Talk:Renal System - Abnormalities: Difference between revisions

Revision as of 10:41, 3 June 2010

thought to be a deficiency in caudal mesoderm which in turn leads to the malformation of the urorectal septum and other structures in the pelvic region.
Recent research has also identified the potential presence of a persistent urachus prior to septation of the cloaca (common urogenital sinus).

associated with renal agenesis.

Bladder_Exstrophy

developmental abnormality associated with bladder development.
origins appear to occur not just by abnormal bladder development, but by a congenital malformation of the ventral wall of abdomen (between umbilicus and pubic symphysis).
There may also be other anomolies associated with failure of closure of abdominal wall and bladder (epispadias, pubic bone anomolies).

Multicystic kidney

diffuse cystic malformation of both kidneys
cystic malformations of liver and lung often associated, Often familial disposition
Two types
- Infantile (inconsistent with prolonged survival)
- Adult (less severe and allows survival)
Autosomal dominant PKD disease - recently identified at mutations in 2 different human genes encoding membrane proteins (possibly channels)

(nephroblastoma) Named after Max Wilms, a German doctor who wrote first medical articles 1899
most common type of kidney cancer children
WT1 gene - encodes a zinc finger protein
Both constitutional and somatic mutations disrupting the DNA-binding domain of WT1 result in a potentially dominant-negative phenotype
some blastema cells (mass of undifferentiated cells) persist to form a ‘nephrogenic rest’
Most rests become dormant or regress but others proliferate to form hyperplastic rests
any type of rest can then undergo a genetic or epigenetic change to become a neoplastic rest
can proliferate further to produce a benign lesion (adenomatous rest) or a malignant Wilms’ tumour

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 * asymptomatic renal ectopia
-==Abnormalities==
-[[File:Australian_abnormalities_pie_urogen.png|left]]
-===Horseshoe Kidney===
-[[File:Horseshoe kidney.jpg|thumb|Horseshoe kidney]]
-*  fusion of the lower poles of the kidney.
-* During migration from the sacral region the two metanephric blastemas can come into contact, mainly at the lower pole.
-* The ureters pass in front of the zone of fusion of the kidneys.
-* The kidneys and ureters usually function adequately but there is an increased incidence of upper urinary tract obstruction or infection.
-* Some horseshoe variations have been described as having associated ureter abnormalities including duplications.
 ===Urorectal Septum Malformation===