title: Effect of oral arginine supplementation on exhaled nitric oxide concentration in sickle cell anemia and acute chest syndrome.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  To determine the effect of oral arginine intake on FENO in sickle cell patients with and without history of ACS, and in healthy controls.
  
  
  OBJECTIVE
  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-).
  
  
  METHODS
  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).
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors: Department of Anesthesia and Critical Care Medicine, Nemours Children's Clinic, Jacksonville, Florida 32207, USA. ksulliva@nemours.org
authors: Kevin Joseph
authors: KJ
authors: Sullivan
authors: Niranjan
authors: N
authors: Kissoon
authors: Eric
authors: E
authors: Sandler
authors: Cynthia
authors: C
authors: Gauger
authors: Melanie
authors: M
authors: Froyen
authors: Laurie
authors: L
authors: Duckworth
authors: Martha
authors: M
authors: Brown
authors: Suzanne
authors: S
authors: Murphy
mesh_terms: D056586:Acute Chest Syndrome / Q000188:drug therapy* / Q000378:metabolism*; D000208:Acute Disease; D000284:Administration, Oral; D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000188:drug therapy* / Q000378:metabolism*; D001120:Arginine / Q000008:administration & dosage* / Q000097:blood / Q000493:pharmacokinetics; D001224:Aspartic Acid / Q000097:blood; D001944:Breath Tests; D002648:Child; D002956:Citrulline / Q000097:blood; D005260:Female; D018698:Glutamic Acid / Q000097:blood; D006801:Humans; D008297:Male; D009566:Nitrates / Q000097:blood; D009569:Nitric Oxide / Q000378:metabolism*; D009573:Nitrites / Q000097:blood; D009952:Ornithine / Q000097:blood; D055815:Young Adult
chemical_list: D009566:Nitrates; D009573:Nitrites; D002956:Citrulline; D001224:Aspartic Acid; D009569:Nitric Oxide; D018698:Glutamic Acid; D001120:Arginine; D009952:Ornithine