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Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment.

Halvorson CR, Bremmer MS, Jacobs SC - Int J Nephrol Renovasc Dis (2010)

Bottom Line: Both autosomal dominant and recessive polycystic kidney disease are conditions with severe associated morbidity and mortality.Recent advances in the understanding of the genetic and molecular pathogenesis of both ADPKD and ARPKD have resulted in new, targeted therapies designed to disrupt cell signaling pathways responsible for the abnormal cell proliferation, dedifferentiation, apoptosis, and fluid secretion characteristic of the disease.Herein we review the current understanding of the pathophysiology of these conditions, as well as the current treatments derived from our understanding of the mechanisms of these diseases.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, University of Maryland School of Medicine, Baltimore, MD, USA. chalv001@umaryland.edu

No MeSH data available.


Related in: MedlinePlus

Explanted polycystic kidneys reveal the massive expansion resulting from progressive cystic growth.
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f1-ijnrd-3-069: Explanted polycystic kidneys reveal the massive expansion resulting from progressive cystic growth.

Mentions: Polycystic kidney disease (PKD) is an inherited disorder characterized by cystic expansion of the kidneys producing progressive kidney enlargement and renal insufficiency, in addition to various extrarenal manifestations. The disease can be inherited in autosomal dominant and recessive forms. Autosomal dominant polycystic kidney disease (ADPKD) is characterized by slow but progressive enlargement of the kidneys with renal failure occurring by the fifth to sixth decade of life.1 The disease occurs in approximately 1:800 to 1:1,000 people and accounts for 2.5% of all cases of end-stage renal disease.1,2 Clinically, ADPKD presents over the course of decades with hypertension, flank pain, hematuria, and renal cyst infections in adults. Cyst development and growth is gradual, yet despite the massive growth of the kidneys (Figure 1), the glomerular filtration rate (GFR) in these patients is typically conserved until ages 30–40, followed by a rapid, linear decline after this time.2,3 By the age of 70, 50% of patients with ADPKD will require dialysis or kidney transplantation.4


Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment.

Halvorson CR, Bremmer MS, Jacobs SC - Int J Nephrol Renovasc Dis (2010)

Explanted polycystic kidneys reveal the massive expansion resulting from progressive cystic growth.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3108786&req=5

f1-ijnrd-3-069: Explanted polycystic kidneys reveal the massive expansion resulting from progressive cystic growth.
Mentions: Polycystic kidney disease (PKD) is an inherited disorder characterized by cystic expansion of the kidneys producing progressive kidney enlargement and renal insufficiency, in addition to various extrarenal manifestations. The disease can be inherited in autosomal dominant and recessive forms. Autosomal dominant polycystic kidney disease (ADPKD) is characterized by slow but progressive enlargement of the kidneys with renal failure occurring by the fifth to sixth decade of life.1 The disease occurs in approximately 1:800 to 1:1,000 people and accounts for 2.5% of all cases of end-stage renal disease.1,2 Clinically, ADPKD presents over the course of decades with hypertension, flank pain, hematuria, and renal cyst infections in adults. Cyst development and growth is gradual, yet despite the massive growth of the kidneys (Figure 1), the glomerular filtration rate (GFR) in these patients is typically conserved until ages 30–40, followed by a rapid, linear decline after this time.2,3 By the age of 70, 50% of patients with ADPKD will require dialysis or kidney transplantation.4

Bottom Line: Both autosomal dominant and recessive polycystic kidney disease are conditions with severe associated morbidity and mortality.Recent advances in the understanding of the genetic and molecular pathogenesis of both ADPKD and ARPKD have resulted in new, targeted therapies designed to disrupt cell signaling pathways responsible for the abnormal cell proliferation, dedifferentiation, apoptosis, and fluid secretion characteristic of the disease.Herein we review the current understanding of the pathophysiology of these conditions, as well as the current treatments derived from our understanding of the mechanisms of these diseases.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, University of Maryland School of Medicine, Baltimore, MD, USA. chalv001@umaryland.edu

No MeSH data available.


Related in: MedlinePlus