Myelodysplastic syndromes and idiopathic pulmonary fibrosis: a dangerous liaison.

Previous studies have shown that the co-existence of bone marrow failure and pulmonary fibrosis in a single patient or in a family is suggestive of telomere related genes (TRG) germline mutations. This study presents the genetic background, clinical characteristics, and outcome of a group of five Greek patients co-affected with IPF and MDS. Four out of five patients developed an IPF acute exacerbation that was not reversible. We failed to detect any mutation in the TERT, TERC, DKC1, TINF2, RTEL1, PARN, NAF1, ACD, NHP2 and NOP10 genes in any patient. Moreover, telomere length was normal in the two patients tested. This could suggest that although the co-occurence of IPF and MDS are suggestive of TRG mutation in patients < 65 years old, in the elderly it may occur without germline mutations and could negatively affect prognosis. Physicians should be aware for possible IPF deterioration and therapeutic options for MDS should be wisely considered.

Body mass index is associated with leukotriene inflammation in asthmatics.

BACKGROUND: Obesity and asthma are characterized by the presence of inflammation. Leptin and adiponectin are circulating hormones produced by adipose tissue that regulate several metabolic and inflammatory functions. We aimed to determine whether obesity influences asthmatic inflammation as well as the contribution of leptin or/and adiponectin to a possible linkage between asthmatic and obesity-related inflammation. MATERIALS AND METHODS: One hundred patients with asthma and 60 healthy controls were studied. Subjects who had a comorbid illness that could interfere with the proposed tests were excluded. All subjects were divided into three groups (normal range, pre-obese, obese) according to the criteria of the current WHO international classification for body mass index (BMI). Possible associations between variables expressing airway inflammation, bronchial hyper-responsiveness, systemic inflammation and obesity, as assessed by BMI, were evaluated. Leptin and adiponectin were also measured and were associated with asthma airway and systemic inflammatory variables to elucidate possible associations. RESULTS: Obese patients had significant higher values of LTE(4) /creatinine in urine compared with pre-obese and normal range ones. In a linear regression model, the only significant associations were those between BMI and LTE(4) /creatinine in urine. Using the same model, log leptin and log adiponectin presented positive and negative associations, respectively with LTE(4) /creatinine in urine. No other significant associations were observed in both patients and healthy subjects. CONCLUSIONS: In a selected cohort of asthmatic patients, obesity is significantly associated with increased urinary leukotriene levels. Alterations of leptin/adiponectin balance may be related to the presence of leukotriene inflammation in obese asthmatic patients.

Allergic Conjunctivitis in Patients with Respiratory Allergic Symptoms; a Retrospective Study in Greece.

Here, we report on the prevalence of allergic conjunctivitis and positive skin prick test (SPT) results in relation to respiratory allergic conditions among patients with symptoms of allergies at a respiratory outpatient clinic. A questionnaire survey of symptoms (i.e., asthma-like, rhinitis, and conjunctivitis symptoms) involving 1522 patients was carried out. The responses of 1242 patients indicated that they had allergic conjunctivitis, asthma, rhinitis, or a combination of these conditions, and 869 of these patients underwent SPTs that assessed responses to 40 allergens. Allergic conjunctivitis was found to be very common (40%, 497 out of 1242 patients) among those with symptoms of allergies. Conjunctivitis was slightly more common among women, while rhinitis was more common among men. Patients with both conjunctivitis and rhinitis were more likely to undergo SPTs, and they had a higher rate of positive SPTs. The coexistence of two or more comorbidities increased the risk of having an immunoglobulin E (IgE)-mediated allergy (based on the SPT results) compared to having each of the conditions alone. In conclusion, allergic conjunctivitis can occur either alone or with asthma and/or rhinitis. It is not always accompanied by rhinitis, but the coexistence of these conditions was the strongest indicator of IgE-mediated allergies.

Allergic conjunctivitis and the most common allergens in Northern Greece.

BACKGROUND: Ocular allergies affect a great part of the general population and often co exist with other allergic manifestations. In the present study, the prevalence of allergic conjunctivitis and the commonest allergens in allergic patients at an outpatient unit in Thessaloniki, Greece were evaluated. METHODS: This is a retrospective study regarding allergic patients who referred to an outpatient clinic between the 1st of January of 1996 and the 31st of December 2010. They completed relative questionnaires concerning their allergic condition. The patients who were included in our study had allergic conjunctivitis confirmed by ophthalmologists and were divided into 4 groups. The criteria used were the existence of allergic conjunctivitis alone or with other allergic co- morbidities. The patients then underwent skin prick tests after consent according to current guidelines. RESULTS: The archives of 1239 allergic patients were evaluated and 497 patients (40,11%) who manifested eye allergic symptoms were included in our study. 448 patients (90.14%) had allergic conjunctivitis in conjunction with asthma or rhinitis or both. 370 patients underwent skin prick tests and 284 of them (124 males-160 females) were positive for at least 1 of the 8 tested allergens (76.75%). 166 were positive to a grass mix (58.45%), 130 to olea European (45.77%), 124 to dust mites mix (43.66%), 58 to cypress (20.42%), 71 to parietaria officinalis (25.00%), 67 to cat dander (23.59%), 35 to dog dander (12.32%) and 32 to Altenaria (11.26%). CONCLUSIONS: Symptoms of ocular allergy are very common in patients with allergic rhinitis and asthma. Men had slightly higher percentages of positive skin prick tests, except for dog dander and Altenaria. Conjunctivitis should not be overlooked as an allergic entity when evaluating allergic patients.

Vitamin D in asthma and future perspectives.

Humans have the ability to synthesize vitamin D during the action of ultraviolet (UV) radiation upon the skin. Apart from the regulation of calcium and phosphate metabolism, another critical role for vitamin D in immunity and respiratory health has been revealed, since vitamin D receptors have also been found in other body cells. The term "vitamin D insufficiency" has been used to describe low levels of serum 25-hydroxyvitamin D that may be associated with a wide range of pulmonary diseases, including viral and bacterial respiratory infection, asthma, chronic obstructive pulmonary disease, and cancer. This review focuses on the controversial relationship between vitamin D and asthma. Also, it has been found that different gene polymorphisms of the vitamin D receptor have variable associations with asthma. Other studies investigated the vitamin D receptor signaling pathway in vitro or in experimental animal models and showed either a beneficial or a negative effect of vitamin D in asthma. Furthermore, a range of epidemiological studies has also suggested that vitamin D insufficiency is associated with low lung function. In the future, clinical trials in different asthmatic groups, such as infants, children of school age, and ethnic minorities are needed to establish the role of vitamin D supplementation to prevent and/or treat asthma.

An unusual cause of spontaneous pneumothorax.

Bronchopulmonary sequestration is a congenital pulmonary malformation consisting of a non-functioning region of lung parenchyma that receives systemic arterial blood supply. The authors present the case of a 31-year-old male who presented with spontaneous pneumothorax as the initial clinical manifestation of an intralobar bronchopulmonary sequestration. There was a persistent air leak through the chest tube which remained in the pleural cavity for 12 days. CT revealed an area of increased opacity, with focal calcifications and a small eccentric cavity in the posterior basal region of the left lower lobe. Aortography established the final diagnosis.

Exhaled NO and exhaled breath condensate pH in the evaluation of asthma control.

BACKGROUND: Asthma is a chronic inflammatory airways disorder. However, no biomarker of airways inflammation has been included in the assessment of asthma control. OBJECTIVE: To evaluate exhaled NO (FeNO) and exhaled breath condensate (EBC) pH in patients with asthma according to the level of control, and their performance in the identification of not well-controlled patients. METHODS: FeNO and EBC pH after Argon deaeration were measured in 274 consecutive patients. Asthma control was evaluated by two asthma specialists blinded to FeNO and pH measurements according to GINA guidelines, as well as by asthma control test (ACT) and asthma control questionnaire (ACQ). RESULTS: FeNO was higher and EBC pH was lower in patients with not well-controlled compared to controlled asthma. In ROC analysis, FeNO presented an AUC of 0.790 for the identification of not well-controlled asthma performing better in non-smokers; EBC pH presented an AUC of 0.791 for the identification of not well-controlled asthma, performing better in smokers. The performance of both biomarkers was inferior to that of ACT and ACQ. FeNO values >30 ppb presented positive predictive values (PPV) > 0.85 with the exception of smokers treated with inhaled corticosteroids. EBC pH values ≤7.20 presented PPV >0.80 in all groups. The presence of FeNO >30 ppb and/or EBC pH ≤7.20 was indicative of not well-uncontrolled asthma in 88.3% of the patients. CONCLUSION: FeNO and EBC pH levels may identify patients with not well-controlled asthma. However, their performance was inferior to clinical judgment and may be limited to selected subgroups of asthmatic patients.