autosomal dominant polycystic kidney disease


Also found in: Dictionary, Thesaurus, Acronyms.

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.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.

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.
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
References in periodicals archive ?
Cadnapaphornchai et al., "Predictors of autosomal dominant polycystic kidney disease progression," Journal of the American Society of Nephrology, vol.
Grantham, "Perinephric hemorrhage in autosomal dominant polycystic kidney disease: Ct and MR findings," Journal of Computer Assisted Tomography, vol.
Positron-emission computed tomography in cyst infection diagnosis in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol 2011; 6: 1644-1650, doi: 10.2215/CJN.06900810.
Why kidneys fail in autosomal dominant polycystic kidney disease [internet].
Discuss how the FNP PCP should best treat and monitor a patient with autosomal dominant polycystic kidney disease.
[4.] Chapman AB, Johnson AM, Gabow PA: Intracranial aneurysms in patients with autosomal dominant polycystic kidney disease: How to diagnose and who to screen.
Autosomal dominant polycystic kidney disease is among the most prevalent dominant human disorders, affecting between 1 and 1000 and 1 and 3000 individuals worldwide.
We announced in May 2019 that the first patient was enrolled in a registrational Phase 3 trial called FALCON, an international, multi-center, randomized, double-blind, placebo-controlled trial studying the safety and efficacy of bardoxolone in approximately 300 patients with autosomal dominant polycystic kidney disease. The FDA has provided guidance that an improvement in retained eGFR versus placebo at one year may support accelerated approval under subpart H, and that data demonstrating an improvement versus placebo in retained eGFR after two years may support full approval.
Overall, the PHOENIX trial studied 103 patients who had one of four rare forms of CKD, including autosomal dominant polycystic kidney disease, IgA nephropathy, type 1 diabetic CKD, and FSGS.
Autosomal Dominant Polycystic Kidney Disease (ADPKD) has an incidence of 1 in 800 live births.
United States-based Kadmon has commenced the Phase IIa portion of a Phase Ib/IIa trial of KD020, the company's orally bioavailable small molecule kinase inhibitor of Src, HER2, EGFR and VEGFR2/KDR, in autosomal dominant polycystic kidney disease, it was reported yesterday.
CT of liver cysts in patients with autosomal dominant polycystic kidney disease. Acta Radiol.

Full browser ?