title: Elevated exhaled carbon monoxide concentration in hemoglobinopathies and its relation to red blood cell transfusion therapy.
abstract: In this study, the authors examined a possible role of measurements of end-tidal carbon monoxide (CO), corrected for inhaled CO (ETCOc), as a noninvasive screening tool for hemoglobinopathies and as an indicator for when transfusions would be required in patients receiving chronic transfusions. ETCOc measurements were obtained in subjects with sickle cell disease (n = 18), thalassemia (n = 21), and healthy controls (n = 62). ETCOc values less than 3 parts per million (ppm) yielded a positive predictive value of 93% and negative predictive value of 94% in identifying hemoglobinopathies. Subsequently, 7 subjects with thalassemia had laboratory parameters and ETCOc measured over 2 transfusion cycles. ETCOc values were 4.90 +/- 0.32 ppm (mean +/- SD), with 89% of values being above normal (>or=3 ppm). Pretransfusion ETCOc levels significantly correlated with pretransfusion reticulocyte count (r = .96, P <.001), but not with pretransfusion hemoglobin (r = .44, P = .16) or pretransfusion soluble transferrin receptors (sTfR, r = .52, P = .10). In conclusion, we found that patients with hemoglobinopathies have ETCOc values above the range for healthy controls and ETCOc measurements can be used as an adjunct to hemoglobin measurements to determine the proper timing of transfusions.
authors.affiliation: Department of Gastroenterology, Children's Hospital & Research Center Oakland, Oakland, California 94609, USA.
authors.forename: Ellen Butensky
authors.initials: EB
authors.lastname: James
authors.forename: Hendrik J
authors.initials: HJ
authors.lastname: Vreman
authors.forename: Ronald J
authors.initials: RJ
authors.lastname: Wong
authors.forename: David K
authors.initials: DK
authors.lastname: Stevenson
authors.forename: Elliott
authors.initials: E
authors.lastname: Vichinsky
authors.forename: Laurie
authors.initials: L
authors.lastname: Schumacher
authors.forename: Judith Y
authors.initials: JY
authors.lastname: Hall
authors.forename: Julie
authors.initials: J
authors.lastname: Simon
authors.forename: Daniel W
authors.initials: DW
authors.lastname: Golden
authors.forename: Paul
authors.initials: P
authors.lastname: Harmatz
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000628:therapy; D001944:Breath Tests; D002248:Carbon Monoxide / Q000032:analysis* / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D017707:Erythrocyte Transfusion; D045853:Exhalation / Q000502:physiology*; D005260:Female; D006453:Hemoglobinopathies / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D011237:Predictive Value of Tests; D013789:Thalassemia / Q000175:diagnosis / Q000628:therapy; D055815:Young Adult
chemical_list: D002248:Carbon Monoxide