Extracorporeal membrane oxygenation (ECMO) is life saving continuous cardiopulmonary bypass for cardiac and pulmonary failure. Four to five hundred infants and children require ECMO per year. A major complication of ECMO is the development of an inflammatory mediated capillary leak syndrome with activation of the contact and complement systems, and with fluid and neutrophil accumulation in the lungs and interstitial tissues resulting in multi-organ dysfunction and prolonged time on ECMO. Inflammatory mediators that may play a critical role in capillary leak syndrome on ECMO are the C1 inhibitor, which regulates the activated contact and complement systems, and chemokines which attract and activate white blood cells possilby contribyuting to lung injury and edema. There is limited information on C1 inhibitor or chemokines on bypass, and this information is mainly derived from studies on short term cardiopulmonary bypass for cardiac surgery. Our workng hypotheses are that the C1 inhibitor may be an important regulator of the generalized capillary leak that develops on ECMO and that the release of chemokines may contribute to the lung injury and edema. This pilot study will gather preliminary information to help develop specific hypotheses to design a larger clinical study. Our goal is to measure C1 inhibitor and chemokine levels, and determine if there is an association between C1 inhibitor and the capillary syndrome, and between chemokines and lung edema on ECMO. Future use of C1 inhibitor concentrate administered before and during ECMO therapy may attenuate the development of the capillary leak syndrome with a decrease in lung injury and edema, and with a subsequent decrease in ventilator, ECMO, and hospital days, in addition to the decreased cost of hospitalization.

Project Start
Project End
Budget Start
Budget End
Support Year
Fiscal Year
Total Cost
Indirect Cost
Children's Hospital of Philadelphia
United States
Zip Code
Medina-Gomez, Carolina; Kemp, John P; Dimou, Niki L et al. (2017) Bivariate genome-wide association meta-analysis of pediatric musculoskeletal traits reveals pleiotropic effects at the SREBF1/TOM1L2 locus. Nat Commun 8:121
Agopian, A J; Goldmuntz, Elizabeth; Hakonarson, Hakon et al. (2017) Genome-Wide Association Studies and Meta-Analyses for Congenital Heart Defects. Circ Cardiovasc Genet 10:e001449
Ruan, Alexandra; Tobin, Nicole H; Mulligan, Kathleen et al. (2016) Brief Report: Macrophage Activation in HIV-Infected Adolescent Males Contributes to Differential Bone Loss by Sex: Adolescent Trials Network Study 021. J Acquir Immune Defic Syndr 72:372-5
Medoff-Cooper, Barbara; Irving, Sharon Y; Hanlon, Alexandra L et al. (2016) The Association among Feeding Mode, Growth, and Developmental Outcomes in Infants with Complex Congenital Heart Disease at 6 and 12 Months of Age. J Pediatr 169:154-9.e1
Lappe, Joan M; Watson, Patrice; Gilsanz, Vicente et al. (2015) The longitudinal effects of physical activity and dietary calcium on bone mass accrual across stages of pubertal development. J Bone Miner Res 30:156-64
Ollberding, Nicholas J; Gilsanz, Vicente; Lappe, Joan M et al. (2015) Reproducibility and intermethod reliability of a calcium food frequency questionnaire for use in Hispanic, non-Hispanic Black, and non-Hispanic White youth. J Acad Nutr Diet 115:519-27.e2
Medina-Gómez, Carolina; Chesi, Alessandra; Heppe, Denise H M et al. (2015) BMD Loci Contribute to Ethnic and Developmental Differences in Skeletal Fragility across Populations: Assessment of Evolutionary Selection Pressures. Mol Biol Evol 32:2961-72
Avitabile, Catherine M; Goldberg, David J; Zemel, Babette S et al. (2015) Deficits in bone density and structure in children and young adults following Fontan palliation. Bone 77:12-6
Rutstein, Richard M; Samson, Pearl; Fenton, Terry et al. (2015) Long-term safety and efficacy of atazanavir-based therapy in HIV-infected infants, children and adolescents: the Pediatric AIDS Clinical Trials Group Protocol 1020A. Pediatr Infect Dis J 34:162-7
Trabulsi, Jillian C; Irving, S Y; Papas, M A et al. (2015) Total Energy Expenditure of Infants with Congenital Heart Disease Who Have Undergone Surgical Intervention. Pediatr Cardiol 36:1670-9

Showing the most recent 10 out of 455 publications