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20 results on '"Rubinshtein, Marina"'

4. Mutations in PPCS, Encoding Phosphopantothenoylcysteine Synthetase, Cause Autosomal-Recessive Dilated Cardiomyopathy

Author

Iuso, Arcangela, primary, Wiersma, Marit, additional, Schüller, Hans-Joachim, additional, Pode-Shakked, Ben, additional, Marek-Yagel, Dina, additional, Grigat, Mathias, additional, Schwarzmayr, Thomas, additional, Berutti, Riccardo, additional, Alhaddad, Bader, additional, Kanon, Bart, additional, Grzeschik, Nicola A., additional, Okun, Jürgen G., additional, Perles, Zeev, additional, Salem, Yishay, additional, Barel, Ortal, additional, Vardi, Amir, additional, Rubinshtein, Marina, additional, Tirosh, Tal, additional, Dubnov-Raz, Gal, additional, Messias, Ana C., additional, Terrile, Caterina, additional, Barshack, Iris, additional, Volkov, Alex, additional, Avivi, Camilla, additional, Eyal, Eran, additional, Mastantuono, Elisa, additional, Kumbar, Muhamad, additional, Abudi, Shachar, additional, Braunisch, Matthias, additional, Strom, Tim M., additional, Meitinger, Thomas, additional, Hoffmann, Georg F., additional, Prokisch, Holger, additional, Haack, Tobias B., additional, Brundel, Bianca J.J.M., additional, Haas, Dorothea, additional, Sibon, Ody C.M., additional, and Anikster, Yair, additional

Published
2018
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18. Blood Lactate Levels Differ Significantly Between Surviving and Nonsurviving Patients Within the Same Risk-Adjusted Classification for Congenital Heart Surgery (RACHS-1) Group After Pediatric Cardiac Surgery.

Author

Hazan, Vered Molina, Gonen, Yael, Vardi, Amir, Keidan, Ilan, Mishali, David, Rubinshtein, Marina, Yakov, Yusim, and Paret, Gideon

Subjects
BLOOD lactate, CARDIAC surgery, CONGENITAL heart disease in children, COHORT analysis, POSTOPERATIVE care, BLOOD gases analysis, CARDIOPULMONARY bypass
Abstract

This study aimed to examine the association between lactate levels in the first hours after surgery for congenital heart defects and the results of Risk-Adjusted Classification for Congenital Heart Surgery (RACHS-1) scoring and to evaluate serial lactate levels over time to determine whether they can serve as a supplementary tool for postoperative assessment within the same RACHS-1 group of patients. A retrospective cohort study was performed using data retrieved from a clinical database of 255 children who had surgery for congenital heart defects between 1999 and 2001 at Sheba Medical Center. Lactate levels were measured postoperatively four times (mg/dL units). The last sample was taken at the end of the surgical procedure, and lactate levels were measured at admission to the pediatrics critical care unit, then 6 and 12 h after admission. The lactate level was measured via arterial blood gases. A total of 27 deaths occurred, yielding a mortality rate of 7.4% when Norwood operations were excluded and 10.16% when they were included. The mean initial postoperative lactate level was significantly lower for survivors (42.2 ± 32.0 mg/dL) than for nonsurvivors (85.4 ± 54.1 mg/dL) ( p < 0.01). The serial mean lactate levels decreased progressively for all surviving patients ( r = 0.96) compared with nonsurvivors ( r = 0.02). The lactate levels correlated with the RACHS-1 subgroups at each time point ( r > 0.96 for all). The Pearson correlations between postoperative lactate levels (last lactate measurement taken in the operating room) and cardiopulmonary bypass (CPB) duration ( r = 0.549), clamp duration ( r = 0.586), and the inotropic score ( r = 0.466) ( p < 0.001 for all) were significantly positive. The correlations between the maximum lactate levels (during the first 12 postoperative hours) and CPB duration ( r = 0.496), clamp duration ( r = 0.509), and the inotropic score ( r = 0.633) ( p < 0.001 for all) were extremely positive. The early elevation of lactate levels in RACHS-1 subgroups 1 to 3 were highly correlated with poor prognosis and death ( p < 0.03). In addition, the lactate levels differed significantly between survivors and nonsurvivors within the same RACHS-1 subgroup. The survivors in RACHS-1 subgroups 1 to 3 had lower mean lactate levels than the nonsurvivors in this group ( P = 0.011), and this also held true for the survivors and nonsurvivors in RACHS-1 subgroups 4 to 6 ( P = 0.026). Lactate levels differed significantly between survivors and nonsurvivors within the same RACHS-1 subgroup. This combination allows the targeting of appropriately intensive interventions and therapies toward the sickest patients. [ABSTRACT FROM AUTHOR]

Published
2010
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20. Cardiac failure in very long chain acyl-CoA dehydrogenase deficiency requiring extracorporeal membrane oxygenation (ECMO) treatment: A case report and review of the literature.

Author

Katz S, Landau Y, Pode-Shakked B, Pessach IM, Rubinshtein M, Anikster Y, Salem Y, and Paret G

Abstract

Fatty acid oxidation (FAO) defects often present with multi-system involvement, including several life-threatening cardiac manifestations, such as cardiomyopathy, pericardial effusion and arrhythmias. We report herein a fatal case of cardiac dysfunction and rapid-onset tamponade following an acute illness in a neonate with molecularly proven very long chain acyl-CoA dehydrogenase (VLCAD) deficiency (harboring the known del799&#95;802 mutation), requiring 15 days of extracorporeal membrane oxygenation (ECMO) treatment. As data regarding the use of ECMO in FAO defects in general, and VLCAD in particular, are scarce, we review the literature and discuss insights from in vitro models and several successful reported cases.

Published
2016
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