Advances in the cell biology and genetics of human kidney malformations.
Advances in the cell biology and genetics of human kidney malformations.
复制标题
人类肾脏畸形的细胞生物学和遗传学进展。
DOI：
--
复制
发表时间：
1998
影响因子：
13.6
通讯作者：
P. Winyard
中科院分区：
文献类型：
--
作者：
A. Woolf;P. Winyard
近年来，肾畸形的生物学研究取得了进展。我们的重点是人类疾病，但我们将在相关时使用动物范例。哺乳动物的肾脏来源于两个后肾组成部分（1-5）：输尿管芽，形成集合管和尿路上皮，以及肾间充质，形成肾单位。人类后肾在妊娠5周时出现，肾小球在妊娠9周时首次形成（图1）。肾单位的产生，直到大约34周，与成熟继续出生后。小鼠后肾在受精后11 ~ 12 d发生，生后2 wk继续肾发生.术语“肾畸形”描述了各种异常（4-5）。在“肾发育不全”中，肾脏缺失。在“肾发育不良”中，分支不良的导管终止于囊肿，并被未分化的细胞和化生软骨包围。“再生障碍性”肾是一个微小的发育不良的雏形，而“多囊性发育不良肾”是由囊肿扩张。“囊性发育不良肾”是指具有一些功能性肾单位的发育不良器官。在“肾发育不全”中，器官较小，肾单位比正常少;当肾单位较大时，这种情况称为“少大肾”（6）。下尿路畸形包括肾盏扭曲和发育不全，以及与梗阻或膀胱输尿管反流相关的肾积水和输尿管积水。肾脏畸形的定义可以扩展到显微镜下的异常，如“肾小管发育不全”，其中近端小管异常形成（7）。虽然多囊性疾病可能表现为畸形，但它们是终末上皮分化的疾病（8-10），在本报告中将不作进一步讨论。肾畸形可能是偶发性或家族性的，单独出现或作为多器官综合征的一部分，通常影响中枢神经、心血管和骨骼系统（4，11）;伴随的肺发育不全通常继发于羊水过少。肾畸形发生率的确定存在偏倚：一些调查排除了新生儿死亡;单侧疾病通常在临床上无症状（12）;很少有组织学检查;
In recent years there have been advances in the biology of renal malformations. Our focus is on human disease, but we will use animal paradigms when relevant. The mammalian kidney derives from two metanephric components (1-5): ureteric bud, which forms collecting ducts and uroepithelium, and renal mesenchyme, which forms nephrons. The human metanephros appears at 5 wk gestation, and glomeruli first form by 9 wk (Figure 1). Nephrons are generated until approximately 34 wk, with maturation continuing postnatally. Murine metanephroi arise 1 1 to I 2 d after fertilization, and nephrogenesis continues 2 wk postnatally. The term “kidney malformation” describes diverse anomalies (4-5). In “renal agenesis” the kidney is absent. In “renal dysplasia,” poorly branched ducts terminate in cysts and are surrounded by undifferentiated cells and metaplastic cartilage. An “aplastic” kidney is a tiny dysplastic rudiment, and the “multicystic dysplastic kidney” is distended by cysts. “Cystic dysplastic kidney” refers to a dysplastic organ with some functioning nephrons. In “renal hypoplasia” the organ is small with fewer nephrons than normal; when nephrons are large the condition is called “oligomeganephronia” (6). Lower urinary tract malformations include calyceal distortions and hypoplasia, as well as hydronephrosis and hydroureter associated with obstruction or vesicoureteric reflux. The definition of renal malformation can be extended to microscopic abnormalities such as “tubular dysgenesis,” in which proximal tubules form abnormally (7). Although polycystic diseases may present as malformations, they are disorders of terminal epithelial differentiation (8-10) and will not be discussed further in this report. Renal malformations may occur sporadically or be familial, appearing in isolation or as part of a multiorgan syndrome commonly affecting central nervous, cardiovascular, and skeletal systems (4, 1 1 ); accompanying lung hypoplasia is often secondary to oligohydramnios. Ascertainment of the occurrence of renal malformations is subject to bias: Some surveys exclude neonatal deaths; unilateral disease is often clinically silent ( 12); there is rarely access to tissue for histology; in
登录
查看更多内容
体外肾器官发生需要后肾转化生长因子-α。
DOI：
10.1152/ajprenal.1992.262.4.f533
发表时间：
1992
期刊：
The American journal of physiology
影响因子：
--
作者：
Rogers,SA;Ryan,G;Hammerman,MR
通讯作者：
Hammerman,MR
Alpha 3 beta 1 整合素在肾脏和肺器官发生中起着至关重要的作用。
DOI：
10.1242/dev.122.11.3537
发表时间：
1996
期刊：
Development (Cambridge, England)
影响因子：
--
作者：
Kreidberg,JA;Donovan,MJ;Goldstein,SL;Rennke,H;Shepherd,K;Jones,RC;Jaenisch,R
通讯作者：
Jaenisch,R
新生儿输尿管梗阻中的生长因子和细胞凋亡。
DOI：
10.1681/asn.v781098
发表时间：
1996
期刊：
Journal of the American Society of Nephrology : JASN
影响因子：
--
作者：
Chevalier,RL
通讯作者：
Chevalier,RL
影响因子：
4.6
作者：
C. Mendelsohn;D. Lohnes;D. Décimo;T. Lufkin;M. Lemeur;P. Chambon;M. Mark
通讯作者：
C. Mendelsohn;D. Lohnes;D. Décimo;T. Lufkin;M. Lemeur;P. Chambon;M. Mark
DOI：
10.1016/0169-328x(89)90076-4
发表时间：
1989-12-01
期刊：
MOLECULAR BRAIN RESEARCH
影响因子：
--
作者：
SCHWANZELFUKUDA, M;BICK, D;PFAFF, DW
通讯作者：
PFAFF, DW