Sickle Cell Anemia Research - Genetics, Causes, Symptoms

Sickle Cell Anemia Research Today is a free monthly online journal that collates and summarizes the latest research about Sickle Cell Anemia, including details on genetics, causes, symptoms.


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Impact of acute chest syndrome on lung function of children with sickle cell disease.

Sylvester KP, Patey RA, Milligan P, Rafferty GF, Broughton S, Rees D, Thein SL, Greenough A

Departments of Child Health, Health and Life Sciences, and Haematological Medicine, King's College London School of Medicine at Guy's, King's College, and St Thomas' Hospitals, London, United Kingdom.

OBJECTIVE: To test the hypothesis that children with sickle cell disease (SCD) who experienced an acute chest syndrome (ACS) hospitalization episode would have worse lung function than children with SCD without ACS episodes. STUDY DESIGN: Forced expiratory volume in 1 second (FEV(1)); forced vital capacity (FVC); FEV(1)/FVC ratio; peak expiratory flow (PEF); forced expiratory flow at 25% (FEF(25)), 50% (FEF(50)), and 75% (FEF(75)) of FVC; airway resistance (Raw); and lung volumes were compared in 20 children with ACS and 20 aged-matched children without ACS (median age, 11 years; range, 6 to 16 years). Fourteen age-matched pairs were assessed before and after bronchodilator use. RESULTS: The mean Raw (P = .03), TLC (P = .01), and RV (P = .003) were significantly higher in the group with ACS than in the group without ACS. There were no significant differences in the changes in lung function test results in response to bronchodilator administration between the 2 groups, but the children with ACS had a lower FEF(25) (P = .04) and FEF(75) (P = .03) pre-bronchodilator use and a lower mean FEV(1)/FVC ratio (P = .03) and FEF(75) (P = .03) post-bronchodilator use. CONCLUSIONS: Children with SCD who experienced an ACS hospitalization episode had significant differences in lung function compared with those who did not experience ACS episodes. Our results are compatible with the hypothesis that ACS episodes predispose children to increased airway obstruction.

Published 24 July 2006 in J Pediatr, 149(1): 17-22.
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Sickle Cell Anemia Research Today Archive:

Volume 1 (2006)
  Issue 1 (February)
  Issue 2 (March)
  Issue 3 (April)
  Issue 4 (May)
  Issue 5 (June)
  Issue 6 (July)
  Issue 7 (August)
  Issue 8 (September)
  Issue 9 (October)
  Issue 10 (November)
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Volume 2 (2007)
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  Issue 4 (April)
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Volume 3 (2008)
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  Issue 5 (May)
  Issue 6 (June)
  Issue 7 (July)
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Hope and Destiny: A Patient's and Parent's Guide to Sickle Cell Disease and Sickle Cell Trait

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