Abstract

Despite advances in neonatal care, the mortality for congenital diaphragmatic hernia (CDH) remains high. The high mortality in these infants has been attributed to the combination of pulmonary hypoplasia and pulmonary hypertension. The investigators hypothesize that anomalous development of the pulmonary surfactant system may contribute to the impaired lung function in CDH. Moreover the investigators propose that exogenous surfactant therapy (EST), which has been shown to improve pulmonary gas exchange and pulmonary mechanics in surfactant deficient premature infants, might also benefit full term infants with CDH. The overall objective of this study is to characterize the status of the pulmonary surfactant system and to determine the efficacy of EST in the newborn lamb model of CDH. These experiments will determine the efficacy of EST in mitigating the decreased pulmonary gas exchange and abnormal pulmonary mechanics associated with CDH.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Research Project (R01)
Project #
1R01HL049977-01A3
Application #
2226053
Study Section
Special Emphasis Panel (ZRG2-RAP (01))
Project Start
1995-04-01
Project End
1998-02-28
Budget Start
1995-04-01
Budget End
1996-02-29
Support Year
1
Fiscal Year
1995
Total Cost
Indirect Cost

  Institution

Name
State University of New York at Buffalo
Department
Surgery
Type
Schools of Medicine
DUNS #
038633251
City
Buffalo
State
NY
Country
United States
Zip Code
14260

  Publications

Kapur, P; Holm, B A; Irish, M S et al. (1999) Lung physiological and metabolic changes in lambs with congenital diaphragmatic hernia after administration of prenatal maternal corticosteroids. J Pediatr Surg 34:354-6
Kapur, P; Holm, B A; Irish, M S et al. (1999) Tracheal ligation and mechanical ventilation do not improve the antioxidant enzyme status in the lamb model of congenital diaphragmatic hernia. J Pediatr Surg 34:270-2
Ting, A; Glick, P L; Wilcox, D T et al. (1998) Alveolar vascularization of the lung in a lamb model of congenital diaphragmatic hernia. Am J Respir Crit Care Med 157:31-4
Irish, M S; Glick, P L; Russell, J et al. (1998) Contractile properties of intralobar pulmonary arteries and veins in the fetal lamb model of congenital diaphragmatic hernia. J Pediatr Surg 33:921-8
Karamanoukian, H L; O'Toole, S J; Holm, B A et al. (1997) Making the most out of the least: new insights into congenital diaphragmatic hernia. Thorax 52:209-12
O'Toole, S J; Karamanoukian, H L; Irish, M S et al. (1997) Tracheal ligation: the dark side of in utero congenital diaphragmatic hernia treatment. J Pediatr Surg 32:407-10
O'Toole, S J; Karamanoukian, H L; Sharma, A et al. (1996) Surfactant rescue in the fetal lamb model of congenital diaphragmatic hernia. J Pediatr Surg 31:1105-8;discussion 1108-9
O'Toole, S J; Karamanoukian, H L; Morin 3rd, F C et al. (1996) Surfactant decreases pulmonary vascular resistance and increases pulmonary blood flow in the fetal lamb model of congenital diaphragmatic hernia. J Pediatr Surg 31:507-11
O'Toole, S J; Sharma, A; Karamanoukian, H L et al. (1996) Tracheal ligation does not correct the surfactant deficiency associated with congenital diaphragmatic hernia. J Pediatr Surg 31:546-50
Karamanoukian, H L; Peay, T; Love, J E et al. (1996) Decreased pulmonary nitric oxide synthase activity in the rat model of congenital diaphragmatic hernia. J Pediatr Surg 31:1016-9

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