Increased chloride reabsorption as an inherited renal tubular defect in familial type II pseudohypoaldosteronism
Article Abstract:
Hyperkalemia, an increase in the amount of potassium ions in the blood, is a common electrolyte imbalance, and may be due to a wide variety of different causes. Among these causes is hypoaldosteronism, which is a reduction in the mineralocorticoid hormone aldosterone, which stimulates the kidney to retain sodium and excrete potassium. Kidney disease may also cause hyperkalemia. In pseudohypoaldosteronism, however, the levels of aldosterone may be normal or elevated, and there is no evidence of kidney disease. In type I pseudohypoaldosteronism, the hyperkalemia results from a defect in the responsiveness of the kidney to aldosterone. A second form of pseudohypoaldosteronism, type II, has been described, and it has been suggested that in this case, the excess of potassium in the blood results from a defect in the transport of chloride ions. Researchers have now reported the cases of three patients, a father and two affected children. In normal individuals, an infusion of sodium ions will result in an increase in potassium in the urine. Sodium ions, however, must always be associated with some counterion, such as bicarbonate, sulfate, or chloride. It is therefore possible to tease apart the physiological effects of sodium ions and chloride ions by comparing, for example, an infusion of sodium bicarbonate with an infusion of sodium chloride. Such a test was performed on the patients in the present study, and it was found that infusion of sodium bicarbonate or sodium sulfate resulted in a normal excretion of potassium, while the infusion of an equivalent amount of sodium chloride did not. The results provide further support to the belief that defective chloride transport may be responsible for type II pseudohypoaldosteronism. Furthermore, the case studies suggest that the defect in chloride transport may be inherited, apparently as an autosomal dominant trait. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1991
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Abdominal aortic aneurysm
Article Abstract:
An aneurysm is an abnormal dilatation, or widening, in a blood vessel wall. It may be due to a weakness in the wall itself or a congenital defect. With hypertension, or high blood pressure, the force of blood increases as it circulates within vessels; when there is an abnormality in a vessel wall (caused by atherosclerosis, infection, trauma or a congenital defect) the vessel is more susceptible to the effects of hypertension. The abdominal aorta, the main artery that carries blood from the heart to the lower extremities, is a common site for aneurysms. Abdominal aortic aneurysms often rupture unpredictably. One half of all patients with an abdominal aortic aneurysm die within the first year of diagnosis; 90 percent die within 5 years; and almost all patients die less than 10 years from the time of diagnosis. The risk of death from a ruptured aneurysm increases with the size of the aneurysm and the severity of hypertension. Surgically replacing the aneurysm with healthy tissue has increased the long-term survival of patients whose aneurysms have not ruptured. Most patients who have had a ruptured abdominal aortic aneurysm report that they were not aware that they had an aneurysm. Most abdominal aortic aneurysms are found and diagnosed incidentally, when X-rays are performed for other reasons. Both ultrasound and computerized tomography imaging studies are considered excellent diagnostic methods for evaluating aneurysms. Diagnosis is made by measuring several levels of the abdominal aorta. The decision for surgery is based on the size of the aneurysm, the degree of hypertension, and the general health status of the patient. Screening persons who have known risk factors for developing aneurysms should be considered and studied further. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1989
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Abdominal aortic aneurysm
Article Abstract:
Abdominal aortic aneurysm is a sac formed by the dilation of the wall of the abdominal aorta to a diameter at least 50% greater than normal. The incidence of abdominal aneurysms appears to have tripled, increasing from 12.2 per 100,000 people in 1951 to 36.2 per 100,000 by 1980. The death rate from ruptured aneurysms is over 90%, but if caught early, reconstructive surgery can significantly save lives and enhance quality of life. Ultrasound testing can detect almost all abdominal aneurysms, and selective screening of patients aged 55 to 80 with high blood pressure and a history of abdominal aortic aneurysm may be useful. Surgery is the only form of treatment and is generally recommended for patients with symptomatic aneurysms or for patients with asymptomatic aneurysms that are greater than 5 centimeters in diameter.
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1993
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