Long-Term Follow-Up on Health-Related Quality of Life After Mechanical Circulatory Support in Children.

OBJECTIVE: The objective of this study was to evaluate health-related quality of life in long-term survivors of mechanical circulatory support after acute cardiopulmonary failure. DESIGN: Prospective follow-up study. SETTING: Single-institutional in a center for congenital heart disease and pediatric cardiology. PATIENTS: Fifty patients who underwent 58 mechanical circulatory support therapies in our institution from 2001 to 2012. Median age was 2 (0-213) months, and median supporting time was 5 (1-234) days. Indication groups: 1) extracorporeal life support in low cardiac output: 30 cases (52%); 2) extracorporeal cardiopulmonary resuscitation: 13 cases (22%); 3) extracorporeal membrane oxygenation in acute respiratory distress syndrome: four cases (7%); and 4) ventricular assist devices: 11 cases (19%). INTERVENTIONS: Health-related quality of life was measured using standardized questionnaires according to the age group and completed by either parent proxies in children under 7 years old or the survivors themselves. MEASUREMENTS AND MAIN RESULTS: Fifty percentage of the patients were discharged home, and 22 long-term survivors (44%) were studied prospectively for health-related quality of life. Median follow-up period was 4.5 (0.3-11.3) years. Median age at follow-up was 5 (0.6-29) years old. Nineteen long-term survivors filled in the health-related quality of life questionnaires and were classified into three age groups: 0-4 years (n = 7): median health-related quality of life score, 69 (59-86) points; 4-12 years (n = 7): median health-related quality of life score, 50 (48-85) points; older than 12 years (n = 5): median health-related quality of life score, 90 (80-100) points. CONCLUSION: Long-term survivors' health-related quality of life as reported by their parents is lower than that of healthy children. However, the self-assessed health-related quality of life of the patients older than 12 years in our group is comparable to a healthy control population.

Effect of sphingosine on Ca2+ entry and mitochondrial potential of Jurkat T cells--interaction with Bcl2.

Triggers of Jurkat T cell apoptosis include sphingosine and ceramide. Sphingosine and ceramide further inhibit capacitative Ca2+ entry (ICRAC), an effect leading to inactivation but not death of Jurkat T cells. Mitochondria are key organelles in the machinery leading to apoptosis and on the other hand have been shown to participate in the regulation of Ca2+ entry. The present experiments were performed to explore whether treatment of Jurkat T cells with sphingosine leads to apoptosis and reduced Ca2+ entry and whether those effects are sensitive to expression of the antiapoptotic protein Bcl2, localized in the outer mitochondrial membrane. Exposure of Jurkat T cells to 10 microM spingosine was according to DiOC6 fluorescence followed by mitochondrial depolarization and according to Fura-red/Fluo-3 fluorescence followed by decreased capacitative Ca2+ entry. Mitochondrial depolarization was significantly delayed in cells overexpressing wild type Bcl2 or Bcl2 targeted to the mitochondrial membrane, whereas no significant influence on mitochondrial depolarization was observed in cells expressing Bcl2 lacking the membrane targeting motif or Bcl2 targeted to the endoplasmatic reticulum. In contrast to mitochondrial potential, the blunting of capacitative Ca2+ entry following sphingosine treatment was not sensitive to mitochondrial Bcl2 expression. In conclusion sphingosine exposure leads to both, mitochondrial depolarization and inhibition of capacitative Ca2+ entry. Mitochondrial Bcl2 reverses the effect on mitochondria but not on Ca2+ entry and thus leads to dissociation of those two sequelae of sphingosine treatment.