The renin-angiotensin-aldosterone system and autosomal dominant polycystic kidney disease
Article Abstract:
Polycystic kidney disease is characterized by the development of many cysts, sac-like structures that contain fluid or solid material, in the kidney. Hypertension (high blood pressure) is found in 50 to 75 percent of patients with this disorder and has been putatively linked to inadequate blood flow (ischemia) to the kidney caused by compression of the renal vessels by cysts. This study investigates the possibility that hypertension in polycystic kidney disease is produced by pathologic alterations of the renin-angiotensin-aldosterone system. The active kidney enzyme renin is produced from an inactive form, prorenin, that transforms the liver protein angiotensinogen to angiotensin I, which is then converted by angiotensin-converting enzyme to angiotensin II. This factor, angiotensin II, constricts blood vessels and activates the production and release of the adrenal hormone aldosterone, which regulates sodium, potassium, and chloride metabolism. Through a prospective study of polycystic kidney disease, patients' blood and urine levels of these hormones and other factors have been measured and related to blood pressure readings. Various drugs that block specific metabolic steps in this hormonal system were used to identify elements of the system that affected hypertension. The renin-angiotensin-aldosterone system is found to be significantly stimulated in hypertensive polycystic kidney disease patients. Increased renin release may be related to kidney ischemia. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1990
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Intracranial aneurysms in autosomal dominant polycystic kidney disease
Article Abstract:
Asymptomatic intracranial aneurysms (bulges in arteries in the head) may occur often in patients with polycystic kidney disease. Polycystic kidney disease is a genetic disorder that affects different organs including the kidneys, heart and gastrointestinal tract. Among 88 patients with polycystic kidney disease who underwent angiography and/or a computed tomographic (CT) scan, four (4%) had an intracranial aneurysm. The occurrence of intracranial aneurysms among members of the general population has been reported at 1%. Among the four patients with intracranial aneurysms, three had more than one aneurysm. The average age of these patients was 42 years old, and two had a family history of ruptured aneurysms. Twenty-five percent of the patients with polycystic kidney disease who underwent angiography suffered complications, compared with 10% of 220 hospital patients without polycystic kidney disease. Individuals with a family history of intracranial aneurysms should be screened every five to 10 years.
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1992
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Autosomal dominant polycystic kidney disease
Article Abstract:
Autosomal dominant polycystic kidney disease is characterized by enlarged kidneys and the development of numerous cysts on the kidneys. The term autosomal dominant refers to the pattern of genetic transmission of the disease. Other manifestations of the disease include the development of cysts in the spleen, pancreas, liver, ovaries, gastrointestinal tract and central nervous system. Patients who are less than 16 years old rarely develop cysts outside the kidneys. However, the chance of cysts occurring in areas other than the kidneys increases with age. Diagnosis is usually made using ultrasonography, computed tomography or magnetic resonance imaging. Diagnosis usually requires that at least three cysts are present on the kidneys and that there is a family history of the disease. Many patients have a mild form of the disease, but others can have severe pain and many complications, including kidney failure.
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1993
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