Interpretation of renal volume in autosomal dominant polycystic kidney disease and relevant clinical implications.
Summary of "Interpretation of renal volume in autosomal dominant polycystic kidney disease and relevant clinical implications."
Autosomal dominant polycystic kidney disease (ADPKD) is the most common life-threatening hereditary disease of the kidney. It presents with progressive enlargement of the kidneys with numerous cysts that distort the parenchyma and result in progressive decline in kidney function. Autosomal dominant polycystic kidney disease is genetically modified with the responsible genes localized to separate loci on chromosome 16 (PKD1 gene), accounting for the majority of ADPKD cases, and chromosome 4 (PKD2 gene), accounting for the remainder. This review discusses the current understanding of the pathogenesis of ADPKD, focusing on renal volume and its pivotal role on the manifestations of the disease. Specifically, activation of the renin-angiotensin-aldosterone system, hypertension, left ventricular hypertrophy, kidney function deterioration, pain, and hematuria are examined as consequences of renal volume increase. Recent developments on diagnostic modalities and criteria of the ADPKD are also discussed.
Affiliation
Department of Medicine, University of Colorado Denver, Aurora, Colorado, USA. robert.schrier@ucdenver.edu.
Journal Details
This article was published in the following journal.
Name: Iranian journal of kidney diseases
ISSN: 1735-8582
Pages: 1-8
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21189426
- DOI: http://dx.doi.org/
Medical and Biotech [MESH] Definitions
Polycystic Kidney, Autosomal Dominant
Kidney disorders with autosomal dominant inheritance and characterized by multiple CYSTS in both KIDNEYS with progressive deterioration of renal function.
Polycystic Kidney Diseases
Hereditary diseases that are characterized by the progressive expansion of a large number of tightly packed CYSTS within the KIDNEYS. They include diseases with autosomal dominant and autosomal recessive inheritance.
Renal Insufficiency, Chronic
Conditions in which the KIDNEYS perform below the normal level for more than three months. Chronic kidney insufficiency is classified by five stages according to the decline in GLOMERULAR FILTRATION RATE and the degree of kidney damage (as measured by the level of PROTEINURIA). The most severe form is the end-stage renal disease (CHRONIC KIDNEY FAILURE). (Kidney Foundation: Kidney Disease Outcome Quality Initiative, 2002)
Polycystic Kidney, Autosomal Recessive
A genetic disorder with autosomal recessive inheritance, characterized by multiple CYSTS in both KIDNEYS and associated LIVER lesions. Serious manifestations are usually present at BIRTH with high PERINATAL MORTALITY.
Hepatorenal Syndrome
Functional KIDNEY FAILURE in patients with liver disease, usually LIVER CIRRHOSIS or portal hypertension (HYPERTENSION, PORTAL), and in the absence of intrinsic renal disease or kidney abnormality. It is characterized by intense renal vasculature constriction, reduced renal blood flow, OLIGURIA, and sodium retention.
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