Carbon Monoxide Transfer Factor Research Articles

Expiratory Venous Volume and Arterial Tortuosity are Associated with Disease Severity and Mortality Risk in Patients with COPD: Results from COSYCONET.

The aim of this study was to evaluate the association between computed tomography (CT) quantitative pulmonary vessel morphology and lung function, disease severity, and mortality risk in patients with chronic obstructive pulmonary disease (COPD). Participants of the prospective nationwide COSYCONET cohort study with paired inspiratory-expiratory CT were included. Fully automatic software, developed in-house, segmented arterial and venous pulmonary vessels and quantified volume and tortuosity on inspiratory and expiratory scans. The association between vessel volume normalised to lung volume and tortuosity versus lung function (forced expiratory volume in 1 sec [FEV1]), air trapping (residual volume to total lung capacity ratio [RV/TLC]), transfer factor for carbon monoxide (TLCO), disease severity in terms of Global Initiative for Chronic Obstructive Lung Disease (GOLD) group D, and mortality were analysed by linear, logistic or Cox proportional hazard regression. Complete data were available from 138 patients (39% female, mean age 65 years). FEV1, RV/TLC and TLCO, all as % predicted, were significantly (p < 0.05 each) associated with expiratory vessel characteristics, predominantly venous volume and arterial tortuosity. Associations with inspiratory vessel characteristics were absent or negligible. The patterns were similar for relationships between GOLD D and mortality with vessel characteristics. Expiratory venous volume was an independent predictor of mortality, in addition to FEV1. By using automated software in patients with COPD, clinically relevant information on pulmonary vasculature can be extracted from expiratory CT scans (although not inspiratory scans); in particular, expiratory pulmonary venous volume predicted mortality. NCT01245933.

Optimizing cardiopulmonary rehabilitation duration for long COVID patients: an exercise physiology monitoring approach.

The presence of prolonged symptoms after COVID infection worsens the workability and quality of life. 200 adults with long COVID syndrome were enrolled after medical, physical, and mental screening, and were divided into two groups based on their performance. The intervention group (n = 100) received supervised rehabilitation at Department of Pulmonology, Semmelweis University with the registration number 160/2021 between 01/APR/2021-31/DEC/2022, while an age-matched control group (n = 100) received a single check-up. To evaluate the long-term effects of the rehabilitation, the intervention group was involved in a 2- and 3-month follow-up, carrying out cardiopulmonary exercise test. Our study contributes understanding long COVID rehabilitation, emphasizing the potential benefits of structured cardiopulmonary rehabilitation in enhancing patient outcomes and well-being. Significant difference was found between intervention group and control group at baseline visit in pulmonary parameters, as forced vital capacity, forced expiratory volume, forced expiratory volume, transfer factor for carbon monoxide, transfer coefficient for carbon monoxide, and oxygen saturation (all p < 0.05). Our follow-up study proved that a 2-week long, patient-centered pulmonary rehabilitation program has a positive long-term effect on people with symptomatic long COVID syndrome. Our data showed significant improvement between two and three months in maximal oxygen consumption (p < 0.05). Multidisciplinary, individualized approach may be a key element of a successful cardiopulmonary rehabilitation in long COVID conditions, which improves workload, quality of life, respiratory function, and status of patients with long COVID syndrome.

Reduced lung function during childhood in identical twins with discordant fetal growth: a cohort study

Fetal growth restriction (FGR) can negatively affect lung development, leading to increased respiratory morbidity and reduced lung function later in life. Studies regarding the impact of FGR on lung function in singletons are influenced by genetic, obstetric, and maternal factors. To overcome these confounding factors, we aim to investigate lung function in identical twins with selective FGR (sFGR). Lung function assessments were performed in identical twins with sFGR born in our centre between March 1, 2002, and December 31, 2017, aged between 5 and 17 years. sFGR was defined as birthweight discordance ≥20%. Outcome measures consisted of forced expiratory volume in 1s (FEV1), forced vital capacity (FVC), and transfer factor for carbon monoxide (DLCO) and were compared between the smaller and larger twin. Thirty-nine twin pairs performed spirometry of sufficient quality. Median gestational age at birth was 34.3 (interquartile range (IQR) 32.1-36.0) weeks with median birthweights of 1500 (IQR 1160-1880) grams and 2178 (IQR 1675-2720) grams for the smaller and larger twin, respectively. Smaller twins had significantly lower z-scores for FEV1 (-0.94 versus-0.41, p=0.0015), FVC (-0.56 versus-0.06, p<0.0001) and DLCO (-0.50 versus 0.00, p<0.0001) compared to larger co-twins. Although being genetically identical, sFGR in identical twins is associated with a reduction in static and dynamic lung volume and a reduction in lung diffusion, even when taking the reduced lung volume into account. This indicates that adverse growth conditions in utero negatively affect lung development and function, potentially contributing to an increase in respiratory morbidities later in life. The Dutch Heart Foundation and The Bontius Foundation.

The effectiveness of glucocorticoid treatment in post-COVID-19 pulmonary involvement

RationalePersistent respiratory symptoms following Coronavirus Disease 2019 (COVID-19) are associated with residual radiological changes in lung parenchyma, with a risk of development into lung fibrosis, and with impaired pulmonary function. Previous studies hinted at the possible efficacy of corticosteroids (CS) in facilitating the resolution of post-COVID residual changes in the lungs, but the available data is limited.AimTo evaluate the effects of CS treatment in post-COVID respiratory syndrome patients.Patients and methodsPost-COVID patients were recruited into a prospective single-center observational study and scheduled for an initial (V1) and follow-up visit (V2) at the Department of Respiratory Medicine and Tuberculosis, University Hospital Olomouc, comprising of pulmonary function testing, chest x-ray, and complex clinical examination. The decision to administer CS or maintain watchful waiting (WW) was in line with Czech national guidelines.ResultsThe study involved 2729 COVID-19 survivors (45.7% male; mean age: 54.6). From 2026 patients with complete V1 data, 131 patients were indicated for CS therapy. These patients showed significantly worse radiological and functional impairment at V1. Mean initial dose was 27.6 mg (SD ± 10,64), and the mean duration of CS therapy was 13.3 weeks (SD ± 10,06). Following therapy, significantly better improvement of static lung volumes and transfer factor for carbon monoxide (DLCO), and significantly better rates of good or complete radiological and subjective improvement were observed in the CS group compared to controls with available follow-up data (n = 894).ConclusionBetter improvement of pulmonary function, radiological findings and subjective symptoms were observed in patients CS compared to watchful waiting. Our findings suggest that glucocorticoid therapy could benefit selected patients with persistent dyspnea, significant radiological changes, and decreased DLCO.

Cumulative occupational exposure to gases and fumes is associated with impairment in lung function and disease-related quality of life in a German COPD patient cohort

ObjectivesThe impact of occupational exposures on lung function impairments and quality of life (QoL) in patients with chronic obstructive pulmonary disease (COPD) was analysed and compared with that of smoking.MethodsData...

Physical exercise in patients with testicular cancer treated with bleomycin, etoposide and cisplatin chemotherapy: pulmonary and vascular endothelial function—an exploratory analysis

PurposeBleomycin, etoposide, and cisplatin combination chemotherapy (BEP) improves the survival of patients with testicular cancer, but is associated with potentially life-threatening toxicities like pneumonitis and thromboembolic events. This study explored the effects of physical exercise in patients with testicular cancer during or after BEP-chemotherapy on pulmonary and vascular endothelial toxicity.MethodsIn this post hoc analysis of a multicenter randomized clinical trial (NCT01642680), patients with metastatic testicular cancer scheduled to receive BEP-chemotherapy were randomized to a 24-week exercise intervention, initiated during (group A) or after BEP-chemotherapy (group B). Endpoints were pulmonary function (forced vital capacity (FVC), forced expiratory volume in one second (FEV1), lung transfer-coefficient and transfer factor for carbon monoxide (KCO, DLCO) and markers of vascular endothelial dysfunction (von Willebrand factor (vWF) and factor VIII).ResultsThirty patients were included. Post-chemotherapy, patients declined less in FVC, FEV1 and DLCO in group A compared to group B. Post-chemotherapy, vWF and factor VIII were significantly lower in group A compared to group B. After completion of exercise, started either during BEP-chemotherapy or thereafter, no between-group differences were found. At 1-year post-intervention, significant between-group differences were found in favour of group A in DLCO and KCO.ConclusionsPatients who exercised during BEP-chemotherapy better preserved FVC, FEV1 and DLCO, measured directly post-chemotherapy and 1-year post-intervention (DLCO, KCO). This coincided with less increase in vWF and factor VIII measured directly post-chemotherapy. These data support a beneficial role of a physical exercise intervention during BEP-chemotherapy on pulmonary and vascular damage in patients with testicular cancer.Trial registryOptimal Timing of Physical Activity in Cancer Treatment (ACT) Registry URL: https://clinicaltrials.gov/ct2/show/NCT01642680. Trial registration number: NCT01642680.

Functional disorders of the respiratory system in combat chest injury

Background. Gunshot injuries occupy one of the leading places in a number of injuries (combat injuries) that occur in military conflicts.&#x0D; Aim. To study the effect of gunshot wounds of the chest (penetrating and non-penetrating), as well as closed chest trauma on the respiratory function.&#x0D; Materials and methods. The study included 78 patients with a diagnosis of combined trauma, including a gunshot penetrating/non-penetrating wound of the chest or closed chest injuries. The median period from injury to the time of lung function tests was 17 [15; 22] days.&#x0D; Results. A prospective cross-sectional study was performed. All patients (100% males) underwent spirometry, body plethysmography, and diffusion test. The total sample was divided into two groups: group 1 (median age 27.5 [23.0; 36.0] years) 68 patients with gunshot penetrating chest wound, group 2 (median age 26.5 [20.0; 30.5] years) 10 patients with gunshot non-penetrating wound or closed chest trauma. In group 1, the medians of the vital capacity, forced vital capacity, inspiratory capacity, forced exhalation volume in 1 second, lung diffusion capacity (determined by the value of the carbon monoxide transfer-factor DLCO) and alveolar volume were reduced. In group 2, pulmonary ventilation parameters were within normal values while the median of DLCO was reduced. There were no statistically significant differences between the groups.&#x0D; Conclusion. Both gunshot penetrating wounds of the chest and gunshot non-penetrating wounds of the chest and closed chest injuries have a negative impact, including on the respiratory function, to a greater extent on lung diffusion capacity. It is necessary to create programs of drug correction and longer rehabilitation treatment of this category of patients.

No long-term effect of past Pneumocystis jirovecii pneumonia on pulmonary function in people with HIV.

To assess the impact of past Pneumocystis jirovecii pneumonia (PJP) on the pulmonary diffusion capacity in people with HIV (PWH) with a history of advanced immunodeficiency. Prospective cross-sectional study. Adult PWH with past PJP >1 year ago were included as the study group. The control group consisted of PWH with a nadir CD4+ lymphocyte count <200 cells/mm3, matched by age, sex, smoking status and time since HIV diagnosis. All PWH completed a pulmonary function test (PFT) consisting of pre-bronchodilation spirometry, body plethysmography and single-breath carbon monoxide transfer factor (TLCO) measurement. TLCO, diffusion impairment (defined as a TLCO Z-score <-1.645), total lung capacity (TLC) and forced expiratory volume in one second/forced vital capacity (FEV1/FVC) Z-scores were assessed. Multivariable regression analyses were conducted with Z-scores and odds of diffusion impairment as outcomes. PFTs of 102 participants were analyzed, 51 of whom had past PJP with a median of ten years since PJP. Mean TLCO Z-score and diffusion impairment rate did not differ significantly between groups (P = 0.790; P = 0.650). Past PJP was not independently associated with TLCO Z-score [β = 0.14; 95% confidence interval (CI) -0.30-0.57], diffusion impairment (odds ratio 1.00; 95% CI 0.36-2.75) nor TLC or FEV1/FVC Z-scores, whereas current (vs. never) smoking was associated with more diffusion impairment and lower TLCO Z-scores. In our study, past PJP was not associated with long-term diffusion impairment. Our findings suggest that smoking plays a more important role in persistent pulmonary function impairment whereas PJP-related changes seem to be reversible.

Association between DLCO index and the severity of heart failure: a cross-sectional study

Background The prognostic role of diffusing capacity of the lung for carbon monoxide (DLCO) in heart failure has not been thoroughly investigated. Therefore, this study aimed to evaluate DLCO variation in different systolic and diastolic heart failure stages. Methods This was a prospective cross-sectional study on 51 patients with systolic (reduced LVEF) or diastolic (preserved LVEF) chronic heart failure (CHF). All patients underwent a standard DLCO test. The associations between the severity of heart failure and reduced carbon monoxide transfer factor (TLCO), carbon monoxide transfer coefficient (KCO), and alveolar volume (VA) were investigated. Data were analysed using SPSS software version 16. p-Values below 0.05 were considered statistically significant. Results The mean age of participants was 59.29 ± 14.91 years, with 72% of the study population being male. Systolic heart failure was observed in 47% of patients, diastolic heart failure in 18%, and a mixed systolic and diastolic pattern in 35%. There were significant differences between TLCO percentage in patients with CHF types and the New York Heart Association (NYHA) functional classes (p = 0.042). Overall, an ejection fraction (EF) of less than 25% correlated with 3%, 53%, and 0.78 declines in TLCO, KCO%, and KCO index, respectively. Conclusion Despite the lack of statistically significant differences between DLCO indices and CHF severity, decreased DLCO parameters correlated with reduced EF. Therefore, DLCO testing might be helpful to predict HF severity.

A Systematic Review and Metaanalysis of Predictors of Mortality in Idiopathic Inflammatory Myopathy-Associated Interstitial Lung Disease.

Idiopathic inflammatory myopathy (IIM)-associated interstitial lung disease (ILD) can range from rapidly progressive disease with high mortality to indolent disease with minimal morbidity. This systematic review and metaanalysis describe immunological, clinical, and radiographical predictors of mortality in IIM-ILD. MEDLINE and Embase database searches were completed on October 18, 2021, to identify articles providing survival data according to baseline characteristics in patients with concurrent IIM and ILD. Prognostic factors common to more than 5 papers were included in the metaanalysis using a random-effects model to report odds ratios (ORs) for binary variables and Hedges g for continuous variables. Risk of bias was assessed using the Newcastle-Ottawa Scale score and the Egger test for publication bias. From 4433 articles, 62 papers were suitable for inclusion; among these studies, 38 different variables were considered. The OR for risk of death regarding the presence of anti-melanoma differentiation-associated protein 5 (MDA5) antibodies was 6.20 (95% CI 3.58-10.71), and anti-tRNA synthetase antibodies were found to be protective (OR 0.24, 95% CI 0.14-0.41). Neither antinuclear antibodies, anti-52-kDa Ro antigen antibodies, nor SSA significantly altered mortality, nor was MDA5 titer predictive. Examples of prognostic factors that are significantly associated with mortality in this study include the following: age; male sex; acute/subacute onset; clinically amyopathic dermatomyositis; dyspnea; ulceration; fever; raised C-reactive protein, ferritin, lactate dehydrogenase, alveolar to arterial O2 (A-aO2) gradient, ground-glass opacity on high-resolution computed tomography (HRCT), and overall HRCT score; and reduced albumin, lymphocytes, ratio of partial pressure of oxygen in the arterial blood to fraction of inspired oxygen (PF ratio), percentage predicted transfer factor for carbon monoxide, and percentage predicted forced vital capacity. Baseline surfactant protein-D and Krebs von den Lungen-6 levels were not predictors of mortality. Many mortality risk factors were identified, though heterogeneity was high, with a low quality of evidence and a risk of publication bias. Studies regarding anti-MDA5 antibody-positive disease and and those from East Asia predominate, which could mask risk factors relevant to other IIM subgroups or populations.

Childhood ‘bronchitis’ and respiratory outcomes in middle-age: a prospective cohort study from age 7 to 53 years

BackgroundChronic bronchitis in childhood is associated with a diagnosis of asthma and/or bronchiectasis a few years later, however, consequences into middle-age are unknown.ObjectiveTo investigate the relationship between childhood bronchitis and...

Lower transfer factor of the lung for carbon monoxide in women with a patent foramen ovale.

What is the central question of this study? Do individuals with a patent foramen ovale (PFO+ ) have a lower lung transfer factor for carbon monoxide than those without (PFO- )? What is the main finding and its importance? We found a lower rate constant for carbon monoxide uptake in PFO+ compared with PFO- women, which was physiologically relevant (≥0.5 z-score difference), but not for PFO+ versus PFO- men. This suggests that factors independent of the PFO are responsible for our findings, possibly inherent structural differences in the lung. The transfer factor of the lung for carbon monoxide (TLCO ) measure assumes that all cardiac output flows through the pulmonary circuit. However, right-to-left blood flow through a shunt can result in a lower transfer factor than predicted. A patent foramen ovale (PFO) is a potential source of right-to-left shunt that is present in ∼35% of the population, but the effect of PFO on TLCO is unknown. We sought to determine the effect of PFO on the TLCO . We conducted a retrospective analysis of TLCO data from 239 (101 women) participants. Anthropometrics and lung function, including spirometry, plethysmography and TLCO , were compiled from our previously published work. Women, but not men, with a PFO had a significantly lower TLCO and rate constant for carbon monoxide uptake (KCO ) (percentage of predicted and z-score) than women without a PFO. Women and men with a PFO had normal alveolar volumes that did not differ from those without a PFO. Correcting the data for haemoglobin in a subset of subjects did not change the results (n=58; 25 women). The lower KCO in women with versus without a PFO was physiologically relevant (≥0.5 z-score difference). There was no effect of PFO in men. This suggests that factors independent of the PFO are responsible for our findings, possibly inherent structural differences in the lung.

Differential Effects of Cannabis and Tobacco on Lung Function in Mid-Adult Life.

Rationale: Evidence suggests that the effects of smoking cannabis on lung function are different from tobacco. However, long-term follow-up data are scarce and mostly based on young adults. Objectives: To assess the effects of cannabis and tobacco on lung function in mid-adult life. Methods: Cannabis and tobacco use were reported at ages 18, 21, 26, 32, 38, and 45 years in a population-based cohort study of 1,037 participants. Spirometry, plethysmography, and carbon monoxide transfer factor were measured at age 45. Associations between lung function and cannabis use were adjusted for tobacco use. Measurements and Main Results: Data were available from 881 (88%) of 997 surviving participants. Cumulative cannabis use was associated with lower FEV1/FVC ratios, owing to a tendency toward higher FVCs. Cannabis use was also associated with higher TLC, FRC, residual volume, and Va along with lower midexpiratory flows, airway conductance, and transfer factor. Quitting regular cannabis use between assessments was not associated with changes in spirometry. Conclusions: Cannabis use is associated with higher lung volumes, suggesting hyperinflation. There is evidence of increased large-airway resistance and lower midexpiratory airflow, but impairment of FEV1/FVC ratio is because of higher FVC. This pattern of effects is different to those of tobacco. We provide the first evidence that lifetime cannabis use may be associated with impairment of gas transfer.

Therapeutic decisions in a cohort of patients with idiopathic pulmonary fibrosis: a multicenter, prospective survey from Poland.

Background:Pirfenidone and nintedanib are considered as the standard of care in idiopathic pulmonary fibrosis (IPF), but there is no consensus as to which of these two agents should be regarded as first-line treatment.Objective:To provide real-world data on therapeutic decisions of pulmonary specialists, particularly the choice of the antifibrotic drug in patients with IPF.Methods:This was a multicenter, prospective survey collecting clinical data of patients with IPF considered as candidates for antifibrotic treatment between September 2019 and December 2020. Clinical characteristics and information on the therapeutic approach were retrieved. Statistical evaluation included multiple logistic regression analysis with stepwise model selection.Results:Data on 188 patients [74.5% male, median age 73 (interquartile range, 68–78) years] considered for antifibrotic therapy were collected. Treatment was initiated in 138 patients, while 50 patients did not receive an antifibrotic, mainly due to the lack of consent for treatment and IPF severity. Seventy-two patients received pirfenidone and 66 received nintedanib. Dosing protocol (p < 0.01) and patient preference (p = 0.049) were more frequently associated with the choice of nintedanib, while comorbidity profile (p = 0.0003) and concomitant medication use (p = 0.03) were more frequently associated with the choice of pirfenidone. Age (p = 0.002), lung transfer factor for carbon monoxide (TLCO) (p = 0.001), and gastrointestinal bleeding (p = 0.03) were significantly associated with the qualification for the antifibrotic treatment.Conclusion:This real-world prospective study showed that dose protocol and patient preference were more frequently associated with the choice of nintedanib, while the comorbidity profile and concomitant medication use were more frequently associated with the choice of pirfenidone. Age, TLCO, and history of gastrointestinal bleeding were significant factors influencing the decision to initiate antifibrotic therapy.

The association of six-minute walk work and other clinical measures to cardiopulmonary exercise test parameters in pulmonary vascular disease.

IntroductionIn pulmonary vascular disease exercise, abnormalities can include reduced exercise capacity, reduced oxygen pulse and elevated VE/VCO2. The association of clinical measures such as six-minute walk work, haemodynamics, lung function and echocardiogram to peak VO2, O2 pulse and VE/VCO2 has not been fully investigated in pulmonary vascular disease.AimsTo determine the relationship of six-minute walk work and other clinical measures to peak VO2, peak O2 pulse and VE/VCO2. Additionally, to investigate the ability to predict peak VO2 from six-minute walk work and other clinical parameters.MethodsClinical data was retrospectively analysed from 63 chronic thromboembolic pulmonary hypertension (CTEPH) and 54 chronic thromboembolic disease (CTED) patients. Six-minute walk test measures, haemodynamics, lung function and echocardiographic measures were correlated with peak VO2, peak O2 pulse and VE/VCO2. Predictive equations were developed to predict peak V̇O2 in both CTEPH and CTED cohorts and subsequently validated.ResultsA number of clinical parameters correlated to peak VO2, peak O2 pulse and VE/VCO2. Six-minute walk work and transfer factor for carbon monoxide demonstrated the strongest correlation to peak VO2 and peak O2 pulse. The validation of the predictive equations showed a variable level of agreement between measured peak VO2 and calculated peak VO2 from the predictive equations.ConclusionSix-minute walk work and additionally a number of clinical test parameters were associated to peak VO2, peak O2 pulse and VE/VCO2. Six-minute walk work and transfer factor for carbon monoxide were particularly highly correlated to peak VO2 and similarly to peak oxygen pulse. The validation of the predictive equations showed a variable level of agreement and therefore may have limited clinical applicability.

Prevalence of reduced carbon monoxide transfer factor in smokers with normal spirometry

We report the prevalence of reduced levels of carbon monoxide transfer factor (TLCO) in middle-aged current or ex-smokers with normal spirometry. Spirometry and TLCO measurements were performed and we identified 391 subjects aged 40–60 years, with a significant smoking history and normal spirometry. In this group, 96 subjects (24%) had TLCO measuremements below the lower limit of normal when using the newly established Global Lung Initiative (GLI) reference equations. The measurement of TLCO should be considered as part of the standard assessment of smokers.

The impact of changing to the Global Lung Function Initiative reference equations for transfer factor in paediatrics.

The Global Lung Function Initiative (GLI) all-age reference equations for carbon monoxide transfer factor were published in 2017 and endorsed by the European Respiratory Society and American Thoracic Society. In order to understand the impact of these new reference equations on the interpretation of results in children referred from haematology and oncology paediatric services, we retrospectively analysed transfer factor results from any paediatric patient referred from haematology/oncology in the period 2010–2018. We examined transfer factor of the lung for carbon monoxide (TLCO), transfer coefficient of the lung for carbon monoxide (KCO) and alveolar volume (VA) from 241 children (age range 7–18 years, 130 male). The predicted values from Rosenthal and GLI were plotted against height. The difference in interpretation of results was analysed by looking at the percentage of patients below the lower limit of normal for each parameter. Overall, the Rosenthal predicted values for TLCO were higher than those predicted by GLI. Predicted KCO using Rosenthal was higher in all observations. In contrast, the Rosenthal predicted VA was generally lower than the GLI value. The GLI predicted values for transfer factor show considerable differences compared with currently used paediatric UK reference values, differences that will have a significant impact on interpretation of results.

"Official ERS technical standards: Global Lung Function Initiative reference values for the carbon monoxide transfer factor for Caucasians." Sanja Stanojevic, Brian L. Graham, Brendan G. Cooper, Bruce R. Thompson, Kim W. Carter, Richard W. Francis and Graham L. Hall on behalf of the Global Lung Function Initiative T LCO working group. Eur Respir J 2017; 50: 1700010.

"Official ERS technical standards: Global Lung Function Initiative reference values for the carbon monoxide transfer factor for Caucasians." Sanja Stanojevic, Brian L. Graham, Brendan G. Cooper, Bruce R. Thompson, Kim W. Carter, Richard W. Francis and Graham L. Hall on behalf of the Global Lung Function Initiative T LCO working group. Eur Respir J 2017; 50: 1700010.

Lung function in Birt-Hogg-Dub\xe9 syndrome: a retrospective analysis of 96 patients

BackgroundBirt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant disorder caused by mutations in the FLCN gene coding for folliculin. Its clinical expression includes cutaneous fibrofolliculomas, renal tumors, multiple pulmonary cysts, and recurrent spontaneous pneumothoraces. Data on lung function in BHD are scarce and it is not known whether lung function declines over time. We retrospectively assessed lung function at baseline and during follow-up in 96 patients with BHD.ResultsNinety-five percent of BHD patients had multiple pulmonary cysts on computed tomography and 59% had experienced at least one pneumothorax. Mean values of forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), FEV1/FVC ratio, and total lung capacity were normal at baseline. Mean (standard deviation) residual volume (RV) was moderately increased to 116 (36) %pred at baseline, and RV was elevated > 120%pred in 41% of cases. Mean (standard deviation) carbon monoxide transfer factor (DLco) was moderately decreased to 85 (18) %pred at baseline, and DLco was decreased < 80%pred in 33% of cases. When adjusted for age, gender, smoking and history of pleurodesis, lung function parameters did not significantly decline over a follow-up period of 6 years.ConclusionsCystic lung disease in BHD does not affect respiratory function at baseline except for slightly increased RV and reduced DLco. No significant deterioration of lung function occurs in BHD over a follow-up period of 6 years.

Long-term Hodgkin Lymphoma Survivors: A Glimpse of What Happens 10 Years After Treatment

IntroductionThis retrospective study was focused on 96 patients (median age at diagnosis, 35 years) with newly diagnosed Hodgkin lymphoma (HL) treated at the University Hospital of Bari (Italy) between 2005 and 2008, to evaluate the outcome and the long-term toxicity. Patients and MethodsFirst-line chemotherapy was ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) in all patients; 49 (51%) patients had undergone radiotherapy. At the end of treatment, 75 (78%) patients were in complete remission (CR); 18 (24%) of 75 patients relapsed after first-line treatment; 20 (21%) underwent autologous hematopoietic stem cell transplantation, and 3 (3%) underwent allogeneic stem cell transplantation. ResultsAfter a median follow-up of 12 years, 85 (88%) patients are alive in CR, and 11 (14%) have died (2 of a second neoplasia, 1 of infection, and 8 of the disease). The 140-month Kaplan-Meier survival estimates were 86%. Three women became pregnant and each gave birth to a healthy child. The most prevalent chronic conditions at last follow-up were: a reduction in lung transfer factor for carbon monoxide (40%), fatigue (31%), hypothyroidism (30%), and infertility (16%). ConclusionsResults of this study offer indications about how long after the initial treatment excess deaths from causes other than HL begin to occur. However, challenges remain, namely establishing the optimal time to begin screening for potential late complications and developing better surveillance guidelines. Further work is needed to identify risk factors that may predict specific late effects.