title: Clinical correlates of acute pulmonary events in children and adolescents with sickle cell disease.
abstract: OBJECTIVE
  We aimed to identify risk factors for acute pulmonary events in children and adolescents in the Pulmonary Hypertension and the Hypoxic Response in SCD (PUSH) study.
  
  
  METHODS
  Patients with hemoglobin SS (n = 376) and other sickle cell genotypes (n = 127) aged 3-20 yrs were studied at four centers in a cross-sectional manner. A subgroup (n = 293) was followed for a median of 21 months (range 9-35).
  
  
  RESULTS
  A patient-reported history of one or more acute pulmonary events, either acute chest syndrome (ACS) or pneumonia, was obtained in 195 hemoglobin SS patients (52%) and 51 patients with other genotypes (40%). By logistic regression, history of acute pulmonary events was independently associated with patient-reported history of asthma (P < 0.0001), older age (P = 0.001), >3 severe pain episodes in the preceding 12 months (P = 0.002), higher tricuspid regurgitation velocity (TRV) (P = 0.028), and higher white blood cell (WBC) count (P = 0.043) among hemoglobin SS patients. History of acute pulmonary events was associated with >3 severe pain episodes (P = 0.009) among patients with other genotypes. During follow-up, 43 patients (15%) had at least one new ACS episode including 11 without a baseline history of acute pulmonary events. History of acute pulmonary events (odds ratio 5.0; P < 0.0001) and younger age (odds ratio 0.9; P = 0.007) were independently associated with developing a new episode during follow-up.
  
  
  CONCLUSIONS
  Asthma history, frequent pain, and higher values for TRV and WBC count were independently associated with history of acute pulmonary events in hemoglobin SS patients and frequent pain was associated in those with other genotypes. Measures to reduce pain episodes and control asthma may help to decrease the incidence of acute pulmonary events in SCD.
authors.affiliation: Howard University, Washington, DC, USA.
authors.forename: Rabindra
authors.initials: R
authors.lastname: Paul
authors.forename: Caterina P
authors.initials: CP
authors.lastname: Minniti
authors.forename: Mehdi
authors.initials: M
authors.lastname: Nouraie
authors.forename: Lori
authors.initials: L
authors.lastname: Luchtman-Jones
authors.forename: Andrew
authors.initials: A
authors.lastname: Campbell
authors.forename: Sohail
authors.initials: S
authors.lastname: Rana
authors.forename: Onyinye
authors.initials: O
authors.lastname: Onyekwere
authors.forename: Deepika S
authors.initials: DS
authors.lastname: Darbari
authors.forename: Olaid
authors.initials: O
authors.lastname: Ajayi
authors.forename: Manuel
authors.initials: M
authors.lastname: Arteta
authors.forename: Gregory
authors.initials: G
authors.lastname: Ensing
authors.forename: Craig
authors.initials: C
authors.lastname: Sable
authors.forename: Niti
authors.initials: N
authors.lastname: Dham
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Kato
authors.forename: Mark T
authors.initials: MT
authors.lastname: Gladwin
authors.forename: Oswaldo L
authors.initials: OL
authors.lastname: Castro
authors.forename: Victor R
authors.initials: VR
authors.lastname: Gordeuk
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D005838:Genotype; D006451:Hemoglobin, Sickle / Q000235:genetics*; D006801:Humans; D000860:Hypoxia; D008171:Lung Diseases / Q000150:complications* / Q000175:diagnosis*; D008297:Male; D012044:Regression Analysis; D012307:Risk Factors; D013997:Time Factors; D014652:Vascular Diseases / Q000150:complications / Q000175:diagnosis; D055815:Young Adult
chemical_list: D006451:Hemoglobin, Sickle
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