Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia

Deepika S. Darbari, Onyinye Onyekwere, Mehdi Nouraie, Caterina P. Minniti, Lori Luchtman-Jones, Sohail Rana, Craig Sable, Gregory Ensing, Niti Dham, Andrew Campbell, Manuel Arteta, Mark T. Gladwin, Oswaldo Castro, James G. Taylor VI, Gregory J. Kato, Victor Gordeuk

Research output: Contribution to journalArticle

59 Citations (Scopus)

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. Study design: 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.

Original languageEnglish (US)
Pages (from-to)286-290
Number of pages5
JournalJournal of Pediatrics
Volume160
Issue number2
DOIs
StatePublished - Feb 2012
Externally publishedYes

Fingerprint

Sickle Cell Anemia
Pain
Tricuspid Valve Insufficiency
Thalassemia
L-Lactate Dehydrogenase
Hemoglobins
Fetal Hemoglobin
Iron Overload
Hydroxyurea
Ferritins
Pulmonary Hypertension
Iron
Prospective Studies
Pediatrics
Delivery of Health Care
Serum

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia. / Darbari, Deepika S.; Onyekwere, Onyinye; Nouraie, Mehdi; Minniti, Caterina P.; Luchtman-Jones, Lori; Rana, Sohail; Sable, Craig; Ensing, Gregory; Dham, Niti; Campbell, Andrew; Arteta, Manuel; Gladwin, Mark T.; Castro, Oswaldo; Taylor VI, James G.; Kato, Gregory J.; Gordeuk, Victor.

In: Journal of Pediatrics, Vol. 160, No. 2, 02.2012, p. 286-290.

Research output: Contribution to journalArticle

Darbari, DS, Onyekwere, O, Nouraie, M, Minniti, CP, Luchtman-Jones, L, Rana, S, Sable, C, Ensing, G, Dham, N, Campbell, A, Arteta, M, Gladwin, MT, Castro, O, Taylor VI, JG, Kato, GJ & Gordeuk, V 2012, 'Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia', Journal of Pediatrics, vol. 160, no. 2, pp. 286-290. https://doi.org/10.1016/j.jpeds.2011.07.018
Darbari, Deepika S. ; Onyekwere, Onyinye ; Nouraie, Mehdi ; Minniti, Caterina P. ; Luchtman-Jones, Lori ; Rana, Sohail ; Sable, Craig ; Ensing, Gregory ; Dham, Niti ; Campbell, Andrew ; Arteta, Manuel ; Gladwin, Mark T. ; Castro, Oswaldo ; Taylor VI, James G. ; Kato, Gregory J. ; Gordeuk, Victor. / Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia. In: Journal of Pediatrics. 2012 ; Vol. 160, No. 2. pp. 286-290.
@article{f85647e9ccbc47778e8c4fc5856742bd,
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. Study design: 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.",
author = "Darbari, {Deepika S.} and Onyinye Onyekwere and Mehdi Nouraie and Minniti, {Caterina P.} and Lori Luchtman-Jones and Sohail Rana and Craig Sable and Gregory Ensing and Niti Dham and Andrew Campbell and Manuel Arteta and Gladwin, {Mark T.} and Oswaldo Castro and {Taylor VI}, {James G.} and Kato, {Gregory J.} and Victor Gordeuk",
year = "2012",
month = "2",
doi = "10.1016/j.jpeds.2011.07.018",
language = "English (US)",
volume = "160",
pages = "286--290",
journal = "Journal of Pediatrics",
issn = "0022-3476",
publisher = "Mosby Inc.",
number = "2",

}

TY - JOUR

T1 - Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia

AU - Darbari, Deepika S.

AU - Onyekwere, Onyinye

AU - Nouraie, Mehdi

AU - Minniti, Caterina P.

AU - Luchtman-Jones, Lori

AU - Rana, Sohail

AU - Sable, Craig

AU - Ensing, Gregory

AU - Dham, Niti

AU - Campbell, Andrew

AU - Arteta, Manuel

AU - Gladwin, Mark T.

AU - Castro, Oswaldo

AU - Taylor VI, James G.

AU - Kato, Gregory J.

AU - Gordeuk, Victor

PY - 2012/2

Y1 - 2012/2

N2 - 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. Study design: 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.

AB - 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. Study design: 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.

UR - http://www.scopus.com/inward/record.url?scp=84855643775&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84855643775&partnerID=8YFLogxK

U2 - 10.1016/j.jpeds.2011.07.018

DO - 10.1016/j.jpeds.2011.07.018

M3 - Article

C2 - 21890147

AN - SCOPUS:84855643775

VL - 160

SP - 286

EP - 290

JO - Journal of Pediatrics

JF - Journal of Pediatrics

SN - 0022-3476

IS - 2

ER -