Ferrokinetic and hematologic studies in cystic fibrosis patients

Journal Article · · Am. J. Pediatr. Hematol./Oncol.; (United States)

OSTI ID:5502816

Wagener, J S; McNeill, G C; Taussig, L M; Corrigan, J J; Lemen, R

We investigated 28 cystic fibrosis (CF) patients to determine why hypoxia from their obstructive pulmonary disease does not produce polycythemia. Oxygen saturation was lower and erythropoietin levels were higher in CF patients than in 25 age-comparable reference subjects (90.8% and 47 mimu vs. 94.7% and 29 mimu, p less than 0.01). Hematocrit and red blood cell (RBC) indices were not different between groups. Serum vitamin and iron levels, ferrokinetics, RBC volume, and RBC survival were studied in 10 of the 28 CF patients. Total iron-binding capacity and vitamin E levels were low, and serum iron, ferritin, vitamin B12, and folate levels were normal in these patients. Red blood cell survival was minimally decreased in six patients although there was no other evidence for hemolysis. Ferrokinetics (/sup 59/Fe) indicated a reduction in total erythropoiesis in only two patients. Plasma volume was high-normal in five and above normal in four CF patients; RBC mass was increased appropriately for each patient's degree of hypoxia, when compared to healthy individuals living at different altitudes. These results suggest that CF patients are able to compensate for hypoxia by increasing RBC mass; however, an expanded plasma volume prevents a detectable rise in hematocrit.

• Research Organization: Pediatric Section, University of Arizona Health Sciences Center, Tucson
• OSTI ID: 5502816
• Journal Information: Am. J. Pediatr. Hematol./Oncol.; (United States), Journal Name: Am. J. Pediatr. Hematol./Oncol.; (United States) Vol. 5:2; ISSN APHOD
• Country of Publication: United States
• Language: English

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550901* -- Pathology-- Tracer Techniques
59 BASIC BIOLOGICAL SCIENCES
AMINO ACIDS
AROMATICS
AZAARENES
BETA DECAY RADIOISOTOPES
BETA-MINUS DECAY RADIOISOTOPES
BIOLOGICAL MATERIALS
BLOOD
BLOOD CELLS
BLOOD CHEMISTRY
BODY FLUIDS
CARBOXYLIC ACIDS
CHROMIUM ISOTOPES
DAYS LIVING RADIOISOTOPES
DISEASES
DRUGS
ELEMENTS
ERYTHROCYTES
ERYTHROPOIETIN
EVEN-ODD NUCLEI
FOLIC ACID
HEMATINICS
HEMATOLOGIC AGENTS
HEREDITARY DISEASES
HETEROCYCLIC COMPOUNDS
HYDROXY COMPOUNDS
INTERMEDIATE MASS NUCLEI
IRON
IRON 59
IRON ISOTOPES
ISOTOPE APPLICATIONS
ISOTOPES
MATERIALS
METALS
NONMETALS
NUCLEI
ORGANIC ACIDS
ORGANIC COMPOUNDS
ORGANIC NITROGEN COMPOUNDS
OXYGEN
PATIENTS
PTERIDINES
RADIOISOTOPES
TRACER TECHNIQUES
TRANSITION ELEMENTS
VITAMIN B GROUP
VITAMIN B-12
VITAMIN E
VITAMINS