Kategoriak: All - genetic - hypertension

arabera Michael McGlue 13 years ago

345

Congenital kidney anomolies

Various kidney disorders, both congenital and acquired, can lead to a range of complications including the development of cysts and renal cell carcinoma. Dialysis-associated cysts, often filled with clear fluid, can increase the risk of carcinoma in long-term dialysis patients.

Congenital kidney anomolies

Small Shrunken Kidney

Large size Kidney

Congenital/Aquired kidney anomolies

Aquired

Acquired (dialysis-associated) cysts
Associated with development of renal cell carcinoma – occurring in 7% of patients dialyzed for 10 years or more
Cortical and medullary location Numerous; filled with clear fluid

Renal Medullary Diseases

Familial Juvenile Nephronopthisis
Corticomedullary Junction cysts
Autosomal recessive with 3 genes (NPH1,NPH2 &NPH3)
Adult onset Medullary
Most common genetic cause of end-stage kidney disease in children and young adults
Cysts are at the corticomedullary Junction
Present with Salt wasting, polyuria

Polycystic Kidney

Juvenile Polycyctic Kidney ARPKD
Perinatal lethal is the most common Associated with portal hypertension
Clinical subtype depends upon % of collecting duct involment.
Defect in the gene that codes for Fibrocystin Results in Dilated collecting ducts (spongelike)
Autosomal Recessive (PKHD 2) Chromosome 6
Adult Polycystic Kidney
Cysts are found in Corticomedullary area
Bilateral Presentation in 4,5,6th decade
ADult or (Autosomal Dominant)
PKD 1 gene (Polycystin 1)
Death is common (10%) from a berry aneurysism
Hepatic cysts are most common co-manifestations along with Hypertension and miral valve prolapse and flank pain & hematuria