Sullivan Kevin Joseph, Kissoon Niranjan, Sandler Eric, Gauger Cynthia, Froyen Melanie, Duckworth Laurie, Brown Martha, Murphy Suzanne
Department of Anesthesia and Critical Care Medicine, Nemours Children's Clinic, Jacksonville, Florida 32207, USA.
J Pediatr Hematol Oncol. 2010 Oct;32(7):e249-58. doi: 10.1097/MPH.0b013e3181ec0ae5.
Decreased exhaled nitric oxide levels (FE(NO)) have been described in patients with sickle cell disease (SCD) and a history of acute chest syndrome (ACS) when compared with non-ACS controls. Oral arginine supplementation has been shown to increase FE(NO) in healthy participants, but its effect in SCD patients is not known.
To determine the effect of oral arginine intake on FENO in sickle cell patients with and without history of ACS, and in healthy controls.
No differences in the FE(NO) increase were seen in SCD patients with a history of ACS (ACS+) compared with healthy controls (HC) and SCD patients without history of ACS (ACS-).
ACS+ (n=6), ACS- (n=9), and HC (n=7) patients were studied. At baseline, and after the administration of escalating doses of oral L-arginine (0.1, 0.2, and 0.4 g/kg), serial measurements were made of the following: FE(NO), plasma concentrations of arginine, ornithine, citrulline, aspartate, glutamate, arginine/ornithine ratio, nitrite, nitrate, heart rate (HR), respiratory rate (RR), blood pressure (BP), oxygen saturation (SpO2), forced expiratory volume in 1 second (FEV1), and forced vital capacity (FVC).
At baseline, FE(NO) did not differ among the groups. ACS- and ACS+ groups were deficient in arginine, and had decreased FEV1, FVC, and SaO2 when compared with HC patients. After arginine supplementation, FE(NO), arginine, ornithine, citrulline, nitrite, and the arginine/ornithine ratio increased similarly in all groups. Changes from baseline for HR, BP, SpO2, RR, FEV1, and FVC were minimal and similar in all groups.
In contrast to our earlier study, ACS+ patients had similar FE(NO) values when compared with ACS- and HC patients. All SCD patients were arginine deficient at baseline and showed impairment in respiratory physiology when compared with HC patients. After arginine supplementation, FE(NO) concentration increased in all groups to a similar degree, and lung function and physiologic parameters were minimally affected. The physiologic significance of alterations in FE(NO) in SCD patients and its relationship to ACS predilection requires further delineation.
与无急性胸部综合征（ACS）病史的对照组相比，镰状细胞病（SCD）且有急性胸部综合征病史的患者呼出一氧化氮水平（FE(NO)）降低。口服精氨酸补充剂已被证明可提高健康参与者的FE(NO)，但其对SCD患者的影响尚不清楚。
确定口服精氨酸摄入对有无ACS病史的镰状细胞病患者及健康对照者FE(NO)的影响。
有ACS病史的SCD患者（ACS+）与健康对照者（HC）及无ACS病史的SCD患者（ACS-）相比，FE(NO)升高无差异。
研究了ACS+组（n = 6）、ACS-组（n = 9）和HC组（n = 7）患者。在基线时以及给予递增剂量的口服L-精氨酸（0.1、0.2和0.4 g/kg）后，对以下指标进行系列测量：FE(NO)、精氨酸、鸟氨酸、瓜氨酸、天冬氨酸、谷氨酸的血浆浓度、精氨酸/鸟氨酸比值、亚硝酸盐、硝酸盐、心率（HR）、呼吸频率（RR）、血压（BP）、血氧饱和度（SpO2）、一秒用力呼气量（FEV1）和用力肺活量（FVC）。
在基线时，各组间FE(NO)无差异。与HC患者相比，ACS-组和ACS+组精氨酸缺乏，FEV1、FVC和SaO2降低。补充精氨酸后，所有组的FE(NO)、精氨酸、鸟氨酸、瓜氨酸、亚硝酸盐和精氨酸/鸟氨酸比值均有相似程度的升高。所有组中HR、BP、SpO2、RR、FEV1和FVC相对于基线的变化极小且相似。
与我们早期的研究相反，ACS+患者与ACS-患者及HC患者相比，FE(NO)值相似。所有SCD患者在基线时精氨酸缺乏，与HC患者相比呼吸生理功能受损。补充精氨酸后，所有组的FE(NO)浓度均有相似程度的升高，肺功能和生理参数受影响极小。SCD患者FE(NO)改变的生理意义及其与ACS易感性的关系需要进一步阐明。
