Is normalisation of serum potassium and magnesium always necessary in Gitelman Syndrome for a successful obstetric outcome?

Basu, Ansu; Dillon, Raymond D.; Taylor, Roy; Davison, James M.; Marshall, Sally M.

doi:10.1111/j.1471-0528.2004.00148.x

Cited by 36 publications

(41 citation statements)

References 18 publications

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“…Na q had no detectable effect on hK8 activity at up to 1 M. The K q concentration in serum (3.4-5.0 mM; Basu et al, 2004) is much lower than the concentration required for hK8 activation ( Figure 5A). Therefore, these metal ions are not expected to influence hK8 activity under physiological conditions.…”

Section: Discussionmentioning

confidence: 87%

Activation and enzymatic characterization of recombinant human kallikrein 8

Kishi

Cloutier²,

Kündig³

et al. 2006

Biological Chemistry

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Human kallikrein 8 (hK8), whose gene was originally cloned as the human ortholog of a mouse brain protease, is known to be associated with diseases such as ovarian cancer and Alzheimer's disease. Recombinant human pro-kallikrein 8 was activated with lysyl endopeptidaseconjugated beads. Amino-terminal sequencing of the activated enzyme demonstrated the cleavage of a 9-aa propeptide from the pro-enzyme. The substrate specificity of activated hK8 was characterized using synthetic fluorescent substrates. hK8 showed trypsin-like specificity, as predicted from sequence analysis and enzymatic characterization of the mouse ortholog. All synthetic substrates tested containing either arginine or lysine at P1 position were cleaved by hK8. The highest k cat /K m value of 20=10 3 M -1 s -1 was observed with Boc-Val-Pro-Arg-7-amido-4-methylcoumarin. The activity of hK8 was inhibited by antipain, chymostatin, and leupeptin. The concentration for 50% inhibition by the best inhibitor, antipain, was 0.46 mM. The effect of different metal ions on the enzyme activity was analyzed. Whereas Na q had no effect on hK8 activity, Ni 2q and Zn 2q decreased the activity and Ca 2q , Mg 2q , and K q had a stimulatory effect. Ca 2q was the best activator, with an optimal concentration of approximately 10 mM.

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Section: Discussionmentioning

confidence: 87%

Activation and enzymatic characterization of recombinant human kallikrein 8

Kishi

Cloutier²,

Kündig³

et al. 2006

Biological Chemistry

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“…Despite increased daily supplementation in anticipation of complications, serum levels of potassium and magnesium decreased in the second trimester and both women became symptomatic. Basu et al [3] have also described the difficulty in correcting potassium and magnesium levels in a patient with Gitelman syndrome.…”

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confidence: 95%

“…However, psychosomatic symptoms may represent manifestations of suppressed negative reactions to the aging process, rather than being caused primarily by ovarian failure. This may explain the ethnic and sociocultural variations in menopausal symptomatology [2], and also suggests that attitude to aging may be an important determinant of menopausal symptomatology in any society [3]. The present study was conducted to determine the age at menopause, symptoms of menopause, the effects of menopause on quality of life, and the attitude toward menopause of Igbo women from South Eastern Nigeria.…”

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confidence: 99%

Gitelman syndrome in pregnancy

Haan

Geers

Berghout

2008

Intl J Gynecology & Obste

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Gitelman syndrome is a rare autosomal recessive inherited disorder caused by inactivating mutations in the gene for the thiazide-sensitive sodium-chloride cotransporter in the distal renal tubule. The resulting renal potassium and magnesium wasting and decreased calcium excretion are similar to that seen with the use of thiazide diuretics. Treatment is based on potassium and magnesium supplementation, usually combined with spironolactone or a nonsteroidal anti-inflammatory drug [1]. Although often asymptomatic, cramps and muscle weakness can occur and this may affect delivery.A 24-year-old woman with Gitelman syndrome became symptomatic in the second trimester of her first pregnancy when serum potassium and magnesium levels dropped; intravenous supplementation was given (Fig. 1). Assessment in week 38 of pregnancy revealed oligohydramnios, partial placenta previa, and intrauterine growth restriction. A healthy female neonate weighing 3970 g was delivered at 38 weeks by Figure 1 Transthoracic echocardiography in the apical 4-chamber view demonstrating pericardial effusion with clot (arrow). Abbreviations: RV, right ventricle; LV, left ventricle.

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“…Les patientes avec le GS ne répondent pas bien à l'indométha-cine, contrairement au syndrome de Bartter, et sont mieux traitées avec une supplémentation orale au long cours de magnésium et de potassium. L'utilisation de diurétiques dits épargneurs de potassium (amiloride, spironolactone) aurait un intérêt en cas de GS [6,7].…”

Section: Discussionunclassified

“…Une revue de la littérature concernant le syndrome de Gitelman et grossesse ne retrouve que seulement sept cas de grossesses chez quatre patientes différentes porteuses d'un GS (Tableau 2) [6-9]. Basu et al en 2004 [7], puis Talaulikar [9] en 2005 rapportaient des grossesses chez des patientes porteuses d'un GS avec de grandes difficultés à obtenir des kaliémies et des magnésémies à des taux normaux malgré une supplémentation à fortes doses, mais avec des issues de grossesses sans complications maternelles ou néonatales [7]. Jones et Dorrell [8] avaient précédemment rapporté, en 1998, deux grossesses successives chez une même patiente porteuse d'un GS avec une supplémentation orale par potassium et magnésium à fortes doses et des grossesses uniquement marquées par un oligoamnios.…”

Section: Discussionunclassified