Mild Obstruction Research Articles

Hyperostosis Frontalis Interna and Temporalis Interna: A Rare Case Report

Hyperostosis is a slow-growing benign bone tumour often seen in the bones of the cranial vault, more commonly found in elderly females. It is an incidental finding noted during radiological examination. The clinical manifestation of such tumour depends on its location inside the cranial cavity: the proximity to the paranasal sinuses, brain tissue, nerves, or blood vessels, etc. Its clinical findings may range from mild obstruction of the paranasal sinuses or blood vessels to severe compression of the surrounding cranial nerves. Here a rare case of hyperostosis of the frontal and temporal bones found during a routine cadaveric dissection in the Department of Anatomy is presented. Causes of the formation of such unusually enlarged bone masses inside the cranial cavity and their clinical presentation are discussed.

Emerging phenotypes of pulmonary hypertension associated with COPD: a field guide.

Purpose of reviewPulmonary hypertension (PH) is a common complication of chronic obstructive lung disease (COPD), but clinical presentation is variable and not always ’proportional’ to the severity of the obstructive disease. This review aims to analyze heterogeneity in clinical features of PH-COPD, providing a guide for diagnosis and management according to phenotypes.Recent findingsRecent works have focused on severe PH in COPD, providing insights into the characteristics of patients with predominantly vascular disease. The recently recognized ’pulmonary vascular phenotype’, characterized by severe PH and mild airflow obstruction with severe hypoxemia, has markedly worse prognosis and may be a candidate for large trials with pulmonary vasodilators. In severe PH, which might be best described by a pulmonary vascular resistance threshold, there may also be a need to distinguish patients with mild COPD (pulmonary vascular phenotype) from those with severe COPD ('Severe COPD-Severe PH’ phenotype).SummaryCorrect phenotyping is key to appropriate management of PH associated with COPD. The lack of evidence regarding the use of pulmonary vasodilators in PH-COPD may be due to the existence of previously unrecognized phenotypes with different responses to therapy. This review offers the clinician caring for patients with COPD and PH a phenotype-focused approach to diagnosis and management, aimed at personalized care.

THE PREVALENCE OF SMALL AIRWAYS DYSFUNCTION IN ASTHMATICS WITH AIRFLOW OBSTRUCTION

TYPE: Abstract TOPIC: Allergy and Airway PURPOSE: To evaluate the prevalence and characteristics of small airways dysfunction (SAD) in adult asthmatic patients with persistent airflow obstruction as measured by FEV1/FVC. METHODS: Spirometry records of adult patients diagnosed with asthma were retrospectively obtained from the pulmonary function unit of a tertiary hospital in the West Midlands, United Kingdom. The 2012 Global Lung Initiative equations were used to produce % predicted values and z-scores. Airflow obstruction was defined as a z-score of <-1.645 in FEV1/FVC. The SAD was defined as a z-score of <-1.645 in MMEF. The z-score for FEV1 was used to categorize the severity of the obstruction as per the ARTP guidelines. Demographics were also obtained to assess the characteristics of patients with and without SAD. RESULTS: A total of 395 records were obtained, of which 337 (85.3%) had evidence of SAD as measured by MMEF. Although most patients with mild airflow obstruction had evidence of SAD (63.1%), it was the only severity group with a prevalence of less than 100%. SAD was more prevalent in females (89.53%) than males (81.37%) (p= 0.032). No statistical differences were found between smoking history, age, weight, height, or ethnicity when compared to those with and without SAD. CONCLUSIONS: As measured by MMEF, the prevalence of SAD is high in asthmatic patients with physiological airflow obstruction and is more common in females. CLINICAL IMPLICATIONS: In asthmatic patients with airflow obstruction, therapies that also target small airways should be considered in the treatment plan. DISCLOSURE: Nothing to declare. KEYWORD: Asthma

E-Cigarette Use, Small Airway Fibrosis, and Constrictive Bronchiolitis

BackgroundVaping, including the use of electronic cigarettes (e-cigarettes), has become increasingly prevalent, yet the associated long-term health risks are largely unknown. Given the prevalence of use, particularly among adolescents early in their lifespan, it is vital to understand the potential chronic pathologic sequelae of vaping.MethodsWe present the cases of four patients with chronic lung disease associated with e-cigarette use characterized by clinical evaluation, with pulmonary function tests (PFTs), chest high-resolution computed tomography (HRCT), endobronchial optical coherence tomography (EB-OCT) imaging, and histopathologic assessment.ResultsEach patient presented with shortness of breath and chest pain in association with a 3- to 8-year history of e-cigarette use, with mild progressive airway obstruction on PFTs and/or chest HRCT findings demonstrating evidence of air trapping and bronchial wall thickening. EB-OCT imaging performed in two patients showed small airway–centered fibrosis with bronchiolar narrowing and lumen irregularities. The predominant histopathologic feature on surgical lung biopsy was small airway–centered fibrosis, including constrictive bronchiolitis and MUC5AC overexpression in all patients. Patients who ceased vaping had a partial, but not complete, reversal of disease over 1 to 4 years.ConclusionsAfter thorough evaluation for other potential etiologies, vaping was considered to be the most likely common causal etiology for all patients due to the temporal association of symptomatic chronic lung disease with e-cigarette use and partial improvement in symptoms after e-cigarette cessation. In this series, we associate the histopathologic pattern of small airway–centered fibrosis, including constrictive bronchiolitis, with vaping, potentially defining a clinical and pathologic entity associated with e-cigarette use. (Funded in part by the National Institutes of Health.)

Lung health after tuberculosis: clinical and functional assessment in post-pulmonary tuberculosis Egyptian patients

BackgroundSome of the pulmonary tuberculous patients who completed their medication course experience lung function impairment which may influence their quality of life. However, the pattern and nature of post-tuberculosis lung impairment are still not identified. Thus, the aim of this study was to determine the type and degree of lung physiology changes in previously treated tuberculous patients which latter may affect their quality of life, thus helping clinicians for early diagnosis of lung impairment, especially in tuberculous endemic areas.ResultsAdults (≥18 years) who were treated from first attack pulmonary tuberculosis were included in the study. Demographic data, respiratory symptoms, smoking history, comorbidities, Medical Research Council dyspnea score (MRC), spirometry, chest radiography, and oxygen saturation were collected. One hundred ninety-seven participants were finally included in the study. The mean age was 49.50±13.26 years with males representing 75.6% of total patients. MRC dyspnea scale grading showed that about 13.2% and 5.1% of patients suffer from dyspnea grade II and grade III, respectively. Main respiratory symptoms were cough in 38.1%, sputum production in 24.9%, and hemoptysis in 6.6% cases. Normal spirometry was documented in 53.3%, obstructive pattern in 31.98%, and mixed pattern in 7.11%, whereas 7.61% cases had a restrictive pattern. According to ATS staging of obstructive pattern, 27 patients (42.8%) had mild obstruction, 22 (34.9%) patients had moderate obstruction, 13 (20.6%) patients had moderate to severe obstruction, and one patient (1.6%) had severe obstruction.ConclusionsImpairment of respiratory function after tuberculosis is one of the causes of chronic lung disease that is underestimated, especially in endemic countries. This impairment in function occurs early in the course of the diseases and even without symptoms, affecting the quality of life. Thus, clinicians should encourage patients for an earlier visit to a respiratory clinic for follow-up and further management if needed. Guidelines for the management of cured tuberculous patients are urgently needed.Trial registrationClinicalTrials.gov, NCT05097638. Registered on October 28, 2021—retrospectively registered

A Rare Case of Giant Unruptured Sinus of Valsalva Aneurysm

Sinus of Valsalva aneurysm is a rare congenital disorder that usually presents with rupture of the aneurysm into adjacent cardiac structures. It usually arises from the right sinus of Valsalva which ruptures into the right ventricle or right atrium (RA). Aneurysms may also arise from the noncoronary cusp that rupture into RA. Rarely, an unruptured aneurysm may compress or dissect into the adjacent structures and produce obstruction to either left or right ventricular outflow or produce conduction blocks. It may also intrude upon valves affecting valvular function. Here is a case of an unruptured giant aneurysm arising from the right sinus of Valsalva burrowing into the interventricular septum (IVS) causing conduction defect in the form of left bundle branch block with first degree atrioventricular block. Through IVS, it protruded into the left ventricular (LV) outflow tract (LVOT) giving rise to a cystic appearance causing mild LVOT obstruction and mild-to-moderate aortic regurgitation. The aneurysm also affected mitral valve function causing severe mitral regurgitation (MR) including characteristic diastolic MR due to prolonged PR interval. There was no regional wall motion abnormality indicating the absence of coronary arterial obstruction by the aneurysm. Left atrial and LV dilatation was present along with LV systolic dysfunction. Pulmonary arterial systolic pressure was approximately 48 mmHg as calculated from tricuspid regurgitation (TR) gradient. Like MR, TR also showed diastolic TR.

Hypertrophic obstructive cardiomyopathy complicated with acute myocardial infarction and diffuse fibrosis: surgery or not?

BackgroundHypertrophic cardiomyopathy with extreme hypertrophy, biventricular obstruction and diffuse myocardial fibrosis complicated by myocardial infarction in the absence of obstructive coronary artery disease (MINOCA) is a rare phenotype. Evidence and guideline recommendations are still lacking for a treatment strategy.Case presentationEmergency coronary angiography was performed in a 38-year-old man with a 2-year history of nonobstructive hypertrophic cardiomyopathy (HCM) presenting with acute myocardial infarction. The coronary angiogram yielded no stenotic lesions but showed a diffusely dilated left descending artery with slow blood flow. All evidence from biomarker analysis, electrocardiography, echocardiography, and imaging supported the diagnosis of acute myocardial infarction in the left ventricular anterior wall. The echocardiogram demonstrated severe interventricular and apical hypertrophy, severe left ventricular outflow tract obstruction and mild right ventricular outflow tract obstruction. Cardiac magnetic resonance imaging showed a concentric morphological subtype of HCM with diffuse late gadolinium enhancement in the left ventricle. Extended septal myectomy was performed 1 month later, and the patient recovered well.ConclusionsHypertrophic obstructive cardiomyopathy with acute myocardial infarction is an indication for coronary angiography. Septal reduction surgery could be performed cautiously in HCM patients with extreme hypertrophy, biventricular obstruction and diffuse myocardial fibrosis complicated by MINOCA to improve the patient’s symptoms.

Impact of the incorrect use of lead drapes on staff and patient doses in interventional radiology

To evaluate the usefulness of commercially available scatter reduction drapes in mitigating staff exposure in interventional radiology and the potential harmful effects of drape malpositioning in terms of exposure levels to both patients and staff. An anthropomorphic phantom was irradiated on an angiography device under three scenarios: no drape and correct and incorrect drape positioning. Different levels of incorrect drape positioning relative to the field-of-view (FOV) were evaluated: slight, mild and severe. Real-time dosimeter systems (positioned on the operator’s eye, chest and thyroid) were used to evaluate accumulative doses and dose rates. Different obstruction levels were evaluated and compared to the observer’s perception. Additionally, patient exposure was evaluated for all scenarios using a dose area product (DAP). Up to a mild obstruction, by using the drape a dose reduction of up to 86% was obtained while a severe obstruction produced a 1000% increase in exposure, respectively for all dosimeter positions compared to the use of no drape. A similar order of magnitude was observed for patient exposure. Good agreement was obtained for the observer perception of the FOV obstruction up to 25% of the FOV; for larger obstructions, an overestimate of the obstruction was observed. Patient lead drapes can reduce staff doses in interventional radiology procedures even when mildly malpositioned and obscuring the FOV. Special attention to protective drape positioning is necessary, since the severe obstruction of the FOV results in a large increase in both operator and patient exposure.

320 cardiac MDCT angiography in preoperative assessment of TOF and its variants: Does it worth it?

BackgroundTetralogy of Fallot is the most common cyanotic congenital heart disease encountering a large spectrum of anatomical presentations with differing surgical approaches, and long-term outcomes ranging from ventricular septal defect with limited aortic overriding and mild pulmonary obstruction to a critical form of VSD and pulmonary atresia. TOF variants include PA/VSD, TOF/CAVC, TOF/DORV and TOF with absent pulmonary valve. Also, it may be accompanied with many associated intracardiac and extracardiac anomalies that may be of value when imaging and planning the surgical procedure.ResultsOur study included 22 cases of classic TOF, 18 with PA/VSD (12 were of type A, 5 were of type B and 1 was of type C), 3 with TOF/CAVC and 7 TOF/DORV. Sub-valvular RVOTO was detected in 94% of patients. A statistically significant difference was depicted between Echocardiography and MDCT in detecting supravalvular RVOTO, however no statistically significant difference was found in sub-valvular and valvular RVOTO detection. MDCT could efficiently characterize pulmonary arterial tree with statistically significant difference between both Echocardiography and MDCT in assessment of main, right and left pulmonary arteries with P value = 0.036,0.014 and 0.023 respectively. With calculation of Mc-Goon ratio in all patients, it was favorable (> 1.2) in 33 patients (66%). MDCT entailed 19 patients with PDA versus 15 depicted by Echocardiography and 25 MAPCAs per 11patients compared 8 MAPCAs per 7 patients detected by Echocardiography. Right sided aortic arch was found in 10 patients and 24 patients showed abnormal branching pattern. Coronary artery abnormalities were identified in eight patients. MDCT showed 100% sensitivity and 100% specificity in depicting aortic, coronary and other associated extracardiac vascular anomalies.ConclusionMDCT offers comprehensive anatomical assessment of TOF, and its variants providing superiority over echocardiography and comparable results to cardiac catherization with 100% sensitivity and specificity in evaluation of associated extracardiac vascular anomalies as well as pulmonary arteries characterization. It is worth using MDCT routinely in combination with echocardiography for the preoperative assessment of TOF and its variants representing a less invasive option than conventional catherization with lower radiation exposure.

Pulmonary Function Tests in Patients with Chronic Rhinosinusitis : A Comparative Study

Coexistence of upper and lower respiratory diseases has long been known. Since chronic rhinosinusitis and pulmonary diseases are closely related this study was made to observe the pulmonary function of patients with chronic rhinosinusitis. Methods: The study was conducted in the Department of ENT and Pulmonary Medicine, IGMC Shimla on 27 adult patients of chronic rhinosinusitis and 26 age matched healthy control subjects between November 2018 and November 2019. The pulmonary function tests were performed using a spirometry and included Vital Capacity (VC) , Forced vital capacity (FVC) , Forced expiratory volume in one second (FEV1) and Forced expiratory volume in one second by Forced vital capacity ratio (FEV1/FVC). Results: Following observations were made from the results of the study, among controls majority of the subjects i.e 23 (92%) had a normal PFT with only 1 patient (4%) had mild and another 1 patient (4%) had moderate restriction on PFT. Where as among the patients group 10 patients (37%) had a normal pre-operative baseline PFT. Another 10 (37%) and 7 patients (25.9%) had mild and moderate obstruction respectively.

Differences between FEV6, FVC and VC at the diagnosis of obstructive ventilatory defect

IntroductionThe diagnosis of airway obstruction can be made through FEV1/FVC ratio <0.7 or FEV1/VC ratio < lower limit of normality (LLN). Several authors advocate that FEV1/FEV6 ratio is an alternative to diagnosing obstructive ventilatory defect, while others have determined that the best cut-off for this ratio (best combined sensitivity and specificity) is 0.73. ObjectiveTo evaluate the non-inferiority of FEV1/FEV6 ratio < 0.73 when compared to FEV1/FVC ratio < 0.7 and FEV1/VC < LLN in diagnosing airway obstruction. MethodsA retrospective analysis of the medical records from patients who underwent spirometry or plethysmography in a university central hospital from June 1st to December 31st, 2018 was carried out. Only medical records which included FEV1/FVC < 0.7 or FEV1/VC < LLN were selected, and these results were compared to FEV1/FEV6 ratio. ResultsA total of 526 patients with obstructive ventilatory defect were identified by one of the two ratios described. Of these, 95.1%, 87.4% and 88.6% were obstructive by FEV1/FVC, FEV1/VC, and FEV1/FEV6 ratio, respectively. The positive predictive value (PPV) of FEV1/FEV6 in relation to FEV1/FVC ratio was 99.6% (p < 0.001) with a diagnostic efficacy of 92.8%, whereas the PPV of FEV1/FEV6 in relation to FEV1/VC was 91.0% (p < 0.001) and diagnostic efficacy was 85.2%. Most false negatives, comparing FEV6 with the other two tests, were found in patients with FEV1 > 70% (mild obstruction) and in individuals aged >50 years. ConclusionsFEV1/FEV6 < 0.73 may be a good alternative ratio, as it is non-inferior to FEV1/VC and FEV1/FVC in diagnosing obstructive ventilatory defect.

The adverse effects of solid biomass fuel exposure on lung functions in non-smoking female population.

ABSTRACTBackground:Though, smoking is the leading cause of chronic obstructive pulmonary disease worldwide, the household air pollution due to use of solid biomass fuel is considered as a major risk factor for the development of obstructive lung disease. The aim of the study was to assess the effect of solid biomass fuel exposure on lung functions in non-smoking female population.Methods:A hospital based, descriptive cross sectional study was carried out among 140 non-smoking female patients aged 40 or more and who had been exposed to solid biomass fuel. These patients underwent spirometry to assess their lung function and were classified as obstructive, restrictive or mixed. Modified medical research council (mMRC) dyspnoea scale for symptom assessment, 6-minute walk test (6 MWT) to determine the exercise capacity and Cumulative exposure index to assess the duration of exposure were also done.Results:All 140 (100%) patients having abnormal lung function, 4 (2.86%) had restrictive pattern, 5 (3.57%) had mixed pattern and 131 (93.57%) had obstructive pattern. Of 131 patients having obstructive pattern, 11 had mild obstruction, 49 had moderate obstruction, 39 had severe obstruction and 32 had very severe obstruction. Most commonly used biomass fuel was wood (43.57%). All the patients had shortness of breath, whereas cough was present in only 35.71% cases. 77 (55%) patients presented with a dyspnoea of mMRC grade 3 and above.Conclusion:Cumulative exposure index for solid biomass fuel is directly proportional to the severity of lung impairment as well as the symptom severity.

Repair with annuloplasty only of balanced bileaflet mitral valve prolapse with severe regurgitation.

Repair of severe mitral valve and mitral regurgitation (MR) in patients with degenerative bileaflet pathology can be challenging. Initial results with a ring-only repair (ROR) approach have shown promising results, but long-term outcomes in larger series are lacking. We report on outcomes of ROR in severe MR secondary to bileaflet prolapse, including Barlow's disease. Eighty patients with degenerative multi-segment bileaflet disease underwent ROR for severe MR with a predominantly central regurgitant jet indicating balanced bileaflet prolapse. The main outcome measure of this study was long-term recurrent MR probability. Secondary outcomes were late mortality, reoperation and in-hospital complications. The mean age was 53 ± 15 years and 54% were males. The mean ejection fraction was 59.2 ± 6.6, 24% and 40% had atrial fibrillation. Barlow's disease was found in 77% of the patients. Minimally invasive surgery was performed in 15 patients (19%). There were no perioperative mortalities or cerebrovascular events in the entire cohort. Post-repair mild outflow tract obstruction (systolic anterior motion) was observed in 4 patients (5%) after ROR. In a mean follow-up of 60 ± 48 months, there was 1 case of death. At follow-up, there was 1 (1%) reoperation due to recurrent MR, and 4 patients who had recurrent moderate or more MR. The 10-year freedom from recurrent MR was 97%. None had severe MR at the latest follow-up. In patients with severe MR and a central regurgitant jet secondary to balanced multi-segment bileaflet mitral valve prolapse, ROR is a simple and efficient approach providing excellent long-term results without a substantial risk of systolic anterior motion.

97 An unusual combination of rare congenital heart defects in a 70 years old lady complaining worsening dyspnea

Abstract Methods and results A 70-year-old woman presented to our outpatient clinic complaining of worsening dyspnoea in the last 3 months. She had a medical history of hypertension, diabetes, dyslipidemia, and paroxysmal atrial fibrillation. We performed a comprehensive evaluation starting with a transthoracic echocardiogram that showed a dilatation of right ventricle with normal function, severe pulmonary regurgitation, and moderate tricuspid regurgitation with estimated pulmonary artery systolic pressure of 55 mmHg; the left ventricle had normal dimension and function, with mild aortic and mitral regurgitation, and a subaortic membrane which caused a mild obstruction (maximum gradient 17 mmHg). The cardiac magnetic resonance (CMR) confirmed the enlargement of the right ventricle and of the pulmonary artery trunk (51 mm) and the severity of pulmonary regurgitation (regurgitant fraction of 41%). CMR also clearly showed the VSD just below the subaortic membrane and the left to right shunt with a jet that appeared to proceed straight from the left ventricle through the pulmonary valve (Figure 1A). The estimated Qp/Qs was 1.6 and no intramyocardial late enhancement was present. Pulmonary pressures and pulmonary vascular resistance were normal at the right heart catheterization and the Qp/Qs ratio calculated invasively was 1.45. Considering patient high-risk profile for coronary artery disease, a coronary angiography was also performed showing an abnormal origin of the right coronary artery (RCA) from the mid-portion of the left anterior descending coronary artery (LAD) with two significant stenosis: one involving the bifurcation of RCA and the other the mid-portion of the LAD (Figure 1B). The coronary computed tomography angiography (CCTA) showed a benign course of the RCA anterior to the pulmonary artery towards the auriculoventricular groove (Figure 1C, D). Taking into account all these findings, multidisciplinary heart team decided to perform a cardiac surgery intervention of pulmonary valve and trunk replacement, closure of ventricular septal defect and two coronary bypass grafts on LAD and RCA. Conclusions This case represents a combination of some rare congenital heart abnormalities where multimodality cardiovascular imaging techniques were essential to establish a proper diagnosis and to plan an adequate surgical repair. We hypothesize that the peculiar orientation of the VSD jet may have caused the pulmonary trunk dilatation considering that neither the shunt, nor the pulmonary pressure appear to have been of sufficient magnitude to cause it. Pulmonary ectasia and the damage inflicted by the jet to the cusps of the valve have led to the severe valvular insufficiency. While aortic and tricuspid regurgitation are known to be associated with VSD, to the best of our knowledge this is the first report of pulmonary regurgitation secondary to VSD.

The role of inspiratory capacity and tidal flow in diagnosing exercise ventilatory limitation in Cystic Fibrosis

BackgroundExercise ventilatory limitation conventionally defined by reduced breathing reserve (BR) may underestimate the effect of lung disease on exercise capacity in patients with mild to moderate obstructive lung diseases. ObjectiveTo investigate whether ventilatory limitation may be present despite a normal BR in Cystic Fibrosis (CF). MethodsTwenty adult CF patients (age 16-58y) with a wide range of pulmonary obstruction severity completed a symptom-limited incremental exercise test on a cycle ergometer. Operating lung volumes were derived from inspiratory capacity (IC) measurement during exercise and exercise tidal flow volume loop analysis.Results: six patients had a severe airway obstruction (FEV1<45% predicted) and conventional evidence of ventilatory limitation (low BR). Fourteen patients had mild to moderate-severe airway obstructive (FEV1 46–103% predicted), and a normal BR [12–62 L/min, BR% (17–40)]. However, dynamic respiratory mechanics demonstrated that even CF patients with mild to moderate-severe lung disease had clear evidence of ventilatory limitation during exercise. IC was decreased by (median) 580 ml (range 90–1180 ml) during exercise, indicating dynamic hyperinflation. Inspiratory reserve volume at peak exercise was 445 ml (241–1350 ml) indicating mechanical constraint on the respiratory system. The exercise tidal flow met or exceeded the expiratory boundary of the maximal flow volume loop over 72% of the expiratory volume (range 40–90%), indicating expiratory flow limitation. ConclusionReduced BR as a sole criterion underestimates ventilatory limitation during exercise in mild to moderate-severe CF patients. Assessment of dynamic respiratory mechanics during exercise revealed ventilatory limitation, present even in patients with mild obstruction.

Cyanosis in a Previously Well Child.

Cyanosis in a Previously Well Child.

OCCUPATIONAL ASTHMA: A CASE WITH PROLONGED SYMPTOMS POST EXPOSURE

TOPIC: Occupational and Environmental Lung Diseases TYPE: Medical Student/Resident Case Reports INTRODUCTION: Occupational asthma is one of the significant public health problems. We present a patient exposed to substances at her workplace, which eventually caused her to develop sensitivity to several other substances and unemployment. CASE PRESENTATION: A 67-year-old female who worked an administrative job at an organization developed progressive dyspnea, wheezing, conjunctivitis, poor concentration, and chronic fatigue within few months of starting work. The patient felt better during weekends, at night times, and when she stayed away from her workplace. In addition, several of her other colleagues also had similar symptoms. An inspection was performed, which revealed that building construction had exposed the employees to several chemicals such as phenol, ethylbenzene, formaldehyde, and glycol. The building was eventually closed due to this. The patient of ours visited several pulmonologists and occupational medicine specialists concerned about her respiratory symptoms over a span of 25years. Over several years, spirometry revealed mild obstruction and restriction. The patient's symptoms improved for a short duration after she stopped working at that facility and with the use of inhaled corticosteroids. Subsequently, her symptoms returned and she developed marked sensitivity to several other agents such as smoke, pollen, odors, hair products, cleaning agents making it difficult for her to step out of the house. She came to our clinic for pulmonary disability evaluation. Further history revealed that she is a lifetime non-smoker, did not have asthma before the exposure history at the workplace. After prolonged investigation and tests, she was deemed unable to work due to her inability to avoid complete exposure to irritants and chemicals at any workplace. DISCUSSION: Occupational asthma is of two types, immunological and non-immunological. The first is sensitization to a stimulant over a while and has a latency period before the overt manifestation of symptoms. The non-immunological type is caused due to sudden high-level exposure to specific irritants with rapid onset of symptoms. Clinical history plays a significant role in recognizing this association early. As mentioned in our case, a typical history is that the patient has symptoms while at work and feels better outside of the workplace. Diagnosis and treatment of immunological type are challenging. Due to latency, there is prolonged and persistent exposure, increasing their risk of developing severe disease, as seen in our case. Management is to avoid exposure to sensitizing agents in addition to bronchodilators and steroids similar to non-occupational asthma. CONCLUSIONS: This case illustrates the potential long-term effects that occupational asthma may have on the socio-economic, physical, and mental status of a patient. Early identification and appropriate intervention can help prevent severe sensitization and disability. REFERENCE #1: Mapp CE, Boschetto P, Maestrelli P, Fabbri LM. Occupational Asthma. American Journal of Respiratory and Critical Care Medicine. 2005;172(3):280-305. REFERENCE #2: Tiotiu AI, Novakova S, Labor M, et al. Progress in Occupational Asthma. Int J Environ Res Public Health. 2020;17(12). REFERENCE #3: Tarlo SM, Lemiere C. Occupational asthma. N Engl J Med. 2014;370(7):640-649. DISCLOSURES: No relevant relationships by Bhanusowmya Buragamadagu, source=Web Response No relevant relationships by Bharath Ganesh, source=Web Response No relevant relationships by Aliza Khanam, source=Web Response No relevant relationships by Michael Korman, source=Web Response No relevant relationships by Rakin Rashid, source=Web Response

ENDOBRONCHIAL HAMARTOMA: A MISDIAGNOSIS OF COPD

TOPIC: Procedures TYPE: Medical Student/Resident Case Reports INTRODUCTION: Hamartomas are benign tumors composed of various mesenchymal cells including cartilage, connective tissue, bone and fat. Pulmonary hamartomas constitute about 8% of all benign lung neoplasms and have an incidence of 0.025-0.32%. They typically occur peripherally involving the lung parenchyma and rarely occur as endobronchial tumors. Here, we present a case of endobronchial hamartoma presenting as dyspnea and chronic cough. CASE PRESENTATION: A 61-year-old male non-smoker presented for 6 months of chronic productive cough. His chest x-ray was unremarkable. Pulmonary function testing was notable for mild obstruction with a forced expiratory volume in one second (FEV1) of 84.9%, forced vital capacity (FVC) of 102.1%, FEV1/FVC ratio of 63.74% and normal DLCO without bronchodilator response. He was initially diagnosed with chronic obstructive pulmonary disease (COPD) and prescribed a daily steroid inhaler and as needed short acting bronchodilator. Despite compliance with his inhalers, he continued to have persistent cough and was referred to a pulmonologist. A computed tomography scan of the chest showed two endobronchial soft tissue lesions in the right bronchus intermedius nearly occluding the bronchus intermedius. Diagnostic bronchoscopy showed a lobulated, mobile endobronchial mass obstructing approximately 85% of the lumen. Endobronchial biopsies returned as fragments of mature hyaline cartilage with adipose tissue and benign bronchial epithelium compatible with pulmonary hamartoma. He underwent rigid bronchoscopy and tumor resection with laser cautery to the base by thoracic surgery. Pathology confirmed the diagnosis of pulmonary hamartoma. After resection, he had improvement of his dyspnea and cough. DISCUSSION: Endobronchial hamartomas account for only 10% of pulmonary hamartomas. Most pulmonary hamartomas are asymptomatic as they typically involve the parenchyma. Endobronchial hamartomas most commonly present as post obstructive pneumonia or hemoptysis. Less frequently, non-specific pulmonary symptoms such as cough, wheezing, and/or dyspnea can be the presenting sign and can lead to a misdiagnosis of asthma or COPD. CONCLUSIONS: Although endobronchial tumors are common, endobronchial hamartomas are very rare. Most endobronchial lesions are malignant. Endobronchial hamartomas are typically slow growing and therefore often incidentally discovered on chest imaging. In some cases, they may be poorly or not visualized on chest radiograph. Patients with nonspecific pulmonary symptoms who fail standard management with an unremarkable chest radiograph should prompt further detailed chest imaging to rule out structural causes such as endobronchial hamartomas. The gold standard for management of symptomatic endobronchial hamartomas is rigid bronchoscopy with resection and laser therapy. REFERENCE #1: Cosío, Broja et al. "Endobronchial Hamartoma.” Chest. Vol. 122;1 (2002): 202-205. REFERENCE #2: Ahmed, Saman et al. "Endobronchial hamartoma; a rare structural cause of chronic cough.” Respiratory medicine case reports. Vol. 22 (2017): 224-227. REFERENCE #3: Stevic, Ruza, and Branislava Milenkovic. "Tracheobronchial tumors.” Journal of thoracic disease vol. 8,11 (2016): 3401-3413. DISCLOSURES: No relevant relationships by Kathy Chan, source=Web Response No relevant relationships by George Moran, source=Web Response No relevant relationships by Sudhir Rajan, source=Web Response No relevant relationships by Eduardo Solbes, source=Web Response

A DUAL DIAGNOSIS: CYCLOPHOSPHAMIDE TOXICITY AND DIFFUSE ALVEOLAR-SEPTAL AMYLOIDOSIS CAUSING HYPOXEMIA

TOPIC: Diffuse Lung Disease TYPE: Fellow Case Reports INTRODUCTION: Patients with cardiac amyloidosis have multiple possible causes of hypoxia including pulmonary edema, infection, drug toxicity, and pulmonary involvement of amyloidosis. Treatment varies significantly for these different etiologies. This broad differential can make diagnosis challenging and bronchoscopy is often needed to establish a definitive diagnosis. CASE PRESENTATION: A 72 yo male with cardiac AL amyloidosis presented with fever and hypoxic respiratory failure. He completed 4 cycles of cyclophosphamide, bortezomib, and dexamethasone 2 weeks prior to presentation. Right heart catheterization (RHC) demonstrated normal pressures. CT chest showed diffuse ground glass opacities (GGO) and basilar predominant interlobular septal thickening. He was diuresed but continued to require supplemental oxygen. Bronchoscopy with BAL and transbronchial biopsy was performed. BAL WBC was 121 with 48% lymphocytes and 48% macrophages. Infectious evaluation returned negative. Transbronchial biopsy returned with lambda-predominant plasma cell infiltrate and amyloid deposition. He was started on prednisone with improvement in symptoms and liberation from supplemental oxygen after 3 weeks of therapy. PFTs obtained at follow-up showed mild obstruction and moderately reduced DLCO. DISCUSSION: Pulmonary toxicity is a known complication of cyclophosphamide therapy that results in pneumonitis, typically occurring weeks after administration. CT imaging shows scattered or diffuse GGOs. Pathology demonstrates diffuse alveolar damage. Case series have shown improvement with drug cessation and steroids. Diffuse alveolar-septal amyloidosis (DASA) is 1 of several patterns of pulmonary amyloidosis. DASA is associated with AL amyloidosis. DASA is a common pathologic finding but is rarely symptomatic unless severe enough to impair gas exchange. CT imaging shows interlobular septal thickening, reticular opacities, and GGOs. Pathology reveals amyloid deposition within the alveolar septum and vessel walls and a plasma cell infiltrate. Treatment is systemic therapy for amyloidosis. This patient with cardiac amyloidosis and cyclophosphamide use was a diagnostic challenge. Given his fever, normal RHC, and failure to improve with diuresis, there was concern for infection vs cyclophosphamide toxicity. Infectious evaluation with BAL was negative and he was started on steroids with improvement in symptoms. However, pathology returned with findings consistent with DASA.His clinical presentation and rapid improvement with prednisone indicate a component of cyclophosphamide toxicity causing hypoxemia. His pathology findings and persistent reduction in DLCO suggest DASA also contributed to his hypoxemia. CONCLUSIONS: DASA and cyclophosphamide toxicity are uncommon causes of respiratory failure and have nonspecific findings on CT imaging. Distinguishing these etiologies can be challenging without bronchoscopy and histological samples. REFERENCE #1: Khoor A, Colby TV. Amyloidosis of the Lung. Arch Pathol Lab Med. 2017 Feb;141(2):247-254. doi: 10.5858/arpa.2016-0102-RA. PMID: 28134587. REFERENCE #2: Malik SW, Myers JL, DeRemee RA, Specks U. Lung toxicity associated with cyclophosphamide use. Two distinct patterns. Am J Respir Crit Care Med. 1996 Dec;154(6 Pt 1):1851-6. doi: 10.1164/ajrccm.154.6.8970380. PMID: 8970380. REFERENCE #3: Rossi SE, Erasmus JJ, McAdams HP, Sporn TA, Goodman PC. Pulmonary drug toxicity: radiologic and pathologic manifestations. Radiographics. 2000 Sep-Oct;20(5):1245-59. doi: 10.1148/radiographics.20.5.g00se081245. PMID: 10992015. DISCLOSURES: No relevant relationships by David Dennis, source=Web Response No relevant relationships by David Zaas, source=Web Response

Changes in energy metabolism and respiration in different\xa0tracheal narrowing in rats

Why obstructive sleep apnea (OSA) treatment does not completely restore healthy metabolic physiology is unclear. In rats, the need for respiratory homeostasis maintenance following airway obstruction (AO) is associated with a loss of thermoregulation and abnormal metabolic physiology that persists following successful obstruction removal. Here, we explored the effect of two different types of tracheal narrowing, i.e., AO and mild airway obstruction (mAO), and its removal on respiratory homeostasis and metabolic physiology. We show that after ten weeks, mAO vs. AO consumes sufficient energy that is required to maintain respiratory homeostasis and thermoregulation. Obstruction removal was associated with largely irreversible increased feeding associated with elevated serum ghrelin, hypothalamic growth hormone secretagogue receptor 1a, and a phosphorylated Akt/Akt ratio, despite normalization of breathing and energy requirements. Our study supports the need for lifestyle eating behavior management, in addition to endocrine support, in order to attain healthy metabolic physiology in OSA patients.