MEDI7111 Lecture Notes - Lecture 14: Calcification, Kidney Stone Disease, Extracellular Fluid
Congenital & Developmental Abnormalities
Congenital abnormalities of the urinary system can either be hereditary or due to a defect
acquired in utero. ~10% of these malformations are significant and are the leading cause of
ESRF and CKD in children. Hydronephrosis is the most common renal abnormality (~30%
of abnormalities).
Location of the malformation:
Kidney (hypoplasia, dysplasia – may be associated with reflux)
Collection system (Hydronephrosis, megaureter, double ureter)
Bladder (ureterocoele, VUR)
Urethra (posterior urethral valves)
Horse-shoe Kidney
Occurs from fusion of the lower poles of the kidney
Usually associated with double ureter
Predominantly no dysfunction of the kidney (slightly increased risk of infection)
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Cystic Dysplasia
Occurs from abnormality in metanephric differentiation
Ureto-pelvic obstruction results in upper and lower renal tract symptoms
Can be unilateral or bilateral
Clinically presents as a flank mass
Excellent prognosis if unilateral, renal failure is expected if bilateral
Medullary Cystic Kidney Disease
Medullary Sponge Kidney
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Document Summary
Congenital abnormalities of the urinary system can either be hereditary or due to a defect acquired in utero. ~10% of these malformations are significant and are the leading cause of. Hydronephrosis is the most common renal abnormality (~30% of abnormalities). Kidney (hypoplasia, dysplasia may be associated with reflux) Occurs from fusion of the lower poles of the kidney. Predominantly no dysfunction of the kidney (slightly increased risk of infection) Ureto-pelvic obstruction results in upper and lower renal tract symptoms. Excellent prognosis if unilateral, renal failure is expected if bilateral. Msk is a common adult condition that is usually identified incidentally. The pathogenesis is unknown and renal function is not impaired by the condition. Secondary complications include calcification, haematuria, infection and urinary calculi. Nephronothisis often presents in childhood and is a progressive disorder of medullary cysts at the cortico-medullary junction. Primarily the distal tubule is affected with basement membrane destruction, tubular atrophy and interstitial fibrosis leading to renal insufficiency.
