Airway Inflammation and Lung Function in Sickle Cell Disease

De, Aliva; Agrawal, Sabhyata; Morrone, Kerry; Zhang, Jinghang; Bjorklund, Nicole L.; Manwani, Deepa; Rastogi, Deepa

doi:10.1089/ped.2019.1014

Cited by 13 publications

(10 citation statements)

References 60 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Conversely, also children with anemic diseases such as sickle cell disease ( 74 , 75 ) or beta-thalassemia major ( 76 – 79 ) (CGMF- 201801200B0) are more likely to have atopic diseases and suffer from asthma ( 80 , 81 ), indicating that iron itself is involved in the etiology of atopic diseases. Adults and children diagnosed with atopic dermatitis are also at greater risk of developing associated autoimmune diseases such as Crohn’s disease, pernicious anemia, autoimmune hypothyroidism, rheumatoid and psoriatic arthritis, vitiligo, and alopecia areata ( 82 ).…”

Section: Micronutrientsmentioning

confidence: 99%

Lack of iron, zinc, and vitamins as a contributor to the etiology of atopic diseases

et al. 2023

View full text Add to dashboard Cite

Micronutritional deficiencies are common in atopic children suffering from atopic dermatitis, food allergy, rhinitis, and asthma. A lack of iron, in particular, may impact immune activation with prolonged deficiencies of iron, zinc, vitamin A, and vitamin D associated with a Th2 signature, maturation of macrophages and dendritic cells (DCs), and the generation of IgE antibodies. In contrast, the sufficiency of these micronutrients establishes immune resilience, promotion of regulatory cells, and tolerance induction. As micronutritional deficiencies mimic an infection, the body’s innate response is to limit access to these nutrients and also impede their dietary uptake. Here, we summarize our current understanding of the physiological function of iron, zinc, and vitamins A and D in relation to immune cells and the clinical consequences of deficiencies in these important nutrients, especially in the perinatal period. Improved dietary uptake of iron is achieved by vitamin C, vitamin A, and whey compounds, whereas zinc bioavailability improves through citrates and proteins. The addition of oil is essential for the dietary uptake of beta-carotene and vitamin D. As for vitamin D, the major source comes via sun exposure and only a small amount is consumed via diet, which should be factored into clinical nutritional studies. We summarize the prevalence of micronutritional deficiencies of iron, zinc, and vitamins in the pediatric population as well as nutritional intervention studies on atopic diseases with whole food, food components, and micronutrients. Dietary uptake via the lymphatic route seems promising and is associated with a lower atopy risk and symptom amelioration. This review provides useful information for clinical studies and concludes/emphasizes that a healthy, varied diet containing dairy products, fish, nuts, fruits, and vegetables as well as supplementing foods or supplementation with micronutrients as needed is essential to combat the atopic march.

show abstract

Section: Micronutrientsmentioning

confidence: 99%

Lack of iron, zinc, and vitamins as a contributor to the etiology of atopic diseases

et al. 2023

View full text Add to dashboard Cite

show abstract

“…Moreover, downstream products of the haemolytic arginine-nitric oxide pathway may contribute to asthma and airway remodelling [32], as well as the increased levels of cysteinyl leukotrienes and leukotriene B 4 in SCD patients (involved in eosinophilic asthma and neutrophilic inflammation, respectively) [33,34]. Overall, these mechanisms result in increased levels of T-helper 1, T-helper 2 and monocytic inflammatory markers in patients with SCD and asthma or asthma-like features compared with non-SCD subjects with atopic asthma [35].…”

Section: Asthma and Wheezingmentioning

confidence: 99%

“…poor compliance to ICS, use of wrong inhaler, poor inhaler technique), comorbidities (e.g. allergic rhinitis, dysfunctional breathing) or the presence of a predominant non-eosinophilic component of airway inflammation [35], or may indicate the need to reconsider the appropriateness of diagnosis. Given the potential role of the leukotriene pathway in the pathogenesis of SCD-related morbidity [46], we often use cysteinyl leukotriene receptor antagonist (montelukast) for chronic asthma prophylaxis in this group.…”

Section: Asthma and Wheezingmentioning

confidence: 99%

Management of chronic respiratory complications in children and adolescents with sickle cell disease

Arigliani

Gupta

2020

Eur Respir Rev

View full text Add to dashboard Cite

Sickle cell disease (SCD) is a life-threatening hereditary blood disorder that affects millions of people worldwide, especially in sub-Saharan Africa. This condition has a multi-organ involvement and highly vascularised organs, such as the lungs, are particularly affected. Chronic respiratory complications of SCD involve pulmonary vascular, parenchymal and airways alterations. A progressive decline of lung function often begins in childhood. Asthma, sleep-disordered breathing and chronic hypoxaemia are common and associated with increased morbidity. Pulmonary hypertension is a serious complication, more common in adults than in children. Although there is a growing attention towards respiratory care of patients with SCD, evidence regarding the prognostic meaning and optimal management of pulmonary issues in children with this condition is limited.This narrative review presents state-of-the-art evidence regarding the epidemiology, pathophysiology and therapeutic options for chronic respiratory complications commonly seen in paediatric patients with SCD. Furthermore, it highlights the gaps in the current knowledge and indicates future directions for studies that aim to improve our understanding of chronic respiratory complications in children with SCD.

show abstract

“…Acute chest syndrome (ACS), an acute complication in SCD [ 11 ], is frequently precipitated by pulmonary infections and inflammation, and complicated by factors such as fat emboli to the lungs, pulmonary infarction, hypoventilation from pain and regional atelectasis [ 7 ]. A range of pulmonary function test (PFT) abnormalities have been described in SCD including obstructive defects, airway hyper-responsiveness, restrictive defects and diffusion defects [ 12 – 18 ]. The consequences of ACS on future lung function have not been well characterized, with contradictory reports of an association of ACS with a decline in lung function [ 19 – 23 ].…”

Section: Introductionmentioning

confidence: 99%

Acute chest syndrome, airway inflammation and lung function in sickle cell disease

Williams

Yao

et al. 2023

PLoS ONE

Self Cite

View full text Add to dashboard Cite

Background Acute chest syndrome (ACS) is an acute complication in SCD but its effects on lung function are not well understood. Inflammation is a key component of SCD pathophysiology but with an unclear association with lung function. We hypothesized that children with ACS had worse lung function than children without ACS and aimed to investigate the association of lung function deficits with inflammatory cytokines. Methods Patients enrolled in a previous 2-year randomized clinical trial who had consented to future data use, were enrolled for the present exploratory study. Patients were categorized into ACS and non-ACS groups. Demographic and clinical information were collected. Serum samples were used for quantification of serum cytokines and leukotriene B4 levels and pulmonary function tests (PFTs) were assessed. Results Children with ACS had lower total lung capacity (TLC) at baseline and at 2 years, with a significant decline in forced expiratory volume in 1 sec (FEV1) and mid-maximal expiratory flow rate (FEF25-75%) in the 2 year period (p = 0.015 and p = 0.039 respectively). For children with ACS, serum cytokines IL-5, and IL-13 were higher at baseline and at 2 years compared to children with no ACS. IP-10 and IL-6 were negatively correlated with PFT markers. In multivariable regression using generalized estimating equation approach for factors predicting lung function, age was significantly associated FEV1 (p = 0.047) and ratio of FEV1 and forced vital capacity (FVC)- FEV1/FVC ratio (p = 0.006); males had lower FEV1/FVC (p = 0.035) and higher TLC (p = 0.031). Asthma status was associated with FEV1 (p = 0.017) and FVC (p = 0.022); history of ACS was significantly associated with TLC (p = 0.027). Conclusion Pulmonary function abnormalities were more common and inflammatory markers were elevated in patients with ACS, compared with those without ACS. These findings suggest airway inflammation is present in children with SCD and ACS, which could be contributing to impaired pulmonary function.

show abstract

Airway Inflammation and Lung Function in Sickle Cell Disease

Cited by 13 publications

References 60 publications

Lack of iron, zinc, and vitamins as a contributor to the etiology of atopic diseases

Lack of iron, zinc, and vitamins as a contributor to the etiology of atopic diseases

Management of chronic respiratory complications in children and adolescents with sickle cell disease

Acute chest syndrome, airway inflammation and lung function in sickle cell disease

Contact Info

Product

Resources

About