Conn's syndrome in pregnancy successfully treated with amiloride

Al-Ali, N. A.; Elsandabesee, D; Steel, S. A.; Roland, J. M.

doi:10.1080/01443610701667098

Cited by 23 publications

(12 citation statements)

References 2 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…These observations reinforce the safety profile of amiloride during pregnancy and support our hypothesis that the decrease in intrauterine growth observed in our patient's child was likely due to uncontrolled hypertension or other factors, rather than to amiloride. These results are further corroborated by the observations of Al-Ali et al [12], who described a pregnancy complicated by primary aldosteronism and adrenal adenoma in which amiloride proved to be a safe alternative to the mineralocorticoid antagonist spironolactone that is contraindicated in pregnancy.…”

Section: Discussionsupporting

confidence: 82%

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

Caretto

Primerano

Novara

et al. 2014

Case Reports in Obstetrics and Gynecology

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 82%

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

Caretto

Primerano

Novara

et al. 2014

Case Reports in Obstetrics and Gynecology

View full text Add to dashboard Cite

show abstract

“…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

View full text Add to dashboard Cite

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.

show abstract

“…Presenting symptoms in the UKOSS cases included headache, dizziness and intermittent numbness of the hands and feet. In previously reported cases the symptoms included headache, palpitations, muscle weakness, oedema, fatigue and nausea. All UKOSS cases had hypertension in the first trimester with systolic values ranging from 146 to 175 mmHg and diastolic values from 100 to 113 mmHg.…”

Section: Resultsmentioning

confidence: 95%

“…The majority of phaeochromocytoma cases were captured in two systematic reviews 3,6 as well as in three additional case reports, 7-9 providing a total of 123 additional phaeochromocytomas for comparison. For primary aldosteronism there was one systematic review 10 as well as 15 individual cases, [10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] resulting in 34 cases for comparison. For Cushing's syndrome, there were two systematic reviews 25,26 as well as two individual cases 9,27 (156 cases in total).…”

Section: Data Collectionmentioning

confidence: 99%