Lung function in infants with sickle cell disease

Koumbourlis, Anastassios C.; Hurlet‐Jensen, Anne; Bye, Michael R.

doi:10.1002/(sici)1099-0496(199710)24:4<277::aid-ppul6>3.0.co;2-h

Cited by 75 publications

(53 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…There are a few studies, including Koumbourlis et al, that validate related findings of the presence of abnormal Lung (2014) 192:385-393 391 lung function in infants with SCD long before the onset of ACS episodes [41]. Similarly, no discernible association between lung function and prior ACS episodes existed in adolescents with SCD [42].…”

Section: Impact Of Medication Status Gv and Acsmentioning

confidence: 89%

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

Williams

Nussbaum

Yoonessi

et al. 2014

Lung

View full text Add to dashboard Cite

show abstract

Section: Impact Of Medication Status Gv and Acsmentioning

confidence: 89%

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

Williams

Nussbaum

Yoonessi

et al. 2014

Lung

View full text Add to dashboard Cite

show abstract

“…2,7,31 Moreover, this leukocytosis correlates well with severity of disease and early mortality in 2 large studies, 5,6 although a recent study did not find relationship. 48 Also seen is increased AH in children 9,11,49 and infants 50 in SCD. Moreover, LTB 4 26,51 and LTE 4 27 levels are found to be increased in SCD at baseline, which further increased during episodes of VOC and ACS.…”

Section: Discussionmentioning

confidence: 99%

Placenta growth factor induces 5-lipoxygenase–activating protein to increase leukotriene formation in sickle cell disease

Patel

Gonsalves

Yang

et al. 2009

Blood

View full text Add to dashboard Cite

IntroductionInflammation is increasingly recognized as central to the pathophysiology of sickle cell disease (SCD) and is manifest as leukocytosis, elevated levels of inflammatory cytokines, and activation of neutrophils, monocytes, and endothelial cells. [1][2][3][4] It is present at steady state and is strongly associated with acute painful events, acute chest, and early mortality. 5,6 Current evidence strongly suggests that inflammation contributes to the endothelial cell dysfunction, potentiates vasoocclusion, and may also give rise to the airway hyperreactivity (AH) that often accompanies SCD. [7][8][9][10] Also intriguing is the spectrum of lung disease seen in this patient population, which spans from an increased incidence of AH and obstructive lung disease in children, [11][12][13] to restrictive lung disease and pulmonary vascular remodeling, which is associated with pulmonary hypertension in adults. [14][15][16][17][18] Leukotrienes (LT) mediate both inflammation and AH. 19-22 5-Lipoxygenase (5-LO) and its activating partner, 5-lipoxygenase activating protein (FLAP), catalyze the production of LT from arachidonic acid (AA) by generating 5-hydroperoxyeicostatraenoic acid (5-HPETE) and leukotriene A 4 (LTA 4 ). LTA 4 is the pivotal intermediate from which other LTs (ie, LTB 4 and cysteinyl LT [CysLT], LTC 4 , LTD 4 , and LTE 4 ) are formed. 20 LTB 4 is one of the most potent chemoattractant for neutrophils, eosinophils, and mediator of inflammation. CysLT, on the other hand, are potent bronchoconstrictors that play an important role in edema, inflammation, and mucus secretion in asthma and were previously termed "slow releasing substances." 23 LT play an important role in the pathogenesis of inflammatory disorders, specifically asthma, rheumatoid arthritis, and inflammatory bowel disease. [19][20][21] Studies by Bigby and coworkers 24,25 have shown that both tumor necrosis factor-␣ (TNF-␣) and lipopolysaccharide (LPS) induce the expression of FLAP in THP-1 cells. These studies showed the importance of nuclear factor-B (NF-B) and CCAAT/enhancer binding protein (C/EBP) transcription factors in the LPS-mediated FLAP expression. 24 LTB 4 levels are higher in SCD patients at steady state, which are further increased in vasoocclusive pain crises (VOC) and acute chest syndrome (ACS). 26 Very recently, increased LTE 4 has been observed in patients with SCD, which is associated with a higher incidence of pain. 27 However, less is understood about how LTs are increased in SCD at the molecular level.Placenta growth factor (PlGF) is an angiogenic growth factor with similar effects on endothelium as vascular endothelial growth factor (VEGF) and is primarily expressed by placental trophoblasts. [28][29][30] More recently, we and others show that erythroid cells, but not other hematopoietic cells, produce PlGF, and its expression is high in SCD and thalassemia. 31,32 VEGFR1 is its cognate receptor and is expressed on endothelial cells, alveolar epithelial cells, mast cells, and monocytes. We have previously shown that...

show abstract

“…Restrictive and obstructive lung function abnormalities are common. Restrictive lung disease is associated with increasing age (Sylvester et al, 2004;MacLean et al, 2008) and obstructive defects can occur even in young children with SCD (Koumbourlis et al, 2001(Koumbourlis et al, , 1997. Hence, it is important to understand the etiology of such abnormalities; mechanistic insights are necessary to better inform therapy to hopefully prevent SCD pulmonary complications.…”

Section: Introductionmentioning

confidence: 99%

Lung function, transfusion, pulmonary capillary blood volume and sickle cell disease

Lunt¹,

McGhee

Robinson

et al. 2016

Respiratory Physiology & Neurobiology

View full text Add to dashboard Cite

Lung function abnormalities occur in children with sickle cell disease (SCD) and may be associated with elevated pulmonary blood volume. To investigate that association, we determined whether blood transfusion in SCD children acutely increased pulmonary capillary blood volume (PCBV) and increased respiratory system resistance (Rrs5). Measurements of Rrs5 and spirometry were made before and after blood transfusion in 18 children, median age 14.2 (6.6-18.5) years. Diffusing capacity for carbon monoxide and nitric oxide were assessed to calculate the PCBV. Post transfusion, the median Rrs5 had increased from 127.4 to 141.3% predicted (p<0.0001) and pulmonary capillary blood volume from 39.7 to 64.1 ml/m2 (p<0.0001); forced expiratory volume in one second (p=0.0056) and vital capacity (p=0.0008) decreased. The increase in Rrs5 correlated with the increase in PCBV (r=0.50, p=0.0493). Increased pulmonary capillary blood volume may at least partially explain the lung function abnormalities in SCD children.

show abstract

Lung function in infants with sickle cell disease

Cited by 75 publications

References 20 publications

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

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

Placenta growth factor induces 5-lipoxygenase–activating protein to increase leukotriene formation in sickle cell disease

Lung function, transfusion, pulmonary capillary blood volume and sickle cell disease

Contact Info

Product

Resources

About