Document Detail

Ferrokinetic and hematologic studies in cystic fibrosis patients.
MedLine Citation:
PMID:  6614395     Owner:  NLM     Status:  MEDLINE    
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 (59Fe) 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.
J S Wagener; G C McNeill; L M Taussig; J J Corrigan; R Lemen
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The American journal of pediatric hematology/oncology     Volume:  5     ISSN:  0192-8562     ISO Abbreviation:  Am J Pediatr Hematol Oncol     Publication Date:  1983  
Date Detail:
Created Date:  1983-10-28     Completed Date:  1983-10-28     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  7908071     Medline TA:  Am J Pediatr Hematol Oncol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  153-60     Citation Subset:  IM    
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MeSH Terms
Anoxia / physiopathology
Chromium Radioisotopes / diagnostic use
Cystic Fibrosis / blood*,  physiopathology
Diphosphoglyceric Acids / blood
Erythrocyte Aging
Erythrocyte Volume
Erythropoietin / metabolism
Iron / metabolism*
Iron Radioisotopes / diagnostic use
Oxygen / blood
Plasma Volume
Grant Support
Reg. No./Substance:
0/Chromium Radioisotopes; 0/Diphosphoglyceric Acids; 0/Iron Radioisotopes; 11096-26-7/Erythropoietin; 138-81-8/2,3-Diphosphoglycerate; 7439-89-6/Iron; 7782-44-7/Oxygen

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