title: Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia.
abstract: OBJECTIVE
  To identify factors associated with frequent severe vaso-occlusive pain crises in a contemporary pediatric cohort of patients with sickle cell anemia (SCA) enrolled in a prospective study of pulmonary hypertension and the hypoxic response in sickle cell disease.
  
  
  METHODS
  Clinical and laboratory characteristics of children with SCA who had ≥3 severe pain crises requiring health care in the preceding year were compared with those of subjects with <3 such episodes.
  
  
  RESULTS
  Seventy-five children (20%) reported ≥3 severe pain episodes in the preceding year, and 232 (61%) had none. Frequent pain episodes were associated with older age (OR, 1.2; 95% CI, 1.1-1.3; P < .0001), α-thalassemia trait (OR 3.5; 1.6-6.7; P = .002), higher median hemoglobin (OR 1.7; 95% CI: 1.2-2.4; P < .003), and lower lactate dehydrogenase concentration (OR 1.82; 95% CI: 1.07-3.11; P = .027). Children with high pain frequency also had an increased iron burden (serum ferritin, 480 vs 198 μg/L; P = .006) and higher median tricuspid regurgitation jet velocity (2.41 vs 2.31 m/s; P = .001). Neither hydroxyurea use nor fetal hemoglobin levels were significantly different according to severe pain history.
  
  
  CONCLUSIONS
  In our cohort of children with SCA, increasing age was associated with higher frequency of severe pain episodes as were α-thalassemia, iron overload, higher hemoglobin and lower lactate dehydrogenase concentration, and higher tricuspid regurgitation velocity.
authors: Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
authors: Deepika S
authors: DS
authors: Darbari
authors: Onyinye
authors: O
authors: Onyekwere
authors: Mehdi
authors: M
authors: Nouraie
authors: Caterina P
authors: CP
authors: Minniti
authors: Lori
authors: L
authors: Luchtman-Jones
authors: Sohail
authors: S
authors: Rana
authors: Craig
authors: C
authors: Sable
authors: Gregory
authors: G
authors: Ensing
authors: Niti
authors: N
authors: Dham
authors: Andrew
authors: A
authors: Campbell
authors: Manuel
authors: M
authors: Arteta
authors: Mark T
authors: MT
authors: Gladwin
authors: Oswaldo
authors: O
authors: Castro
authors: James G
authors: JG
authors: Taylor
authors: Gregory J
authors: GJ
authors: Kato
authors: Victor
authors: V
authors: Gordeuk
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D015415:Biomarkers; D002648:Child; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D066298:In Vitro Techniques; D019190:Iron Overload / Q000503:physiopathology; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008297:Male; D010146:Pain / Q000097:blood / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology; D011446:Prospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D014262:Tricuspid Valve Insufficiency / Q000209:etiology / Q000503:physiopathology; D014652:Vascular Diseases / Q000097:blood / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology; D017085:alpha-Thalassemia / Q000503:physiopathology
chemical_list: D015415:Biomarkers; D006454:Hemoglobins; D007770:L-Lactate Dehydrogenase