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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
11
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pubmed:dateCreated |
1994-11-2
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pubmed:abstractText |
To study the effect of fetal hemoglobin (Hb F) on the formation of dense and irreversibly deformed cells in patients with sickle cell disease (SCD), we investigated the distribution of Hb F and Hb F cells (FC, cells having HbF) and the difference in the degree of irreversible deformation between FC and non-Hb F cells (NFC) in relation to their cell density. We used immunofluorescence to define FC and image analysis to quantify the degree of deformation. Blood samples from 16 patients with Hb F levels ranging from 4 to 24% were separated into less dense [top (T), d < 1.11 g/mL] and dense [bottom (B), d > or = 1.11 g/mL] fractions. We found higher percentages of Hb F and FC in top fractions, suggesting that FC are hydrated more than NFC. Quantitative shape analysis demonstrated that the irreversible deformation of FC in the top fraction [FC(T)] was not significantly different from that of NFC in the fraction [NFC(T)] (p = 0.163), but rather that the irreversible deformation of FC in the bottom fraction [FC(B)] was much milder than for NFC(B) (p < 0.001). The order of the degree of irreversible deformation was NFC(B) > FC(B) > NFC(T) = FC(T). These results clearly demonstrated that FC are less susceptible to both irreversible deformation and dehydration than NFC, suggesting that in circulation, the sickling process plays a major role in cell dehydration. Additionally, we found that the ratio of NFC(B) to FC(B) decreased as a patient's Hb F increased (r = 0.84) and that NFC(B) = FC(B) when Hb F was about 20%.
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pubmed:grant | |
pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Oct
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pubmed:issn |
0301-472X
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
22
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
1058-63
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pubmed:dateRevised |
2007-11-14
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pubmed:meshHeading |
pubmed-meshheading:7523162-Adolescent,
pubmed-meshheading:7523162-Anemia, Sickle Cell,
pubmed-meshheading:7523162-Cell Separation,
pubmed-meshheading:7523162-Centrifugation, Density Gradient,
pubmed-meshheading:7523162-Child,
pubmed-meshheading:7523162-Child, Preschool,
pubmed-meshheading:7523162-Erythrocyte Deformability,
pubmed-meshheading:7523162-Fetal Hemoglobin,
pubmed-meshheading:7523162-Humans,
pubmed-meshheading:7523162-Infant
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pubmed:year |
1994
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pubmed:articleTitle |
Quantitative analysis of the degree of irreversible deformation of F cells and non-F cells and its relationship to cell density in sickle cell disease.
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pubmed:affiliation |
Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia 19104.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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