Maternal Bartter’s syndrome in pregnancy treated by amiloride

Deruelle, Philippe; Dufour, P.; Magnenant, Eric; Courouble, Nicolas; Puech, F

doi:10.1016/j.ejogrb.2004.01.030

Cited by 23 publications

(8 citation statements)

References 6 publications

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“…Our observation is in concordance with those reported in other published case reports of women treated with amiloride during pregnancy without complications. Disease backgrounds were different from LS, being Bartter's syndrome [8, 9] and Gitelman's syndrome [10] and the maximum dose of amiloride used was 30 mg daily, which is higher than that we used in our patient [8, 9]. Amiloride was effective and well tolerated also in a pregnant woman with aldosteronism secondary to bilateral adrenal hyperplasia [11].…”

Section: Discussionmentioning

confidence: 94%

A Therapeutic Challenge: Liddle’s Syndrome Managed with Amiloride during Pregnancy

Caretto

Primerano

Novara

et al. 2014

Case Reports in Obstetrics and Gynecology

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Liddle's syndrome (LS) is a rare heritable form of hypertension that often affects young patients. It is caused by gain-of-function mutations of the kidney epithelial sodium channel (ENaC) and it is classically associated with hypokalemia and suppression of renin and aldosterone. LS is characterized by responsiveness to ENaC inhibitors but not to mineralocorticoid receptor inhibitors. Consequently the most effective treatment is amiloride. This drug is not used in pregnancy, as it has not been sufficiently studied during gestation. However for pregnant LS patient amiloride is the most effective drug in decreasing blood pressure. Herein we report the case of a LS patient, who has been followed up by a multidisciplinary teamwork during her first pregnancy. Hypertension worsened after the 25th week of gestation and amiloride was safely administered, firstly in combination with hydrochlorothiazide (the only formulation commercially available in Italy) and, thereafter, as a single drug. Genetic testing was performed in the patient's family in order to support diagnosis and clinical management.

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Section: Discussionmentioning

confidence: 94%

A Therapeutic Challenge: Liddle’s Syndrome Managed with Amiloride during Pregnancy

Caretto

Primerano

Novara

et al. 2014

Case Reports in Obstetrics and Gynecology

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“…(Obstet Gynecol 2011;117:512-6) DOI: 10.1097/AOG.0b013e3182075317 I nherited hypokalemic salt-losing renal tubular disorder is an overlapping set of closely related rare diseases. Affected patients present with normal or low blood pressure, hyperreninemia, metabolic alkalosis, and especially hypokalemia.…”

Section: Background: the Management Of Inherited Hypokalemia Has Imprmentioning

confidence: 99%

Pregnancy in Inherited Hypokalemic Salt-Losing Renal Tubular Disorder

Mascetti

Bettinelli

Simonetti

et al. 2011

Obstetrics &Amp; Gynecology

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“…These pregnancies resulted in healthy offspring. [28][29][30] Plasminogen is produced predominantly by the liver and circulates at slightly elevated plasma concentrations in normal human pregnancies. 31 The present study confirms significant excretion of plasminogen 18,19 and shows active plasmin in urine from PE patients.…”

Section: Discussionmentioning

confidence: 99%

Urinary Plasmin Activates Collecting Duct ENaC Current in Preeclampsia

et al. 2012

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In nephrotic syndrome, plasminogen is aberrantly filtered from plasma to the urinary space and activated along the tubular system. In vitro, plasmin increases ENaC current by proteolytic cleavage of the γ-subunit. It was hypothesized that preeclampsia is associated with plasmin-dependent ability of tubular fluid to activate ENaC. Urine was sampled from 16 preeclamptic (PE) patients and 17 normotensive pregnant women (Ctrl). Urine was analyzed for plasmin(ogen), creatinine, albumin, aldosterone, Na + , K + , proteolytic activity, and for its effect on inward current in cortical collecting duct cells (M1 cells) by whole-cell patch clamp. In PE, urine plasmin(ogen): creatinine ratio was elevated 40-fold (geometric mean, 160 versus 4 µg/g; P <0.0001) and urine aldosterone: creatinine ratio was suppressed to 25% of Ctrl (geometric mean, 27 versus 109 µg/g; P <0.001). A significant negative correlation was found in PE between urinary plasmin(ogen) and aldosterone ( P <0.05). In PE, proteolytic activity was detected at 90 to 75 kD by gelatin zymography in 14 of 16 patients and confirmed by serine protease assay. Immunoblotting showed active plasmin in PE urine. Whole-cell inward current increased in M1 cells on exposure to urine from PE (173±21%; n=6; P <0.001). The increase in current was abolished by amiloride (2 μmol/L; P <0.001), α 2 -antiplasmin (1 μmol/L; P <0.001), and heat denaturation ( P <0.001). Preeclampsia is associated with urinary excretion of plasmin(ogen) and plasmin-dependent activation of ENaC by urine. Proteolytic activation of ENaC by plasmin may contribute to Na + retention and hypertension in preeclampsia.

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Maternal Bartter’s syndrome in pregnancy treated by amiloride

Cited by 23 publications

References 6 publications

A Therapeutic Challenge: Liddle’s Syndrome Managed with Amiloride during Pregnancy

A Therapeutic Challenge: Liddle’s Syndrome Managed with Amiloride during Pregnancy

Pregnancy in Inherited Hypokalemic Salt-Losing Renal Tubular Disorder

Urinary Plasmin Activates Collecting Duct ENaC Current in Preeclampsia

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