Elucidation of mechanism of MCDK and optimal treatment of obstructive uropathy in the Fetal Lamb

MCDK机制的阐明和胎羔梗阻性尿路病的最佳治疗

• 批准号: 14571709
• 负责人: KITAGAWA Hiroaki
• 金额: $ 2.24万
• 依托单位: St.Marianna University School of Medicine
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2002
• 资助国家: 日本
• 起止时间: 2002 至 2003
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-14571709/
• 关键词: Dysplastic Kidney; Potter Syndrome; Obstructive Uropathy; Ftal Surgery; 水腎症; 胎児手術; 多嚢胞性異型性腎(MCDK); 胎児水腎症; Potter症候群

Purpose : It is still unclear how urinary tract obstruction alters normal nephrogenesis ad leads to renal dysplasia. We created an obstructive uropathy model in fetal lambs ad reviewed the pathology of the obstructed kidney to determine the optimal timing for decompression of the obstruction.Methods : A obstructive uropathy was created in fetal lambs at 60 days gestation by ligating the urethra and urachus. They were delivered 20-31 days later by caesarian section. The kidneys were processed for histological examination.Results : Thirty four 60-day lambs were operated upon. Dysplastic changes were noted in 25 fetuses and 24 fetuses had cysts in the nephrogenic zone. The cystic components in multicystic dysplastic kidneys (MCDK) are mainly in the proximal tubules. Conclusions : In utero urinary tract obstruction causes reduction of numbers of functioning nephrons and produces cysts in the nephrogenic zone, and in the deeper cortex. These cysts and dilated proximal tubules suppress new nephron formation. Twenty days after obstruction there were early features of dysplasia but the nephrogenic zones were still present. We conclude that early shunting may salvage renal function.

目的：尿路梗阻是如何改变正常肾发生和导致肾发育不良的，目前尚不清楚。我们建立了一个梗阻性尿路病模型在胎羊广告审查梗阻肾的病理，以确定最佳的减压时机obstruction.Methods：梗阻性尿路病在胎羊在妊娠60天结扎尿道和脐尿管。他们在20-31天后通过剖腹产分娩。结果：对34只60日龄羔羊进行了手术。在25个胎儿中观察到发育不良变化，24个胎儿在肾源区有囊肿。多囊性发育不良肾（MCDK）的囊性成分主要位于近端小管。结论：子宫内尿路梗阻导致功能性肾单位数量减少，并在生肾区和深层皮质产生囊肿。这些囊肿和扩张的近端小管抑制了新肾单位的形成。梗阻后20天，有早期发育不良的特点，但肾区仍然存在。我们的结论是，早期分流可以挽救肾功能。

项目成果

Kevin C.Pringle: "Renal dysplasia produced by obstructive uropathy in the fetal lamb"Pathology. 35. 518-821 (2003)

Kevin C.Pringle：“胎儿羔羊梗阻性尿路病引起的肾发育不良”病理学。

Hiroaki Kitagawa, Kevin C.Pringle, Junki Koike, Jane Zukkoro, Yasuji Seki, Shinichirou Fujiwaki, Hideaki Sato, Hideki Nagae, Koonosuke Nakada: "Optimal Treatment of Obstructive Uropathy in the Fetal Lamb"Journal of Pediatric Surgery. 38. 1785-1789 (2003)

Hiroaki Kitakawa、Kevin C.Pringle、Junki Koike、Jane Zukkoro、Yasuji Seki、Shinichirou Fujiwaki、Hideaki Sato、Hideki Nagae、Koonosuke Nakada：“胎儿羔羊梗阻性尿路病的最佳治疗”小儿外科杂志。

Hiroaki Kitagawa, Kevin C Pringle, Junki Koike, Jane Zucollo, Yuriko Sato, Hideaki Sato, Takeshi Aoba, Yasuji Seki, Koonosuke Nakada: "Fetal hydrops in obstructive uropathy resolves after vesicostomy formation. Is this cause and effect?"Pediatric Surgery

Hiroaki Kitakawa、Kevin C Pringle、Junki Koike、Jane Zucollo、Yuriko Sato、Hideaki Sato、Takeshi Aoba、Yasuji Seki、Koonosuke Nakada：“梗阻性尿路病中的胎儿积水在膀胱造口术形成后会消失。这是因果关系吗？”小儿外科

Kevin C.Pringle, Jane Zuccollo, Hiroaki Kitagawa, Junki Koike, Brett Delahunt: "Renal dysplasia produced by obstructive uropathy In the fetal lamb"Pathology. 35. 518-521 (2003)

Kevin C.Pringle、Jane Zuccollo、Hiroaki Kitakawa、Junki Koike、Brett Delahunt：“胎儿羔羊中阻塞性尿路病引起的肾发育不良”病理学。

H.Kitagawa: "Optimal timing of prenatal treatment of obstructive uropathy in the fetal lamh"Journal of Pediatric Surgery. Vol:36, No.12. 1785-1789 (2003)

H.Kitakawa：“胎儿羔羊梗阻性尿病产前治疗的最佳时机”小儿外科杂志。