Charles R Esther

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Pulmonary lymphangiectasia (PL) is a rare disease characterized by dilation of the normal lung lymphatics. The disease was previously associated with a poor prognosis. However, several recent case reports and case studies indicate that survival is possible in the severe neonatal form of PL, and the prognosis is good for infants who present after the(More)
Extracellular UDP-sugars promote cellular responses by interacting with widely distributed P2Y(14) receptors, but the mechanisms by which these molecules are released from cells are poorly understood. Given the active role of UDP-sugars in glycosylation reactions within the secretory pathway, we hypothesized that UDP-sugar release includes an exocytotic(More)
BACKGROUND Although nontuberculous mycobacteria (NTM) are recognized pathogens in cystic fibrosis (CF), associations with clinical outcomes remain unclear. METHODS Microbiological data was obtained from 1216 CF patients over 8years (481+/-55patients/year). Relationships to clinical outcomes were examined in the subset (n=271, 203+/-23 patients/year) with(More)
, 246ra96 (2014); 6 Sci Transl Med et al. Deborah M. Cholon CFTR in cystic fibrosis F508 ∆ Potentiator ivacaftor abrogates pharmacological correction of Editor's Summary potentiators make CFTR less stable, accelerating the removal of this channel from the cell membrane. and Veit and coauthors now show that combining the two types of drugs does not work(More)
Primary pulmonary lymphangiectasia (PPL) is a rare disorder of unknown aetiology characterised by dilatation of the pulmonary lymphatics. PPL is widely reported to have a poor prognosis in the neonatal period and little is known about the clinical features of patients who survive the newborn period. The current authors report the outcome in nine patients(More)
BACKGROUND Adenosine and related purines have established roles in inflammation, and elevated airway concentrations are predicted in patients with COPD. However, accurate airway surface purine measurements can be confounded by stimulation of purine release during collection of typical respiratory samples. METHODS Airway samples were collected(More)
Purinergic signalling regulates airway defence mechanisms, suggesting that extracellular purines could serve as airway inflammation biomarkers in cystic fibrosis (CF). The purines adenosine triphosphate (ATP), adenosine diphosphate (ADP), adenosine monophosphate (AMP) and adenosine were measured in sputum from 21 adults (spontaneously expectorated from(More)
Exhaled breath condensate (EBC) collection is a simple and non-invasive method to sample airway secretions, but analysis is limited by extensive and variable dilution of airway secretions within the condensate. To overcome this limitation, we developed a sensitive and specific liquid chromatography/tandem mass spectrometry (LC/MS/MS) method to(More)
The physiological relevance of the purinergic signaling network for airway defenses is emerging through cumulating reports of abnormal ATP and adenosine (ADO) levels in the airway secretions of patients with asthma, chronic pulmonary obstructive diseases, cystic fibrosis and idiopathic pulmonary fibrosis. The consequences for airway defenses range from(More)
RATIONALE Although airway inflammation begins early in life in children with chronic respiratory diseases, current methods to assess this inflammation are invasive and entail significant risk. Measurement of exhaled breath condensate (EBC) purines and other biomarkers offers a less invasive method to assess airway inflammation; however, the feasibility and(More)