Clinical correlates of acute pulmonary events in children and adolescents with sickle cell disease

Rabindra Paul, Caterina P. Minniti, Mehdi Nouraie, Lori Luchtman-Jones, Andrew Campbell, Sohail Rana, Onyinye Onyekwere, Deepika S. Darbari, Olaid Ajayi, Manuel Arteta, Gregory Ensing, Craig Sable, Niti Dham, Gregory J. Kato, Mark T. Gladwin, Oswaldo L. Castro, Victor R. Gordeuk

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Objectives: 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.

Original languageEnglish (US)
Pages (from-to)62-68
Number of pages7
JournalEuropean Journal of Haematology
Volume91
Issue number1
DOIs
StatePublished - Jul 2013
Externally publishedYes

Fingerprint

Sickle Cell Anemia
Lung
Sickle Hemoglobin
Pain
Acute Chest Syndrome
Genotype
Tricuspid Valve Insufficiency
Asthma
Leukocyte Count
Odds Ratio
Pulmonary Hypertension
Pneumonia
Logistic Models
Incidence

Keywords

  • Acute chest syndrome
  • Asthma
  • Pain
  • Sickle cell disease
  • Vaso-occlusive crisis

ASJC Scopus subject areas

  • Hematology

Cite this

Clinical correlates of acute pulmonary events in children and adolescents with sickle cell disease. / Paul, Rabindra; Minniti, Caterina P.; Nouraie, Mehdi; Luchtman-Jones, Lori; Campbell, Andrew; Rana, Sohail; Onyekwere, Onyinye; Darbari, Deepika S.; Ajayi, Olaid; Arteta, Manuel; Ensing, Gregory; Sable, Craig; Dham, Niti; Kato, Gregory J.; Gladwin, Mark T.; Castro, Oswaldo L.; Gordeuk, Victor R.

In: European Journal of Haematology, Vol. 91, No. 1, 07.2013, p. 62-68.

Research output: Contribution to journalArticle

Paul, R, Minniti, CP, Nouraie, M, Luchtman-Jones, L, Campbell, A, Rana, S, Onyekwere, O, Darbari, DS, Ajayi, O, Arteta, M, Ensing, G, Sable, C, Dham, N, Kato, GJ, Gladwin, MT, Castro, OL & Gordeuk, VR 2013, 'Clinical correlates of acute pulmonary events in children and adolescents with sickle cell disease', European Journal of Haematology, vol. 91, no. 1, pp. 62-68. https://doi.org/10.1111/ejh.12118
Paul, Rabindra ; Minniti, Caterina P. ; Nouraie, Mehdi ; Luchtman-Jones, Lori ; Campbell, Andrew ; Rana, Sohail ; Onyekwere, Onyinye ; Darbari, Deepika S. ; Ajayi, Olaid ; Arteta, Manuel ; Ensing, Gregory ; Sable, Craig ; Dham, Niti ; Kato, Gregory J. ; Gladwin, Mark T. ; Castro, Oswaldo L. ; Gordeuk, Victor R. / Clinical correlates of acute pulmonary events in children and adolescents with sickle cell disease. In: European Journal of Haematology. 2013 ; Vol. 91, No. 1. pp. 62-68.
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abstract = "Objectives: 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.",
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T1 - Clinical correlates of acute pulmonary events in children and adolescents with sickle cell disease

AU - Paul, Rabindra

AU - Minniti, Caterina P.

AU - Nouraie, Mehdi

AU - Luchtman-Jones, Lori

AU - Campbell, Andrew

AU - Rana, Sohail

AU - Onyekwere, Onyinye

AU - Darbari, Deepika S.

AU - Ajayi, Olaid

AU - Arteta, Manuel

AU - Ensing, Gregory

AU - Sable, Craig

AU - Dham, Niti

AU - Kato, Gregory J.

AU - Gladwin, Mark T.

AU - Castro, Oswaldo L.

AU - Gordeuk, Victor R.

PY - 2013/7

Y1 - 2013/7

N2 - Objectives: 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.

AB - Objectives: 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.

KW - Acute chest syndrome

KW - Asthma

KW - Pain

KW - Sickle cell disease

KW - Vaso-occlusive crisis

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