autosomal dominant polycystic kidney disease


autosomal dominant polycystic kidney disease

A common (1:400–1:1000) autosomal dominant condition (OMIM:173910), which causes 6–9% of end-stage renal disease in developed countries. ADPKD first appears in adults with upper quadrant tenderness; extrarenal disease is due to defective extracellular matrix, with hepatic cysts, diverticulosis, berry and abdominal aneurysms, annuloaortic ectasia and valvular regurgitation.
Clinical findings
Acute or subacute onset of azotaemia and hypertension, due to increased activity of the renin-angiotensin-aldosterone (RAA) system, possibly related to the ischaemic pressure induced by the expanding cysts.
Lab
Anaemia, increased ESR, increased WBCs.
 
Diagnosis
Ultrasonography.

autosomal dominant polycystic kidney disease

ADPKD A common–1:400-1:1000 AD condition, which causes 6-9% of ESRD in developed countries Clinical Acute or subacute onset of azotemia and HTN, due to ↑ activity of the RAA system, possibly related to the ischemic pressure induced by the expanding cysts; ADPKD first appears in adults with upper quadrant tenderness; extrarenal disease is due to defective extracellular matrix, with hepatic cysts, diverticulosis, berry and abdominal aneurysms, annuloaortic ectasia, valvular regurgitation, anemia, ↑ ESR, ↑ WBCs Diagnosis Ultrasonography. See Polycystic kidneys.