Grasemann C, Ratjen F, Schnabel D, Reutershahn E, Vester U, Grasemann H
Children's Hospital, University of Duisburg-Essen, Germany.
Eur Respir J. 2008 Apr;31(4):815-21. doi: 10.1183/09031936.00103907. Epub 2007 Dec 19.
Airway nitric oxide production is decreased in cystic fibrosis. As growth hormone therapy has been shown to increase nitric oxide production in growth hormone-deficient patients, it may also affect nitric oxide production in patients with cystic fibrosis. The objective of the present study was to investigate the effect of growth hormone therapy on systemic and airway nitric oxide formation in patients with cystic fibrosis. Nitric oxide metabolites in serum and urine, amino acid concentrations in serum and sputum, as well as exhaled nitric oxide, were measured in children with cystic fibrosis before, during and after 1 yr of treatment with human growth hormone. Nitric oxide metabolite concentrations increased significantly in serum and urine during the treatment period. Serum amino acid concentrations (including l-arginine, the substrate for nitric oxide synthases) also increased during treatment. The systemic bioavailability of l-arginine for nitric oxide synthases, expressed as ratio of l-arginine/l-ornithine+lysine, remained unchanged. In contrast, l-arginine concentrations in sputum decreased significantly during growth hormone treatment, as did exhaled nitric oxide levels. Treatment with growth hormone in children with cystic fibrosis decreases exhaled nitric oxide by reducing the concentration of l-arginine in the airways.
囊性纤维化患者气道一氧化氮生成减少。由于生长激素治疗已被证明可增加生长激素缺乏患者的一氧化氮生成，因此它也可能影响囊性纤维化患者的一氧化氮生成。本研究的目的是探讨生长激素治疗对囊性纤维化患者全身和气道一氧化氮生成的影响。在接受人生长激素治疗1年之前、期间和之后，对囊性纤维化儿童的血清和尿液中的一氧化氮代谢产物、血清和痰液中的氨基酸浓度以及呼出一氧化氮进行了测量。治疗期间血清和尿液中的一氧化氮代谢产物浓度显著增加。治疗期间血清氨基酸浓度（包括一氧化氮合酶的底物L-精氨酸）也有所增加。以L-精氨酸/L-鸟氨酸+赖氨酸的比值表示的一氧化氮合酶的L-精氨酸全身生物利用度保持不变。相比之下，生长激素治疗期间痰液中的L-精氨酸浓度显著降低，呼出一氧化氮水平也显著降低。囊性纤维化儿童接受生长激素治疗可通过降低气道中L-精氨酸的浓度来降低呼出一氧化氮水平。
