Introduction and objective: Left-heart dysfunction and pulmonary vasculopathy are increasingly recognized as contributing factors of exercise capacity limitation in interstitial fibrosing lung disease (IFLD). Moreover, the clinical significance of exercise pulmonary hypertension (ePH) in pulmonary and cardiac diseases has been documented, representing a risk factor for decreased exercise capacity and survival, progression to resting pulmonary hypertension (PH) and overall clinical worsening. We conducted a prospective study aiming at: (a) assessing the prevalence of PH and ePH in a cohort of 40 functionally limited patients with IFLD, (b) determining the post-capillary (postC) or pre-capillary (preC) etiology of either PH or ePH in this cohort, and (c) examining the correlations between invasively and non-invasively measured exercise variables among hemodynamic groups.

Purpose: A(1)Antitrypsin deficiency (AATD) pathogenic mutations are expanding beyond the PI*Z and PI*S to a multitude of rare variants. Aim: to investigate genotype and clinical profile of Greeks with AATD. Methods: Symptomatic adult-patients with early-emphysema defined by fixed airway obstruction and computerized-tomography scan and lower than normal serum AAT levels were enrolled from reference centers all over Greece. Samples were analyzed in the AAT Laboratory, University of Marburg-Germany. Results: Included are 45 adults, 38 homozygous or compound heterozygous for pathogenic variants and 7 heterozygous. Homozygous were 57.9% male, 65.8% ever-smokers, median (IQR) age 49.0(42.5-58.5) years, AAT-levels 0.20(0.08-0.26) g/L, FEV1(%predicted) 41.5(28.8-64.5). PI*Z, PI*Q0, and rare deficient allele's frequency was 51.3%, 32.9%,15.8%, respectively. PI*ZZ genotype was 36.8%, PI*Q0Q0 21.1%, PI*MdeficientMdeficient 7.9%, PI*ZQ0 18.4%, PI*Q0Mdeficient 5.3% and PI*Zrare-deficient 10.5%. Genotyping by Luminex detected: p.(Pro393Leu) associated with M-Heerlen (M1Ala/M1Val); p.(Leu65Pro) with M-Procida; p.(Lys241Ter) with Q0(Bellingham); p.(Leu377Phefs*24) with Q0(Mattawa) (M1Val) and Q0(Ourem) (M3); p.(Phe76del) with M-Malton (M2), M-Palermo (M1Val), M-Nichinan (V) and Q0(LaPalma) (S); p.(Asp280Val) with P-Lowell (M1Val)(;) P-Duarte (M4)(,) Y-Barcelona (p.Pro39His). Gene-sequencing (46.7%) detected Q0(GraniteFalls), Q0(Saint-Etienne), Q0(Amersfoort(M1Ala),) M-W & uuml;rzburg, N-Hartfordcity and one novel-variant (c.1A>G) named Q0(Attikon).Heterozygous included PI*MQ0(Amersfoort(M1Ala),) PI*MMProcida, PI*Mp.(Asp280Val), PI*MOFeyzin. AAT-levels were significantly different between genotypes (p = 0.002). Conclusion: Genotyping AATD in Greece, a multiplicity of rare variants and a diversity of rare combinations, including unique ones were observed in two thirds of patients, expanding knowledge regarding European geographical trend in rare variants. Gene sequencing was necessary for genetic diagnosis. In the future the detection of rare genotypes may add to personalize preventive and therapeutic measures. (c) 2023 Sociedade Portuguesa de Pneumologia. Published by Elsevier Esparta, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Background: A1-antitrypsin (A1AT) deficiency (D) pathogenic mutations are expanding beyond the Z and S to a multitude of rare variants. Objectives: to investigate genotype and clinical profile of Greek patients with A1ATD. Methods: Samples were analyzed in the A1AT Laboratory, University of Marburg, Germany. Patients were enrolled for early emphysema. Results: Included are 37 adults, 56.8% male, 62.2% ever-smokers, diagnosed at a median age (IQR) of 48.5 (42.25-57.0) years with A1AT levels of 0.20(0.08-0.30) g/L. FEV1(% predicted), FEV1/FVC%, FEF25-75% and DLCO (% predicted) were 40.9 (28.0-62.0), 50.5 (38.6-63.0), 14 (9.0-29.5) and 42.5 (30.9-65.0) respectively. Frequency of Z, Q0 and M-like alleles was 51.4%, 32.4% and 16.2% respectively. Fourteen patients (37.8%) presented PI*ZZ genotype, 8 (21.6%) PI*Q0Q0, 3 (8.10%) PI*M-like/M-like, 6 (16.2%) PI*ZQ0, 2 (5.4%) PI*Q0/M-like and 4 (10.8%) PI*Z/M-like genotypes. Sequencing demonstrated Q0 alleles as Q0Bellingham, Q0Amersfoort, Q0Granitefalls, Q0Saint-Etienne and Q0Mattawa and M-like variants as MHeerlen, MProcida, MMalton and MWürzburg. In patients with smoke exposures, A1AT levels correlated to FEV1%predicted [p=0.032, r=0.468], DLCO% predicted [p=0.021, r=0.610] and age at diagnosis [p=0.013, r=0.499]. Q0Q0 genotype was associated with lower A1AT and FEF25-75% values, compared to all other variants [p<0.001 and p=0.030] respectively. Conclusion: Almost half of the A1ATD in Greece are non-Z ultra-rare pathogenic A1AT variants, confirming an established North-South European geographical trend in Z variant A1ATD. Thus, in addition to early diagnosis of A1ATD, the detection of this rare genotypes is mandatory to personalize preventive and therapeutic measures.

Adult PAP patients experience similar #COVID19 rates to the general population, and high rates of hospitalisation and deaths, underscoring their vulnerability and the need for measures to prevent infection. The impact of iGM-CSF must be considered. https://bit.ly/3M0wKnZ.

Allergic diseases are recognized as a burden on the public health. They stand as one of the most common chronic diseases, especially in developed countries. Therefore, the objective of this study is to evaluate the association between the development of atopic allergy and the presence of food allergy in children, and food consumption.This multidisciplinary cross-observational epidemiological study was conducted among 1199 schoolchildren who were recruited in 4th grade and 5th grade (9–11 years old from Marseille). Data were collected by means of a standardized epidemiological questionnaire with a medical assessment focusing on allergic diseases, and questions on lifestyle and child nutrition (FFQ).During the last 12 months, prevalence of allergic diseases were shown as follows: 41% of children presented allergic rhinitis symptoms, 24% reported having asthma related symptoms, while 28% suffered of eczema and 7% complained of food allergy. There was a significant association between food allergy and asthma symptoms (P-value < 0.001, IC = [2.29–7.69]), eczema (P-value < 0.001, IC = [2.37–8.32]) and allergic rhinitis (P-value < 0.001, IC = [2.32–8.17]). Regarding dietary habits, the multivariate analysis shows that the consumption of vegetables (ORa = 0.72 [0.64–0.74]) and fish (ORa = 0.74 [0.50–1.09], ORa = 0.66 [0.47–0.94] respectively) are associated decreased risk of developing asthma and allergic rhinitis symptoms, while fruit consumption (ORa = 0.68 [0.45–1.03]) is associated with a decrease in eczema symptoms.This study shows that the presence of a food allergy is strongly associated with allergic diseases. Indeed, the consumption of vegetables, fruits and fish seems to have a protective effect on the occurrence of these diseases.

BACKGROUND:Oxidant-antioxidant imbalance may play an important role in the development and progression of bronchial asthma. However, the role of blood antioxidants especially in asthma exacerbation has not been fully discussed.OBJECTIVE:This study examines a part of the intracellular antioxidant defense mechanism in asthmatic patients admitted to hospital due to severe exacerbation of their disease.METHODS:Peripheral blood Erythrocyte Superoxide Dismutase (SOD) activity was measured in 38 patients (33 men - 5 women, with a mean age of 56 +/- 2.8 yrs), using a colorimetric method. On the days of admission and discharge the Forced Expiratory Volume in 1 second (FEV1) and the Partial arterial Oxygen pressure (PaO2) were recorded and correlated with SOD activity at the same time.RESULTS:A statistically significant decrease of SOD activity was observed on the day of admission compared to SOD activity on the day of discharge (43.64 +/- 31.78 vs. 96.16 +/- 54.05 units/ml, p < 0.001), suggesting the presence of oxidative stress during an asthma attack. A statistically significant correlation was observed between FEV1 on admission and SOD activity at the same time (r = 0.57, p < 0.001). Furthermore, SOD activity on admission was correlated with PaO2 on discharge (r = 0.55, p < 0.001), as well as SOD on discharge with PaO2 on discharge (r = 0.53, p = 0.001).CONCLUSIONS:Decreased systemic erythrocyte SOD activity was observed during asthma attacks. This activity was correlated with severity criteria such as FEV1 and PaO2. Therefore, it seems that measurement of SOD activity could be a useful tool in the evaluation of an asthma attack. The supplementary administration of antioxidants in the future needs further clarification.

Purpose A retrospective study on the prevalence of conjunctivitis and the commonest allergens in Northern Greece. Methods From the archives of an outpatient unit in Thessaloniki-Greece, the patients with conjunctivitis were evaluated. The patients underwent skin prick tests (SPTs) according to current guidelines. The patients were divided into 4 groups: Group A : Patients with conjunctivitis alone, Group B: patients with asthma and conjunctivitis, Group C: patients with rhinitis and conjunctivitis, Group D: patients with all three co-morbidities. Results 49 out of 1239 allergic patients had only conjunctivitis, 102 conjunctivitis and asthma, 117 conjunctivitis and rhinitis and 229 all three co-morbidities. Conclusion In the Northern Greek population allergic conjunctivitis is common. There was no significant statistical difference between men and women in the aforementioned groups.

Background Specific challenges provide a better understanding of the underlying inflammatory mechanisms in asthma. The aim of this study was to evaluate the immediate and late phase asthmatic response after specific nasal challenge (SNC) in asthmatic patients sensitive to Alternaria.

OBJECTIVES Pulmonary involvement of varying etiology is common in collagen vascular diseases (CVDs). Bronchoalveolar lavage fluid (BALF) cell differentials reveal information on the immune mechanisms involved in the CVDs. The aim of the present study was to evaluate BALF cell populations in CVD-associated ILD and to investigate possible correlation with pulmonary function. METHODS Fifty-seven patients (26 male and 31 female, mean age ± SD: 54.68±12.18 years) with CVD-associated interstitial lung disease were studied. Patients were divided into 6 groups based on underlying CVD. The study population also included a group of 10 healthy controls. BALF was examined in all individuals. Cell density, total cell number and differential cell count were recorded. BALF lymphocyte subsets were analysed by dual flow cytometry. Pulmonary function was assessed in all patients. RESULTS BALF differential cell count did not differ significantly among the different groups. Scleroderma patients showed the highest percentage of CD19 cells (p<0.001). The NK and NKT cell percentages were significantly higher in systemic lupus erythematosus and in Sjögren, respectively, compared to other CVDs and controls (p=0.001 and p<0.001). Also BALF neutrophil percentage correlated negatively with FVC (r=-0.356, p=0.011) and FEV1 (r=-0.336, p=0.017) and BALF NKT cell percentage correlated negatively with pO2 (r=-0.415, p=0.003). CONCLUSIONS Important variations observed in BALF cell populations suggest the implication of NK and NKT cells in the pathogenesis of lung involvement in CVDs.

Background Increased oxidative and inflammatory markers have been reported in lung cancer patients, but relatively few studies have investigated the presence of antioxidants both in the local lung environment and in the systemic circulation. Furthermore, it is hypothesized that the immune system activation in vivo is regulated by the redox environment. Objectives To investigate local and systemically circulating antioxidant and inflammatory mediators in lung cancer patients and potential correlations between them. Methods Forty two male patients (mean age 65±8 years) with primary lung cancer were studied. Sixteen age and smoking history matched male subjects without any evidence of malignancy served as controls. Total antioxidant status (TAS) and glutathione (GSH), as well as interleukin-1a (IL-1a), interleukin-6 (IL-6) and tumor necrosis factor alpha (TNF-α) were measured in bronchoalveolar lavage fluid (BALF) and serum samples. Results A statistically significant increase of TAS and GSH in BALF was observed in lung cancer patients compared to healthy subjects (0.27±0.24 vs. 0.12±0.02 mmol/L, p=0.02 and 7.56±4.29 vs. 4.62±2.23 μmol/L, p=0.01 respectively). Statistically significant correlations in cancer patients were observed in BALF between TAS and a. IL-1α (r=0.87, p<0.001), b. IL-6 (r=0.52, p=0.001) and c. TNF-α (r=0.67, p<0.001). Conclusions Alteration in antioxidant and inflammatory mediator status was found in lung cancer patients both in serum and in BALF compared to healthy subjects matched for smoking history. Moreover, a positive correlation was observed between antioxidants and pro-inflammatory cytokines, but only locally and not systematically.

Small cell lung cancer is characterized by rapid growth and early metastasis. Despite its sensitivity to cytotoxic therapy, until now treatments have failed to control or cure this disease in most patients. Orbital metastases are a rare manifestation of systemic malignancies. Breast and lung cancers represent more than two thirds of the primary cancer sites. Metastases to the eye and orbit develop in approximately 0.7-12% of patients with lung cancer. Here, we report a rare case of exophthalmos as the first manifestation of a metastatic carcinoma due to small cell lung cancer, and a 6-months follow-up with complete exophthalmic response to chemotherapy.

The aim of this study was to prospectively evaluate the prevalence of pulmonary hypertension (PH) in patients with idiopathic pulmonary fibrosis (IPF). One hundred thirty-nine patients (101 male, mean age = 68.6 ± 9 years), with confirmed IPF and who were admitted to eight Pulmonary Departments in Greece between November 2005 and December 2006 were included in the study. Pulmonary artery systolic pressure (PASP) was estimated by echocardiography, and PH was defined as PASP > 36 mmHg. We compared demographics, pulmonary function tests, NYHA functional status, 6-min walk distance (6MWD), B-type natriuretic peptide (BNP), PaO2, and P(A-a)O2 at rest data between patients with PH and without PH (PASP ≤ 36 mmHg). Increased estimated right ventricular systolic pressure was present in 55% of patients (mean PASP = 47.1 ± 11.2 mmHg vs. 30.3 ± 3.8 mmHg, respectively). Patients with PH had a lower but not statistically significant DLCO (47.1 ± 18.8 vs. 52.5 ± 20.1), lower PaO2 at rest (64.6 ± 12.2 vs. 71.1 ± 11.3, P = 0.004), and lower mean 6MWD (282 ± 118 vs. 338 ± 91, P = 0.007). Significant differences were also observed in the NYHA functional status between the two groups (P = 0.02). Statistically significant correlations were observed between PASP and PaO2 at rest (r = −0.331, P = 0.00), P(A-a)O2 at rest (r = 0.494, P = 0.00), 6MWD (r = −0.264, P = 0.01), SpO2 at rest (r = −0.293, P = 0.00), SpO2 at the end of exercise (r = −0.364, P = 0.00), and also BNP values (r = 0.319, P = 0.01). Moreover, PaO2 (P = 0.02), P(A-a)O2 (P = 0.005), and SpO2 at the end of exercise (P = 0.023) were independent predictors of the presence of estimated PH. Using Doppler echocardiography as a screening tool for the estimation of PH, we found that PH is common in patients with IPF. Gas exchange parameters at rest and exercise desaturation might indicate underlying PH in IPF.

BACKGROUND Bronchoalveolar lavage (BAL) is a useful bronchoscopic technique. Studies in "normal" children are limited. AIM To provide data on BAL reference values from Greek children and compare BAL cellular and noncellular components in children with inflammatory and non-inflammatory lung diseases. METHODS Seventy two children, aged 2.5 months to 16 years, underwent diagnostic bronchoscopy and BAL. Patients were divided in two groups whether lung inflammation was absent or present. Differential cytology, flow cytometry for lymphocyte subsets and cytokine and chemokine measurements were performed on BAL fluid. RESULTS Alveolar macrophages were the predominant cellular population in normal children. Patients with inflammatory pneumonopathies had significantly more neutrophils. There was no difference in lymphocyte subpopulations. Values of CD4+/CD8+ ratio in BAL was similar to that reported in adults. Levels of IL-8 and TNF- alpha were significantly higher in children with inflammatory lung diseases. CONCLUSION This study provides the first data on BAL of "normal" Greek children. BAL from patients with pulmonary inflammation was characterised by neutrophilia. Finally, we propose that measurement of IL-8 and TNF-a levels in BAL could help in early identification of inflammation in the tracheobronchial tree.

Defoliation produced by abiotic factors and the subsequent re-growth can reduce sugar beet (Beta vulgaris L.) sucrose content and final sugar yield. Field experiments were conducted during 2003 and 2004 growing seasons in the farm of Hellenic Sugar Industry SA, Larissa factory, central Greece. Three sugar beet cultivars (Rival, Europa and Corsica) were ordinary irrigated till the beginning of July and then left without irrigation for a month. Three defoliation levels (control-C, moderate-MD and severe-SD) were performed at early August and irrigation was simultaneously started to promote re-growth. Four samplings were conducted (before defoliation, 15, 30 and 40 days after defoliation) to determine the changes in physiological and productive traits. Yields were lower in 2003 compared to 2004 because sugar beets were grown under more stressful conditions due to the delayed sowing, the higher temperatures and the lower rainfall. Both defoliation level and cultivar had significant effects on physiological and productive traits after re-growth only in 2003. The late-season cultivar, Corsica, showed better LAI maintenance compared to Europa and Corsica and had the greatest performance after re-growth in regard to fresh root weight and sugar yield. Also, this cultivar showed the least decrease of sucrose percentage in fresh root weight and juice purity mainly due to the stable potassium (K) concentration and limited increase of sodium (Na) accumulation in roots. Corsica consumed the least root a-amino N for its re-growth. Quantitative and qualitative traits were negatively affected only by the SD treatment. Plants suffered from MD treatment gradually recovered during growing season. This study demonstrates that under Mediterranean conditions, the adverse effects of re-growth on sugar beet yield and quality depend on the growing conditions and they can be restricted by the selection of an appropriate cultivar.

Background: Eosinophilic lung diseases comprise a group of heterogeneous pulmonary disorders linked by increased eosinophils in bronchoalveolar lavage fluid (BALF). There is supporting evidence that natural killer (NK) cells participate in the regulation of eosinophilic inflammation. Objective: Our aim was to investigate the relationship between eosinophils and NK cells in BALF in patients with different interstitial lung diseases (ILDs) focusing on eosinophilic pneumonias. Methods: Of 114 patients who presented with increased BALF eosinophils (>5%), 74 patients were classified into the following groups: 27 had eosinophilic pneumonia (EP), 17 had idiopathic pulmonary fibrosis (IPF), 16 had hypersensitivity pneumonitis (HSP) and 14 had cryptogenic organizing pneumonia (COP/BOOP). Total BALF cells, cell density and cell differential counts were assessed and lymphocyte subsets CD3+, CD4+, CD8+, CD19+, CD3–CD16/56+ (NK) and CD3+CD16/56+ (NKT) were determined by flow cytometry. Results: Significant differences were observed in the percentages of lymphocytes (p < 0.001) and CD3+CD16/56+ cells (p = 0.023) among patient groups. In patients with EP, the percentage of eosinophils correlated positively with the number of CD3–CD16/56+ cells (r = 0.522, p = 0.005), the percentage of CD3–CD16/56+ cells (r = 0.690, p < 0.001), and the absolute count of CD3+CD16/56+ absolute cells (r = 0.609, p = 0.001). However, in patients with IPF, HSP or COP/BOOP, no correlation between the percentage of eosinophils and CD3–CD16/56+ or CD3+CD16/56+ cells was observed. Conclusions: Eosinophil inflammation seems to develop through a different pathway in EP compared to other ILDs.