Soluble transferrin receptors and tissue oxygenation in non anaemic cystic fibrosis patients.
BACKGROUND
Chronic pulmonary disease and progressive tissue hypoxia are major causes of morbidity and mortality in cystic fibrosis (CF). Normally the body adapts to tissue hypoxia by increasing the red cell mass and decreasing the Hb-O(2) affinity. These adaptations are commonly observed in patients with cyanotic heart disease and individuals living at high altitude. However, patients with CF not only have an impaired erythroid response to hypoxia, but also are frequently anaemic.


METHODS
In order to evaluate erythroid marrow activity and tissue oxygenation in 37 patients with CF we measured: the haematological and blood chemistry parameters; including red cell indices, ferritin, erythropoietin (Epo) and soluble transferrin receptors (sTfR) levels; arterial blood gases, P(50) and oxygen release to the tissues (O(2)(R)) and the 2,3-BPG levels.


RESULTS
The main results showed that a) patients with CF have a mild degree of tissue hypoxia which is expressed by the moderately decreased of P(50) and O(2)(R) values and the relative increase of Epo level, b) 2,3-BPG synthesis in patients with CF is normal and c) sTfR levels are significantly increased (3-fold normal) in patients with CF compared to normal controls.


CONCLUSIONS
The above observations indicate that erythroid marrow activity in patients with CF is increased.
Christoforou
Elena
E
Haematology Laboratory, Aghia Sophia Children's Hospital, Athens, Greece.
Papassotiriou
Ioannis
I
Skarmoutsou
Christophille
C
Doudounakis
Stavros
S
Stamoulakatou
Alexandra
A
Kanavakis
Emmanuel
E
D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D001784:Blood Gas Analysis; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000097:blood* / Q000150:complications; D004912:Erythrocytes / Q000378:metabolism; D004921:Erythropoietin / Q000097:blood; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D000860:Hypoxia / Q000097:blood / Q000209:etiology; D007223:Infant; D008297:Male; D010101:Oxygen Consumption / Q000502:physiology*; D011990:Receptors, Transferrin / Q000097:blood*; D012720:Severity of Illness Index
D015415:Biomarkers; D011990:Receptors, Transferrin; D004921:Erythropoietin; D005293:Ferritins