08-11-2017, 10:12 AM
Autosomal Dominant polycystic kidney disease. Multisystemic and progressive disorder.
Pathophysiology is: DEFECT IN EPITHELIAL CELL DIFFERENTIATION
S/S around 40s, Hypertension ( renin production), Renal Failure ( BUN and creatinine).
S/S Anemia (Secondary to a Failure of renal eryhtropietin production).
Associated with Hepatic Cysts, Berry Aneuryms in the circle of Willis (Subarachnoid Hemorrhage).
Hypertension cases in this Pt will best Tx with ACE inhibitors.
MAJOR EXTERNAL COMPLICATIONS OF ADULT POLYCYSTIC KIDNEY DISEASE (ADPKD)
1) Cardiac Valve disease (seen 25 to 30 %: most often mitral valve prolapse and aortic regurgitation)
2) Hepatic Cysts (seen in 10% of patients).
3) Cerebral Aneurysms (seen in 4 to 10% of patients).
4) Colonic Diverticula and Abdominal wall and inguinal hernia.
Pathophysiology is: DEFECT IN EPITHELIAL CELL DIFFERENTIATION
S/S around 40s, Hypertension ( renin production), Renal Failure ( BUN and creatinine).
S/S Anemia (Secondary to a Failure of renal eryhtropietin production).
Associated with Hepatic Cysts, Berry Aneuryms in the circle of Willis (Subarachnoid Hemorrhage).
Hypertension cases in this Pt will best Tx with ACE inhibitors.
MAJOR EXTERNAL COMPLICATIONS OF ADULT POLYCYSTIC KIDNEY DISEASE (ADPKD)
1) Cardiac Valve disease (seen 25 to 30 %: most often mitral valve prolapse and aortic regurgitation)
2) Hepatic Cysts (seen in 10% of patients).
3) Cerebral Aneurysms (seen in 4 to 10% of patients).
4) Colonic Diverticula and Abdominal wall and inguinal hernia.