舒巴坦，后使用头孢地尼）使肿块缩小（2周时为1.1×0.8×1.1cm）。升高的甲胎蛋白（AFP，888.27ng/ml）恢复正常，排除了恶性肿瘤。
该病例突出了XGP作为发热性肾肿块婴儿重要鉴别诊断的地位。组织病理学、宏基因组测序及延长随访相结合证实，局灶性XGP通过靶向抗生素治疗可成功处理，避免肾切除术。
