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83 (74�C92), P?=?0.001], emotional [63 (55�C72) vs. 84 (74�C93), P?Angiogenesis inhibitor reducing lung function decline in children with CF. The cost of IV antibiotics was reduced by ?66,384 ($104,000) in 2010 when compared with 2009. Such cost-benefit may have implications for workforce planning and service provision. Pediatr Pulmonol. 2012; 47:1235�C1241. ? 2012 Wiley Periodicals, Inc. ""Endothelium-derived nitric oxide (NO) and endothelin (ET)-1 interact to regulate the vascular tone in pulmonary hypertension (PH). We investigated the protective effects of an orally active, dual endothelin converting enzyme (ECE)/neutral endopeptidase (NEP) inhibitor/CGS 26393 on pulmonary vascular remodeling and pulmonary expressions of ET-1 and endothelial nitric oxide synthase (eNOS) during the development of PH secondary to cardiac dysfunction. Significant increases in the mean pulmonary arterial pressure, pulmonary arteriolar medial thickness, and pulmonary expression of ET-1 were seen in rats subjected to aortic banding TRIB1 for 4 weeks, compared with sham-operated rats. Treatment with CGS 26393 (30?mg/kg, twice daily, p.o.) began on 1 day after aortic banding. CGS 26393 treated rats had lower mean pulmonary arterial pressure (15?��?1?mmHg, mean?��?SEM, P?MK-2206 mw that CGS 26393 could offer preventive effects on the development of PH by ameliorating pulmonary remodeling, decreasing ET-1 production, and up-regulating eNOS and cGMP in aorta-banded rats. However, the molecular mechanisms by which treatment with CGS 26393 results in altered expressions of eNOS and cGMP awaits further investigation. Pediatr Pulmonol. 2010;45:1076�C1085. ? 2010 Wiley-Liss, Inc. ""Growth failure is a common and complicated process in children with cystic fibrosis (CF). Growth hormone, which is becoming a more commonly used agent in such patients, has demonstrated beneficial effects aside from increased growth velocity.