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动脉粥样硬化伴糖尿病病人,主动脉粥样硬化严重、肾动脉狭窄,肾脏出现结节性肾小球硬化和肾硬化。终末期肾时,可进行肾移植治疗。移植的肾位于盆腔,因为技术上容易实现。通常也无需摘除患病者的肾。本例,病人发生了慢性移植排斥反应,可见移植肾出现局灶性出血、轻度肿胀。

In this case, severe atherosclerosis in a patient with diabetes mellitus led to severe aortic atherosclerosis with renal arterial stenosis as well as nephrosclerosis and nodular glomerulosclerosis of the kidneys. The end stage renal disease was treated with renal transplantation. The transplant kidney is placed in the pelvis because this is technically easier and there is usually no point in trying to remove the native kidneys. In this case, the patient developed chronic rejection and that is why focal hemorrhages are seen and the kidney is slightly swollen.

正常足月儿肾脏,可见胚胎期肾小叶;光滑的皮质表面有脂肪组织。

These are normal term infant kidneys. Note the fetal lobulations and the smooth cortical surfaces with some attached adipose tissue.

这是妊娠中期,估计为25周胎儿肾。切面正常,可见肾盂、肾盏,皮质、髓质分界清楚。

These fetal kidneys (from a gestation estimated at 25 weeks in the second trimester) demonstrate a normal cut surface. Note the pelvis and calyces. Note the well-defined corticomedullary junctions.

婴儿因羊水过少导致的肺发育不良在孕23周早产后死亡。羊水过少是由多囊肾引起胎儿排尿显著减少造成的。双侧肾脏增大,几乎占满了肝脏以下的腹腔。在本例,肉眼和组织学表现均符合隐性遗传性多囊肾。

This child died soon after premature birth at 23 weeks gestation from pulmonary hypoplasia as a result of oligohydramnios. The oligohydramnios resulted from markedly diminished fetal urine output as a consequence of polycystic kidney disease. Note the bilaterally enlarged kidneys that nearly fill the abdomen below the liver. The histologic appearance in this case, coupled with the gross appearance., was consistent with recessive polycystic kidney disease (RPKD).

隐性遗传性多囊肾的切面。囊肿相当小,但广泛分布于实质。因此,本病通常表现为对称性,两侧肾脏明显增大。因为它是常染色体隐性遗传的,所以它在家族中的发病率是25%。受累婴儿大多存活不长。

Here is a cut section of a kidney with recessive polycystic kidney disease (RPKD). Note that the cysts are fairly small but uniformly distributed throughout the parenchyma so that the disease is usually symmetrical in appearance, with both kidneys markedly enlarged. The recurrence risk for this disease is, of course, 25% because of the autosomal recessive inheritance pattern. Affected babies usually do not survive long.

隐性遗传性多囊肾镜下见囊肿布满了实质,很难找到肾小球。右侧许多囊肿变长,从肾脏的中间向外周放射状排列,呈轮辐状。

Here is the microscopic appearance of recessive polycystic kidney disease (RPKD). Note that the cysts fill most of the parenchyma, and it is hard to find glomeruli. Many of the cysts are elongated and radially arranged from the center of the kidney on the right, much like spokes on a wagon wheel.

这不是肾脏,隐性遗传性多囊肾还伴有先天性肝纤维化。图示,门管区扩张,周围的胆管呈放射状排列。肝实质内的许多色暗的成串的细胞是髓质外的岛状肝细胞增生。这是胎儿肝脏的典型表现。

You're right, this isn't kidney, but remember that recessive polycystic kidney disease also manifests with congenital hepatic fibrosis, as seen here in which a portal area is expanded with increased bile ducts radially arranged around the perimeter. The many dark clusters of cells in the hepatic parenchyma are islands of extramedullary hematopoiesis typical for fetal liver.

多囊性发育不良肾,须与隐性遗传性多囊肾区分,尽管它比隐性遗传性多囊肾常见,但本病仅散在发病,不具有明确的遗传性。囊肿较大具大小不等,通常为单侧。如果是双侧,常是不对称的,可能也会象隐性遗传性多囊肾那样,发生羊水过少及其它并发症。

This is a multicystic dysplastic kidney. This condition must be distinguished from RPKD because it occurs only sporadically and not with a defined inheritance pattern, though it is more common than RPKD. The cysts are larger and variably sized. Often, it is unilateral. If bilateral, it is often asymmetric. If bilateral, oligohydramnios and its complications can ensue, just as with RPKD.

多囊性发育不良肾也称为多囊肾发育不良,切开发现,大小不等的囊肿取代了肾实质。本例,种病变是单侧的,在婴儿期发现后当作肿块手术切除了。既使只有一个肾,肾脏的代偿能力也足以满足需要。

A multicystic dysplastic kidney (also known as cystic renal dysplasia) has been sectioned to reveal the variably sized cysts that replace the renal parenchyma. In this case, the disease was unilateral and presented in infancy as a mass lesion which was removed surgically. Even with one kidney, there is enough renal reserve capacity to live a normal life.

多囊性发育不良肾(多囊肾发育不良)的显微镜下特点:囊肿较大,内层为扁平立方上皮,受累实质纤维化,内有岛状的蓝色软骨和少量的小球。

The microscopic appearance of multicystic dysplastic kidney (cystic renal dysplasia, or Potter's type II) is characterized by large cysts lined by flattened cuboidal epithelium and an intervening parenchyma that is fibrotic with islands of bluish cartilage and rare glomeruli.


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