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Cited by 13 publications
References 16 publications
Two brothers had evidence of Bartter's syndrome. Rapid intravenous infusions of saline reverted angiotensin insensitivity and elevated plasma renin activity to normal levels, suggesting that secondary stimulation of the renin-angiotensin-aldosterone system by a decreased extracellular volume (ECFV) may be a major defect in these patients. The data indicate that chronic ECFV depletion exists as a consequence of impaired distal sodium reabsorption. However, inexorable sodium loss does not occur, presumably due to enhanced proximal sodium reabsorption. The patients also exhibited renal wasting of potassium, in part independent of the effects of aldosterone. Bartter's syndrome appears to be the result of renal tubular defects in the handling of sodium and potassium. Evidence presented suggests this defect lies beyond the proximal part of the nephron.Bartter' s syndrome1 encompasses a group of patients with a unique form of hyperaldosteronism. These patients, many of whom have been seen as children, frequently present with growth retardation, polyuria, and weakness, and have been de¬ scribed in a recent review.2 They have been observed to be normotensive and without edema, hypokalemic, in¬ sensitive to the pressor effect of in¬ fused angiotensin (angiotensin insensitivity) and to have increased plasma renin activity (PRA), in¬ creased aldosterone production, and hyperplasia of the juxtaglomerular apparatus of the kidney. Unlike pa¬ tients with primary hyperaldosteronism who are hypertensive, these patients have normal blood pressure, and in several instances postural hypotension.3 4 A further contrast to primary hyperaldosteronism is the presence of high PRA and angioten¬ sin insensitivity.5 Their normal blood pressure also serves to distinguish Bartter's syndrome patients from those patients with renal artery ste¬ nosis or malignant hypertension who otherwise have a similar increase of PRA and angiotensin insensitivity.The metabolic abnormalities of Bart¬ ter's syndrome are similar to those of the familial hypomagnesemic disease described by Gitelman, et al." Bart¬ ter's syndrome patients very closely resemble, by clinical and laboratory examination, patients with secondary hyperaldosteronism from blood loss, salt deprivation, diuretics, or gas¬ trointestinal fluid loss, and, except for the absence of edema, those patients with secondary hyperaldosteronism associated with hypoalbuminemic states or congestive heart failure. The degree of hypokalemia is generally more severe in Bartter's syndrome than in the usual form of secondary hyperaldosteronism. Despite these similarities, attempts to identify a salt-losing defect to produce volume contraction and secondary aldosteronism or attempts to correct the ab¬ normalities of the renin-angiotensinaldosterone system by restoring a
Two brothers had evidence of Bartter's syndrome. Rapid intravenous infusions of saline reverted angiotensin insensitivity and elevated plasma renin activity to normal levels, suggesting that secondary stimulation of the renin-angiotensin-aldosterone system by a decreased extracellular volume (ECFV) may be a major defect in these patients. The data indicate that chronic ECFV depletion exists as a consequence of impaired distal sodium reabsorption. However, inexorable sodium loss does not occur, presumably due to enhanced proximal sodium reabsorption. The patients also exhibited renal wasting of potassium, in part independent of the effects of aldosterone. Bartter's syndrome appears to be the result of renal tubular defects in the handling of sodium and potassium. Evidence presented suggests this defect lies beyond the proximal part of the nephron.Bartter' s syndrome1 encompasses a group of patients with a unique form of hyperaldosteronism. These patients, many of whom have been seen as children, frequently present with growth retardation, polyuria, and weakness, and have been de¬ scribed in a recent review.2 They have been observed to be normotensive and without edema, hypokalemic, in¬ sensitive to the pressor effect of in¬ fused angiotensin (angiotensin insensitivity) and to have increased plasma renin activity (PRA), in¬ creased aldosterone production, and hyperplasia of the juxtaglomerular apparatus of the kidney. Unlike pa¬ tients with primary hyperaldosteronism who are hypertensive, these patients have normal blood pressure, and in several instances postural hypotension.3 4 A further contrast to primary hyperaldosteronism is the presence of high PRA and angioten¬ sin insensitivity.5 Their normal blood pressure also serves to distinguish Bartter's syndrome patients from those patients with renal artery ste¬ nosis or malignant hypertension who otherwise have a similar increase of PRA and angiotensin insensitivity.The metabolic abnormalities of Bart¬ ter's syndrome are similar to those of the familial hypomagnesemic disease described by Gitelman, et al." Bart¬ ter's syndrome patients very closely resemble, by clinical and laboratory examination, patients with secondary hyperaldosteronism from blood loss, salt deprivation, diuretics, or gas¬ trointestinal fluid loss, and, except for the absence of edema, those patients with secondary hyperaldosteronism associated with hypoalbuminemic states or congestive heart failure. The degree of hypokalemia is generally more severe in Bartter's syndrome than in the usual form of secondary hyperaldosteronism. Despite these similarities, attempts to identify a salt-losing defect to produce volume contraction and secondary aldosteronism or attempts to correct the ab¬ normalities of the renin-angiotensinaldosterone system by restoring a
Two girls with hypokalemic and hypochloremic metabolic alkalosis and failure to thrive were found to have Bartter syndrome at ages 9 and 6 months. Both had normal blood pressures despite substantial elevation of plasma renin activity and evidence of secondary hyperaldosteronism. A similarity in facial features, including prominent forhead, a large head, triangular facies with drooping mouth, and large eyes and pinnae, was noted in these two infants and in published pictures of other infants with the syndrome. Although the normotension associated with substanital elevation of plasma renin activity and hyperaldosteronism in Bartter syndrome has been considered the effect of hypovolemia, a normal or slightly elevated plasma volume was found in these infants, suggesting that in certain cases an alternate mechanism for the depressed response to renin may be present.
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