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A case of a congenital unilateral multicystic kidney and a review of 126 cases in Japan

机译:日本先天性单侧多囊肾1例并复查126例

摘要

A 3-year-old boy was admitted to our hospital with complaints of left flank mass and growth retardation. Examinations with IVP (Fig. 1), retrograde pyelography (Fig. 4) and cystoscopic examination (Fig. 3) showed a left nonopacified kidney, and right hydroureteronephrosis due to stenosis of the midureter. Two large cystic patterns were found in the left kidney by ultrasonic scanning tomography (Fig. 2); and, the left kidney was found to be occupied by a round and homogeneously low density mass by CT scanning (Fig. 6). The left renal artery was not demonstrated on the aortogram (Fig. 5). The patient was diagnosed to have a left congenital unilateral multicystic kidney, and laparotomy was performed. The left kidney was easily removed, and the part with stenosis in the right ureter was removed, then end-to-end anastomosis was performed. Grossly, two large cysts (upper and lower part) and connective tissues were found in the left kidney (Fig. 7), and the vessels of the renal pedicle were filiform. No luminal formation of the renal pelvis or ureter was found. The epithelium of the cyst wall (Fig. 8) was deciduated leaving the connective tissue and smooth muscle, as shown by the histological examination. Histological examination of the parenchymal tissue (Fig. 9) between the two cysts showed primitive glomeruli and renal tubules among abundant connective tissue, but no cartilage tissue. Statistic examination and discussion have been made of 126 cases of congenital multicystic kidney reported in Japan.
机译:一个3岁男孩因左胁腹肿块和生长迟缓而入院。 IVP检查(图1),逆行肾盂造影(图4)和膀胱镜检查(图3)检查显示,由于输尿管中段狭窄,左肾不透明,右输尿管肾盂积水。超声扫描断层扫描在左肾中发现两个大的囊性样变(图2)。通过CT扫描发现左肾被圆形且均质的低密度肿块占据(图6)。主动脉造影未显示左肾动脉(图5)。患者被诊断患有左先天性单侧多囊肾,并进行了剖腹手术。左肾很容易被切除,右输尿管狭窄的部分被切除,然后进行端到端吻合。大体上,在左肾中发现两个大的囊肿(上部和下部)和结缔组织(图7),并且肾蒂的血管为丝状。未发现肾盂或输尿管的腔形成。组织学检查显示,囊肿壁的上皮(图8)脱落,结缔组织和平滑肌脱落。对两个囊肿之间的实质组织进行组织学检查(图9),发现结缔组织中有原始的肾小球和肾小管,但没有软骨组织。对日本报告的126例先天性多囊肾进行了统计检查和讨论。

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