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Erschienen in: Lung 3/2014

01.06.2014

Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

verfasst von: Sophia N. Williams, Eliezer Nussbaum, Leila Yoonessi, Tricia Morphew, Inderpal Randhawa

Erschienen in: Lung | Ausgabe 3/2014

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Abstract

Purpose

The pulmonary complications of sickle cell disease (SCD) are a leading cause of morbidity and mortality (MacLean et al. Am J Respir Crit Care Med 178:1055–1059, 2008; Klings et al. Am J Respir Crit Care Med 173:1264–1269, 2006; National Heart, Lung, and Blood Institute, 2009). Despite this recognition, predictive markers of lung dysfunction progression remain elusive (Klings et al. Am J Respir Crit Care Med 173:1264–1269, 2006; Platt et al. N Engl J Med 330:1639–1644, 1994; Caboot et al. Curr Opin Pediatr 20:279–287, 2008; Field et al. Am J Hematol 83:574–576, 2008; Shirlo et al. Peadiatr Respir Review 12:78–82, 2011). This study was designed describe the longitudinal progression and identify specific markers that influence bronchial disease in SCD.

Methods

A retrospective, chart review of 89 patients with SCD was conducted. All patients underwent spirometry in conjunction with body plethysmography as part of routine care. Eleven lung function variables were assessed, five of which were selected to establish patterns of normal, obstructive, restrictive, or mixed obstructive-restrictive physiology (Klings et al. Am J Respir Crit Care Med 173:1264–1269, 2006; Field et al. Am J Hematol 83:574–576, 2008).

Results

In the unadjusted model, forced expiratory volume in one second (FEV1)% of predicted trended downward with age, while total lung capacity (TLC)% of predicted showed a bimodal distribution and carbon monoxide diffusion capacity corrected for hemoglobin (DLCOcor)% of predicted remained stable. Adjusting for acute chest syndrome (ACS) episodes, medication status, and growth velocity (GV), the final model demonstrated that the downward trend between FEV1% of predicted with age was further influenced by the latter two factors.

Conclusions

Initial decline in FEV1% of predicted is associated with worsening pulmonary dysfunction over time. Independent of ACS episodes, the factors most influential on the progression of FEV1% predicted include the introduction of medications as well as the promotion of adequate prepubertal growth. Efforts to ensure normal prepubertal GV and treatment with bronchodilators, such as short-acting beta2 agonists and inhaled corticosteroids (ICS), should be considered at an early age to delay progression of pulmonary dysfunction.
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Metadaten
Titel
Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease
verfasst von
Sophia N. Williams
Eliezer Nussbaum
Leila Yoonessi
Tricia Morphew
Inderpal Randhawa
Publikationsdatum
01.06.2014
Verlag
Springer US
Erschienen in
Lung / Ausgabe 3/2014
Print ISSN: 0341-2040
Elektronische ISSN: 1432-1750
DOI
https://doi.org/10.1007/s00408-014-9572-y

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