作者：Michael McGlue 13 年以前
359
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.
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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
