The relationship between fetal hemoglobin and disease severity in children with sickle cell anemia
dc.contributor.author | Odenheimer, Daniel J. | en_US |
dc.contributor.author | Sarnaik, Sharada A. | en_US |
dc.contributor.author | Whitten, Charles F. | en_US |
dc.contributor.author | Rucknagel, Donald L. | en_US |
dc.contributor.author | Sing, Charles F. | en_US |
dc.date.accessioned | 2006-04-28T16:48:00Z | |
dc.date.available | 2006-04-28T16:48:00Z | |
dc.date.issued | 1987-07 | en_US |
dc.identifier.citation | Odenheimer, Daniel J.; Sarnaik, Sharada A.; Whitten, Charles F.; Rucknagel, Donald L.; Sing, Charles F. (1987)."The relationship between fetal hemoglobin and disease severity in children with sickle cell anemia." American Journal of Medical Genetics 27(3): 525-535. <http://hdl.handle.net/2027.42/38246> | en_US |
dc.identifier.issn | 0148-7299 | en_US |
dc.identifier.issn | 1096-8628 | en_US |
dc.identifier.uri | https://hdl.handle.net/2027.42/38246 | |
dc.identifier.uri | http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=retrieve&db=pubmed&list_uids=2443006&dopt=citation | en_US |
dc.description.abstract | A study was conducted in a sample of 140 children with sickle cell anemia to evaluate the relationship between hematological variables (%HbF, %HbA2, %Hb, and mean cell volume) and disease severity. A patient's severity status was determined by whether he/she was hospitalized, had a transfusion, and/or had a pain crisis at 2 evaluation periods; the first was based on a patient's history taken at the initial assessment visit to the Wayne State Comprehensive Sickle Cell Center, and the second was based on a 1–3 year follow-up at the center. Fetal hemoglobin was a strong predictor of a patient's hospitalization and transfusion status. A decrease in %HbF of 4.76% (one SD of %HbF) was associated with a 3.58 fold (95% confidence interval, 1.18–7.28) greater odds of being hospitalized both prior to initial assessment and on follow-up, compared to not being hospitalized at either evaluation. Similarly, a decrease in %HbF of 4.76% was associated with a 5.56 fold (95% confidence interval, 1.67–18.96) greater odds of having a transfusion both prior to initial assessment and on follow-up compared to not having a transfusion at either evaluation. Patients who were both hospitalized and transfused at initial assessment and on follow-up (n = 12) had a mean %HbF of 7.59%, while patients who were not hospitalized or transfused at either evaluation (n = 19) had a mean %HbF of 13.61%. Fetal hemoglobin was not a significant predictor of pain crises in this sample of patients. None of the other hematological variables were significant predictors of disease severity in this study. The strong relationship between %HbF and disease severity identified in this study suggests that a single %HbF measurement may be useful in predicting important aspects of the clinical course of children with sickle cell anemia. | en_US |
dc.format.extent | 740558 bytes | |
dc.format.extent | 3118 bytes | |
dc.format.mimetype | application/pdf | |
dc.format.mimetype | text/plain | |
dc.language.iso | en_US | |
dc.publisher | Wiley Subscription Services, Inc., A Wiley Company | en_US |
dc.subject.other | Life and Medical Sciences | en_US |
dc.subject.other | Genetics | en_US |
dc.title | The relationship between fetal hemoglobin and disease severity in children with sickle cell anemia | en_US |
dc.type | Article | en_US |
dc.rights.robots | IndexNoFollow | en_US |
dc.subject.hlbsecondlevel | Genetics | en_US |
dc.subject.hlbtoplevel | Health Sciences | en_US |
dc.subject.hlbtoplevel | Science | en_US |
dc.description.peerreviewed | Peer Reviewed | en_US |
dc.contributor.affiliationum | Department of Human Genetics, University of Michigan, Ann Arbor, Michigan | en_US |
dc.contributor.affiliationum | Department of Human Genetics, University of Michigan, Ann Arbor, Michigan | en_US |
dc.contributor.affiliationum | Department of Human Genetics, University of Michigan, Ann Arbor, Michigan ; Department of Human Genetics, University of Michigan, Ann Arbor, MI 48109-0618 | en_US |
dc.contributor.affiliationother | Comprehensive Sickle Cell Center and Pediatrics, Wayne State University, Detroit, Michigan | en_US |
dc.contributor.affiliationother | Comprehensive Sickle Cell Center and Pediatrics, Wayne State University, Detroit, Michigan | en_US |
dc.identifier.pmid | 2443006 | en_US |
dc.description.bitstreamurl | http://deepblue.lib.umich.edu/bitstream/2027.42/38246/1/1320270305_ftp.pdf | en_US |
dc.identifier.doi | http://dx.doi.org/10.1002/ajmg.1320270305 | en_US |
dc.identifier.source | American Journal of Medical Genetics | en_US |
dc.owningcollname | Interdisciplinary and Peer-Reviewed |
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