Polycystic kidney disease classification: Difference between revisions
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*[[Autosomal recessive]] polycystic kidney disease | *[[Autosomal recessive]] polycystic kidney disease | ||
[[Autosomal dominant]] polycystic kidney disease (ADPKD) is generally a late-onset disorder characterized by progressive cyst development and bilaterally enlarged kidneys with multiple cysts. Kidney manifestations in this disorder include renal function abnormalities, [[hypertension]], renal [[pain]], and [[renal insufficiency]]. Approximately 50% of patients with ADPKD have [[end stage renal disease]] ([[ESRD]]) by the age of 60. ADPKD is a systemic disease with cysts in other organs such as the [[liver]] (which may lead to [[cirrhosis]]), [[seminal vesicles]], pancreas, and [[arachnoid mater]] and non-cystic abnormalities such as intracranial [[aneurysms]] and [[dolichoectasias]], dilatation of the [[aortic]] root and dissection of the [[thoracic aorta]], [[mitral valve prolapse]], and abdominal wall hernias. | '''[[Autosomal dominant]] polycystic kidney disease''' (ADPKD) is generally a late-onset disorder characterized by progressive cyst development and bilaterally enlarged kidneys with multiple cysts. Kidney manifestations in this disorder include renal function abnormalities, [[hypertension]], renal [[pain]], and [[renal insufficiency]]. Approximately 50% of patients with ADPKD have [[end stage renal disease]] ([[ESRD]]) by the age of 60. ADPKD is a systemic disease with cysts in other organs such as the [[liver]] (which may lead to [[cirrhosis]]), [[seminal vesicles]], pancreas, and [[arachnoid mater]] and non-cystic abnormalities such as intracranial [[aneurysms]] and [[dolichoectasias]], dilatation of the [[aortic]] root and dissection of the [[thoracic aorta]], [[mitral valve prolapse]], and abdominal wall hernias. | ||
[[Autosomal recessive]] polycystic kidney disease (ARPKD) is much rarer than ADPKD and is often fatal in utero or during the first month of life. The signs and symptoms of the condition are usually apparent at birth or in early infancy. | '''[[Autosomal recessive]] polycystic kidney disease''' (ARPKD) is much rarer than ADPKD and is often fatal in utero or during the first month of life. The signs and symptoms of the condition are usually apparent at birth or in early infancy. | ||
==References== | ==References== | ||
Revision as of 13:23, 20 August 2012
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]
Overview
Classification
- Autosomal dominant polycystic kidney disease
- Autosomal recessive polycystic kidney disease
Autosomal dominant polycystic kidney disease (ADPKD) is generally a late-onset disorder characterized by progressive cyst development and bilaterally enlarged kidneys with multiple cysts. Kidney manifestations in this disorder include renal function abnormalities, hypertension, renal pain, and renal insufficiency. Approximately 50% of patients with ADPKD have end stage renal disease (ESRD) by the age of 60. ADPKD is a systemic disease with cysts in other organs such as the liver (which may lead to cirrhosis), seminal vesicles, pancreas, and arachnoid mater and non-cystic abnormalities such as intracranial aneurysms and dolichoectasias, dilatation of the aortic root and dissection of the thoracic aorta, mitral valve prolapse, and abdominal wall hernias.
Autosomal recessive polycystic kidney disease (ARPKD) is much rarer than ADPKD and is often fatal in utero or during the first month of life. The signs and symptoms of the condition are usually apparent at birth or in early infancy.