Jan Sulc

Bronchoalveolar lavage fluid cellular characteristics, functional parameters and cytokine and chemokine levels in interstitial lung diseases.

Idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis (HP) and sarcoidosis belong to interstitial lung diseases (ILD) where an imbalance of regulatory, profibrotic and antifibrotic cytokines is hypothesized. The relationship of bronchoalveolar lavage (BAL) fluid (BALF) cytokines, BALF cell profile and ILD course is supposed. The aim of our study was to correlate BALF cytokine and chemokine levels with BALF cellular characteristics and lung function parameters in different ILD. Twenty‐two sarcoidosis, seven IPF and 11 HP patients underwent lung function tests and BAL. The BALF differential cell counts and superficial cell markers were characterized, and MCP‐1, MIP‐1α, MIP‐1β, RANTES, epithelial neutrophil‐activating protein (ENA)‐78, FGF, G‐CSF, GM‐CSF, IFN‐γ, interleukin (IL)‐1α, IL‐1RA, IL‐1β, ‐2β, ‐4β, ‐5β, ‐6β, ‐8β, ‐10β, ‐17β, tumour necrosis factor (TNF)‐α, thromobopoietin (Tpo) and vascular endothelial growth factor (VEGF) values measured. The BALF VEGF values were highest in sarcoidosis (P = 0.0526). IL‐1RA values were higher in IPF and HP compared with sarcoidosis (P = 0.0334). IL‐8/ENA‐78 ratio positively correlated with BALF neutrophil counts in IPF (r = 0.89, P = 0.04). Vital capacity and TLCO values positively correlated with VEGF and negatively with IL‐8 BALF levels in all ILDs but the correlations were most significant in sarcoidosis group. We suppose that VEGF plays a role in ILDs’ early phases and has rather angiogenic than profibrotic effect. On the contrary, IL‐8 is probably upregulated in advanced ILDs with prominent fibrosis and marked lung functions decline. We state that BALF VEGF, IL‐8 and ENA‐78 levels and IL‐8/ENA‐78 ratio could become useful markers of ILDs’ phase, activity and prognosis. They might also be helpful in treatment modality choice.

Stabilizing function of the diaphragm: dynamic MRI and synchronized spirometric assessment

The aim was to describe diaphragmatic behavior during postural limb activities and examine the ventilatory and stabilizing functions of the diaphragm. Thirty healthy subjects were examined in the supine position using a dynamic MRI system assessed simultaneously with specialized spirometric readings. The diaphragmatic excursions (DEs) were measured at three diaphragmatic points in the sagittal plane; the diaphragm positions (DPs) as related to a reference horizontal baseline were determined. Measurements were taken during tidal breathing (TB) and isometric flexion of upper or lower extremities against external resistance together with TB. Mean DE in both upper and lower postural limb activities was greater compared with the TB condition (P < 0.05), with the effect greater for lower limb activities. Inspiratory DPs in the upper and lower extremity activities were lower compared with TB alone (P < 0.01). Expiratory DP was lower only for lower extremity activities (P < 0.01). DP was most affected at the apex of the crescent and crural (posterior) portion of the diaphragm. DEs correlated strongly with tidal volume (Vt) in all conditions. Changes in DEs relative to the initial value were minimal for upper and lower extremities but were related to lower values of Vt (P < 0.03). Significant involvement of the diaphragm in the limb postural activities was found. Resulting DEs and DPs differed from the TB conditions, especially in lower extremity activities. The differences between the percent changes of DEs vs. Vt found for lower extremity activities were confirmed by both ventilatory and postural diaphragm recruitment in response to postural demands.

Correlation of IL-1alpha and IL-4 Gene Polymorphisms and Clinical Parameters in Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a serious disease characterized with progressive scarring of the lungs in which a genetic background is supposed. We have tested correlation of promotor regions of IL‐1alpha and IL‐4 gene polymorphisms with clinical parameters in IPF.

Abnormalities in pulmonary function in infants with high-risk congenital diaphragmatic hernia

AIMS The aim of the study was to analyze lung growth and abnormality of infant pulmonary function tests (IPFT) in congenital diaphragmatic hernia (CDH) survivors younger than three years of age with respect to unfavorable prognostic factors. METHODS Thirty high-risk CDH survivors at the age of 1.32±0.54 years, body weight 9.76±1.25 kg were examined using IPFT: tidal breathing analysis, baby resistance/compliance, whole baby body plethysmography and rapid thoraco-abdominal compression. Gore-Tex patch was used in 13% of patients (GORE group). Pulmonary hypertension was diagnosed and managed in 13% (iNO group). Standard protocols and appropriate reference values were used and obtained data were statistically analysed. RESULTS High incidence of peripheral airway obstruction (70%), increased value of functional residual capacity (FRCp) 191.3±24.5 mL (126.5±36.9 % predicted; P < 0.0005), increased value of effective airway resistance (Reff) 1.71±0.93 kPa.L(-1).s (144.4±80.1 % predicted; P < 0.01) and decreased specific compliance of the respiratory system (Crs/kg) 14.1±2.3 mL.kPa.kg(-1) (i.e., 76.1±20.1 % predicted, P < 0.0005) was noted in infants with CDH in comparison with reference values. Increased value of FRCp was found in GORE group (165.7±51.9 versus 120.4±31.2, P < 0.02) and in iNO group (183.1±52.6 versus 117.8±25.7 mL; P < 0.0005). CONCLUSION A high incidence of peripheral airway obstruction, an increased value of FRCp and decreased specific compliance of the respiratory system was noted in infants with CDH. Unfavorable prognostic factors (Gore-Tex patch, pulmonary hypertension) correlate with more severe alteration of pulmonary function in infants.