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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of Liddle's syndrome were found in a brother and sister. Both showed typical hypokalemic hypertension without hyperaldosteronism. These cases showed similar responses in various pharmacological tests and their symptoms of hypokalemic and hypertension were relieved by triamterene. And in a family survey, the father appeared to be affected. This seems to be the first report on this syndrome in Japan.
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PMID:Two cases of pseudoaldosteronism (Liddle's syndrome) in siblings. 114 90

A 79-year old man with hypertension, hypokalemic metabolic alkallosis, hyporeninemia and hypoaldosteronemia was studied. Blood pressure fell and serum potassium returned to normal after sodium restriction and the administration of triamterene. Serum DHEA, DOC, corticosterone, 18-OH-corticosterone, 11-deoxy-cortisol, cortisol were within normal range. Adrenal CT scanning did not reveal an adrenal tumor. The excretions of urinary kallikrein and prostaglandin E2 were within the normal range. Although an atrophic juxtaglomerular apparatus and arteriosclerotic change were observed by renal biopsy, there was no evidence of hypokalemic nephropathy. From these results, it is suggested that this patient has a defect in handling sodium and potassium in the distal tubules. This is similar to that observed in Liddle's syndrome.
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PMID:[A 79-year old man with hypertension, hypokalemia, hyporeninemia and hypoaldosteronemia similar to Liddle's syndrome]. 159 40

A 22-yr-old female suffering from hypertension, hypokalemic alkalosis and suppressed plasma renin activity was studied. The plasma aldosterone concentration (PAC) ranged between subnormal and normal levels while the other adrenal mineralocorticoids were normal. Examinations through computed tomography and ultrasonography showed no abnormal findings. For differential diagnosis, dexamethasone, spironolactone and triamterene were administered. Triamterene alone corrected the abnormalities in this case, and the therapeutic effect was further enhanced by sodium restriction. Therefore, the present case is strongly suggested to be one of Liddle's syndrome, which is characterized by a primary defect in renal tubular sodium handling and can be corrected with triamterene. However, the patient in our study is different from the first reported case in which aldosterone secretion was estimated to be low. Analysis of the changes in PAC has shown that PAC is parallel with the level of plasma progesterone in accordance with the rhythm of the menstrual cycle and, in the follicular phase, PAC is rather low. It is concluded that the patient was suffering from Liddle's syndrome, and it is assumed that PAC is not always subnormal and, as same as in normal females, PAC may change in accordance with the rhythm of the menstrual cycle in a female case of Liddle's syndrome.
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PMID:Plasma aldosterone level in a female case of pseudohyperaldosteronism (Liddle's syndrome). 265 10

Liddle's syndrome was diagnosed in a 72-year-old man who presented clinically with hypertension and muscle weakness. This disorder has been characterized by hyporeninemic hypoaldosteronism, hypertension, hypokalemia and enhanced erythrocyte sodium influx. Administration of spironolactone failed to correct the hypertension and electrolyte abnormality, which subsequently improved with triamterene therapy and a low salt diet. However, suppression of the renin-angiotensin-aldosterone system remained unchanged after this treatment. In addition, an atrophic juxtaglomerular apparatus and hypertensive lesions in the arterioles were confirmed by kidney biopsy after triamterene therapy. Therefore, a process of intrinsic hyperactive distal sodium reabsorption, probably affected by aldosterone-independent sodium transport into erythrocytes, appears to be important in the pathogenesis of this syndrome. Triamterene therapy, which usually is performed in patients with this disease, might not be the ultimate therapy in the future even if electrolyte abnormalities were to be improved temporarily.
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PMID:Liddle's syndrome, an uncommon form of hyporeninemic hypoaldosteronism: functional and histopathological studies. 355 Jan 46

Important advances have been made in understanding the role of the renin-angiotensin-aldosterone system in the pathogenesis and diagnosis of hypertensive disorders. Measurement of plasma renin activity (PRA) and aldosterone is very important in the assessment of secondary hypertension. Hypertensions with increased PRA include renovascular hypertension, some cases of unilateral and bilateral renal parenchymal disease, malignant hypertension, hypertension associated with oral contraceptive agents, and renin-secreting tumors. Hypertension with decreased PRA is observed in four recognized types of primary aldosteronism: adenoma, bilateral hyperplasia, indeterminate aldosteronism, and glucocorticoid-responsive aldosteronism. Other conditions with hypertension and depressed PRA include ACTH and DOC secreting tumors, primary hyperpituitarism, syndromes of 17-hydroxylase and 11-beta-hydroxylase deficiency. Liddle's syndrome, licorice abuse, exogenous administration of mineralocorticoids, and preeclampsia.
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PMID:The renin-angiotensin-aldosterone system in primary and secondary hypertension. 611 55

Liddle's syndrome was diagnosed in a 23-yr-old Chinese girl with hypertension and hypokalemia by the presence of suppressed renin and negligible plasma and urinary aldosterone secretion. Adrenal corticosteroids, including aldosterone, were suppressed by dexamethasone and stimulated by ACTH. While spironolactone was ineffective, triamterene (2,4,7-triamino-6-phenyl-pteridine) treatment corrected the hypertension and hypokalemia and restored PRA to normal provided that sodium intake was not excessive. During long term treatment with triamterene, blood pressure was extremely sensitive to salt intake, increasing promptly with high intake and decreasing with low salt intake. As a result of the chronic hypervolemia and sodium retention consequent upon the patient's persistent high salt intake and increased renal tubular sodium reabsorption, plasma renin and aldosterone remained low. Erythrocyte sodium concentration and membrane permeability were increased. Triamterene with salt restriction was able to lower the intracellular sodium concentration but did not correct the increased sodium permeability. This suggests that there is an abnormality of sodium transport in Liddle's syndrome which affects the erythrocytes as well as the renal tubular cells.
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PMID:The effect of triamterene and sodium intake on renin, aldosterone, and erythrocyte sodium transport in Liddle's syndrome. 626 54

A 24-yr-old woman with hypertension, hypokalemic alkalosis, low plasma renin and hypoaldosteronism was studied. Plasma aldosterone, renin and potassium returned to normal and blood pressure fell after sodium restriction or the administration of triamterene. Thiazide therapy also normalized her blood pressure while dexamethasone, spironolactone and furosemide did not improve her symptoms. Plasma aldosterone levels were low and responded poorly to a short term ACTH injection, but responded well to the maximal adrenal stimulation by ACTH-Z. Plasma levels of cortisol, corticosterone and deoxycorticosterone were within the normal range. Adrenal scintigram with 131I-adosterol and abdominal computed axial tomography did not reveal the presence of a sizeable adrenal tumor. In addition, the urinary kallikrein excretion was low after sodium restriction and showed no response to saline infusion. These findings suggest that the excessive secretion of unusual mineralocorticoids may not exist in this case. From these observations and the results of the therapeutic responses to the diuretic agents, we conclude that the primary cause of the disorder of this patient seems to be a renal defect in the distal tubule in handling sodium and potassium which is similar to that in Liddle's syndrome.
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PMID:Hypertension, hypokalemia and hypoaldosteronism with suppressed renin: a clinical study of a patient with Liddle's syndrome. 627 44

In the past few years, a number of key insights have been made concerning the genetic basis of hypertension and blood pressure regulation. The genes responsible for two Mendelian forms of hypertension, glucocorticoid-remediable aldosteronism and Liddle's syndrome, were identified. In addition, research into the role of the renin-angiotensin system in blood pressure regulation has further implicated the angiotensinogen and angiotensin-converting enzyme loci in hypertension and its complications, such as myocardial infarction. Finally, several new candidate genes for hypertension have been identified through the use of genome scanning and contemporary gene expression assays in model organisms.
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PMID:The genetics of hypertension. 754 32

Sensitivity of blood pressure to dietary salt is a common feature in subjects with hypertension. These features are exemplified by the mendelian disorder, Liddle's syndrome, previously shown to arise from constitutive activation of the renal epithelial sodium channel due to mutation in the beta subunit of this channel. We now demonstrate that this disease can also result from a mutation truncating the carboxy terminus of the gamma subunit of this channel; this truncated subunit also activates channel activity. These findings demonstrate genetic heterogeneity of Liddle's syndrome, indicate independent roles of beta and gamma subunits in the negative regulation of channel activity, and identify a new gene in which mutation causes a salt-sensitive form of human hypertension.
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PMID:Hypertension caused by a truncated epithelial sodium channel gamma subunit: genetic heterogeneity of Liddle syndrome. 755 Mar 15

Genetic approaches are setting the stage for a new understanding of the biological mechanisms underlying blood pressure regulation and hypertension. Recently, substantial progress has been made in elucidating the molecular mechanisms that cause several rare forms of hypertension, including hypertension arising from mutations in the 11 beta-hydroxylase gene, Liddle's syndrome, and glucocorticoid-suppressible aldosteronism. New results have been obtained on the possible role of the angiotensinogen gene, and other candidate genes, such as the angiotensin II receptor Type I gene and the Sa gene, in human hypertension. Investigation of experimental models of hereditary hypertension have also been important in unravelling the genetic complexity of the disease.
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PMID:The genetic basis of hypertension. 760 49


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