Airway obstruction can be better predicted using Global Lung Function Initiative spirometry reference equations in Marfan syndrome.

Marfan syndrome is a genetic disorder of the connective tissue, including involvement of the lungs.Pulmonary function test was performed in 32 asymptomatic adult Marfan patients using European Community for Coal and Steel (ECCS) and Global Lung Function Initiative (GLI) reference values.Using GLI equations for reference, significantly lower lung function values were noted for forced vital capacity (FVC) (87.0 ± 16.6% vs. 97.1 ± 16.9%; P < 0.01) and forced expiratory volume in the first second (FEV1) (79.6 ± 18.9% vs. 88.0 ± 19.1%; P < 0.01) predicted compared to ECCS. Obstructive ventilatory pattern was present in 25% of the cases when calculating with GLI lower limit of normal (LLN), and it was significantly more common in men as compared to women (n = 6, 50% vs. n = 2, 10%; P = 0.03).GLI is more suitable to detect early ventilatory changes including airway obstruction in young patients with special anatomic features, and should be used as a standard way of evaluation in asymptomatic Marfan population.

Antibody-Mediated Rejection in a Multiple Lung Transplant Patient: A Case Report.

Lung transplant is an effective way to treat many end-stage lung diseases. However, one of the main barriers of allograft organ transplant is still the immunologic rejection of transplanted tissue, which is a response of the HLA molecules. Rejection is a complex process involving both T-cell-mediated delayed-type hypersensitivity reactions and antibody-mediated hypersensitivity reactions to histocompatibility molecules on foreign grafts. We report the case of a 25-year-old female patient with cystic fibrosis who underwent 2 lung transplants because of her initial diagnosis and appearance of bronchiolitis obliterans syndrome after the first transplant. Only 13 months after the second transplant, despite the therapies applied, a new rejection occurred associated with high mean fluorescent intensity donor-specific antibody levels, which resulted later in the death of the patient. The present case draws attention to the importance of matching HLA molecules between donor and recipient in addition to immunosuppressive therapy.

Relation of circulating T cell profiles to airway inflammation and asthma control in asthmatic pregnancy.

Asthmatic inflammation during pregnancy poses a risk for maternal and fetal morbidities. Circulating T cell immune phenotype is known to correlate with airway inflammation (detectable by fractional concentration of nitric oxide present in exhaled breath (FENO)) in non-pregnant allergic asthmatics. The aim of this study was to assess the relationship of peripheral T cell phenotype to FENO and clinical variables of asthma during pregnancy.We examined 22 pregnant women with allergic asthma in the 2nd/3rd trimester. The prevalence of Th1, Th2, regulatory T (Treg) and natural killer (NK) cell subsets was identified with flow cytometry using cell-specific markers. FENO, Asthma Control Test (ACT) total score and lung function were evaluated.Peripheral blood Th1, Th2, Treg, and NK cell prevalence were not significantly correlated to airway inflammation assessed by FENO in asthmatic pregnant women (all cells p > 0.05; study power > 75%). However, an inverse correlation was detected between Th2 cell prevalence and ACT total scores (p = 0.03) in asthmatic pregnancy.Blunted relationship between T cell profile and airway inflammation may be the result of pregnancy induced immune tolerance in asthmatic pregnancy. On the other hand, increased Th2 response impairs disease control that supports direct relationship between symptoms and cellular mechanisms of asthma during pregnancy.

Airway pathogens during the first year after lung transplantation: a single-center experience.

After lung transplantation, a high level of immunosuppression is needed to prevent rejection. This demand renders recipients more sensitive to infections. As pulmonary infections are a major clinical problem during the first postoperative year after lung transplantation, preventive treatment and regular surveillance examinations are needed for immediate, adequate therapy. We describe the airway pathogens registered during the first posttransplantation year among our 12 lung transplant recipients since December 2008. Samples were obtained for microbiologic analysis from the upper and lower respiratory tracts and from serum as part of routine care. During the first year after transplantation the most frequent pathogens were fungi (Candida albicans 82%; Aspergillus 50%), Pneumocystis (8%), gram-negative bacteria (Pseudomonas spp 60%; Klebsiella 25%, Acinetobacter 17%; Escherichia Coli 17%; and Enterococcus faecalis 25%), and Staphylococcus aureus (50%, including methicillin-resistant strains 25%). This pathogen spectrum in the first postoperative year after lung transplantation was similar to other centers. Colonization with Pseudomonas or fungi presented early and was prevalent among our patients.

Severe mixed sleep apnea after bilateral lung transplantation in a cystic fibrosis patient: a case report.

BACKGROUND: Lung transplantation is the only treatment for end-stage lung disease in selected patients. After lung transplantation, patient recovery is often slow owing to severe underlying diseases in the patient producing hypoxemia before, during, and after surgery, as well as infections and rejection episodes. Postoperative breathing and ventillatory disorders are also associated with diaphragmatic dysfunction and/or phrenic nerve damage. METHODS: Herein we have reported a case of a 35-year-old man undergoing bilateral lung transplantation owing to worsening of chronic respiratory failure from cystic fibrosis. After uncomplicated surgery, weaning was delayed due to nighttime dyspnea and hypoxemia attributed to diaphragm dysfunction. After improvement of diaphragm function, the symptoms persisted, requiring noninvasive nocturnal ventilatory support. Polysomnography confirmed severe mixed sleep apnea. RESULTS: Effective treatment with noninvasive bi-level positive airway pressure spontaneous/timed mode (BiPAP S/T) ventilation during the nights rendered the patient symptom free. Polysomnography confirmed successful treatment. CONCLUSION: Disordered breathing while sleeping is common after solid organ transplantation. BiPAP S/T ventilator therapy was effective to the treat dominantly central sleep apnea in our patient.

Cisplatin nephrotoxicity aggravated by cardiovascular disease and diabetes in lung cancer patients.

Ageing lung cancer patients may be at increased risk of Cisplatin (Cp) nephrotoxicity, because of comorbidities leading to accelerated ageing of the kidneys. Therefore, the Cp-induced impairement of renal function was compared between no comorbidity (NC) and hypertension plus ischaemic heart disease (CD) patients or others having diabetes mellitus plus ischaemic heart disease (DMIH). In a preliminary study, glomerular filtration rate (GFR) was measured by clearance of technetium 99m-labelled diethylene-thiamine penta-acetate in 38 lung cancer patients with normal serum creatinine concentration ([creat]). Then, the incidence of nephrotoxicity was analysed retrospectively over 1st-4th cycles of Cp treatment among 242 lung cancer patients with initially normal [creat]. GFR was repeatedly estimated using calculated creatinine clearance. Pre-treatment GFR was 57 ± 3 mL·min⁻¹·m⁻² in those with normal (n = 15) and 42 ± 2 mL·min⁻¹·m⁻² in those with pathologically increased (n = 23) [creat] any time following their 2nd-4th Cp cycle (p < 0.05). The retrospective analysis revealed that Cp-induced nephrotoxicity developed in 7.5% of the NC (n = 80), in 20.9% of the CD (n = 110) and in 30.8% of the DMIH (n = 52) subgroups. Within the overall dropout rate from further Cp chemotherapy, nephrotoxicity was responsible in 14% of NC, 38% in CD and 75% in DMIH patients. A major portion of our ageing lung cancer patients suffered from comorbidities leading to reduced renal resistance to Cp nephrotoxicity.

Increased interferon-gamma- and interleukin-4-synthesizing subsets of circulating T lymphocytes in pregnant asthmatics.

BACKGROUND: Pregnancy frequently interferes with the course of bronchial asthma, and asthmatic pregnant women experience less successful pregnancies. T lymphocytes synthesizing IL-4 or IFN-gamma are important in allergic mechanisms of the airways as well as in materno-fetal immunity. OBJECTIVE: We hypothesized that pregnancy (a T helper-2 polarized state) of asthmatics will enhance the number of circulating T2 lymphocytes, but decrease the subset-producing IFN-gamma (T1 lymphocytes) and thereby cause a culminating T2 dominance with possible clinical consequences. METHODS: IL-4- or IFN-gamma-producing T lymphocytes were determined by flow cytometry in healthy (n=8) and asthmatic (n=13) non-pregnant women and healthy (n=18) and asthmatic (n=48) pregnant women of similar chronological and gestational (2nd-3rd trimester) age and asthma severity (Global Initiative for Asthma II-III). RESULTS: In the blood of non-pregnant women--healthy or asthmatic--the numbers of IL-4- and IFN-gamma+ T cells were very low (<10/microL blood). In contrast, in asthmatic pregnant women, the cell counts were 182+/-27 and 39+/-6 for IFN-gamma+ and IL-4+ T cells/microL blood, respectively (both P<0.05 vs. respective control values of non-pregnant asthmatics). Within the asthmatic pregnant group, significant negative correlations were revealed between the numbers of IFN-gamma+ or IL-4+ T cells and maternal peak expiratory flow as well as birth weight of newborns (both P<0.05). CONCLUSION: These data show a previously unknown immunological interference between asthma and pregnancy. The culminating proliferation of IFN-gamma+ and IL-4+ T lymphocytes may potentially impair maternal airway symptoms as well as fetal development.