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

Williams, Sophia; Nussbaum, Eliezer; Yoonessi, Leila; Morphew, Tricia; Randhawa, Inderpal

doi:10.1007/s00408-014-9572-y

Cited by 7 publications

(9 citation statements)

References 35 publications

(70 reference statements)

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“…A similar prevalence was found in a retrospective cohort study conducted by Williams et al, 33 who demonstrated that 35.9% of the patients had a diagnosis of asthma and that a decline in FEV 1 as a percentage of predicted is associated with progression to pulmonary dysfunction. These findings underscore the need for the recognition of asthma, as well as the importance of longitudinal follow-up of pulmonary function, in this population.…”

Section: Discussionsupporting

confidence: 82%

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

et al. 2016

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Objective:To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods:This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome. Results:Of the 64 patients who underwent spirometry, 15 (23.4%) showed abnormal results: restrictive lung disease, in 8 (12.5%); and obstructive lung disease, in 7 (10.9%). Of the 69 patients who underwent the six-minute walk test, 18 (26.1%) showed abnormal results regarding the six-minute walk distance as a percentage of the predicted value for age, and there was a ≥ 3% decrease in SpO2 in 36 patients (52.2%). Abnormal pulmonary function was not significantly associated with any of the other variables studied, except for hypoxemia and restrictive lung disease. Conclusions:In this sample of children and adolescents with sickle cell disease, there was a significant prevalence of abnormal pulmonary function. The high prevalence of respiratory disorders suggests the need for a closer look at the lung function of this population, in childhood and thereafter.

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Section: Discussionsupporting

confidence: 82%

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

et al. 2016

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“…Treatments such as short-acting beta2 agonists and/or inhaled corticosteroids, are known to delay progression of pulmonary dysfunction (Williams et al, 2014). Treatments such as short-acting beta2 agonists and/or inhaled corticosteroids, are known to delay progression of pulmonary dysfunction (Williams et al, 2014).…”

Section: Discussionmentioning

confidence: 99%

“…The risk factors identified in our study could have possible implications for clinicians. Treatments such as short-acting beta2 agonists and/or inhaled corticosteroids, are known to delay progression of pulmonary dysfunction (Williams et al, 2014). In young children with SS homozygous or Sb 0 compound heterozygous SCD, the treatment of bronchial obstruction, or adenoidectomy and/or tonsillectomy could possibly modify the risk and course of CV.…”

Section: Discussionmentioning

confidence: 99%

Clinical and haematological risk factors for cerebral macrovasculopathy in a sickle cell disease newborn cohort: a prospective study

et al. 2016

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Children with sickle cell disease (SCD) have a significant vascular morbidity, especially cerebral macrovasculopathy (CV), detectable by transcranial Doppler. This study aimed to identify risk factors for CV using longitudinal biological and clinical data in a SCD newborn cohort followed at the Robert Debre Reference centre (n = 375 SS/Sβ(0) ). Median follow-up was 6·8 years (2677 patient-years). Among the 59 children presenting with CV, seven had a stroke. Overall, the incidence of CV was 2·20/100 patient-years [95% confidence interval (95% CI): 1·64-2·76] and the incidence of stroke was 0·26/100 patient-years (95% CI: 0·07-0·46). The cumulative risk of CV by age 14 years was 26·0% (95% CI: 20·0-33·3%). Risk factors for CV were assessed by a Cox model encompassing linear multivariate modelling of longitudinal quantitative variables. Years per upper-airway obstruction [Hazard ratio (HR) = 1·47; 95% CI: 1·05-2·06] or bronchial obstruction (HR = 1·76; 95% CI: 1·49-2·08) and reticulocyte count (HR = 1·82 per 50 × 10(9) /l increase; 95% CI: 1·10-3·01) were independent risk factors whereas fetal haemoglobin level (HR = 0·68 per 5% increase; 95% CI: 0·48-0·96) was protective. Alpha-thalassaemia was not protective in multivariate analysis (ancillary analysis n = 209). Specific treatment for upper or lower-airway obstruction and indirect targeting of fetal haemoglobin and reticulocyte count by hydroxycarbamide could potentially reduce the risk of CV.

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“…The authors also observed a history of asthma in one third of SCD patients, which is double the prevalence observed in the population of children and adolescents in the city of Belo Horizonte (17.8%), according to the International Study of Asthma and Allergies in Childhood . These findings underscore the need for the recognition of asthma, as well as the importance of longitudinal follow‐up of pulmonary function, in this population . This may also indicate that several inflammatory mechanisms are involved in the genesis of airway obstruction in SCD …”

Section: Discussionmentioning

confidence: 99%

Surfactant protein D as a marker for pulmonary complications in pediatric patients with sickle cell disease: Relation to lung function tests

Tantawy

Adly

Ebeid

et al. 2019

Pediatric Pulmonology

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Background: Surfactant protein D (SP-D) is considered a candidate biomarker for lung integrity and for disease progression. Aim:We determined the level of SP-D in children and adolescents with SCD and assessed its possible relation to pulmonary complications and lung function.Methods: Serum SP-D levels were assessed in 50 SCD patients compared with 30 healthy controls. High-resolution computerized tomography (HRCT) of the chest was done. Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV 1 ), FEV 1 /FVC% and forced expiratory flow rate during 25-75% of expiration (FEF25-75%) were determined.Results: SP-D was significantly higher in SCD patients than controls, particularly patients with sickle cell anemia than those with sickle β-thalassemia. SP-D levels were significantly associated with increasing severity of interstitial lung disease. The highest SP-D levels were observed among patients with restrictive lung disease followed by mixed type then obstructive lung disease. SP-D was positively correlated to HbS and serum ferritin while negatively correlated to duration of hydroxyurea treatment and parameters of pulmonary functions. ROC curve analysis revealed that SP-D cutoff value 720 ng/mL could significantly detect the presence of abnormal pulmonary function among SCD patients with 82% sensitivity and 88% specificity. Logistic regression analysis showed that SP-D is an independent factor related to abnormal pulmonary function in SCD.Conclusions: SP-D may be a promising biomarker for screening of SCD patients for risk of later pulmonary complications. K E Y W O R D S iron overload, pulmonary function tests, sickle cell disease, surfactant protein-D

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

Abstract: Purpose The pulmonary complications of sickle cell disease (SCD) are a leading cause of morbidity and mortality (MacLean et al.

Cited by 7 publications

References 35 publications

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

Clinical and haematological risk factors for cerebral macrovasculopathy in a sickle cell disease newborn cohort: a prospective study

Surfactant protein D as a marker for pulmonary complications in pediatric patients with sickle cell disease: Relation to lung function tests

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