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Acute chest syndrome is associated with history of asthma in hemoglobin SC disease
Author(s) -
Poulter Elana Y.,
Truszkowski Piotr,
Thompson Alexis A.,
Liem Robert I.
Publication year - 2011
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22900
Subject(s) - medicine , acute chest syndrome , hemoglobin , wheeze , asthma , hemoglobin f , disease , anemia , pediatrics , gastroenterology , fetal hemoglobin , sickle cell anemia , pregnancy , fetus , biology , genetics
Background Acute chest syndrome (ACS) is a frequent cause of morbidity and leading cause of death among individuals with sickle cell disease. Yet, ACS in hemoglobin SC disease is not well characterized. The objective of this study was to compare the presentation and clinical course of ACS in individuals with hemoglobin SC disease to that observed in individuals with hemoglobin SS disease. Procedure We retrospectively reviewed 71 inpatient episodes of ACS in patients with hemoglobin SC disease over a 20‐year period. Continuous and categorical data from index cases were compared with that from 71 control cases of ACS in patients with hemoglobin SS disease. Results Median length of hospitalization was shorter for hemoglobin SC episodes when compared to hemoglobin SS episodes (3.0 vs. 5.0 days, P < 0.001). In comparison to hemoglobin SS patients, a greater proportion of hemoglobin SC patients had a previous history of asthma or wheezing (50.7 vs. 33.8%, OR = 2.01 [1.02–3.96], P = 0.041) and a respiratory complaint of wheeze (11.3 vs. 2.8%, OR = 4.38 [0.90–21.4], P = 0.049). In our multivariate regression model, time to ACS diagnosis and total number of days of oxygen supplementation were independent predictors of length of hospitalization in both hemoglobin SC and SS episodes of ACS. Conclusions ACS appears to be less severe in children with hemoglobin SC disease compared to that in children with SS disease. Asthma and wheezing may represent more significant risk factors for the development of ACS in children with hemoglobin SC disease. Pediatr Blood Cancer 2011; 57: 289–293. © 2010 Wiley‐Liss, Inc.