Department of Pediatrics, Kindai University Faculty of Medicine, 377-2 Ohno-higashi, Osakasayama-shi, Osaka, 589-8511, Japan.
Department of Pathology, Jikei University School of Medicine, Minato-ku, Tokyo, Japan.
BMC Nephrol. 2021 Feb 5;22(1):55. doi: 10.1186/s12882-021-02249-6.
The association of hypertension with congenital renal hypoplasia has been established. We report a case of an infant who underwent nephrectomy for hypertension.
Magnetic resonance imaging for the mother revealed fetal renal masses, and fetal multicystic dysplastic kidney was suspected. Following birth, the baby developed hypertension. Numerous investigations revealed that the left kidney was non-functional, and she was initiated on benazepril hydrochloride. However, because the drug response was poor, the left kidney was removed at the age of 7 months. Examination of the renal specimen revealed abrupt transition from normal to atrophic cortex with lobar atrophy and cysts. Tubular atrophy, marked abnormal blood vessels with wall thickening, gathered immature glomeruli, and parenchymal destruction were observed. Renin was partially localized in the proximal tubules and the parietal epithelium of the Bowman's capsule in the immature glomeruli. We speculated that an abnormal vascular structure and irregular renin localizations may be the cause of hypertension. Serum renin and aldosterone levels gradually reduced post-surgery, reaching normal levels on the 90th postoperative day. A long follow-up is needed due to the possibility of the child developing hypertension in the future.
This is a case of an infant with MCDK, which discusses the clinicopathological features based on the pathophysiological analysis, including renin evaluation.
高血压与先天性肾发育不全有关。我们报告了一例因高血压而行肾切除术的婴儿病例。
母亲的磁共振成像显示胎儿肾肿块,疑似胎儿多囊性发育不良肾。出生后,婴儿出现高血压。大量检查显示左肾功能丧失,她开始服用盐酸贝那普利。然而,由于药物反应不佳,她在 7 个月大时切除了左肾。肾脏标本检查显示,正常皮质突然过渡到萎缩,伴有小叶萎缩和囊肿。观察到管状萎缩、伴有壁增厚的明显异常血管、聚集的不成熟肾小球和实质破坏。肾素部分定位于不成熟肾小球的近端小管和鲍曼囊的壁细胞。我们推测异常的血管结构和不规则的肾素定位可能是导致高血压的原因。术后血清肾素和醛固酮水平逐渐降低,术后第 90 天恢复正常。由于孩子将来可能会发展为高血压,因此需要进行长期随访。
这是一例患有 MCDK 的婴儿病例,根据病理生理学分析讨论了临床病理特征,包括肾素评估。