Abnormal Pulmonary Function Tests Research Articles

Progressive systemic sclerosis in Egyptian patients: Clinical characteristics and nail fold capillaroscopy

Aim of the workTo present the clinical and laboratory disease characteristics in Egyptian patients with progressive systemic sclerosis (SSc) and their nail fold capillaroscopy findings. Patients and methods50 SSc patients were included. For all patients detailed history taking and clinical and rheumatological examination were performed. The modified Rodnan skin score (mRSS) and nail fold capillaroscopy were assessed. ResultsThe mean age was 41.4 ± 12.7 years (range 16–67 years) and median disease duration was 5 years (range 1–40 years). Eight patients (16%) were males and 42 (84%) females (M:F 1:5.25). Skin tightness was present in all patients; mean mRSS was 17.5 ± 10.4 (ranges 4–45). 44 (88%) patients had abnormal pulmonary function tests; 42 (84%) restrictive and 2 (4%) obstructive; 25 (50%) presented with interstitial lung disease. Pulmonary artery hypertension was recorded in 9 (18%) patients. Renal manifestations were the least common recorded system involvement (20%). Antinuclear antibodies were positive in 98%, rheumatoid factor in 4%, antitopoisomerase I in 36% and anticentromere in 8% of patients. 96% of patient had abnormal nail fold capillaroscopy. There was a significant negative correlation between mRSS with forced expiratory volume (r = −0.46, p = 0.001), forced volume capacity (r = −0.47, p = 0.001) and positively with the FEV1/FVC (r = 0.3, p = 0.035). ConclusionAlmost all patients have ANA seropositivity, abnormal PFTs and abnormal nailfold capillaroscopy. Antitopoisomerase I antibody seropositivity, ILD, abnormal PFT, worsening skin score, late pattern of nailfold capillaroscopy are more common in dcSSc. Extensive clinical examination and investigations should be considered for early diagnosis and follow up of Egyptian SSc patients.

Characteristics of Progressive Systemic Sclerosis in a Cohort of Egyptian Patients

Objective: To estimate the frequency of epidemiological, clinical and laboratory characteristics of progressive systemic sclerosis in a cohort of Egyptian patients. Methods: Fifty systemic sclerosis patients were included. These patients were subjected to detailed history taking, clinical and rheumatological examination, Measuring the dermal skin thickness by the modified Rodnan skin score (mRSS), Nail fold capillaroscpy (NFC) and the relevant radiological, laboratory and immunological investigations. Results: Our results revealed that the mean age at time of diagnosis was 32.66 ± 13.08 with the disease durations range from 1 to 40 years with a median of five years. Male to female ratio of 1: 5.2 and 20% of patients were smokers. Skin tightness was present in all patients, the mRSS ranges from 4 to 45 with a mean of 17.48 ± 10.44. ANA was detected in 98%, RF was detected 4%, antitopoisomerase I (antitopo I) was detected in 36% and ACA was detected in 8% of patients. 96% of patient had abnormal NFC. There were statistical significant negative correlations between mRSS and both of FEV1% and FVC%. There was also a statistical significant positive correlation between mRSS and FEV1/FVC. Conclusion: This study has shown that almost our Egyptian SSC patients have ANA seropositivity, abnormal pulmonary function tests and abnormal nailfold capillaroscopy (NFC). The study revealed that anti topo I antibody seropositivity, ILD, abnormal pulmonary function tests, worsening skin score, late pattern of NFC are more common in Diffuse Cutaneous Systemic Sclerosis (DCSSC) than Limited Cutaneous Systemic Sclerosis (LCSSC). Also ILD in SSC patients is commonly associated with antitopo I antibody seropositivity, abnormal pulmonary function tests, worsening skin score and late pattern of NFC. Therefore ANA, antitopo I, high resolution CT chest, pulmonary function test, mRSS and NFC should be considered for early diagnosis and follow up of SSC patients.

Development of breath test for pneumoconiosis: a case-control study

BackgroundLipid peroxidation plays an important role in the pathogenesis of pneumoconiosis. Volatile organic compounds (VOCs) generated from lipid peroxidation might be used to detect pneumoconiosis. The objective of this study was to develop a breath test for pneumoconiosis.MethodsA case-control study was designed. Breath and ambient air were analysed by gas chromatography/mass spectrometry. After blank correction to prevent contamination from ambient air, we used canonical discriminant analysis (CDA) to assess the discrimination accuracy and principal component analysis (PCA) to generate a prediction score. The prediction accuracy was calculated and validated using the International Classification of Radiographs of the Pneumoconiosis criteria combined with an abnormal pulmonary function test as a reference standard. We generated a receiver operator characteristic (ROC) curve and calculated the area under the ROC curve (AUC) to estimate the screening accuracy of the breath test.ResultsWe enrolled 200 stone workers. After excluding 5 subjects with asthma and 16 subjects who took steroids or nonsteroidal anti-inflammatory drugs, a total of 179 subjects were used in the final analyses, which included 25 cases and 154 controls. By CDA, 88.8% of subjects were correctly discriminated by their exposure status and the presence of pneumoconiosis. After excluding the VOCs of automobile exhaust and cigarette smoking, pentane and C5-C7 methylated alkanes constituted the major VOCs in the breath of persons with pneumoconiosis. Using the prediction score generated from PCA, the ROC-AUC was 0.88 (95% CI = 0.80—0.95), and the mean ROC-AUC of 5-fold cross-validation was 0.90. The breath test had good accuracy for pneumoconiosis diagnosis.ConclusionThe analysis of breath VOCs has potential in the screening of pneumoconiosis for its non-invasiveness and high accuracy. We suggest that a multi-centre study is warranted and that all procedures must be standardized before clinical application.

Long-term Pulmonary Outcomes in Pediatric Survivors of High-risk Neuroblastoma.

Children with high-risk neuroblastoma are exposed to multimodality therapies early in life and survivors confront late therapy-related toxicities. This study assessed respiratory symptoms, pulmonary function tests (PFTs), and risk factors for abnormalities among survivors. High-risk neuroblastoma survivors followed in the long-term follow-up clinic at Memorial Sloan Kettering Cancer Center were enrolled. Self-administered symptom questionnaires were completed. Medical records were reviewed for treatment information and comorbidities. PFTs included spirometry, plethysmography, and diffusion capacity of the lung for carbon monoxide (DLCO). Thirty-nine survivors participated (median age at study: 11.4 y; median age at diagnosis: 2.3 y; median time since completion of therapy: 5.5 y). Chronic respiratory symptoms were reported for 33%. PFT abnormalities were identified in 79% and included low forced expiratory volume in 1 second (38%), decreased total lung capacity (44%), and abnormal DLCO (67%). PFT abnormalities were mostly mild to moderate. Mean forced vital capacity, forced expiratory volume in 1 second, and total lung capacity were normal and mean DLCO was mildly abnormal. Risks included thoracic surgery, chest radiation therapy, thoracic surgery plus chest radiation therapy, and shorter time since completion of therapy (P<0.05). Although respiratory abnormalities were common, they were mostly mild or moderate. Continued pulmonary surveillance of this at-risk population is warranted.

Pulmonary function in patients with ulcerative colitis and its relationship with disease severity.

Background and AimUlcerative colitis (UC) patients have several extraintestinal and systemic manifestations. As studies on the frequency and predictors of pulmonary involvement in patients with UC are inconsistent, we undertook this prospective study.MethodsEighty‐seven patients with UC (in remission 49, 56.3%, active disease 38, 43.6%, median age: 40 years, range: 16–66, 55, 62.2% males) and 50 healthy controls (median age: 38 years, range: 14–69, 34, 68% males) underwent pulmonary function tests (PFTs) including forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), Tiffeneau value (FEV1/FVC), mid‐expiratory flow rate, and diffusion lung capacity for carbon monoxide with spirometer.ResultsSubjects with UC and control were comparable in age and gender. PFT was abnormal in 24 (27.5%) patients (active disease 15/38, 39.4%, remission 9/49, 18.4%) and 1 (2%) control (P < 0.005). Of the 24 patients with abnormal PFT, small airway, restrictive, and obstructive defects were detected in 12 (50%), 11 (45.8%), and 1 (4.2%) patients, respectively. Patients with abnormal PFT more often had active disease (15/24, 62.5% vs 23/63, 36.5%; P = 0.03). No relation of PFT abnormalities was found with age, sex, duration of disease, body mass index, serum albumin, and hemoglobin levels, and other extraintestinal manifestation (arthritis/arthralgia) and drugs used to treat UC.ConclusionUC patients with active disease have abnormal pulmonary functions with predominant involvement of small airways. Active UC was more often associated with abnormal PFT than the disease in remission.

Pulmonary Function Tests Abnormalities Predictors In Smoker Patients

Background: Smoking remains the most preventable cause of premature death and morbidity in the US and the developed world. Cigarette smoking is the most well known risk factor for accelerating lung function decline in adults. This study aimed to evaluate pulmonary function tests abnormalities predictors in smoker patients Materials and methods: This case-control study was done in Merjan teaching Hospital, Babylon, Iraq, in the period from December 2010 to March 2012. The study included 182 patients who were divided into 114 patients in obstructive group and 68 patients in restrictive group. Duration and amount of smoking were evaluated by pack years. Spirometry was done for each patient. The effects of smoking were compared between obstructive and restrictive chronic lung diseases.&#x0D; Results: The ages of patients in both groups ranged from 31-70 years. Most of patients were males (65% in obstructive group while 58% in restrictive group). There was significant correlation between pack years and degree of dyspnea (functional state),in addition there was significant correlation between pack years and severity of obstructive and restrictive diseases.&#x0D; Conclusions: The results of this study indicate that effects of smoking were more in obstructive group than restrictive group. It was established that lung function (FEV1,FVC and FEV1%FVC) decreases with increasing number of pack years. Lung function was related inversely to pack years of cigarette use.

Study of pulmonary function test in asymptomatic smokers and non-smokers between 30-50 years of age in a tertiary care hospital

Background: Tobacco smoking is widely prevalent all over the world and it continues to rise in developing countries. Smoking has a deleterious effect on pulmonary functions. Smoking is the single most significant risk factor contributing to the development of Chronic obstructive airway diseases (COPD). Spirometry by a trained health professional gives an indication of lung health by measuring airway abnormality. Objectives were to study pulmonary function test (PFT) in smokers and non-smokers between 30-50 years and to study the correlation of PFT with pack years.Methods: Apparently healthy subjects, 50 smokers and 50 non-smokers between 30-50 years without any symptoms were included as subjects. Patients with uncontrolled hypertension, recent myocardial infarction and pulmonary TB were excluded. Ex-smokers were excluded from the study. Patients with acute respiratory illness, severe systemic illness, chest trauma and dementia were also excluded. After proper history taking and clinical examination, measuring height and weight (vitals, pulse rate, respiratory rate, blood pressure) the selected individual was subjected to spirometry using ATS criteria (American Thoracic Society criteria). Spirometry was performed using UNI-EM spirometer. Collected data was analyzed using Statistical Package for the Social Service (SPSS) software version 17.Results: In this study 94 males and 6 females were enrolled as subjects. Maximum number of the patient 49 cases (49%) presented in the age group of 30-35 years, followed by 23 cases (23%) in 36-40 years age group. Of these 49 cases,15 were smokers while the rest 34 were non-smokers (P=0.0007). In 100 cases studied, 39% showed normal PFT and 61% shows abnormal PFT. Among smokers (out of total 50 cases) pulmonary function test (PFT) was abnormal in 36 cases (72%) while in non-smokers, 25 (50%) had abnormal PFT (P-value-0.024). This data indicates that smoking is highly associated with an abnormal PFT pattern. Out of 100 cases both smokers and non-smokers, 11 (73.33%) underweight cases with (BMI&lt;18.4) showed abnormal PFT. In 50 smokers, maximum cases 29 (58%) had normal BMI (18.5-22.9) (P value 0.0002). Abnormal PFT was observed in 25 (50%) non-smokers (P=0.001). Abnormal PFT was seen in 30 (69.77%) smokers with pack years &lt;15 and 6 smokers (85.6%) with pack years &gt;15 (P-value 0.383).Conclusions: Smoking is common in males between 30-35 years age group. Smoking is highly associated with an abnormal PFT. Cessation of smoking should be encouraged and PFTs from time to time in asymptomatic adults both smokers and non-smokers will be useful for early identification of abnormalities.

Pulmonary arterial hypertension screening of systemic sclerosis patients in clinical practice: an independent chart review

Patients with systemic sclerosis (SSc) are at increased risk of pulmonary arterial hypertension (PAH). Guidelines recommend annual screening with pulmonary function testing (PFT) and transthoracic echocardiogram (TTE). Through auditing the charts of 11 rheumatologists associated with McMaster University, we evaluated the proportion of SSc patients without PAH or pulmonary fibrosis who receive annual TTE, PFT, and dyspnea screening. Screening rates between self-identified SSc experts and non-experts were compared. In cases where screening tests were abnormal, charts were reviewed for evidence of cardiologist or respirologist referral. In total, 136 patients’ charts were included. Annual screening for dyspnea was very common (88% of patients, 119/134). Annual PAH screening via TTE (74%, 100/135) and PFT (79%, 107/136) was less common. Annual dyspnea screening, TTE, and PFT were more commonly performed by SSc experts than by non-experts (94% vs. 83%, p = 0.03; 85% vs. 61%, p = 0.002; 93% vs. 62%, p&lt;0.001, respectively). Nearly all patients with an abnormal TTE (10/11, 91%) and PFT (12/14, 86%) received appropriate referrals. Future research should explore reasons for differences in screening rates between SSc experts and non-experts. Given that rheumatologists screen for dyspnea more often than they order PFT and TTE, there may be barriers to ordering these tests that warrant further investigation.

Pulmonary Involvement in Patients with Guillain-Barré Syndrome in Subacute Phase.

Objectives:To evaluate the pulmonary function in Guillain–Barre syndrome (GBS) patients in subacute phase and find clinical correlates of pulmonary dysfunction.Methods:This was a single-center, prospective, cross-sectional, hospital-based study in GBS patients performed in Department of Neurological Rehabilitation at a tertiary care institute. Clinical examination for pulmonary function was done by measuring chest expansion. The pulmonary function tests were carried out by Spirometry kit Microquark Cosmed, Italy. Fatigue was assessed by Fatigue Severity Scale, disability status by Hughes Disability Scale (HDS), and muscle weakness by Medical Research Council sum scores.Statistical Analysis:Statistical analysis was performed by Stata 11. The significance of P value was adjudged against an alpha of 0.05.Results:Twenty-eight patients were included with 17 (61%) men and mean age of 31 years. Median duration of symptoms was 16.5 days. There were 10 (36%) demyelinating and 18 (64%) axonal variants. Twenty-six (93%) patients scored more than 2 on HDS. All study participants reported fatigue. Twenty-two (78.6%) patients had chest expansion of <2.5 cm. Spirometry showed restrictive pulmonary dysfunction in 23 (79%) patients. Significant correlation was found between abnormal pulmonary function test and chest expansion (P = 0.003).Conclusion:Pulmonary dysfunction in GBS is common even during subacute phase. It needs to be identified and managed appropriately for better clinical outcome.

Correlation of pulmonary function and usual interstitial pneumonia computed tomography patterns in idiopathic pulmonary fibrosis

BackgroundLittle is known about presenting ‘inconsistent’ or ‘possible’ usual interstitial pneumonia (UIP) computed tomography (CT) patterns advancing to ‘consistent’ UIP as disease progresses in idiopathic pulmonary fibrosis (IPF). We hypothesized that if ‘consistent’ UIP represented more advanced disease, such a pattern on presentation should also correlate with more severe pulmonary function test (PFT) abnormalities. Material and methodsConsecutive IPF patients (2005–2013) diagnosed by international criteria with baseline PFT and CT were included. Presenting CTs were assessed by three expert radiologists for consensus UIP pattern (‘consistent’, ‘possible’, and ‘inconsistent’). Approximation of individual and combined interstitial abnormalities was also performed with correlation of interstitial abnormalities and UIP CT pattern made with PFT findings and survival. ResultsThree-hundred and fifty patients (70% male) were included with a mean age of 68.3 years. Mean percent predicted forced vital capacity (FVC%) and diffusion capacity (DLCO%) was 64% and 45.5% respectively. Older age and male gender correlated more with ‘consistent’ UIP CT pattern. FVC% was not associated with any UIP pattern but did correlate with total volume of radiologist assessed interstitial abnormalities. DLCO% was lower in those with ‘consistent’ UIP pattern. A ‘consistent’ UIP CT pattern was also not independently predictive of survival after correction for age, gender, FVC%, and DLCO%. ConclusionPFT findings appear to correlate with extent of radiologic disease but not specific morphologic patterns. Whether such UIP patterns represent different stages of disease severity or radiologic progression is not supported by coinciding pulmonary function decline.

P239 Diagnostic potential of MMP-1 and SP-A as biomarkers to distinguish chronic hypersensitivity pneumonitis from Idiopathic pulmonary fibrosis

Introduction: Hypersensitivity pneumonitis (HP) is an inflammatory interstitial lung disease caused by a wide variety of organic particles. In general, the diagnosis of chronic HP represents a challenge for clinicians that need to use a constellation of clinical, laboratory, radiographic and pathological data to assess a confident diagnosis. Importantly, the clinical features cHP can be indistinguishable from those observed in idiopathic pulmonary fibrosis (IPF). Objective: To determine whether serum levels of surfactant protein A (SP-A) and matrix metalloprotease-1 (MMP-1) may help to differentiate cHP from IPF. Methods: This was a cross-sectional study including patients with IPF (n = 31), cHP (n = 31) and 17 healthy subjects. SP-A and MMP-1 serum levels were determined by ELISA and were evaluated for the differential diagnosis between HP and IPF, and also correlated with values of pulmonary function tests at diagnosis. Results: MMP-1 and SP-A were significantly elevated in cHP compared with the other two groups. MMP-1 (HP= 11.7 ± 14 ng/ml versus FPI= 16 ± 20.7 ng/ml, p< 0.001) and versus Controls= (0.4 ± 0.3 ng/ml, p < 0.0001), ANOVA between groups < 0.0001. SP-A (HP= 74 ± 30 pg/ml versus FPI= 54 ± 20 pg/ml, p< 0.02) and versus Controls= (35 ± 6 pg/ml, p < 0.0001), ANOVA between groups < 0.0001. There was no correlation between MMP-1 or SP-A levels with the abnormalities in pulmonary function tests. Conclusions: Our findings indicate that patients with cHP display the highest concentration of MMP-1 and SP-A which may be useful biomarkers to distinguish it from IPF.

Pulmonary function tests do not predict mortality in patients undergoing continuous-flow left ventricular assist device implantation

ObjectivesTo investigate the effect of pulmonary function testing on outcomes after continuous flow left ventricular assist device implantation. MethodsA total of 263 and 239 patients, respectively, had tests of forced expiratory volume in 1 second and diffusing capacity of the lungs for carbon monoxide preoperatively for left ventricular assist device implantations between July 2005 and September 2015. Kaplan-Meier analysis and multivariable Cox regressions were performed to evaluate mortality. Patients were analyzed in a single cohort and across 5 groups. Postoperative intensive care unit and hospital lengths of stay were evaluated with negative binomial regressions. ResultsThere is no association of forced expiratory volume in 1 second and diffusing capacity of the lungs for carbon monoxide with survival and no difference in mortality at 1 and 3 years between the groups (log rank P = .841 and .713, respectively). Greater values in either parameter were associated with decreased hospital lengths of stay. Only diffusing capacity of the lungs for carbon monoxide was associated with increased intensive care unit length of stay in the group analysis (P = .001). Ventilator times, postoperative pneumonia, reintubation, and tracheostomy rates were similar across the groups. ConclusionsForced expiratory volume in 1 second and diffusing capacity of the lungs for carbon monoxide are not associated with operative or long-term mortality in patients undergoing continuous flow left ventricular assist device implantation. These findings suggest that these abnormal pulmonary function tests alone should not preclude mechanical circulatory support candidacy.

Respiratory function in facioscapulohumeral muscular dystrophy 1

To test the hypothesis that wheelchair dependency and (kypho-)scoliosis are risk factors for developing respiratory insufficiency in facioscapulohumeral muscular dystrophy, we examined 81 patients with facioscapulohumeral muscular dystrophy 1 of varying degrees of severity ranging from ambulatory patients to wheelchair-bound patients. We examined the patients neurologically and by conducting pulmonary function tests: Forced Vital Capacity, Forced Expiratory Volume in 1 second, and static maximal inspiratory and expiratory mouth pressures.We did not find pulmonary function test abnormalities in ambulant facioscapulohumeral muscular dystrophy patients. Even though none of the patients complained of respiratory dysfunction, mild to severe respiratory insufficiency was found in more than one third of the wheelchair-dependent patients. Maximal inspiratory pressures and maximal expiratory pressures were decreased in most patients, with a trend that maximal expiratory pressures were more affected than maximal inspiratory pressures. Wheelchair-dependent patients with (kypho-)scoliosis showed the most restricted lung function.Wheelchair-dependent patients with (kypho-)scoliosis are at risk for developing respiratory function impairment. We advise examining this group of facioscapulohumeral muscular dystrophy patients periodically, even in the absence of symptoms of respiratory insufficiency, given its frequency and impact on daily life and the therapeutic consequences.

Preoperative Pulmonary Function Testing Predicts Cough Effectiveness Early After Laparoscopic Bariatric Surgery

Background: Cough is a protective mechanism providing bronchial drainage and helps to protect secretion retention in postoperative patients. Although laparoscopic bariatric surgery significantly decreases postoperative respiratory complications, there is an increase in patients with abnormal pulmonary function test results. The aim of the study was to investigate the relationship between preoperative pulmonary function testing (PFT) variables and cough effectiveness early after laparoscopic bariatric surgery. Methods: Twenty-seven patients (8 male and 19 female, median age 39 years) who had undergone laparoscopic sleeve gastrectomy were enrolled in the study. Preoperative PFT was recorded from patient files. Cough effectiveness was assessed using a portable flow meter to measure peak cough flow (PCF) on the first postoperative day. Results: None of the patients showed an obstructive (median forced expiratory volume in 1 s/forced vital capacity [FEV1/FVC] ratio was 83%), restrictive (median FVC was 92%), o...

Progressive delayed respiratory complications of sulfur mustard poisoning in 43 Iranian veterans, three decades after exposure.

The most common delayed complication of sulfur mustard (SM) poisoning has been observed in the respiratory tracts. It was thus aimed to investigate the delayed respiratory complications in SM-exposed patients around 25 years before the study. Forty-three veterans with more than 25% disability of due to SM poisoning were investigated. Clinical examinations as well as pulmonary function test (PFT) were performed. High-resolution computed tomography (HRCT) of the lungs was done as clinically indicated. Triad of chronic cough, dyspnea, and expectoration were the most common symptoms that were recorded in 88.2%, 88.2%, and 64.7% of the patients, respectively. PFT abnormalities were detected in 44.18% of the patients. Restrictive pattern was the most common (41.86%), while pure obstructive pattern did not detect at all. Mixed pattern was significantly correlated with higher disability percentages among the veterans ( p < 0.001). Significant reverse correlation between the disability percentages and forced expiratory volume in 1 s/forced vital capacity ratio was obtained ( p = 0.010, r = -0.389). Air trapping was the most common abnormality in HRCTs (50%). Bronchiectasis (25%), pulmonary fibrosis (25%), and ground-glass attenuation (16.66%) were other common HRCT findings. Comparing with the previous studies on these patients, more restrictive and mixed pattern were observed. Moreover, bronchiolitis, bronchiectasis, and lung fibrosis were the main pathological findings in these patients.

Effect of increasing body fat on pattern of pulmonary function: A prospective study

Background: Several evidences are available for altered pulmonary function (PF) with increasing body fat as measured by body mass index (BMI). However, consequences of obesity on PF have been reported to vary depending on its severity and pattern. Objectives: To study the effect of increasing body fat (measured by BMI) on the pattern of PF. Materials and Methods: A total of 120 subjects of either sex were studied having BMI ≥30 kg/m2 in the Department of Medicine, GR Medical College, Gwalior, from July 2013 to November 2014. Anthropometric and spirometric parameters were measured using a computerized spirometer. Data were analyzed using IBM SPSS version 20 and expressed as either mean ± standard deviation or percentage. Results: Out of 120 subjects, most of them belong to age group of 41-60 years (55%), were female (52.5%) and had BMI between 30 and 35 kg/m2 (73.33%). The most common PF test (PFT) pattern was restrictive (30.83%). Mean forced expiratory volume in 1 s (FEV1%), forced vital capacity (FVC%), and FEV1/FVC ratio among patients with BMI 30-35 (n = 76), was 78.3 ± 16.3, 77.9 ± 14.5, and 105.6 ± 7.8; with BMI 36-40 (n = 21) was 78.5 ± 21.1, 78.5 ± 19.2, and 105.6 ± 8.2; and with BMI 41-45 kg/m2 (n = 3) was 65.2 ± 18.3, 68.6 ± 16.7, and 99.9 ± 5.7, respectively. Conclusions: Increase in BMI leads to increase in abnormal PFT parameters. Early detection can reduce the abnormal PFT values in obese subject.

Study the Pulmonary Hypertension among Heavy Smokers Young Adult Males before the Clinical Evidences of Chronic Lung Disease

Background: Smoking is a well-known risk factor for development of COPD. Prevalence of smoking is high. Its effect on the pulmonary pressure before the development of COPD still needs to be explored on human model. Aim of the study: The aim of the study is to evaluate the pulmonary hypertension in young heavy smokers adult population prior to the development of the clinical and the abnormal pulmonary function test. Material and Methods: The study was carried out at Al Sader Najaf Teaching Hospital during the period April 2015 to April 2016 where 93 Smokers who smoke at least 2 packets/day for minimal two years period were included in the study with 93 non-smokers used as a control group. The age of the smokers group and non- smokers group was less than 40 year with mean age 25 ± 4.1 for smokers and 24.9 ± 3 for non –smokers. BMI for all was >30. All had their pulmonary function test with the clinical examination to exclude any evidences of the chronic lung disease. Transthoracic Echocardiography and Doppler study was done for the smokers and non-smokers groups to evaluate the Maximum tricuspid valve velocity, the Mean pulmonary pressure gradient and the Pulmonary artery pressure. Results: Mean tricuspid maximum velocity (TGmax) for smokers was 0.9 ± 0.1 and for non-smokers 0.60 ± 0.20 (p value less than 0.001). The mean pulmonary pressure gradient for smokers was 3.4 ± 1.0 and for non-smokers 1.5 ± 0.8 with p value less than 0.001. The mean pulmonary artery pressure for smokers group was 12.2 ± 1.6 and for non-smokers group 7.0 ± 1.2 with p value less than 0.001. Conclusion: There is an increase in the pulmonary arterial pressure among the heavy smokers young adults when was compared with the non-smokers young adults.

FVC, FEV1 and FEV1/FVC% in patients with Major Depressive Disorder

Background: Major Depressive Disorder (MDD) is associated with depressed lung function.Objectives: To observe FVC, FEV1 and FEV1/FVC% in Major Depressive Disorder patients.Methods: This prospective study was carried out in the Department of Physiology, Bangabandhu Sheikh Mujib Medical University (BSMMU) in 2014on 60 newly diagnosed female MDD patients aged 20 to 50 years. They were enrolled from the Department of Psychiatry of BSMMU. Age, BMI, socioeconomic status and occupation matched 30 apparently healthy females were controls.FVC, FEV1 and FEV1/FVC% were assessed by a portable digital spirometer. For statistical analysis, Independent sample ‘t’ test was done.Results: FVC, FEV1, FEV1/FVC% were significantly lower (p&lt;0.001) in all MDD patients than those of control. More over, 54(90%) MDD patients had abnormal pulmonary function test. Among them,5(9.25%) had obstructive,32(59.25%) had restrictive and 17(31.48%) had both restrictive and obstructive disorder.Conclusion: From this study it may be concluded that significant depression of lung function may be associated with MDD, characterized by restrictive, obstructive and mixed type disorders.Bangladesh Soc Physiol. 2016, December; 11(2): 59-62

Cardiopulmonary Fitness and Clinical Outcomes in Adults Followed in the Cooperative Study for Sickle Cell Disease

Introduction: Cardiopulmonary fitness is significantly reduced among individuals with sickle cell disease (SCD). Cardiopulmonary fitness is also an important predictor of morbidity and all-cause mortality in the general population. However, the relationship between fitness and clinical outcomes in SCD has not been well studied. The objectives of this analysis were to: 1) determine the factors associated with fitness in a cohort of adults with SCD, and 2) evaluate the relationship of fitness to hospitalization for pain and acute chest syndrome (ACS) and overall mortality. We hypothesized that clinical factors such as age, sex, hemoglobin, SCD genotype and cardiopulmonary disease significantly affect fitness, and that poor fitness is a predictor of more frequent hospitalizations for pain and ACS and higher mortality in adults with SCD.Methods: A cohort of adults with SCD was constructed from participants enrolled in phase 2 of the Cooperative Study of Sickle Cell Disease (CSSCD) who underwent exercise testing (modified Balke treadmill protocol). Primary measure for fitness was total treadmill duration. Retrospective pain or ACS hospitalization rates were calculated using events in the 3 years prior to exercise testing. Mortality and prospective hospitalization rates for pain and ACS were calculated using events after exercise testing with a minimum 6 month follow-up. Results of pulmonary function testing (PFT), echocardiography, and laboratory testing within 3 years of exercise testing were included in our analysis. Standard descriptive analyses were performed (SPSS V24). Multivariable negative binomial and Cox proportional hazards models were constructed to evaluate the relationship of fitness to ACS and pain hospitalization rates and mortality, respectively. Multivariable linear models were constructed to determine factors associated with fitness.Results: A total of 223 participants had valid exercise testing data (64% female, 70% hemoglobin SS or S/b0 thalassemia, mean age 43.3 ± 7.5 years, mean hemoglobin 9.1 ± 2.2 g/dl, mean follow-up 2.7 ± 0.7 years after exercise testing). Participants completed a mean of 11.6 ± 5.2 min on the treadmill, with 87% completing ≥ 3 stages but only 17% completing all 10 stages. We categorized fitness into tertiles of treadmill duration (5.7 vs. 11.8 vs. 18.1 min, p < 0.001). Age (45.2 vs. 43.1 vs. 41.3 years, p = 0.007), baseline hemoglobin (8.5 vs. 9 vs. 9.8 g/dl, p = 0.003), as well as the proportion of females (77 vs. 71 vs. 40%, p < 0.001) and participants with abnormal PFT (58 vs. 35 vs. 39%, p = 0.008), differed significantly across fitness tertiles. Pain or ACS hospitalization rates during the 3 years prior to exercise testing were not significantly different across fitness tertiles. Using multivariable linear regression, male sex (β = 3.1, p < 0.001), lower age at exercise testing (β = -0.14, p = 0.003), and higher hemoglobin (β = 0.44, p = 0.049) were independently associated with higher fitness, with abnormal PFT trending toward significance (β = -1.28, p = 0.07). In this model, genotype, tricuspid regurgitant jet velocity (TRJV) ≥ 2.5 m/s, and pain and ACS hospitalization rates prior to exercise testing were not significantly associated with fitness. Using a negative binomial regression model, we found that fitness did not predict future pain or ACS episodes after adjustment for age, sex, genotype, hemoglobin and TRJV. Fitness also did not predict survival in our cohort (hazard ratio, 0.97; 95% CI [0.84, 1.13], p = 0.71), in which death was reported in only 9 participants. In our Cox regression model, male sex (HR 7.1; 95% CI [1.3, 38.9]; p = 0.02) and lower hemoglobin (HR 0.56; 95% CI [0.36, 0.88]; p = 0.01) were independent predictors of death, but age at exercise testing, abnormal PFT and TRJV ≥ 2.5 m/s were not.Conclusions: In adults with SCD, lower fitness is significantly associated with female sex, older age, lower hemoglobin and abnormal PFT. Fitness did not predict survival or future pain or ACS events in the CSSCD. Given that cardiopulmonary fitness remains an important predictor of all-cause mortality in the general population, larger scale prospective studies in SCD are needed to evaluate the impact of regular exercise on improving fitness, quality of life, clinical outcomes and mortality in this population. DisclosuresBadawy:Ann & Robert H. Lurie Children’s Hospital of Chicago: Employment; Ann & Robert H. Lurie Children’s Hospital of Chicago: Research Funding; Salveo Health Communications LLC: Consultancy. Payne:National Center on Birth Defects and Developmental Disabilities: Employment. Liem:Ann & Robert H. Lurie Children’s Hospital of Chicago: Employment; Ann & Robert H. Lurie Children’s Hospital of Chicago: Research Funding; National Institute of Health: Research Funding.

Prognostic Significance of Pulmonary Function Test Abnormalities in Patients with Dyskeratosis Congenita

▪Introduction: Pulmonary fibrosis (PF) is a known complication in patients with dyskeratosis congenita (DC), reported in 15-20% of patients. PF is more common in older individuals with DC who may or may not have the classical clinical features of DC. PF in younger DC patients has been reported mainly in the setting of hematopoietic cell transplantation (HCT). There are no prospective studies of pulmonary function tests (PFT) in patients with DC.Objectives: To determine baseline pulmonary function status in patients with DC, association of PFT abnormalities with development or progression of pulmonary disease, and pulmonary outcomes in patients who did or did not receive HCT.Methods: DC patients older than 5 years of age underwent PFTs as part of a prospective IRB-approved cohort study at the NIH from 2002-2015. Data were obtained from spirometry [forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC)], lung volumes [total lung capacity (TLC)] and carbon monoxide diffusion capacity (DLCO). PFT abnormalities were classified as restrictive (normal FEV1/FVC and low TLC), obstructive (low FEV1/FVC and normal TLC) or mixed (low FEV1/FVC and low TLC) according to the American Thoracic Society Task Force guidelines. A low value was < lower limit of normal adjusted for age, sex, race and height. DLCO was also adjusted for hemoglobin. PFT data were analyzed in relation to the DC triad (nail dystrophy, skin pigmentation, leukoplakia), severe bone marrow failure (BMF needing treatment) and telomere length by flow FISH. Patients were followed through 2015. Pulmonary outcomes were compared in those with or without PFT abnormalities at evaluation and in those who did or did not receive HCT. P value <0.05 was significant.Results: Fifty-one patients with DC had PFTs, median age 19 yrs (range 6 - 69). Spirometry was abnormal in 16 patients (31%): 13 had restriction, 2 had obstruction, and 1 had a mixed pattern. DLCO was reduced (<80% of the predicted) in 32/49 patients (65%): 17 had mild (>60%), 13 had moderate (40-60%) and 2 had severely reduced (<40%) DLCO. All patients with abnormal spirometry had reduced DLCO compared with 18/35 (48%) with normal spirometry (p<0.0001). Patients with abnormal spirometry were more likely to have moderate/severe diffusion defects (10/16 vs 5/35; p=0.02), and severe BMF (12/16 vs 10/35; p=0.003) than those with normal spirometry. Telomere length and 2 or more features of the DC triad were not associated with PFT abnormality.Fourteen patients (27%) developed pulmonary disease at a median follow-up of 6 yrs (range 0.1 - 12 yrs) and a median age of 17 yrs (11 - 65): 10 had PF with interstitial honeycomb pattern on chest CT and 4 had pulmonary arteriovenous malformation (AVM) and mild fibrosis. Patients with abnormal spirometry and moderate/severely reduced DLCO were more likely to develop pulmonary disease than those with normal spirometry (12/16 vs 2/35; p<0.0001). Fourteen patients received a HCT (3 prior to and 11 after PFT evaluation) at a median age of 12 yrs (range 3 - 29). Eight of the 14 (57%) developed pulmonary disease 1 - 5 yrs after HCT (median 4 yrs) while 6/37 (16%) non-HCT patients developed PF (p=0.01). Transplanted patients developed pulmonary disease at a younger age than non-transplanted (median age 15 yrs, range 11 - 22 vs 54 yrs, 25 - 65; p=0.001), and had mutations in TINF2 (n=3), RTEL1, DKC1, WRAP53 (1 each); gene unknown (n=2). DC genes mutated in older non-HCT patients with PF were TERC (n=4), TERT (n=1), and unknown (n=1). The independent impact of severe DC phenotype, BMF, and HCT on pulmonary outcome could not be distinguished in young patients as most patients with severe disease had BMF and received HCT. There was no difference in PF among those who did or did not receive radiation for HCT. Four of the 10 deaths were due to PF.Conclusion: More than 30% of patients with DC have restriction and moderate/severe diffusion defect on PFTs, and these are more common in patients with severe BMF. PFT abnormalities are significantly associated with progression to PF, although they may also signal the presence of AVMs, a less common pulmonary manifestation of DC. PF in older patients is a known manifestation of the telomere biology disorder. It is unclear whether pulmonary disease in young patients is a feature of severe DC or the toxicity of HCT preparative regimens. Larger prospective studies are needed to confirm the predictive value of PFTs for future lung disease in patients with DC. DisclosuresNo relevant conflicts of interest to declare.