Studies on the pathogenesis of Bartter's syndrome

Baehler, Richard W.; Work, Jack; Kotchen, Theodore A.; McMorrow, Greg; Guthrie, Gordon P.

doi:10.1016/s0002-9343(80)80022-2

Cited by 61 publications

(24 citation statements)

References 26 publications

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“…Little K ϩ is excreted by the gastrointestinal tract normally; therefore, hypokalemia in magnesium deficiency is likely associated with enhanced renal K ϩ excretion. To support this idea, Baehler et al 5 showed that administration of magnesium decreases urinary K ϩ excretion and increases serum K ϩ levels in a patient with Bartter disease with combined hypomagnesemia and hypokalemia. Similarly, magnesium replacement alone (without K ϩ ) increases serum K ϩ levels in individuals who have hypokalemia and hypomagnesemia and receive thiazide treatment.…”

mentioning

confidence: 99%

Mechanism of Hypokalemia in Magnesium Deficiency

Huang¹,

Kuo²

2007

Journal of the American Society of Nephrology

323

216

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confidence: 99%

Mechanism of Hypokalemia in Magnesium Deficiency

Huang¹,

Kuo²

2007

Journal of the American Society of Nephrology

323

216

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“…Prior to molecular studies, investigators suggested a variety of renal transport disorders [17], including defects in Cl transport, Na transport, or both in proximal tubule [18], thick ascending limb of the loop of Henle [19][20][21], or the cortical distal nephron [22][23][24][25]. Linkage of GS to the locus encoding the thiazide-sensitive NaCl cotransporter was established, and a number of mutations consistent with loss of function were identified in these patients [5].…”

Section: Discussionmentioning

confidence: 99%

Studies on the Pathogenesis of Hypokalemia in Gitelman’s Syndrome: Role of Bicarbonaturia and Hypomagnesemia

et al. 1998

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Objective: Hypokalemia and renal potassium (K) wasting are hallmarks of the group of disorders called Bartter’s syndrome. The presence of hypomagnesemia and a low rate of excretion of calcium are currently used to characterize a subgroup of these patients as having Gitelman’s syndrome (GS) in which the molecular lesion is a defect in the thiazide-sensitive NaCl cotransporter in the distal convoluted tubule. This study was undertaken to examine whether bicarbonaturia or hypomagnesemia exacerbates the kaliuresis in patients with GS. Methods: Six patients with most of the diagnostic features of GS were examined. To examine the role of bicarbonaturia, the transtubular K concentration gradient (TTKG) was assessed before and after an oral load of NH₄Cl which caused the urine pH to be <6. To evaluate the role of hypomagnesemia, the TTKG was examined after an infusion of enough magnesium (Mg) to achieve normal levels of Mg in plasma for close to 24 h. Results: The TTKG remained very high even when the pH of the urine was <6.0. An infusion of Mg caused the TTKG to approach expected values for hypokalemia in 4 of 6 patients. The infusion of Mg was extended in 1 patient who had a sustained high TTKG for 24 h; the TTKG remained elevated for 96 h despite normal plasma Mg levels. Conclusions: Bicarbonaturia does not play a critical role in maintaining the very high TTKG in these patients. The K wasting in 4 of 6 of these patients could largely be attributed to hypomagnesemia and/or Mg depletion. The plasma aldosterone level tended to be higher in patients who did not respond to the infusion of Mg. Therefore, these patients may not represent a homogeneous group with regard to the pathophysiology of their renal K wasting.

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“…Since magnesium divalent ion is predominantly reabsorbed in the thick ascending limb of the loop of Henle by active chloride reabsorption as the major driving force, namely the site where most of distal chloride reabsorption takes place, impairmentof renal magnesium conservation might also coexist in the syndrome (7,8,10). Baehler et al (4) found that infusions of magnesium chloride (875 mg magnesium/day) restored the plasma magnesium level to normal, and corrected a defect in renal potassium wasting, remarkably improved plasma potassium, but failed to normalize PRAand PACin a sporadic case ofBartter's syndrome with hypomagnesemia. However, in familial Bartter's syndrome such studies have yet to be dpne.…”

Section: Discussionmentioning

confidence: 99%

“…However, in familial Bartter's syndrome such studies have yet to be dpne. Moreover, since they used a chloride compoundto supplement magnesium,the changes in urinary chloride excretion were not assessed (4). A large amountof chloride ion might also affect the reabsorption of sodium and potassium in the renal tubule.…”

Section: Discussionmentioning

confidence: 99%

“…A defect in chloride and sodium reabsorption in the thick ascending limb of Henle's loop provides a reasonable explanation for the serial events that characterize this syndrome (2,3). Although hypomagnesemia has also been noted in some of the patients with this syndrome, little is known about its pathophysiological significance (4). Experimental magnesiumdepletion, on the other hand, causes hypopotassemia which is due to an increased renal loss of potassium (5).…”

Section: Introductionmentioning

confidence: 99%

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Pathophysiological Role of Magnesium in Familial Bartter's Syndrome.

et al. 1994

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Westudied three siblings with Bartter's syndrome associated with hypomagnesemia;two of them showing marked hypomagnesemiaand the other mild hypomagnesemia.Urinary potassium, sodium and chloride excretions were determined and distal fractional chloride reabsorption and free water clearance on water loading test were comparedbefore and after magnesiumsupplementation. Baseline urinary potassium and magnesiumexcretions were elevated in spite of the decreased plasma levels, whereas distal fractional chloride reabsorption and free water clearance were depressed in all patients. Magnesiumrepletion resulted in significant decrease in urinary potassium, sodium and chloride and subsequent increase in plasma potassium in all patients. However,neither distal fractional chloride reabsorption nor free water clearance was affected. Hypomagnesemiamay contribute to urinary potassium wasting and aggravate urinary sodium and chloride wasting in familial Bartter's syndrome by a mechanism independent of the defect in free-water formation by the active reabsorption of chloride in Henle's loop. (Internal Medicine 33: 1-5, 1994)

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Studies on the pathogenesis of Bartter's syndrome

Cited by 61 publications

References 26 publications

Mechanism of Hypokalemia in Magnesium Deficiency

Mechanism of Hypokalemia in Magnesium Deficiency

Studies on the Pathogenesis of Hypokalemia in Gitelman’s Syndrome: Role of Bicarbonaturia and Hypomagnesemia

Pathophysiological Role of Magnesium in Familial Bartter's Syndrome.

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