Aborted sudden cardiac death in two patients with Bartter’s/Gitelman’s syndromes

Scognamiglio, R; Negut, Christian; Calò, Lorenzo A.

doi:10.5414/cnp67193

Cited by 46 publications

(48 citation statements)

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“…Six recent case reports support the assumption that hypokalemia may cause dangerous cardiac arrhythmias in Bartter-Gitelman patients [17][18][19][20][21].…”

mentioning

confidence: 89%

Cardiac arrhythmias and rhabdomyolysis in Bartter–Gitelman patients

Cortesi

Lava

Bettinelli³

et al. 2010

Pediatr Nephrol

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Recent data demonstrate that patients affected with hypokalemic salt-losing tubulopathies are prone to acute cardiac arrhythmias and rhabdomyolysis. The tendency to these potentially fatal complications is especially high if chronic hypokalemia is severe, in patients with diarrhea, vomiting or a prolonged QT interval on standard electrocardiography, in patients on drug management with compounds prolonging the electrocardiographic QT interval (including antiarrhythmic agents, some antihistamines, macrolides, antifungals, psychotropics, ß 2 -adrenergic agonists or cisapride), following acute alcohol abuse and during exercise. Cardiac arrhythmias and rhabdomyolysis occur with sufficient frequency in hypokalemic salt-losing tubulopathies to merit wider awareness of their presence and the preparation of specific prevention and management recommendations.

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“…Six recent case reports support the assumption that hypokalemia may cause dangerous cardiac arrhythmias in Bartter-Gitelman patients [17][18][19][20][21].…”

mentioning

confidence: 89%

Cardiac arrhythmias and rhabdomyolysis in Bartter–Gitelman patients

Cortesi

Lava

Bettinelli³

et al. 2010

Pediatr Nephrol

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“…On one hand, a favourable prognosis has been reported for most patients with GS [4,9,12] with a normal life expectancy [4], but a reduced quality of life [3]. On the other hand, in the last decade, cases with life-threatening arrhythmias and sudden cardiac death have been reported [27,28]. Although sudden cardiac death also occurred with normal potassium blood levels, [27] hypokaliemia and QTc prolongation are the most frequently suggested mechanisms of sudden cardiac death caused by arrhythmia [27,28].…”

Section: Introductionmentioning

confidence: 99%

“…On the other hand, in the last decade, cases with life-threatening arrhythmias and sudden cardiac death have been reported [27,28]. Although sudden cardiac death also occurred with normal potassium blood levels, [27] hypokaliemia and QTc prolongation are the most frequently suggested mechanisms of sudden cardiac death caused by arrhythmia [27,28]. Therefore, medications that prolong the QT duration should be avoided in GS patients.…”

Section: Introductionmentioning

confidence: 99%

“…Therefore, medications that prolong the QT duration should be avoided in GS patients. Moreover, GS has been associated with left ventricular dysfunction and exercise-induced reduction of the cardiac index [27,29]. Because of the salt and fluid loss, it can be assumed that GS is also connected with a higher frequency of thromboembolic events.…”

Section: Introductionmentioning

confidence: 99%

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Gitelman syndrome DD thiazide diuretics abuse

Keller

Beule²,

Dippold³

2014

Open Medicine

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AbstractIntroduction. Gitelman syndrome (GS) is a rare inherited disorder. Mutations in SLC12A3 gene that encode tubular Na+Cl-cotransporter (NCCT) cause hypokalemic metabolic alkalosis, salt loss, hypomagnesaemia and hypocalciuria. The symptoms include weakness, vertigo, hypotension, tetany, paresthesia and nausea. Diagnostic criteria are a normal urine concentrating ability, normal glomerular filtration rate (GFR), hypomagnesaemia (<0,65mmol/l), hypokalemia (<3,6mmol/l) and hypocalciuria (<0,1mmol/mmol creatinine). Previously, the diagnosis was made by exclusion. Today, genetic analysis can ensure diagnosis. Thiazide diuretics (TD) abuse with similar abnormalities can make the differential diagnosis difficult. Causal therapy of GS does not exist. The substitution of potassium and magnesium are therapeutic strategies. Case presentation. A 41-year-old obese woman presented at the emergency department with recurrent episodes of hypokalemia with concomitant weakness, muscle cramps, polyuria and collapse. The results of laboratory testing of blood and urine led to the suspected diagnosis of GS. In the follow-up examinations, the results were inconsistent. Therefore, a transient thiazide diuretics abuse was assumed. Discussion. This case demonstrates the difficulties in making the diagnosis of GS on the basis of only clinical and laboratory tests, without the use of genetic analysis. The differentiation between GS and thiazide diuretic abuse is especially difficult.

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“…However, severe hypokalemic rhabdomyolysis or ventricular arrhythmias may occur when acute additional extrarenal potassium and magnesium losses occur (vomiting or diarrhea). [3][4][5][6][7] Lifelong oral potassium and magnesium supplementation is the cornerstone of treatment aiming at relieving symptoms and preventing severe hypokalemia-related complications, including cardiac arrhythmias. 8 However, oral supplementation may be insufficient to fully correct the electrolyte disorders or may have poor gastrointestinal (GI) tolerability, which can limit dosing.…”

mentioning

confidence: 99%

Indomethacin, Amiloride, or Eplerenone for Treating Hypokalemia in Gitelman Syndrome

Blanchard

Vargas‐Poussou

Vallet

et al. 2015

Journal of the American Society of Nephrology

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Patients with Gitelman syndrome (GS), an inherited salt-losing tubulopathy, are usually treated with potassium-sparing diuretics or nonsteroidal anti-inflammatory drugs and oral potassium and magnesium supplementations. However, evidence supporting these treatment options is limited to case series studies.We designed an open-label, randomized, crossover study with blind end point evaluation to compare the efficacy and safety of 6-week treatments with one time daily 75 mg slow-release indomethacin, 150 mg eplerenone, or 20 mg amiloride added to constant potassium and magnesium supplementation in 30 patients with GS (individual participation: 48 weeks). Baseline plasma potassium concentration was 2.860.4 mmol/L and increased by 0.38 mmol/L (95% confidence interval [95% CI], 0.23 to 0.53; P,0.001) with indomethacin, 0.15 mmol/L (95% CI, 0.02 to 0.29; P=0.03) with eplerenone, and 0.19 mmol/L (95% CI, 0.05 to 0.33; P,0.01) with amiloride. Fifteen patients became normokalemic: six with indomethacin, three with eplerenone, and six with amiloride. Indomethacin significantly reduced eGFR and plasma renin concentration. Eplerenone and amiloride each increased plasma aldosterone by 3-fold and renin concentration slightly but did not significantly change eGFR. BP did not significantly change. Eight patients discontinued treatment early because of gastrointestinal intolerance to indomethacin (six patients) and hypotension with eplerenone (two patients). In conclusion, each drug increases plasma potassium concentration in patients with GS. Indomethacin was the most effective but can cause gastrointestinal intolerance and decreased eGFR. Amiloride and eplerenone have similar but lower efficacies and increase sodium depletion. The benefit/risk ratio of each drug should be carefully evaluated for each patient.

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Aborted sudden cardiac death in two patients with Bartter’s/Gitelman’s syndromes

Cited by 46 publications

References 0 publications

Cardiac arrhythmias and rhabdomyolysis in Bartter–Gitelman patients

Cardiac arrhythmias and rhabdomyolysis in Bartter–Gitelman patients

Gitelman syndrome DD thiazide diuretics abuse

Indomethacin, Amiloride, or Eplerenone for Treating Hypokalemia in Gitelman Syndrome

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