title: Increased risk of severe vaso-occlusive episodes after initial acute chest syndrome in children with sickle cell anemia less than 4 years old: Sleep and asthma cohort.
abstract: Previous studies have shown that the highest incidence of acute chest syndrome (ACS) in sickle cell disease occurs in children <4 years old, and a history of ACS at this age is a risk factor for future ACS episodes. However, the interval associated with the highest risk of subsequent ACS or severe pain is not known. Through this mixed retrospective-prospective observational study, the Sleep and Asthma Cohort, we sought to determine the interval after an initial ACS episode during which the majority of children <4 years old are rehospitalized for ACS or severe pain. The cumulative prevalence of rehospitalization for ACS or severe pain within 6 months, 1 years, and 2 years was calculated for children with an initial ACS episode <4 years old and compared to children with an initial ACS episode ≥4 years old. A total of 44.8% and 55.2% of participants had an initial ACS episode <4 years and ≥4 years old (Range: 4-17.7 years), respectively. At 1 year following the initial ACS episode, children <4 years old had a significantly higher cumulative prevalence of rehospitalizations for ACS or pain as compared to children ≥4 years of age, 62.5 and 39.1%, respectively (P = 0.009). After initial ACS episodes, the majority of children <4 years old will be rehospitalized for ACS or severe pain within one year, suggesting the need for a therapeutic intervention for this high-risk group.
authors.affiliation: Doris Duke Clinical Research Mentorship Program, Vanderbilt University School of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.
authors.forename: Leah D
authors.initials: LD
authors.lastname: Vance
authors.forename: Mark
authors.initials: M
authors.lastname: Rodeghier
authors.forename: Robyn T
authors.initials: RT
authors.lastname: Cohen
authors.forename: Carol L
authors.initials: CL
authors.lastname: Rosen
authors.forename: Fenella J
authors.initials: FJ
authors.lastname: Kirkham
authors.forename: Robert C
authors.initials: RC
authors.lastname: Strunk
authors.forename: Michael R
authors.initials: MR
authors.lastname: DeBaun
mesh_terms: D056586:Acute Chest Syndrome / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D001249:Asthma / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010146:Pain / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D010359:Patient Readmission / Q000706:statistics & numerical data; D011446:Prospective Studies; D012189:Retrospective Studies; D012307:Risk Factors; D012890:Sleep