Respiratory Function Tests Research Articles

Efficiency and Reliability of Bronchoscopic Lung Volume Reduction Coil Application in Patients with Severe Emphysema.

In the past years, surgery has been used for the non-medical treatment of severe emphysema. However, in recent years, bronchoscopic lung volume reduction (LVR) treatment has become more preferred because it is less invasive. Bronchoscopic coil treatment is the most frequently applied technique among these methods. The aim of the investigation was to determine the efficacy and safety of bronchoscopic volume reduction coil treatment for patients with severe emphysema. The patients who were performed bronchial volume reduction coil treatment between 2015 and 2017 and were followed in our outpatient clinic were retrospectively examined. They were followed for 1 year at quarterly intervals after the procedure. All the safety and efficacy of the patient's records, including the modified Medical Research Council (MRC) dyspnea score, the St. George's Respiratory Questionnaire (SGRQ) quality of life scale, the 6 min walk distance (6-MWT), pulmonary function tests, and adverse events, were evaluated. Sixteen patients were included in the study. The mean of the preoperative mMRC clinic dyspnea score was 3.38, the mean of the 3rd month's mMRC score was 2.62 (p=0.007), and the mean of the 12th month's mMRC was 2.37 (p=0.003). The preoperative SGRQ quality of life parameter was 71.95±15.7, the 3rd month was 66.7±16.2 (p=0.007), and the 12th month was 62.9±16.4 (p=0.003). Preoperative mean of 6-MWT was 247.25±112.36 m, 3rd month 264.25±95 m (p=0.148), and 12th month 317±122.9 m (p=0.034). Patients' preoperative residual volume was 5.28±1.96 L, 3rd month 4.52±1.35 L (p=0.023), and 12th month 4.545±1.83 L (p=0.163). Patients' preoperative forced expiratory volume in one second, respectively, was 0.79±0.29 L, 3rd month 0.79±0.3 L (p=0.917), and 12th month 0.86±0.3 L (p=0.756). It seems that bronchoscopic LVR coil treatment, which is an effective and reliable procedure that reduces shortness of breath rather than respiratory function test parameters and improves the quality of daily life, will become even more widespread.

Impact of bilateral mediastinal lymph node dissection on pulmonary function during the early postoperative period after curative-intent lung surgery for cancer: a randomized study.

Bilateral lymph node dissection is not a standard surgical treatment for non-small cell lung carcinoma. However, data from anatomical studies showing lymph flow to the contralateral mediastinal lymph nodes have prompted attempts to extend lymph node dissection to the contralateral mediastinum. Little is known about the functional effects of extended lymphadenectomy. This study aimed to determine whether bilateral mediastinal lymphadenectomy (BML) performed as part of lung cancer surgery leads to more severe impairment of respiratory function than standard systematic lymph node dissection (SLND). Respiratory function data were derived from a randomized clinical study (BML-1), which included patients with non-small cell lung cancer (NSCLC) who underwent curative-intent lung resection with standard SLND or BML. Lung function tests were performed preoperatively and on postoperative days 1, 3-5, 7-9, and 30-32. Data from 89 patients were available for the final analysis of the BML-1 study: 40 and 49 underwent BML and SLND, respectively. Complete respiratory function test data were available for 35 patients. The age, sex, Thoracoscore, revised cardiac risk index, dyspnea score, lobar location of the tumor, histology, preoperative pulmonary function test results, and type of lung resection were comparable for both groups. The vital capacities (VCs) were not different on postoperative days 1, 3-5, 7-9, and 30-32 (P=0.49, 0.66, 0.18, and 0.16, respectively). The forced expiratory volumes in 1st second (FEV1) obtained at the same time points were not different (P=0.40, 0.72, 0.81, and 0.32, respectively). BML was not associated with a more severe deterioration of respiratory function than standard (unilateral) lymph node dissection.

Dyspnea assessment in myotonic dystrophy type 1

In myotonic mystrophy type 1 (DM1), combining respiratory symptom screening and respiratory function testing, is crucial to identify the appropriate time for ventilatory support initiation. Dyspnea has been little investigated in DM1. To provide a multidimensional description of dyspnea, questionnaires assessing dyspnea were administered to 34 consecutive adult patients with DM1 (median (25th-75th centile) age of 36 (28–49), Vital Capacity (VC) of 74 (64–87)% of predicted value). Dyspnea scores were low whatever the questionnaire used: Multidimensional Dyspnea Profile score of 2(0–4.7)/50 for dyspnea sensory descriptor and of 0 (0–4.7)/60 for the emotional descriptor, Visual Analogue Scale score of 0 (0–0)/10 in sitting and supine position and Borg score after six-minute walk test (6MWT) of 2.2 (1.8–4.2)/10. Eleven patients (32%) reported disabling dyspnea in daily living (modified Medical Research Council (mMRC) score ≥ 2). In comparison with patients with mMRC score < 2, patients with mMRC score ≥ 2 had a more severe motor handicap (Muscular Impairment Rating score of 4.0 (4.0–4.0) vs 3.0 (2.0–3.5), p<0.01), a lower 6MWT distance (373 (260–424) vs 436 (346–499)m, p = 0.03) and a lower VC (64 (48–74)% vs 75 (69–89)%, p = 0.02). These data suggest that the mMRC scale might be an easy-to-use and useful tool to assess dyspnea in daily living in DM1 patients. However, the interest of integrating the mMRC dyspnea scale in clinical practice to guide therapeutic management of DM1 patients remains to be assessed in further studies.

Survey the proportions of TCM symptoms and patterns in stable COPD patients at University Medical Center HCMC

COPD affects the quality of a patient’s life and leads to death. Identifying TCM symptoms and clinical patterns proportions in the community will make diagnosis and treatment more effective. In Vietnam, there were no epidemiological documents about COPD in TCM. Therefore, this study wished to survey stable COPD patients to find out the proportions of symptoms and patterns in TCM to create a basis for further practice. The survey was conducted as a descriptive cross-sectional study. Participants were stable COPD outpatients at the Clinic and respiratory function test of the University Medical Center HCMC from September to December 2018. According to the survey form based on the TCM diagnostic criteria, the proportions of symptoms and patterns were recorded. After three months, 116 patients were recruited. The proportions of 30 TCM symptoms and signs were recorded. The proportions of patients were as follows: 80.17% met Lung qi deficiency, 76.72% for Lung-kidney qi deficiency, 53.45% for Lung-kidney qi and yin deficiency, 47.41% for Lung-spleen qi deficiency, and 2.59% for not meeting any of the diagnostic criteria. Patterns were overlapping in the same patients. All TCM symptoms and patterns in the diagnostic criteria appeared in COPD patients. The deficiency of the Lung and Kidney was the most common. Most patients were classified into many different patterns with multi organs damage. More multicenter studies with bigger participants numbers are suggested.

UNILATERAL PULMONARY ARTERY ATRESIA/AGENESIS: A CASE REPORT

A twenty-three-year-old male truck driver with a 12-pack year smoking history and no previous history of hospitalization was admitted to the “Shefqet Ndroqi” University Hospital pulmonology department after a cardiologist consultation where he was diagnosed with pulmonary hypertension. Two of the patient’s uncles from his mother’s side died during their pediatric age from congenital heart defects. A contrast-enhanced chest computed tomography was performed, and it showed an interruption of the lumen of the proximal part of the left pulmonary artery (atresia), a reduction of the left lung parenchyma volume, and bilateral pulmonary emphysema. The respiratory functional tests detected a moderate obstructive syndrome, and the 6MWT detected a decrease in arterial partial pressure of oxygen and oxygen saturation level. A diagnosis of unilateral pulmonary artery atresia/agenesis was confirmed.

SUBJECTIVE SLEEP QUALITY IN QUITTERS VERSUS NONQUITTERS ON A TOBACCO SMOKING CESSATION PROGRAM

Amaç&#x0D; Uyku kalitesi ile ilişkili faktörler sigara kullananların bırakma&#x0D; programına uyumları ile ilişkilidir. Bu çalışma,&#x0D; en az bir yıldır sigara kullanımını bırakmada başarılı&#x0D; olan ve olamayan kişiler arasındaki uyku kalitesini&#x0D; karşılaştırmayı amaçlamaktadır.&#x0D; Gereç ve Yöntem&#x0D; Sigara bırakma programında olan toplam 682 kişi Mart&#x0D; 2004-Şubat 2011 tarihleri arasında çalışmaya alınma&#x0D; uygunluğu açısından incelenmiş ve uygun olanlar telefon&#x0D; ile çalışmaya katılmaya davet edilmişlerdir. Davet&#x0D; edilenlerden 106 kişi (Erkek/Kadın 67/39) çalışmaya&#x0D; katılmayı kabul etmiş ve final analizlerine dahil&#x0D; edilmişlerdir. Pittsburgh uyku kalite indeksi skorları,&#x0D; solunum fonksiyon testi ve karbon monoksit ölçümleri&#x0D; başarılı bir şekilde sigarayı bırakanlar (n=42) ve bırakamayanlar&#x0D; (n=64) arasında karşılaştırılmıştır.&#x0D; Bulgular&#x0D; Sigarayı bırakanların bırakmayanlara göre uyku kalitelerinin&#x0D; daha iyi olduğu saptandı. Pittsburgh uyku kalitesi&#x0D; indeksine göre bırakan olguların %23,8’i (10/42),&#x0D; sigarayı bırakmayanların ise sadece %7,8’i (5/64) iyi&#x0D; uyku kalitesi skoruna sahipti (p=0.021). Sigarayı bırakanların&#x0D; vücut ağırlığı ortalama 8,33 kg, vücut kitle&#x0D; indeksi ortalama 3,13 artmıştı (p

Pulmonary Fibrosis Related to Amiodarone-Is It a Standard Pathophysiological Pattern? A Case-Based Literature Review.

Amiodarone hydrochloride is an antiarrhythmic drug, with proven efficacy in prevention and treatment of numerous arrhythmias, atrial fibrillation especially, or ventricular arrhythmias, with a long half-life (55-60 days). The increased risk of developing amiodarone-induced pulmonary fibrosis is directly related to the dose and the duration of the intake. Amiodarone-induced pulmonary toxicity is conditioned by dose, patient's age, and pre-existent pulmonary pathologies. The pattern for drug-induced lung injury may vary in many forms, but the amiodarone can cause polymorphous injuries such as diffuse alveolar damage, chronical interstitial pneumonia, organizing pneumonia, pulmonary hemorrhage, lung nodules or pleural disease. The pathological mechanism of pulmonary injury induced by amiodarone consists of the accumulation of phospholipid complexes in histocytes and type II pneumocytes. Differential diagnosis of pulmonary fibrosis induced by amiodarone is made mainly with idiopathic pulmonary fibrosis, left ventricular failure or infectious disease. Before starting treatment with amiodarone, patients should be informed of potential adverse effects and any new respiratory symptoms should promptly be reported to their family physician or attending physician. The assessment carried out at the initiation of amiodarone treatment should include at least chest X-ray and respiratory function tests and extrapulmonary evaluation.

Smoking-Related Interstitial Fibrosis (SRIF) in Patients Presenting With Diffuse Parenchymal Lung Disease

To describe the clinical, radiologic, and pathologic findings in cases where smoking-related interstitial fibrosis (SRIF) was diagnosed in surgical lung biopsy specimens from patients with clinical and imaging features of diffuse parenchymal lung disease (DPLD). Cases were included in this study if patients had clinical and imaging evidence of DPLD and surgical lung biopsy specimens revealed SRIF. A dedicated multidisciplinary conference was held to correlate clinical, radiologic, and pathologic findings. Six cases met inclusion criteria; all six (five women/one man, aged 42-57 years, mean age 47 years) were either current smokers (five of six) or ex-smokers (one of six) and were evaluated for respiratory symptoms and abnormal pulmonary function tests, most commonly reduced forced vital capacity (n = 3) and diffusing capacity for carbon monoxide (n = 6). The most common imaging abnormalities were bilateral ground-glass opacities, which correlated with histopathologic SRIF. Follow-up of up to 10 years showed stable or improved clinical symptoms, pulmonary function tests, and radiologic findings with smoking cessation (three patients) or a decrease in smoking (three patients). No specific treatments were given, and those treated with empiric corticosteroid tapers did not show discernible responses. SRIF can present as clinically meaningful diffuse parenchymal lung disease in relatively young heavy smokers, characterized by bilateral ground-glass opacities and a stable clinical course.

КЛИНИЧЕСКИЕ ПРОЯВЛЕНИЯ РЕКОНВАЛЕСЦЕНЦИИ У ПАЦИЕНТОВ, ПЕРЕНЕСШИХ НОВУЮ КОРОНАВИРУСНУЮ ИНФЕКЦИЮ COVID-19, В ЗАВИСИМОСТИ ОТ ДЛИТЕЛЬНОСТИ ПОСТКОВИДНОГО ПЕРИОДА И СТЕПЕНИ ПОРАЖЕНИЯ ЛЕГКИХ

Abstract. Introduction. Post-COVID syndrome is a complex of symptoms that develops in almost half of the patients who have had a new coronavirus infection (COVID-19). The frequency of symptom development based on the duration of the post-COVID period, age, gender, and severity of the course of the disease are highly relevant subjects, as they will allow to form approaches to the diagnosis, treatment, and prevention of post-COVID syndrome. Material and methods. 43 patients were included (men – 10, women – 33), age 65 [60–71]. The questionnaire was designed after a comprehensive review of literature from the past studies based on similar objectives. We divided patients in two groups in depending on recovery: up to 3 months (n=23), after to 3 months (n=20). In depend on degree of lung parenchymal abnormalities two groups were formed: less than 25%, more than 25%. Vital signs (respiratory rate, SpO2 , hearth rate) were collected. The tolerance in physical activity we investigate by modified Medical Research Council breathlessness scale – mMRC, six-minute test. Borgscale, hurt rate and oxygen saturation were measured immediately before and after test. Data were analyzed using Statistical Package for Social Sciences version 22.0. Results and discussion. The majority of the patients complained on persistent fatigue (75%), dyspnea (59%), joint pain (45%), anxiety (38%) and chest pain (37%). We didn’t find difference in frequencies of majority of complaints in depend on duration of post- COVID period. In patients with LA&gt;25% mMRC, respiratory rate, Borg scale in six-minute test higher than in patients with LA&lt;25%. Conclusion. In elderly patients, symptoms persisted for more than 3 months after a new coronavirus infection, which is associated with a higher risk of chronic pathological processes in elderly patients. With lung lesion volume of more than 25%, respiratory functional test values are higher than in the group of patients with smaller lesion volume, which is most likely caused by preserved structural damage of lung tissue.

The impact of multistrains of probiotics on Th17-related cytokines in patients with asthma: a randomized, double-blind, placebo-controlled trial

Objective Asthma is known as one of the most common chronic inflammatory diseases characterized by recurrent obstruction and inflammation of the airways. Probiotics are defined as a group of beneficial living microorganisms that are beneficial in many disorders, including allergies. The aim of this study was to investigate the probiotic supplement effects on improvement of clinical asthma symptom and changes in the pattern of Th17-related inflammatory cytokines in asthmatic patients. Methods This was a randomized controlled clinical trial with parallel, double-blind groups. Forty patients with asthma were enrolled and received 1 capsule/day of a probiotic supplement for 8 weeks. Respiratory function tests; and the level of IL-6, IL-17, IL-21 and TGF-β were evaluated at the baseline and end of intervention. Results The results showed that the level of IL-6 and IL-17 in patients after receiving probiotics was reduced and expression of TGF-β was increased as compared to the baseline. Also, the expression of IL-17 and IL-21 in the probiotic group was significantly lower than the placebo group at the end of the intervention. In addition, an improvement in pulmonary function tests and clinical symptoms was observed after receiving probiotics. Conclusions Eight-weeks treatment with a probiotic supplementation suggests that it may effect on Th17 cells-associated IL-6, IL-17 and TGF-β; and Forced Expiratory Volume in 1 s and Forced Vital Capacity. Taken together, these results suggest that probiotics may have the ability to affect neutrophilic asthma and they can possibly be used besides common treatments for patients with neutrophilic asthma.

Effect of Omega-3 Polyunsaturated Fatty Acids Intake on Eosinophil Airway Inflammation in University Athletes.

Though athletes have a high risk of respiratory disorders, effective prevention has not yet to be identified. Omega-3 (n-3) polyunsaturated fatty acids (PUFA) have some practical anti-inflammatory effects in allergy, and therefore may reduce airway inflammation in athletes. This study aimed to assess whether n-3 PUFA intake affects airway inflammation in university athletes. Twenty-three males were divided into three groups: 1) the eosinophilic airway inflammation group (I_PUFA group; fractional exhaled nitric oxide (FeNO ≥ 25 ppb, n = 10); 2) the non-eosinophilic airway inflammation group (N_PUFA group; FeNO < 25 ppb, exhaled carbon dioxide (eCO) ≥ 3.6 ppm, n = 5); and 3) the control group (FeNO < 25 ppb, eCO < 3.6 ppm, n = 8). Participants took supplements containing 260 mg of docosapentaenoic acid and 600 mg of eicosapentaenoic acid (EPA) daily for 3 weeks. Baseline measurements of FeNO, respiratory impedance, respiratory function, dietary intake (food frequency questionnaires), and blood tests were performed. FeNO and respiratory impedance were measured weekly, and the rest were measured after 3 weeks. There was a significant decrease in FeNO levels from baseline at 2 and 3 weeks in the I_PUFA group (54.7 ± 8.5 ppb vs. 45.1 ± 9.1 and 45.4 ± 7.7 ppb; mean ± standard error (SE), P < 0.05). After 3 weeks, FeNO levels remained unchanged in the N_PUFA and control groups, and respiratory impedance and function remained unchanged in all groups. Blood EPA levels significantly increased in the I_PUFA and N_PUFA groups (I_PUFA, 27.7 ± 16.9 vs. 52.1 ± 12.3 µg/mL; N_PUFA, 20.8 ± 8.7 vs. 70.4 ± 36.1 µg/mL; mean ± standard deviation (SD), P < 0.05). No changes were observed in dietary intake over the 3 weeks. n-3 PUFA supplementation for 3 weeks reduced airway inflammation in athletes with FeNO levels ≥ 25 ppb.

Do handgrip strength and dexterity predict respiratory function in neuromuscular disease?

Neuromuscular diseases are acquired or inherited diseases that affect the function of the muscles in our body, including respiratory muscles. We aimed to discover more cost-effective and practical tools to predict respiratory function status, which causes serious problems with patients with neuromuscular disease. The Vignos and Brooke Upper Extremity Functional Scales were used to evaluate functional status for patient recruitment. The handgrip strength and dexterity of patients were measured using a dynamometer and nine-hole peg test. Respiratory function parameters: forced vital capacity, forced expiratory volume in one second, and peak expiratory flow were evaluated using spirometry. The mean age of the 30 patients was 11.5 ± 3.79 years old. Significant relationships were found between nine-hole-peg-test scores and respiratory function parameters on both sides. Significant correlations were found between both handgrip strength and respiratory function parameters (p < 0.05). In the linear regression analysis, it was seen that the forced expiratory volume in 1 second, and peak expiratory flow values could be explained in different percentages (p < 0.05). Handgrip strength and dexterity measurements can be used as indicators for estimating respiratory function parameters in terms of cost and accessibility, although it is known that they will not replace respiratory function tests.

Results of a Series of Epidemiological Investigations on Health Effects in Toner-Manufacturing Workers

A cohort study spanning ten years was conducted to assess the health effects on toner manufacturing workers. A survey consisting of questions investigating subjective respiratory symptoms, current medical history, disease occurrence, dust exposure concentrations at the workplace, respiratory function tests, biochemical and immunological items in blood and urine, and a chest radiograph or chest computed tomography survey was conducted. The results of these surveys have been published in academic journals, and none of the surveys showed any findings suggesting significant health problems in the toner-worker group compared to the non-toner-worker group. The results suggest that the health risks associated with toner handling are not high when the work environment at the toner handling site is well controlled.

Post-COVID Pulmonary Function Test Evaluation.

Since the lung is the most affected organ by COVID-19 disease, we aimed to evaluate the pulmonary function test, presence of hypoxemia, and Post-COVID-19 Functional Status Scale in 3- to 6-month post-COVID period. Post-COVID-19 Functional Status Scale, pulse oxygen saturation, and pulmonary function test were evaluated in 67 outpatients/inpatients after 3-6 months following COVID-19 (positive reverse transcription-polymerase chain reaction on nasopharyngeal swab) disease. Pre-COVID pulmonary function test parameters were available in 33 patients, and these were compared with post-COVID pulmonary function test parameters. We found 20.9% (14 patients) restrictive and 11.9% (8 patients) obstructive patterns in pulmonary function test. Of those with forced vital capacity < 80%, 53.3% were patients without known lung diseases. When pulmonary function test values before and after COVID-19 were compared, only a loss of 130 mL in forced expiratory volume in 1 second was determined (P = .005). About 65.4% of the patients with dyspnea were in the group without a lung disease (P = .002) and 66.7% of patients with forced expiratory volume in 1 second and forced vital capacity of .05). Smoking, hospitalization, oxygen support, and the severity of computed tomography involvement did not impact pulmonary function test. In post-COVID patients, the major disorder in the respiratory function test was determined as a restriction. However, advanced tests such as lung volumes and carbon monoxide diffusing capacity (DLCO) measurement and high-resolution lung tomography are needed to differentiate in terms of physical functional limitation or parenchymal fibrosis.

EOSINOPHILIC ESOPHAGITIS AND CHRONIC COUGH ORIGINATED BEYOND THE RESPIRATORY TREE

Background Chronic cough (CC) as the main symptom of eosinophilic esophagitis (EoE), associated or not with other signs of esophageal dysfunction,has been poorly studied. Objectives Research the frequency of CC symptoms of EoE and their characteristics. Comorbidities, adherence to treatment, and evolution. Methods An observational, prospective study. Variables: Epidemiological, demographics, atopic, endoscopic, histological, comorbidities, symptoms, time of evolution, endoscopic phenotype, respiratory function tests. Adherence, response to treatment, complications, and evolution Results Only 20 patients (5%) with EoE had CC.The mean age:24 years, male 85%.The time of evolution: 5 years. CC: recurrent during the day (never at night), worsened with food intake (during or after), improved when remission EoE and reappeared when active EoE.There were a few severe complications. Conclusion Although the frequency of CC as a symptom in EoE is low,it must be considered,especially if it appears in food intake.

Respiratory and functional tests in the assessment and prognosis of patients with chagas disease

Chagas disease causes an important impact on cardiorespiratory system, functional and working capacity of the patient. Performing a functional assessment on these individuals becomes essential, since the impact of physical limitation is an important factor that is related with other life domains, such as risk of cardiovascular events in a brief period and risk of death. The goal of this work was to perform a literature review to present the evaluative and prognostic value of main valid respiratory and functional tests in patients with Chagas disease, as well as their applicability and reproducibility. A literature search was performed from 1981 to 2020 in Scielo, Pubmed/Medline, Science Direct, Scopus and Web of Science databases. Only complete studies published in English, Portuguese or Spanish were analyzed which specifically discussed the respiratory and functional tests used in patients with Chagas disease. In total, 544 articles were retrieved of which 35 were eligible. The main functional tests approached were Cardiopulmonary Exercise Testing, Incremental Exercise Testing and Six-Minute Walk Test. Several studies show themselves as useful markers for predicting quality of life, mortality and functional impairment, in addition to other patients’ characteristics. Several tests can be used to assess the respiratory and functional impact to patients’ health, as well as its relationship with important domains of their lives. KEY WORDS: Chagas disease; Chagas cardiomyopathy; exercise test; respiratory function tests; prognosis.

Prevalence and implications of exercise pulmonary hypertension in chronic thromboembolic pulmonary disease

Abstract Introduction Recent international statement on chronic thromboembolic pulmonary hypertension proposed the definition of chronic thromboembolic pulmonary disease (CTEPD) and advocated further research regarding its clinical characteristic, natural history, and therapeutic strategy. Exercise pulmonary hypertension (Ex-PH) has been considered a mild degree of pulmonary hypertension (PH) among patients with normal pulmonary hemodynamics at rest. However, the clinical significance of Ex-PH in CTEPD remains unknown. Purpose In the patients with CTEPD, we aimed to verify 1) the prevalence and clinical profiles of Ex-PH, 2) effect of BPA on pulmonary vascular response after exercise in Ex-PH, 3) long-term clinical outcomes of conservative management in non-Ex-PH. Methods We retrospectively reviewed 26 patients with CTEPD (median age 65 years, 38% male), who underwent cardiopulmonary exercise test with right heart catheterization (CPET-RHC). The definitions of CTEPD are the following 1) pulmonary artery occlusion due to organic thrombus confirmed by imaging studies after ≥3 months of anticoagulation, 2) mPAP&amp;lt;25 mmHg and PAWP≤15 mmHg at rest. PQslope was plotted using multipoint plots. Ex-PH was defined by PQ slope&amp;gt;3.0, and the patients were divided into Ex-PH and non-Ex PH groups. Clinical profiles and long-term outcomes were compared between two groups. The patients in Ex-PH groupunderwent CPET-RHC 6–12 months after balloon pulmonary angioplasty (BPA). In Non-Ex-PH group, serial measurements of echocardiography were performed. Results Overall, 5 and 21 patients were categorized as CTEPD with PH (mPAP 21–24mmHg) and without PH (mPAP≤20mmHg), and 14 and 12 were categorized Ex-PH and non-Ex-PH groups, respectively. Although all 5 patients with CTEPD with PH were classified as Ex-PH group (Figure 1), there was no significant difference in baseline hemodynamics at rest between Ex-PH and non-Ex-PH groups (mPAP: 19.5 [18.4–20.6] vs. 17.7 [16.6–18.9] mmHg, PVR: 2.2 [1.7–2.7] vs. 2.3 [1.9–2.8] wood units, P&amp;gt;0.05, respectively). PQ slope was significantly higher in Ex-PH group (4.6 [3.2–6.0] vs. 1.31 [0.2–2.8], p=0.002). There were no differences in respiratory function test, blood gas analysis, and 6-minute walk distance between two groups. There were no major adverse events such as all-cause mortality and hospitalization for PH in overall cohort. Among Ex-PH group, BPA decreased PQslope (4.8 [3.6–6.4] to 2.3 [1.9–3.0], p&amp;lt;0.05). Among no-Ex-PH group, there was no significant change in tricuspid regurgitation pressure gradient (28 [17–33] to 27 [21–36] mmHg, p&amp;gt;0.05) over the 997 [651–1451] days. Conclusion Ex-PH was common in patients with CTEPD, and there were no clinical profiles differentiating Ex-PH from non-Ex-PH, except parameters of CPET-RHC. BPA improved an abnormal pulmonary vascular response to exercise in Ex-PH. The conservative management in non-Ex-PH was feasible. Randomized clinical trials will be needed to further investigate this treatment strategy. Funding Acknowledgement Type of funding sources: None.

Primary ciliary dyskinesia diagnosis management in low-resource setting, a practical approach

Abstract Primary ciliary dyskinesia (PCD) is a congenital disorder with genetic determinism, characterized by chronic infections of the upper and lower respiratory tract. The triad of situs inversus, chronic sinusitis and bronchiectasis is called Kartagener Syndrome. PCD is a predominantly autosomal recessive disease, involving more than 40 mutations of genes. Abnormal cilia movement leads to defective mucociliary clearance resulting in chronic cough, nasal congestion, recurrent otitis, chronic sinusitis, male infertility and other rare conditions. First presented case is one of a 17-year-old girl with chronic treatment for asthma for the last 5 years. Respiratory functional tests were performed: the patient didn’t present variable airflow obstruction. The diagnosis of PCD was suspected because of chronic respiratory infections in a situs-inversus patient. The second case is a 4-year-old patient with a clinical history highly suggestive of ciliary dyskinesia, who received a recommendation for genetic testing to confirm the diagnosis. The third case is an 8-year-old boy with wet cough from neonatal period, bronchiectasis and sinusitis. Both his symptoms and the fact that his adult brother presented situs inversus, chronic sinusitis and bronchiectasis led us to raise suspicion of PCD. These case reports aim to highlight the real-life difficulties in a rather low-resource setting in diagnosing PCD, a genetic syndrome with long term impact on airways. The second objective was outlining the clinical manifestations that are highly suggestive of PCD and also to develop a diagnostic algorithm for a real-life scenario involving a pulmonologist with reduced access to high-performance tools like TEM (transmission electron microscopy) and complex genetic tests. Because not all PCD cases have known genetic mutations and some don’t have ultrastructural abnormalities we could still rely on the old saccharin test in order to select patients that are candidates for nasal NO. A high index of suspicion for PCD should be present in any patient with non-cystic fibrosis bronchiectasis patient. PCD remains a rare and underdiagnosed genetic syndrome in real-life paediatric setting in countries with limited neonatal screening programs. Increasing awareness of this condition and accessible diagnostic tools are desirable in order to find patients with high-likelihood of PCD and to refer these selected patients to specialized centres. Authors provide an age-dependent approach of PCD patients in a low resource setting.

Utility of cytokines CXCL4, CXCL8 and GDF15 as biomarkers in systemic sclerosis

Background and objectivesSystemic sclerosis (SSc) is an autoinmune disease that can affect several organs and its mortality is fundamentally related to its pulmonary involvement. There are some cytokines with high serum levels of patients with SSc. Our goal is to determine the role of CXCL4, CXCL8 and GDF15 in the physiopathology of SSc and whether they can be considered organic damage biomarkers. Patients and methodsObservational case–control study of SSc patients (ACR/EULAR 2013 criteria). Demographic, clinical, analytical, activity, severity, health perception, and disability variables were collected. Moreover, Videocapillaroscopy, Echocardiography and Respiratory Function Test were made. Serum levels of CXCL4, CXCL8 and GDF15 were measured both in SSc patients and in healthy controls. ResultsA total of 42 patients were included (95.4% women), with an average age of 59.2 years and a median of 4 years from diagnosis. We also included 42 healthy controls. We found significantly higher levels of GDF15 in SSc patients than in controls (p<0.001), but no higher CXCL4 or CXCL8 levels. GDF15 was associated with Diffuse SSc, pulmonary arterial hypertension, interstitial lung disease, less forced vital capacity, high titles of antiScl70, disease activity, and dilated loops in capillaroscopy. CXCL4 levels were associated to a higher Rodnan punctuation, while CXCL8 was associated to C4 fraction consumption and tortuosities in capillaroscopy. ConclusionsGDF15 high levels were associated with diffuse SSc, lung impairment, disease activity and changes in capillaroscopy. Moreover, CXCL4 was only associated with skin impairment, while CXCL8 was not related to organic damage.

Enfermedades pulmonares intersticiales

Interstitial lung diseases are a heterogeneous set of illnesses that show pathophysiological similarities with variable symptoms and radiological findings. They have diverse prognoses. Their classification is varied and may be based on their etiology or radiology. According to their etiology, they can be classified as idiopathic, of known etiology, or granulomatous. In regard to their radiology, they can be fibrotic or non-fibrotic. The symptoms are nonspecific, which entails a difficult and late diagnosis. The most common symptom is dyspnea together with persistent dry cough. It can be accompanied by systemic symptoms. The diagnosis is based on symptoms, a physical examination, and additional tests (laboratory, imaging, and respiratory function tests and invasive techniques). Treatment is based on symptom management (oxygen therapy), the prevention of exacerbations (vaccination, rehabilitation), immunosuppression (glucocorticoids, among others), and antifibrotic therapy (nintedanib and pirfenidone). The final step is lung transplantation.