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产前诊断为巨大多囊性发育不良肾,于新生儿期行肾切除术。

Antenatally diagnosed giant multicystic dysplastic kidney resected during the neonatal period.

作者信息

Komura Makoto, Kanamori Yutaka, Sugiyama Masahiko, Nakahara Saori, Kawashima Hiroshi, Hatanaka Akira, Takazawa Yutaka, Goishi Keiji, Igarashi Takashi, Iwanaka Tadashi

机构信息

Department of Pediatric Surgery, The University of Tokyo Hospital, Tokyo, Japan.

出版信息

J Pediatr Surg. 2008 Nov;43(11):2118-20. doi: 10.1016/j.jpedsurg.2008.07.010.

Abstract

At 24 weeks of gestation, a fetus was suspected of having a huge intraabdominal cyst by fetal ultrasound. Multicystic dysplastic kidney (MCDK) was the most probable diagnosis; however, because a solid area was visualized in the large cystic lesion, a neoplasm of the kidney could not be ruled out. A 3529-g boy was born at 35 weeks of gestation by cesarean delivery. Eight days after birth, the tumor was resected. Histopathologic examination confirmed MCDK. The cause of MCDK in this patient was assumed to be ureteral obstruction in early fetal life. These findings suggested that the affected kidney had experienced mesenchyme-to-epithelium transition followed by interaction between the metanephric blastema and ureteral bud.

摘要

妊娠24周时,胎儿超声检查怀疑有巨大腹腔内囊肿。最可能的诊断是多囊性发育不良肾(MCDK);然而,由于在大的囊性病变中可见实性区域,不能排除肾肿瘤。一名3529克的男婴于妊娠35周时剖宫产出生。出生8天后,切除肿瘤。组织病理学检查证实为MCDK。该患者MCDK的病因推测为胎儿早期输尿管梗阻。这些发现提示,患肾经历了间充质到上皮的转化,随后后肾胚基与输尿管芽之间发生了相互作用。

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