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一名儿科患者的黄色肉芽肿性肾盂肾炎。

Xanthogranulomatous pyelonephritis in a pediatric patient.

作者信息

Caixeta Kamile Eller Gusmão, Matos Wallan de Deus Caixeta, Ceranto Augusto Ventura, Silva João Henrique do Amaral E, Barbosa Kellen Cristina Kamimura

机构信息

Universidade Federal do Triângulo Mineiro, Hospital de Clínicas, Pediatria, Uberaba, MG, Brasil.

出版信息

J Bras Nefrol. 2022 Jul-Sep;44(3):452-456. doi: 10.1590/2175-8239-JBN-2020-0246.

Abstract

INTRODUCTION

Xanthogranulomatous pyelonephritis consists of a chronic infectious and inflammatory process of the renal parenchyma, a variant of chronic obstructive pyelonephritis. It is more prevalent in middle-aged adults, rare in pediatric patients, with less than 300 cases reported in children worldwide.

REPORT

Preschooler, aged 2 years and 11 months, male, with 2 months of abdominal distention, increased temperature and intense pallor, associated with microcytic anemia refractory to the use of ferrous sulfate. 1 week before, he had a bulging in his left flank and a hard palpable mass there. Imaging exams (ultrasound and tomography) revealed an overall enlargement of the left kidney, destruction of the renal parenchyma and intense calyceal dilation, forming the "bear's paw" sign, with a staghorn calculus in the pelvis. He underwent treatment with antibiotic therapy and total nephrectomy, with a specimen sent for pathological examination.

DISCUSSION

a disease of uncertain incidence in the pediatric age group, xanthogranulomatous pyelonephritis is more prevalent in male children and affects mainly the left kidney, being frequently associated with the presence of stones. Clinically, it has nonspecific symptoms, the most common being abdominal distension and asthenia. Laboratory exams shows microcytic, leukocytosis, thrombocytosis and increased inflammation, pyuria, hematuria and proteinuria, in addition to bacterial growth in urine culture. The diagnosis is anatomopathological, but it can be hinted by contrasted CT scan, with the classical sign of the "bear's paw". Treatment may include nephrectomy and broad-spectrum antibiotic therapy.

摘要

引言

黄色肉芽肿性肾盂肾炎是肾实质的一种慢性感染性炎症过程,是慢性梗阻性肾盂肾炎的一种变体。它在中年成年人中更为常见,在儿科患者中罕见,全球报道的儿童病例少于300例。

报告

一名2岁11个月的学龄前男性儿童,有2个月的腹胀、体温升高和极度苍白,伴有使用硫酸亚铁难以纠正的小细胞性贫血。1周前,他左侧胁腹膨隆,可触及一个硬肿块。影像学检查(超声和断层扫描)显示左肾整体增大,肾实质破坏,肾盏明显扩张,形成“熊掌”征,肾盂内有鹿角形结石。他接受了抗生素治疗和全肾切除术,并将标本送去做病理检查。

讨论

黄色肉芽肿性肾盂肾炎在儿科年龄组发病率不确定,在男性儿童中更为常见,主要影响左肾,常与结石的存在有关。临床上,它有非特异性症状,最常见的是腹胀和乏力。实验室检查显示小细胞性贫血、白细胞增多、血小板增多和炎症增加,有脓尿、血尿和蛋白尿,此外尿培养有细菌生长。诊断依靠解剖病理学检查,但通过增强CT扫描可提示诊断,其典型征象为“熊掌”征。治疗可能包括肾切除术和广谱抗生素治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a4ec/9518615/df22948ae4b5/2175-8239-jbn-2020-0246-gf01.jpg

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