Infants and Young Children with Cystic Fibrosis Have High Levels of Serum Sialyl Lewisa Antigen

Frates, Ralph C.; Scott, Sandra; Hammond, KeithB.; Brooks, John O.; Richardson, C. Joan; Roberts, Diana

doi:10.1203/00006450-199504000-00013

Cited by 5 publications

(5 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In contrast with the distribution of Clara cells, these cells are located primarily in the higher airway rather than the bronchioles. Although elevated respiratory specific mucin antigens have been described in the circulation in a number of respiratory diseases, including cystic fibrosis, 65 interstitial pneumonia, pulmonary fibrosis, tuberculosis, 66 , 67 lung transplant 68 and ARDS, 69 the manner in which the peptides gain access is unclear.…”

Section: The Ideal Marker Of Alveolocapillary Permeabilitymentioning

confidence: 99%

PARTITIONING LUNG AND PLASMA PROTEINS: CIRCULATING SURFACTANT PROTEINS AS BIOMARKERS OF ALVEOLOCAPILLARY PERMEABILITY^‡

Doyle¹,

Nicholas²,

Bersten

1999

Clin Exp Pharma Physio

View full text Add to dashboard Cite

1. The alveolocapillary membrane faces an extraordinary task in partitioning the plasma and lung hypophase proteins, with a surface area approximately 50-fold that of the body and only 0.1-0.2 micron thick. 2. Lung permeability is compromised under a variety of circumstances and the delineation between physiological and pathological changes in permeability is not always clear. Although the tight junctions of the epithelium, rather than the endothelium, are regarded as the major barrier to fluid and protein flux, it is becoming apparent that the permeability of both are dynamically regulated. 3. Whereas increased permeability and the flux of plasma proteins into the alveolar compartment has dire consequences, fortuitously the flux of surfactant proteins from the airspaces into the circulation may provide a sensitive means of non-invasively monitoring the lung, with important implications for treatment modalities. 4. Surfactant proteins are unique in that they are present in the alveolar hypophase in high concentrations. They diffuse down their vast concentration gradients (approximately 1:1500-7000) into the circulation in a manner that reflects lung function and injury score. Surfactant proteins vary markedly in size (approximately 20-650 kDa) and changes in the relative amounts appear particularly diagnostic with regard to disease severity. Alveolar levels of surfactant proteins remain remarkably constant despite respiratory disease and, unlike the flux of plasma proteins into the alveolus, which may reach equilibrium in acute lung injury, the flux of surfactant proteins is unidirectional because of the concentration gradient and because they are rapidly cleared from the circulation. 5. Ultimately, the diagnostic usefulness of surfactant proteins as markers of alveolocapillary permeability will demand a sound understanding of their kinetics through the vascular compartment.

show abstract

Section: The Ideal Marker Of Alveolocapillary Permeabilitymentioning

confidence: 99%

PARTITIONING LUNG AND PLASMA PROTEINS: CIRCULATING SURFACTANT PROTEINS AS BIOMARKERS OF ALVEOLOCAPILLARY PERMEABILITY^‡

Doyle¹,

Nicholas²,

Bersten

1999

Clin Exp Pharma Physio

View full text Add to dashboard Cite

show abstract

“…This issue has been partially resolved in a few studies. Frates et al [14] found high sialyl Le a antigen levels in young CF patients, but normal levels in patients with asthma and bronchopulmonary dysplasia. In another study, they demonstrated the CA 19-9 levels to be significantly higher in CF patients than in bronchiectatic non-CF patients; all patients of both groups had Pseudomonas in their sputum [15].…”

Section: Discussionmentioning

confidence: 98%

“…Robinson et al [16] found significantly higher serum mucin levels in CF patients than in patients with chronic obstructive pulmonary disease with a strong "bronchitic" component, who produced up to one-half a cup of sputum daily. Elevated serum mucin levels are associated with CF rather than with either purulent or dry non-CF lung disease [14].…”

Section: Discussionmentioning

confidence: 99%

Serum CA 19?9 levels as a diagnostic marker in cystic fibrosis patients with borderline sweat tests

Augarten

Berman

Aviram

et al. 2003

Clinical and Experimental Medicine

View full text Add to dashboard Cite

Patients with normal or borderline sweat tests present a diagnostic challenge. In spite of the availability of genetic analysis and measurement of nasal potential difference, there is still uncertainty in diagnosing cystic fibrosis in some patients. CA 19-9 is a tumor-associated antigen whose levels were previously found to be elevated in some cystic fibrosis patients. We investigated whether serum CA 19-9 levels can contribute to establishing the diagnosis of cystic fibrosis in patients with a borderline sweat test, and evaluated the influence of different clinical variables on CA 19-9 levels. Serum CA 19-9 levels were measured in 82 cystic fibrosis patients grouped according to their genotype and in 38 healthy individuals. Group A included 50 patients who carried two mutations previously found to be associated with a pathological sweat test and pancreatic insufficiency (DeltaF508, W1282X, G542X, N1303K, and S549R). Group B included 13 compound heterozygote cystic fibrosis patients who carried one mutation known to cause mild disease with a borderline or normal sweat test and pancreatic sufficiency (3849+10kb C-->T, 5T). Group C included 38 normal controls. Nineteen cystic fibrosis patients carried at least one unidentified mutation. An association between CA 19-9 levels and age, pulmonary function, pancreatic status, sweat chloride, previous pancreatitis, serum lipase, meconium ileus, distal intestinal obstruction, liver disease, and diabetes was investigated. The distribution of CA 19-9 levels was significantly different between the three groups ( p<0.01); high CA 19-9 levels were found in 60% (30/50) of group Apatients and in 46.6% (6/13) of group B patients, but in only 5.2% (2/38) of the controls. CA 19-9 levels were inversely related to forced expiratory volume in 1 s, while no association was found with the other clinical parameters examined. Our findings suggest that the serum CA 19-9 in cystic fibrosis patients originates in the respiratory system, and has a useful ancillary role, particularly when diagnostic uncertainty exists. Hence, the diagnosis of cystic fibrosis should be considered in patients with borderline sweat tests and high CA 19-9 levels, but normal levels do not exclude cystic fibrosis.

show abstract

“…The human respiratory mucins belong to a broad family of different mucin peptides characterized by diverse carbohydrate side chains. With the development of monoclonal antibodies (mAbs), several studies have been done to assess the relationship of lung diseases and mucins (2)(3)(4)(5)(6). Previous studies suggest that the mucin antigen in the serum of patients with pulmonary diseases is largely of pulmonary origin (2).…”

mentioning

confidence: 99%

“…Previous studies suggest that the mucin antigen in the serum of patients with pulmonary diseases is largely of pulmonary origin (2). Serum mucin levels are increased in cystic fibrosis (CF) patients, and serum levels of mucin-associated antigen correlate with the pulmonary inflammation in these patients (3,4). Kohno and colleagues reported that KL-6 antigen, a human MUC-1 mucin expressed on Type II pneumocytes, was a sensitive serum marker for evaluating the disease activity of interstitial pneumonia and pulmonary fibrosis and the extent of pulmonary tuberculosis (5,6).…”

mentioning

confidence: 99%

Elevated Serum Levels of Mucin-associated Antigen in Patients with Acute Respiratory Distress Syndrome

Shih

Yang

et al. 1997

Am J Respir Crit Care Med

View full text Add to dashboard Cite

Increased serum levels of mucin-associated antigen have been previously demonstrated in patients with cystic fibrosis (CF) and interstitial pneumonia, and in lung-transplant recipients. The present study assessed the serum airway mucin levels in patients with acute respiratory distress syndrome (ARDS). An enzyme-linked immunosorbent assay (ELISA) method with a human-airway-mucin-specific monoclonal antibody (17Q2) was used to measure serum mucin levels in normal subjects, chronic smokers, patients with chronic bronchitis and other pulmonary diseases, patients with acute cardiogenic lung edema, and patients with ARDS. The serum mucin levels measured 9.9 +/- 0.8 ng/ml (mean +/- SEM, n = 59) in normal subjects, 12.7 +/- 1.6 ng/ml (n = 29) in chronic smokers, 21.8 +/- 1.9 ng/ml (n = 28) in patients with chronic bronchitis and other pulmonary diseases, 9.0 +/- 3.1 ng/ml (n = 5) in patients with acute cardiogenic lung edema. The serum mucin level was 53.8 +/- 6.6 ng/ml (n = 13) in patients with ARDS (p < 0.05, as compared with the four other groups). Serial measurements of serum mucin levels were obtained in patients with ARDS. Statistical analysis showed an inverse correlation of serial measurements of serum mucin with static respiratory-system compliance (p = 0.021), an inverse correlation of sequential serum mucin levels and log(Pa(O2)/Fl(O2)) (p = 0.016), and a positive correlation of sequential serum mucin levels and lung injury score (LIS) (p = 0.019). Gel-filtration analysis showed that mucin-associated antigens in ARDS sera were polydispersed and smaller than the antigens in normal sera. This study indicates that an increasing amount of degraded mucin occurs in patients with ARDS.

show abstract

Infants and Young Children with Cystic Fibrosis Have High Levels of Serum Sialyl Lewisa Antigen

Cited by 5 publications

References 15 publications

PARTITIONING LUNG AND PLASMA PROTEINS: CIRCULATING SURFACTANT PROTEINS AS BIOMARKERS OF ALVEOLOCAPILLARY PERMEABILITY^‡

PARTITIONING LUNG AND PLASMA PROTEINS: CIRCULATING SURFACTANT PROTEINS AS BIOMARKERS OF ALVEOLOCAPILLARY PERMEABILITY^‡

Serum CA 19?9 levels as a diagnostic marker in cystic fibrosis patients with borderline sweat tests

Elevated Serum Levels of Mucin-associated Antigen in Patients with Acute Respiratory Distress Syndrome

Contact Info

Product

Resources

About

Infants and Young Children with Cystic Fibrosis Have High Levels of Serum Sialyl Lewisa Antigen

Cited by 5 publications

References 15 publications

PARTITIONING LUNG AND PLASMA PROTEINS: CIRCULATING SURFACTANT PROTEINS AS BIOMARKERS OF ALVEOLOCAPILLARY PERMEABILITY‡

PARTITIONING LUNG AND PLASMA PROTEINS: CIRCULATING SURFACTANT PROTEINS AS BIOMARKERS OF ALVEOLOCAPILLARY PERMEABILITY‡

Serum CA 19?9 levels as a diagnostic marker in cystic fibrosis patients with borderline sweat tests

Elevated Serum Levels of Mucin-associated Antigen in Patients with Acute Respiratory Distress Syndrome

Contact Info

Product

Resources

About

PARTITIONING LUNG AND PLASMA PROTEINS: CIRCULATING SURFACTANT PROTEINS AS BIOMARKERS OF ALVEOLOCAPILLARY PERMEABILITY^‡

PARTITIONING LUNG AND PLASMA PROTEINS: CIRCULATING SURFACTANT PROTEINS AS BIOMARKERS OF ALVEOLOCAPILLARY PERMEABILITY^‡