Common but neglected problem in chronic spontaneous urticaria: Sleep disturbance.

Background: Association of chronic spontaneous urticaria (CSU) with sleep disturbance has not been evaluated in studies that involve a large number of patients. Objective: In this study, we aimed to evaluate the sleep attitude and circadian rhythm in patients with CSU. Methods: As the patient group, recently diagnosed 100 patients with CSU, 100 patients with allergic rhinitis (AR) as the patient control group, and 100 healthy controls (HCs) were included. The Pittsburgh Sleep Quality Index (PSQI) questionnaire, sleep hygiene index (SHI), Epworth Sleepiness Scale (ESS) questionnaire, and the morningness-eveningness questionnaire (MEQ) were filled to assess sleep quality and circadian rhythm. CSU disease activity was evaluated by urticaria activity score-7 (UAS-7). Patients with concomitant diseases, e.g., psychiatric illnesses, that possibly affect sleep status or those who use related medications and at moderate or high risk of obstructive sleep apnea according to the STOP-Bang questionnaire were excluded from the study. Results: PSQI, SHI, and ESS scores were higher, and the MEQ score was lower in patients with CSU and patients with AR than those in the HCs (p < 0.001, for each score). However, the scores were not different among the patients with CSU and the patients with AR. UAS-7 was only correlated with PSQI scores (r = 0.402, p < 0.001). In addition, blood eosinophil counts and the serum C Reactive Protein (CRP) level were correlated with sleep quality (p = 0.02). Conclusion: The poor sleep quality, impaired sleep hygiene, increased daytime sleepiness, and intermediate type of circadian rhythm were observed in the patients with CSU and the patients with AR. Physicians should be aware of sleep problems in patients with CSU that might affect their quality of life and the success of their treatment.

Importance of oxidative stress in the evaluation of acute pulmonary embolism severity.

BACKGROUND: Pulmonary embolism (PE) is a common and potentially life-threatening disorder. Our study was aimed to investigate whether oxidative stress markers can be used as clinical markers in the evaluation of acute PE (APE) severity. METHODS: 47 patients with objectively documented diagnosis of APE were recorded. Of these patients, 14 had low-risk PE, 16 had moderate-risk PE, and 17 had high-risk PE. 21 healthy subjects were also enrolled in this study. Ischemia-modified albumin (IMA), prooxidants-antioxidants balance (PAB), advanced protein oxidation products (AOPPs), and ferric reducing antioxidant power (FRAP) were measured as oxidative stress parameters to evaluate the role of oxidative stress. RESULTS: In the low-risk and moderate-risk APE groups, AOPPs and PAB levels were significantly higher and FRAP levels were significantly lower than those in the control group. AOPPs and IMA levels in the patients with high-risk PE were significantly higher than those in both the low-risk and moderate-risk APE patients. There was a significant correlation between levels of AOPPs and the levels of both IMA (r: 0.462, p < 0.001) and PAB (r:0.378, p < 0.005). Serum FRAP levels were negatively correlated with PAB (r:- 0.683, p < 0.001) and AOPPs levels (r:- 0,384, p < 0.001). There was also a significant positive correlation between the serum IMA and PAB levels. CONCLUSIONS: We clearly demonstrated that reactive oxygen species formation is significantly enhanced in APE. IMA and AOPPs may be used as clinical markers in the evaluation of APE severity in clinical practice. However, further studies with larger patient populations and longer follow-up periods are required to confirm the mechanisms underlying these findings.

The relationship between T-wave peak-to-end interval and hemodynamic parameters in patients with pulmonary arterial hypertension.

BACKGROUND: T-wave peak-to-end interval (TPEI) is a measure of repolarization dispersion on surface electrocardiogram (ECG). TPEI has been reported as a prognostic parameter with heart disorders. In this study, we aimed to evaluate the relationship between echocardiogram-derived right heart parameters, right heart catheterization (RHC) measurements, and TPEI in patients with precapillary pulmonary arterial hypertension (PAH). METHODS: Thirty-eight patients (29 females and 9 males, mean age of 54.9 ± 10.9 years) who had undergone RHC for a preliminary diagnosis of pulmonary hypertension (PH) were included in the study. We performed transthoracic echocardiography (TTE), and resting 12-lead ECG was recorded before RHC. TPEI was measured from leads of V1-V6, DII, DIII, and aVF, and these values are averaged to obtain the global TPEI. RESULTS: Duration of TPEI was significantly correlated with mean PAP, pulmonary vascular resistance (PVR), and cardiac index (CI). Longer TPEI was associated with higher N terminal probrain natriuretic peptide (NT pro-BNP) level, lower 6-min walk distance (6MWD), and lower tricuspid annular plane systolic excursion (TAPSE). CONCLUSION: Prolongation of TPEI could be a new predictor of adverse outcome in PAH and may provide additional prognostic information for patients with PAH.

[Efficiency of nebulised magnesium sulphate in infective exacerbations of chronic obstructive pulmonary disease]. / Kronik obstrüktif akciger hastaligi enfektif alevlenmelerinde nebül magnezyum sülfatin etkinligi.

INTRODUCTION: Conflicting results has been achieved in a small number of clinical studies evaluating the efficiency of magnesium sulphate (MS) in COPD exacerbations. We aimed to investigate the efficiency of nebulised MS in COPD exacerbations. PATIENTS AND METHODS: Twenty patients who met the study criteria were randomized into two groups. All patients were treated with O2, antibiotics and oral corticosteroids. Additionally one group received ipratropium bromide (IB) 500 µg together with MS 151 mg/dose, while the other group received IB together with placebo. The patients were followed-up with forced expiratory volume in 1 second (FEV1) and visual analogue scale dyspnea scores for 48 hours. Peak expiratory flow rates (PEFRs) were measured before and 10, 30, 60 and 120 minutes after each nebule treatment. RESULT: The baseline characteristics of the patients in both groups were similar. The FEV1 values measured at 24 and 48 hours did not show significant changes compared to baseline in both groups. Dyspnea scores in both groups decreased significantly in the first day, and in only MS group in the second day. The % change in the dyspnea score at the end of first day was significantly more in the MS group [-23.8% (13.6)] compared with the placebo group [-9.4% (12.9)] (p= 0.002). The % changes in PEFRs at 10 minutes [4.7 (7.5) and -3.5 (6.0), p= 0.005] and 30 minutes [8.2 (6.7) and 1.3 (5.5), p= 0.03] were significantly greater in the MS group compared with the placebo group on the first day. No side effects developed due to MS. CONCLUSION: Nebulised MS is a cheap, feasible and safe drug that can be added to the standart bronchodilator treatment since it provides additional relief of dyspnea in patients with COPD exacerbations. This needs to be evaluated in future clinical studies including greater number of patients.

Does the 6-minute walk test predict nocturnal oxygen desaturation in patients with moderate to severe COPD?

Patients with chronic obstructive pulmonary disease (COPD) who have nocturnal oxygen desaturation (NOD) can be treated with nocturnal oxygen therapy (NOT) to avoid possible morbidity and mortality. Although there is no definite data recommending NOT alone, our aim is to evaluate the relationship between desaturation during the six-minute walk test (6MWT) and NOD in COPD. Fifty-five stable patients with COPD were enrolled in this study. The 6MWT and nocturnal oximetry were performed. Patients with comorbid diseases and respiratory failure were excluded. In total, 55 patients (49 males and 6 females, mean age: 65.8 ± 8.4 years) were analysed. Twenty-seven of the patients had moderate COPD and the remainder (n = 28) had severe COPD. Three patients (11%) with moderate COPD and 12 patients (42.9%) with severe COPD desaturated during 6MWT (p = 0.003). NOD was observed in five patients with severe COPD (17.9%). There were no patients with NOD in the moderate COPD group. Three (25%) of patients with severe COPD who desaturated during the 6MWT also had NOD. NOD was more common in patients with severe COPD and the patients with higher carbon dioxide levels (p = 0.02 and p = 0.001). Three patients (11%) with moderate COPD desaturated during the 6MWT; however they did not have NOD. Although the sample size in this study was too small to be conclusive, NOD was more common in desaturators during the 6MWT particularly in patients with severe COPD.

(18)F-fluorodeoxyglucose uptake by positron emission tomography in patients with IPAH and CTEPH.

Pulmonary arterial hypertension (PAH) is driven by pathologies associated with increased metabolism such as pulmonary revascularization, vasoconstriction and smooth muscle cell proliferation in pulmonary artery wall. 18-fluorodeoxyglucose positron emission tomography (18FDG-PET) is an imaging technique sensitive to glucose metabolism and might be considered as a non-invasive method for diagnosis due to significant role of inflammation in idiopathic pulmonary artery hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH). The present study aimed to investigate the role of PET/CT imaging of patients with IPAH and CTEPH as an alternative diagnosis method. Demographic characteristics, FDG uptake in lungs, pulmonary artery and right ventricle (RV) of 17 patients (10 IPAH, 7 CTEPH), and 30 controls were evaluated. PET scanning, 6-min walk test, pro-BNP level, right heart catheterization of patients were performed both at the onsert and after 6-month PAH specific treatment. IPAH and CTEPH patients had significantly higher left lung FDG (p = 0.006), right lung FDG (p = 0.004), right atrial (RA) FDG (p < 0.001) and RV FDG (p < 0.001) uptakes than controls. Positive correlation was detected between the RV FDG uptake and the mean pulmonary artery pressure (mPAP) (r = 0.7, p = 0.012) and between the RA FDG uptake and the right atrial pressure (RAP) (r = 0.5, p = 0.02). Increased RV FDG and RA FDG uptakes predicts the presence of pulmonary hypertension and correlates with mPAP and RAP, respectively, which are important indicators in the prognosis of PAH. Further studies are required whether FDG PET imaging can be used to diagnose or predict the prognosis of pulmonary hypertension.

Asthma-chronic obstructive pulmonary disease overlap: Results from a national-multicenter study.

Introduction: Patients with asthma-chronic obstructive pulmonary disease (COPD) overlap (ACO) have a greater disease burden than those with COPD or asthma alone. In this study, it was aimed to determine the prevalence, risk factors, and clinical features of ACO because there are limited national data in Türkiye. Materials and Methods: The study was conducted in a cross-sectional design in nine tertiary-care hospitals. The patients followed with a diagnosis of asthma or COPD for at least one year were enrolled in the study. The frequency of ACO and the characteristics of the patients were evaluated in the asthma and COPD groups. Result: The study included 408 subjects (F/M= 205/203, mean age= 56.24 ± 11.85 years). The overall prevalence of ACO in both groups was 20.8% (n= 85). The frequency was higher in the COPD group than in the asthma group (n= 55; 33.3% vs. n= 22; 9.8%), respectively (p= 0.001). Patients with ACO had similarities to patients with COPD in terms of advanced age, sex, smoking, exposure to biomass during childhood, being born in rural areas, and radiologic features. Characteristics such as a history of childhood asthma and allergic rhinitis, presence of chronic sinusitis, NSAID hypersensitivity, atopy, and high eosinophil counts were similar to those of patients with asthma (p<0.001). The annual decline in FEV1 was more prominent in the ACO group (mean= -250 mL) than in the asthma (mean change= -60 mL) and COPD (mean change= -230 mL) groups (p= 0.003). Conclusions: This study showed that ACO was common among patients with asthma and COPD in tertiary care clinics in our country. ACO should be considered in patients with asthma and COPD who exhibit the abovementioned symptoms.

Diagnosis and Treatment Patterns of Chronic Thromboembolic Pulmonary Hypertension in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia: A Registry Study.

BACKGROUND: Patients with chronic thromboembolic pulmonary hypertension (CTEPH) in countries with limited resources have, to date, been poorly represented in registries. OBJECTIVE: This work assesses the epidemiology, diagnosis, hemodynamic and functional parameters, and treatment of CTEPH in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia. METHODS: A prospective, cohort, phase IV, observational registry with 3-year follow-up (n = 212) in patients aged ≥ 18 years diagnosed with CTEPH was created. Clinical, hemodynamic, and functional parameters were obtained at an initial visit, follow-up visits, and a final visit at the end of 3 years' observation or end of follow-up. Data were recorded on electronic case report forms. Parameters evaluated included 6-minute walking distance (6MWD), use of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), pulmonary hypertension (PH)-targeted therapy, and survival. All statistical analyses were exploratory and descriptive, and were performed in the overall population. RESULTS: The most common symptoms were typical of those expected for CTEPH. Almost 90% of patients underwent right heart catheterization at diagnosis or initial study visit. In total, 66 patients (31%) underwent PEA before the initial visit; 95 patients (45%) were considered operable, 115 (54%) were inoperable, and two (1%) had no operability data. Only 26 patients (12%) had been assessed for BPA at their initial visit. PH-targeted therapy was documented at diagnosis for 77 patients (36%), most commonly a phosphodiesterase type 5 inhibitor (23%). Use of PH-targeted therapy increased to 142 patients (67%) at the initial visit, remaining similar after 3 years. Use of riociguat increased from 6% of patients at diagnosis to 38% at 3 years. Between baseline and end of observation, results for patients with paired data showed an increase in 6MWD. Survival at the end of observation was 88%. CONCLUSIONS: These data highlight the current diagnosis and management of CTEPH in the participating countries. They show that early CTEPH diagnosis remains challenging, and use of off-label PH-targeted therapy is common. CLINICALTRIALS: gov: NCT02637050; registered December 2015.

Risk of venous thromboembolism associated with single and combined effects of Factor V Leiden, Prothrombin 20210A and Methylenetethraydrofolate reductase C677T: a meta-analysis involving over 11,000 cases and 21,000 controls.

Genetic and environmental factors interact in determining the risk of venous thromboembolism (VTE). The risk associated with the polymorphic variants G1691A of factor V (Factor V Leiden, FVL), G20210A of prothrombin (PT20210A) and C677T of methylentetrahydrofolate reductase (C677T MTHFR) genes has been investigated in many studies. We performed a pooled analysis of case-control and cohort studies investigating in adults the association between each variant and VTE, published on Pubmed, Embase or Google through January 2010. Authors of eligible papers, were invited to provide all available individual data for the pooling. The Odds Ratio (OR) for first VTE associated with each variant, individually and combined with the others, were calculated with a random effect model, in heterozygotes and homozygotes (dominant model for FVL and PT20210A; recessive for C677T MTHFR). We analysed 31 databases, including 11,239 cases and 21,521 controls. No significant association with VTE was found for homozygous C677T MTHFR (OR: 1.38; 95 % confidence intervals [CI]: 0.98-1.93), whereas the risk was increased in carriers of either heterozygous FVL or PT20210 (OR = 4.22; 95 % CI: 3.35-5.32; and OR = 2.79;95 % CI: 2.25-3.46, respectively), in double heterozygotes (OR = 3.42; 95 %CI 1.64-7.13), and in homozygous FVL or PT20210A (OR = 11.45; 95 %CI: 6.79-19.29; and OR: 6.74 (CI 95 % 2.19-20.72), respectively). The stratified analyses showed a stronger effect of FVL on individuals ≤ 45 years (p value for interaction = 0.036) and of PT20210A in women using oral contraceptives (p-value for interaction = 0.045). In this large pooled analysis, inclusive of large studies like MEGA, no effect was found for C677T MTHFR on VTE; FVL and PT20210A were confirmed to be moderate risk factors. Notably, double carriers of the two genetic variants produced an impact on VTE risk significantly increased but weaker than previously thought.

Impact of Promising Biomarkers on Severity and Outcome of Acute Pulmonary Embolism.

Background: Acute pulmonary embolism (APE) is a common clinical condition. Its severity ranges from asymptomatic radiological findings to fatal obstructive shock. The potential circulating biomarkers have been studied to predict APE outcomes. This study aimed to explore their predictive power on prognosis in APE. Material and Method: It was a prospective observational study between March 2008 and April 2010. All consecutive patients diagnosed with APE were categorized as massive/high-risk, submassive/moderate-risk, and non-massive/low-risk. Cardiac troponin T (cTnT), myoglobin, N-terminal pro-brain natriuretic peptide (NT-proBNP), heart-type fatty acid-binding protein (H-FABP), growth differentiation factor-15 (GDF-15), and D-dimer levels were measured. Results: Of these patients, 14 (29.8%), 16 (34.0%), and 17 (36.2%) patients were categorized as low-risk, moderate-risk, and high risk-patients, respectively. There was no significant difference between the patient groups categorized based on the risk stratification in terms of demographic and clinical characteristics. The cTnT, myoglobin, HFABP, and D-dimer levels have also not differed significantly between the groups. There was a significant difference between the groups in respect of NT-proBNP and GDF-15 levels (p=0.009 and p=0.037, respectively). Nine (19.1%) patients had died by the 3rd-month follow-up. Adverse events were seen in 26 (55.3%) patients. GDF-15 had the highest area under the curve (AUC) value for predicting any adverse event (cut-off value=9.3 ng/mL, AUC=0.796, CI (confidence interval) 95%: 0.653-0.899). NT-ProBNP was determined as the best predictor for mortality (cut-off value=229.2 pg/mL, AUC=0.889, CI 95%: 0.756-0.964). Conclusion: Higher levels of NT-proBNP and GDF-15 were found to be associated with more severe APE, worse outcomes, and mortality.

Effect of Ground-Based Walk Training in Pulmonary Hypertension.

This study aimed to determine the effect of ground-based walking training on exercise capacity, physical activity, quadriceps muscle strength, and quality of life (QoL) in patients with pulmonary hypertension. A total of 24 patients were included in the study. Patients were randomly assigned to 2 groups as the walking group or the control group. The walking group participated in 30-minute supervised ground-based walking training 2 days/week for 8 weeks. Also, they walked unsupervised at least 1 day/week. The control group received no intervention. The number of weekly steps taken in both groups was recorded using a pedometer. In addition to the sociodemographic and clinic characteristics of the patients, the endurance shuttle walk test, incremental shuttle walk test, and 6-minute walk test were used for the evaluation of exercise capacity, and an activity monitor and pedometer for physical activity, a dynamometer for quadriceps muscle strength, and emPHasis-10 for QoL. After 8 weeks, endurance capacity, maximal exercise capacity, and the number of steps significantly improved in the walking group (p <0.05). The 6-minute walk distance, physical activity, quadriceps muscle strength, and QoL were similar in both groups (p >0.05). The results of the study showed that ground-based walking could improve endurance capacity, maximal exercise capacity, and the number of steps. Quadriceps muscle strength also improved in the walking group. No adverse effects were reported during the training period. Ground-based walking training can be performed safely in patients with pulmonary hypertension.

Extrapulmonary involvement in patients with sarcoidosis in Turkey.

BACKGROUND AND OBJECTIVE: Extrapulmonary sarcoidosis is common, and is almost always associated with concomitant thoracic involvement. Extrapulmonary manifestations vary on the basis of gender, age at presentation and ethnicity. The aim of this study was to investigate extrapulmonary involvement in patients with sarcoidosis in Turkey. METHODS: This study was conducted by Turkish Thoracic Society Clinical Problems Study Group. New cases of sarcoidosis between 1 June 2004 and 31 May 2006 were recorded on electronic case record forms sent to all potential investigators and information about extrapulmonary involvement was collected. RESULTS: One hundred and nineteen of 293 patients (83 female, 36 male, mean age = 45 ± 12 years) had extrapulmonary involvement in this study (40.6%). The median time to diagnosis was 6 months and this was longer than patients with just thoracic sarcoidosis (P = 0.001). Extrapulmonary symptoms were present in 181 (61.8%) patients, and skin lesions, arthralgia and back pain were the commonest (33.4%, 20.8% and 16.4%, respectively). Incidence of organ involvement was independent of age with the exception of ocular involvement, which was higher in those under the age of 40 years (P = 0.007). CONCLUSIONS: Skin and peripheral lymph node involvement were the most common sites of extrapulmonary involvement and ocular involvement was more common in those under the age of 40 years in patients with sarcoidosis in a Turkish population.

Clinical presentations and diagnostic work-up in sarcoidosis: a series of Turkish cases (clinics and diagnosis of sarcoidosis).

Sarcoidosis is an idiopathic granulomatous disease. It usually affects the lung. The diagnosis may be problematic since the known causes of granulomatous inflammation must be excluded. This multicenter study aimed to evaluate the clinical presentations and diagnostic approaches of sarcoidosis. The study protocol was sent via internet, and the participants were asked to send the information (clinical, radiological and diagnostic) on newly diagnosed sarcoidosis cases. 293 patients were enrolled within two years. Pulmonary symptoms were found in 73.3% of the patients, and cough was the most common one (53.2%), followed by dyspnea (40.3%). Constitutional symptoms were occured in half of the patients. The most common one was fatigue (38.6%). The most common physical sign was eritema nodosum (17.1%). The most common chest radiograhical sign was bilateral hilar lymphadenomegaly (78.8%). Staging according to chest X-ray has revealed that most of the patients were in Stage I and Stage II (51.9% and 31.7%, respectively). Sarcoidosis was confirmed histopathologically in 265 (90.4%) patients. Although one-third of the bronchoscopy was revealed normal, mucosal hyperemi (19.8%) and external compression of the bronchial wall (16.8%) were common abnormal findings. The 100% success rate was obtained in mediastinoscopy among the frequently used sampling methods. Transbronchial biopsy was the most frequently used method with 48.8% success rate. Considering sarcoidosis with its most common and also rare findings in the differential diagnosis, organizing the related procedures according to the possibly effected areas, and the expertise of the team would favour multimodality diagnosis.

Factors affecting the recurrence in patients with venous thromboembolism: A retrospective cohort study.

BACKGROUND: The aim of the study was to evaluate the frequency of recurrence and the risk factors for recurrence in patients who were diagnosed with venous thromboembolism. METHODS: Between January 2005 and January 2015, a total of 412 venous thromboembolism patients (164 males, 248 females; mean age: 53.5±16.6 years; range: 19 to 95 years) were retrospectively analyzed. The demographics, underlying risk factors, comorbidities, imaging findings, and treatment data of the patients were recorded. RESULTS: At least one transient/permanent risk factor was found in 341 (82.7%) of the index events, and the other 71 (17.2%) were idiopathic. Recurrence developed in 76 (18.4%) of the patients. The duration of the treatment in the first event was significantly longer in recurrent cases (p=0.007). The recurrence rate in patients diagnosed with only deep vein thrombosis or patients diagnosed with pulmonary thromboembolism + deep vein thrombosis was significantly higher than the patients diagnosed with only pulmonary thromboembolism (24% vs. 14.2%, respectively; p=0.007). The rate of idiopathic venous thromboembolism was higher in recurrent cases than in non-recurrent cases (26.3% vs. 15.2%, respectively; p=0.028). At the end of the first year, the mean D-dimer levels were higher in recurrent cases (p=0.034). Hereditary risk factors were also higher in recurrent cases (39.5% vs. 19.3%, respectively; p=0.031). There was no significant correlation between recurrence and mortality. CONCLUSION: The presence of deep vein thrombosis, idiopathic events, high D-dimer levels at the end of the first year and hereditary risk factors seem to be associated with recurrence.

Clinical efficacy, safety, tolerability, and survival outcome of long-term inhaled iloprost treatment in the management of pulmonary arterial hypertension: Data from prospective multicenter observational OPTION study.

OBJECTIVE: To evaluate clinical efficacy, safety and tolerability of long-term inhaled iloprost treatment in the daily practice for the management of pulmonary arterial hypertension (PAH). METHODS: A total of 115 patients with PAH on inhaled iloprost treatment were included. New York Heart Association (NYHA) functional class, brain natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels, and 6-minute walk distance (6MWD) were recorded at baseline and at 3rd to 24th month visits. Safety and tolerability of iloprost treatment were also evaluated during follow-up, as were the survival, clinical worsening, and the related risk factors. RESULTS: The treatment was associated with an increase in the percentage NYHA functional class II (from 0.0% at enrolment to 36.2% at 24th month visit) patients but no significant difference was noted in 6MWD values. Clinical worsening was observed in 63.5% patients, while survival rate was 69.6%. NT-proBNP levels were significantly higher in non-survivors than in survivors (p=0.042). Cox regression analysis revealed the association of female sex [odds ratio (OR)=0.318; 95% confidence interval (CI), 0.128-0.792; p=0.014] and scleroderma-related PAH (OR=0.347; 95% CI, 0.140-0.860; p=0.022) with significantly lower risk (3.14 fold and 2.88 fold, respectively) of mortality. CONCLUSION: Our findings indicate favorable efficacy, safety, and tolerability of long-term iloprost treatment in the management of PAH, whereas improved NYHA functional class was not accompanied with a significant change in 6MWD values. Patient age was a risk factor for clinical worsening, while female sex, scleroderma subtype, and lower NT-proBNP levels were associated with significantly lower mortality risk.

Sleep apnea in adult myotonic dystrophy patients who have no excessive daytime sleepiness.

PURPOSE: Sleep apnea is common in myotonic dystrophy (MD) and may cause respiratory failure. Most of the sleep studies have been performed in patients with excessive daytime sleepiness (EDS), which is a characteristic and strong predictor of sleep apnea. Therefore, we investigated the prevalence of sleep apnea in adult MD patients who have no EDS. MATERIALS AND METHODS: Epworth Sleepiness Scale was used to exclude EDS and a score over 10 was accepted as an indicator of EDS. Sleep studies of 17 adult MD patients with the Epworth sleepiness scale score < or =10 were retrospectively reviewed. Spirometry (n = 16) and daytime arterial blood gasses were used to evaluate the relationship with nocturnal parameters. RESULTS: On admission to the outpatient chest clinic, seven patients had normal spirometry, and ten had daytime hypercapnia and/or hypoxemia. All but one had sleep apnea (apnea-hypopnea index > or =5 events/h of sleep; mild in five, moderate in seven, and severe in four). Hypopneas were more common than apneas (16.9 +/- 13.2 events/h vs. 4.6 +/- 4.1 events/h). Nocturnal desaturation episodes were very frequent (oxygen desaturation index, 19.7 +/- 20.3/h of sleep). Three patients had central sleep apnea and 13 had obstructive sleep apnea. Body mass index, spirometry parameters (FVC and FEV1) and arterial oxygen tension were moderately correlated with nocturnal oxygenation parameters. Apnea-hypopnea index showed moderate correlation with spirometry parameters (FVC and FEV1). CONCLUSION: Sleep apnea and oxygen desaturations are very common in MD patients who report no excessive daytime sleepiness. Daytime lung function parameters are not sufficiently reliable for screening sleep apnea. Therefore, we recommend routine polysomnography in MD patients.

A randomized controlled trial on inspiratory muscle training in pulmonary hypertension: Effects on respiratory functions, functional exercise capacity, physical activity, and quality of life.

BACKGROUND: Impaired respiratory muscle function may be one of the causes of increased dyspnea, reduced exercise capacity, and physical activity (PA), and poor quality of life in pulmonary hypertension (PH). OBJECTIVE: To investigate the effects of threshold inspiratory muscle training (TIMT) on respiratory functions, functional exercise capacity, PA, and QoL in patients with PH. METHODS: Thirty patients with PH were randomly allocated to a TIMT (n = 15) and sham group (n = 15). Three patients in the sham group could not participate in the program. The TIMT group (n = 15) trained at 30% of the maximal inspiratory pressure (MIP), and the sham group (n = 12) performed at lowest pressure without change in threshold pressure. In both groups, patients performed TIMT at home for 15 min, twice per day, with the MIP load determined by the trainer, and were supervised once weekly at the hospital for eight weeks. The primary outcomes were MIP and maximal expiratory pressure (MEP). The secondary outcome measures included spirometric measurements, six-minute walking distance (6MWD), PA (SenseWear armband and International Physical Activity Questionnaire-Short Form-IPAQ-Short Form), and QoL (Minnesota Living with Heart Failure-MLHF). RESULTS: After the training, changes in MIP (p = 0.023) were higher in the intervention group compared with the sham group. Differences in MEP, FEV1 (%), FVC (%), FEV1/FVC (%), 6MWD, %6MWD, IPAQ-SF, MLHFQ, and armband parameters were not significantly different between the groups (p > 0.05). CONCLUSIONS: The results of the study demonstrated that TIMT could increase MIP and did not improve other parameters of respiratory functions, functional exercise capacity, PA, and QoL in patients with PH.

Frequency of Obstructive Sleep Apnea in Stage I and II Sarcoidosis Subjects Who Had No Corticosteroid Therapy.

OBJECTIVES: The number of studies on the frequency of obstructive sleep apnea (OSA) in subjects with sarcoidosis is low. Therefore, we aimed to investigate the frequency and predictors of OSA in subjects with clinically stable stage I and II sarcoidosis who were not taking corticosteroid and/or immunosuppressive drugs. We also evaluated restless legs syndrome (RLS) and periodic leg movements in sleep (PLMS). MATERIALS AND METHODS: Subjects with clinically stable stage I and II sarcoidosis and not receiving corticosteroid and/or immunosuppressive therapy were included in the study. Upper airway examination, lung function tests (forced vital capacity [FVC], forced expiratory volume in 1 second [FEV1], diffusing capacity of the lungs for carbon monoxide [DLCO]), and polysomnography were performed on all subjects. In addition, subjects' Epworth Sleepiness Scale (ESS) scores and the Pittsburgh Sleep Quality Index (PSQI) were recorded. RESULTS: Of the total number of 46 sarcoidosis subjects (35 women, 11 men; age: 44.4±10.7 years; body mass index (BMI): 29.3±5 kg/m2), 28 (60.9%) were detected with OSA (67.8% mild OSA). The recorded ESS score of the subjects was low (2.6±3.2), whereas the sleep quality was poor in 36.9% of these subjects. Rapid eye movements (REM) related OSA was diagnosed in 14.2% of the OSA subjects. Age was the only factor related to OSA diagnosis in a logistic regression analysis (p=0.048). None of the subjects were diagnosed with RLS and PLMS. CONCLUSION: OSA is common in stage I and II sarcoidosis subjects who did not receive corticosteroid therapy. The frequency of OSA diagnosis increases as the age of the subjects increases. Therefore, sarcoidosis subjects should be evaluated for OSA throughout the follow-up.

Survey of Physician's Knowledge, Attitudes, and Practices Regarding Idiopathic Pulmonary Fibrosis in Turkey.

OBJECTIVES: The aim of this study is to evaluate the approaches of Turkish pulmonologists to the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) in daily clinical practice. MATERIALS AND METHODS: A questionnaire containing 38 questions about the IPF diagnosis and treatment was given to pulmonologists between January 22 and 29, 2018, and the data of 158 physicians who responded to the questionnaire were evaluated. RESULTS: This survey showed that the mean number of patients that physicians followed up and managed annually was 8.3 and 5, respectively. The mean symptom duration before the diagnosis was 9-12 months. Patients were seen on average by three physicians prior to confirmed diagnosis. Almost 80% of the physicians have an opportunity to access a pathologist and radiologist specialized in IPF. However, only 26% of them have an opportunity to access regular multidisciplinary meetings. Although antifibrotics were the most commonly prescribed drugs, approximately 10% of patients were prescribed steroids, N-acetylcysteine, and immunosuppressants. Most of the physicians (81%) were aware of international guidelines; however, the Turkish Thoracic Society IPF Diagnosis and Treatment Consensus Report was read by only 41% of them. CONCLUSION: This survey may lead to the IPF awareness in Turkey, and it may help to close the gaps regarding the diagnosis and treatment.

Isolated laryngo-tracheal amyloidosis presenting as a refractory asthma and longstanding hoarseness.

Respiratory tract amyloidosis is characterized by deposition of amyloid fibril protein at any site along the respiratory tract ranging from the larynx to the pulmonary parenchyma. Usually, it is not associated with primary systemic amyloidosis. We report a case with isolated laryngotracheal amyloidosis, which is relatively a rare condition, together with a history of refractory asthma accompanied with longstanding hoarseness.