Congenital Renal Disease

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Congenital renal disease is a common clinical problem, often found in association 
with other congenital abnormalities. 
The main developmental abnormalities of the kidney can be classified as: 

agenesis, failure of differentiation (renal dysplasia), abnormal anatomic development, 
abnormalities of renal tubular transport, and developmental abnormalities of 
structural elements.
Bilateral renal agenesis occurs as part of Potter's syndrome.
Affected infants have abnormal facies and, frequently, abnomalities of lower 
urinary tract, lungs and nervous system.
Characteristically, because the kidneys are not present to contribute to 
amniotic fluid, there is oligohydramnios in pregnancy.
During development, there may be failure of differentiation of metanephric tissues, 
leadingto renal dysplasia.
This may affect a whole kidney or just one segment, and may be unilateral or bilateral.
The affected areas are replaced by solid and cystic masses in which cartilage 
is usually prominent.
Renal development may be anatomically abnormal, leading to horseshoe kidney; 
the two kidneys are fused across the midline and pelvic kidney, and the kidney is 
sited low in the pelvis.
Several congenital metabolic defects affect the kidney.
These are usually defects in transport of amino acids across tubular epithelium, 
such that they are excreted in the urine.
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