Morais Catarina Granjo, Gomes Sara, Fragoso Ana Catarina, Coelho Janine, Jardim Joana, Barreira João Luís, Pinto-Carvalho Irene, Pinto Helena
Department of Pediatrics, Centro Hospitalar Universitário São João, Porto, Portugal.
Department of Pediatric Surgery, Centro Hospitalar Universitário São João, Porto, Portugal.
J Investig Med High Impact Case Rep. 2022 Jan-Dec;10:23247096211066295. doi: 10.1177/23247096211066295.
Xanthogranulomatous pyelonephritis (XPN) is an uncommon variant of chronic pyelonephritis with a poorly understood pathogenesis and a challenging diagnosis. It is rare in pediatric patients, particularly in the neonatal period. We report the case of an 18-day-old female neonate admitted to the emergency room due to macroscopic hematuria and poor feeding. Urinalysis revealed leukocyturia and she was initially admitted under the clinical suspicion of acute pyelonephritis. Renal ultrasound and magnetic resonance imaging (MRI) revealed a progressive nodular lesion in the middle third of the left kidney. Given the suspicion of renal abscess or neoplasm, the patient was transferred to our tertiary hospital. Urinary catecholamines and tumor markers had normal values. Percutaneous kidney biopsy confirmed XPN. Posterior computed tomography scan excluded extension to neighboring structures. A conservative management with systemic antibiotic therapy was decided. She completed 7 weeks of systemic antibiotic therapy (ampicillin and cefotaxime) with progressive reduction of lesion size and posterior calcification. Follow-up at 3 years was uneventful. The lipid profile and study of neutrophil function were normal. Voiding cystourethrography excluded vesicoureteral reflux. The authors intend to highlight the importance of a high index of suspicion of XPN to allow preoperative diagnosis. Histopathological assessment is mandatory to confirm XPN and exclude other entities mimicked by focal and unilateral progressive disease. There are only a few published cases of optimal clinical evolution solely with broad-spectrum antibiotics; however, this may allow a beneficial nephron-sparing approach in selected patients.
黄色肉芽肿性肾盂肾炎(XPN)是慢性肾盂肾炎的一种罕见变异型,其发病机制尚不清楚,诊断具有挑战性。在儿科患者中罕见,尤其是在新生儿期。我们报告一例18日龄女婴因肉眼血尿和喂养困难入住急诊室。尿液分析显示白细胞尿,最初因临床怀疑急性肾盂肾炎而入院。肾脏超声和磁共振成像(MRI)显示左肾中三分之一处有一进行性结节性病变。鉴于怀疑肾脓肿或肿瘤,患者被转诊至我们的三级医院。尿儿茶酚胺和肿瘤标志物值正常。经皮肾活检确诊为XPN。后位计算机断层扫描排除了病变向邻近结构的扩展。决定采用全身抗生素治疗的保守治疗方法。她完成了7周的全身抗生素治疗(氨苄西林和头孢噻肟),病变大小逐渐减小,后部出现钙化。3年随访无异常。血脂谱和中性粒细胞功能研究正常。排尿性膀胱尿道造影排除了膀胱输尿管反流。作者旨在强调高度怀疑XPN以实现术前诊断的重要性。组织病理学评估对于确诊XPN和排除由局灶性和单侧进行性疾病模拟的其他实体是必不可少的。仅有少数已发表的病例仅通过广谱抗生素就实现了最佳临床转归;然而,这可能为选定患者提供一种有益的保留肾单位的方法。