Severe Obstructive Lung Disease Research Articles

473: MULTISYSTEM INFLAMMATORY SYNDROME IN ADULTS (MIS-A) AFTER COVID-19 INFECTION

Introduction: Multisystem Inflammatory Syndrome was first described in children (MIS-C) after COVID-19 infection, it is characterized by gastrointestinal symptoms, shock, fevers, elevated inflammatory markers, and systolic dysfunction. A few similar presentations have also been reported in young adults designated as Multisystem Inflammatory Syndrome Adult type (MIS-A). Often, due to multi-organ involvement, extensive testing is undertaken with no yield of a clear etiological factor. We present a case of a 23-year-old male who was admitted into the Critical Care Unit for Encephalopathy with multi-organ dysfunction. Description: A 23-year-old male with a medical history of Williams-Campbell syndrome complicated by severe bronchiectasis and obstructive lung disease requiring 2 liters of oxygen at baseline, presented to the hospital with severe Encephalopathy, notably, he tested positive for COVID-19 one month before presentation with no increase in oxygen requirements until hospital presentation. Vitals were otherwise stable. Initial lab values were significant for an elevated AST of 6,620, ALT of 9,540, Creatinine of 4.71, Troponin-I of 3,913, CRP of 19.2, IL-6 of 22.1, and Ammonia of 171. Further investigative workup, including imaging, did not reveal a clear etiology for his presentation. An Echocardiogram however showed left ventricular dysfunction with an ejection fraction of 41%. Management included: broad-spectrum antibiotics which were discontinued after negative infectious workup, steroids for a suspected exacerbation of his lung condition, lactulose, and CRRT being initiated due to worsening renal function which was attributed to cardiac dysfunction leading to Acute Tubular Necrosis. MIS-A was eventually suspected as a diagnosis of exclusion considering the recent history of COVID-19 infection. Steroids were continued leading to gradual improvement of lab values. Discussion: With COVID-19 continuing to make an impact, it is essential to be cognizant of various presentations and sequela. There have been multiple reports of different kinds of sequela, such is our case of MIS-A for which a long steroid taper is the mainstay of treatment. We want to raise awareness in the medical community of the possible consequences of COVID-19 infection such as MIS-A.

AB010. Keloidal scleroderma with isolated intertriginous involvement

A 33-year-old African-American female with scleroderma, Raynaud phenomenon, and possible systemic lupus erythematosus (SLE) presented to dermatology clinic for evaluation of a 1-year history of slowly growing plaques in her bilateral axillae and inguinal region. The patient was initially diagnosed with scleroderma approximately five years prior to presentation following the onset of sclerodactyly, arthralgias, myalgias, and severe obstructive lung disease. Two years prior to presentation, she was started on monthly intravenous immunoglobulin infusions. Her other medications included hydroxychloroquine, mycophenolic acid, cilostazol, and amlodipine. One year ago, she began developing leathery, firm, dark plaques underneath both armpits. The plaques slowly enlarged and new plaques began forming in her inguinal region. They were nonpruritic but mildly tender. On exam, the patient had well-demarcated, linear, hyperpigmented, indurated plaques that coalesced in her bilateral axillae, bilateral inguinal folds, and bilateral peri-inguinal region with no evident secondary scale or crust. She also had sclerodactyly of the digits of her bilateral hands and blue-white discoloration of her 4th digit of the right hand. A 4 mm punch biopsy was performed from her left axilla and was consistent with keloidal morphea/scleroderma. Keloidal scleroderma, also known as nodular scleroderma, is a rare manifestation of systemic sclerosis that is usually characterized by innumerable nodules or keloidal plaques on the trunk. We present a case of isolated intertriginous keloidal scleroderma, an exceedingly uncommon distribution for this condition. A recent literature review of nodular scleroderma by Kassira et al. revealed only one reported case with lesions in intertriginous areas. Given the rarity of the diagnosis, our knowledge of efficacious treatment options remains limited. Possible options for treatment include methotrexate, ultraviolet A light therapy, and narrow-band ultraviolet B light therapy. Teaching point: the patient’s biopsy-proven isolated intertriginous keloidal scleroderma represents an uncommon clinical presentation for an already rare variant of systemic sclerosis.

WHAT'S LUPUS GOT TO DO WITH IT? A CASE OF ACUTE HYPERCAPNIC RESPIRATORY FAILURE IN A PATIENT UNDERGOING KIDNEY TRANSPLANTATION

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that is classically associated with constitutional symptoms, joint pain, and erythematous rash. However, patients with SLE can also have multiple cardiopulmonary comorbidities, making management difficult. In this report we present a patient with severe pulmonary arterial hypertension (PAH) and obstructive lung disease due to SLE who experienced a brief arrest during kidney transplantation. CASE PRESENTATION: A 41-year-old female with PAH and end-stage renal disease (ESRD) secondary to SLE presented in 2015 with the following right heart catheter (RHC) findings: RA 5, PAP 73/24 (40), PCWP of 9 mmHg, cardiac index of 1.93 L/min, and PVR of 9.7 Wood units. Alternative causes of PAH were assessed and ruled out via standard diagnostic testing. Treatment was initiated with sildenafil and inhaled treprostinil, but side effects eventually necessitated transition to ambrisentan and selexipag. A repeat RHC in 2017 showed the following: RA 5, PAP 30/17 (21), PCWP of 12 mmHg, cardiac output 3.12 L/min, PVR of 1.8. These improvements strongly suggested a component of Group 1 PAH that responded well to therapy. The patient was listed for renal transplant, and in Feb 2018 this was attempted but aborted due to intraoperative difficulty ventilating, yielding acute hypercapnic respiratory failure and brief cardiac arrest. After recovery and hospital discharge, repeat evaluation showed stable echocardiogram and RHC findings, but severe obstructive lung disease (FEV1/FVC 54, FEV 1 of .92 (40%), RV 161%, and DLCO 57%). Because the pathophysiology of acute hypercapnic respiratory failure does not correspond with acute PAH exacerbation or right ventricular failure, we suspected that severe air trapping secondary to SLE-induced bronchial inflammation led to her intraoperative respiratory arrest. Treatment of obstructive lung disease was then initiated with a fluticasone/salmeterol inhaler and advanced to 500/50 dosage, which led to significant improvement in subsequent FEV1. The patient was then relisted for renal transplant and underwent successful renal transplantation in Oct 2019. She did not require hemodialysis following the transplant, recovered quickly, and was discharged. At this time, 6 months after surgery, she remains functional class II and continues to do very well in the posttransplant setting. DISCUSSION: In this report, despite successful management of our patient's PAH, it was only after treatment of her concurrent SLE-related bronchitis that she was able to successfully receive a renal transplant. CONCLUSIONS: SLE is a systemic condition that can have multiple concurrent cardiopulmonary abnormalities, including obstructive lung disease due to bronchial inflammation and pulmonary arterial hypertension. Therefore, it is important for clinicians to look carefully at treatment options for each related cardiopulmonary condition. Reference #1: Hachulla E, Jais X, Cinquetti G, et al. Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus. Chest. 2018;153(1):143-151. https://doi.org/10.1016/j.chest.2017.08.014https://doi.org/10.1016/j.chest.2017.08.014 Reference #2: Kimoto M, Sharma TS, Manzi S. 35 - Lupus and the Cardiopulmonary System. In: Wallace DJ, Hahn BH, eds. Dubois' Lupus Erythematosus and Related Syndromes (Ninth Edition). Content Repository Only!; 2019:418-433. https://doi.org/10.1016/B978-0-323-47927-1.00035-9 Reference #3: Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD. Manifestations of systemic lupus erythematosus. Maedica (Buchar). 2011;6(4):330-336. DISCLOSURES: No relevant relationships by dan grinnan, source=Web Response No relevant relationships by Meagan Shinbashi, source=Web Response

INITIATION OF ELEXACAFTOR-TEZACAFTOR-IVACAFTOR LEADING TO LUNG TRANSPLANTATION

SESSION TITLE: Medical Student/Resident Genetic and Developmental Disorders Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene resulting in impaired chloride conductance, a dehydrated airway surface liquid, and multi-organ dysfunction (1). The highly effective CFTR modulator elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA) has shown efficacy in people with the ∆F508 mutation (2). We report a case of a patient who developed acute respiratory failure with hypoxia and hypercapnia requiring lung transplantation after starting ELX/TEZ/IVA. CASE PRESENTATION: A 23-year-old male with CF (∆F508/621+1G–>T) complicated by very severe obstructive lung disease with a ppFEV1 of 32% presented with hypoxemia and productive cough three days after starting treatment with ELX/TEZ/IVA. Physical exam revealed bilateral crackles and bilateral lower extremity pitting edema. Labs showed WBC 20.8 and IgE 563. Sputum culture was positive for Pseudomonas aeruginosa. CT chest revealed extensive upper lobe cystic bronchiectasis with mucus plugging and consolidation. Patient was started on culture-directed antimicrobials and ELX/TEZ/IVA was held. Patient improved clinically, and on day 5 of hospitalization, he was restarted on ELX/TEZ/IVA at a reduced dose. Within 13 hours, he developed worsening hypoxemia and hypercapnia requiring high flow oxygen and intermittent noninvasive positive pressure ventilation and was transferred to a lung transplant center, where he was listed for lung transplantation and eventually transplanted. DISCUSSION: ELX/TEZ/IVA is usually well tolerated; adverse events leading to discontinuation of the drug only occurred in 1% of study participants (2). The most commonly reported adverse event was an infective pulmonary exacerbation, likely because pulmonary exacerbations are frequently observed in CF (2). The temporal relationship between starting ELX/TEZ/IVA to onset of symptoms, both initially and after restarting the drug despite being on appropriate antimicrobial therapy, suggests an adverse effect directly related to the drug rather than a classic pulmonary exacerbation. This observation may be the result of significant CFTR upregulation in a patient with very severe obstructive lung disease. Since individuals with a ppFEV1 < 40% were not studied, more data is needed to determine if closer monitoring is warranted around the time of initiation with ELX/TEZ/IVA in this more severe cohort. CONCLUSIONS: ELX/TEZ/IVA is an extremely effective and overall safe drug for around 90% of individuals with CF (1). Our case necessitates asking if individuals with more severe CF should undergo closer monitoring with hospitalization when initiating treatment. Reference #1: Ratjen F, Döring G. Cystic fibrosis. Lancet. 2003;361(9358):681-9. Reference #2: Middleton PG et al. Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. N Engl J Med. 2019;381(19):1809-1819. DISCLOSURES: No relevant relationships by Christopher Barrios, source=Web Response No relevant relationships by Travis Homan, source=Web Response No relevant relationships by Stephanie Link, source=Web Response

A RARE PRESENTATION FOR PULMONARY EMBOLISM

SESSION TITLE: Monday Fellow Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: With the evolution of diagnostic and treatment modalities for venous thrombo-embolic disease we have seen a significant reduction in the case fatality rate as well as a steady increase in the total number of cases diagnosed worldwide. While pulmonary infarction is an uncommon complication of PE, it's further evolution to cavitaty formation is exceedingly rare and we would like to present and discuss such a case. CASE PRESENTATION: Our case is that of a 68 year old gentleman with a known history of severe obstructive lung disease and HIV who initially presented to the hospital with complaints of acute severe dyspnea. He was diagnosed with a pneumothorax, and after successful chest tube placement a non contrast computer tomography (CT) scan of his chest showed a cavitary lesion in the superior segment of the right lower lobe. Subsequent testing also revealed an acinetobacter baumanii infection for which he was treated with a prolonged course of antibiotics. Three months after the initial admission, he returned to the hospital with dyspnea at which time a CT of his chest with contrast showed extensive bilateral PE with radiological signs of chronicity and a new left lower lobe infiltrate with cavitation. The distribution of his chronic PE was consistent with both his previously noted right lung and current left lung cavities. During his second hospitalization, no causative organisms could be isolated however he was treated empirically with antibiotics. His PE was treated with anticoagulation and he was successfully followed in the outpatient setting with serial CT imaging which showed resolution of both cavitary lesions and no new PE's on subsequent CT scans. DISCUSSION: The dual blood supply to the lungs make pulmonary infarction an uncommon sequela of PE. Less than 1% of PE's have been noted to lead to cavitation after infarction. While multiple risk factors have been noted for cavitation after a PE, including old age, chronic lung disease, a history of heart failure and the size of the infarct area, the average time to cavitation is 2-3 weeks. Areas of infarct are usually aseptic however secondary infections can lead to hastening of the formation of a cavitary lesion. Our patient presented to the hospital with a formed cavity implying that he had suffered from the initial PE a few weeks before. While cavity formation after pulmonary infarcts has been well described in the literature, our case is unique because of the presentation as well as because he developed multiple cavitations at different times over the course of treatment which is an extremely unusual and rare clinical course for a PE and has not been previously noted in the literature. CONCLUSIONS: While cavitary lesions are a rare complication of pulmonary infarcts, PE must always be considered as part of the differential diagnosis. Early appropriate treatment can prevent complications that have been noted with acute and chronic PE. Reference #1: Sonmez, M., Aboussouan, L. S., Farver, C. A. R. O. L., Murthy, S. C., & Kaw, R. (2018). Pulmonary infarction due to pulmonary embolism. Reference #2: Konstantinides, Stavros V., et al. "Management of pulmonary embolism: an update.” Journal of the American College of Cardiology 67.8 (2016): 976-990. Reference #3: Koroscil, Matthew T., and Timothy R. Hauser. "Acute pulmonary embolism leading to cavitation and large pulmonary abscess: a rare complication of pulmonary infarction.” Respiratory medicine case reports 20 (2017): 72-74. DISCLOSURES: No relevant relationships by Taaran Cariappa Ballachanda Subbaiah, source=Web Response No relevant relationships by Jason George, source=Web Response

Defining severe obstructive lung disease in the biologic era: an endotype-based approach.

Severe obstructive lung disease, which encompasses asthma, chronic obstructive pulmonary disease (COPD) or features of both, remains a considerable global health problem and burden on healthcare resources. However, the clinical definitions of severe asthma and COPD do not reflect the heterogeneity within these diagnoses or the potential for overlap between them, which may lead to inappropriate treatment decisions. Furthermore, most studies exclude patients with diagnoses of both asthma and COPD. Clinical definitions can influence clinical trial design and are both influenced by, and influence, regulatory indications and treatment recommendations. Therefore, to ensure its relevance in the era of targeted biologic therapies, the definition of severe obstructive lung disease must be updated so that it includes all patients who could benefit from novel treatments and for whom associated costs are justified. Here, we review evolving clinical definitions of severe obstructive lung disease and evaluate how these have influenced trial design by summarising eligibility criteria and primary outcomes of phase III randomised controlled trials of biologic therapies. Based on our findings, we discuss the advantages of a phenotype- and endotype-based approach to select appropriate populations for future trials that may influence regulatory approvals and clinical practice, allowing targeted biologic therapies to benefit a greater proportion and range of patients. This calls for co-ordinated efforts between investigators, pharmaceutical developers and regulators to ensure biologic therapies reach their full potential in the management of severe obstructive lung disease.

Bitter Taste Receptors for Asthma Therapeutics

Clinical management of asthma and chronic obstructive pulmonary disease (COPD) has primarily relied on the use of beta 2 adrenergic receptor agonists (bronchodilators) and corticosteroids, and more recently, monoclonal antibody therapies (biologics) targeting specific cytokines and their functions. Although these approaches provide relief from exacerbations, questions remain on their long-term efficacy and safety. Furthermore, current therapeutics do not address progressive airway remodeling (AR), a key pathological feature of severe obstructive lung disease. Strikingly, agonists of the bitter taste receptors (TAS2Rs) deliver robust bronchodilation, curtail allergen-induced inflammatory responses in the airways and regulate airway smooth muscle (ASM) cell proliferation and mitigate features of AR in vitro and in animal models. The scope of this review is to provide a comprehensive and systematic insight into our current understanding of TAS2Rs with an emphasis on the molecular events that ensue TAS2R activation in distinct airway cell types and expand on the pleiotropic effects of TAS2R targeting in mitigating various pathological features of obstructive lung diseases. Finally, we will discuss specific opportunities that could help the development of selective agonists for specific TAS2R subtypes in the treatment of asthma.

Effects of Sirolimus on Lung function in patients with Lymphangioleiomyomatosis.

Background:Lymphangioleiomyomatosis (LAM) is a progressive lungs disease that affects women at reproductive years. Sirolimus inhibits mammalian target of rapamycin (mTOR) and its administration in past studies was hopeful in treatment of patients with LAM. The aim of this study was to evaluate sirolimus therapy on lung function in LAM patients.Methods:We conducted a trial to evaluate the effect of sirolimus on six patients with LAM who had severe or very severe obstructive lung disease, and one-year follow up. Maintenance level of Sirolimus was 10-15 ng/ml. Serial visits (including physical examination, evaluation of signs and symptoms of disease and adverse events due to treatment), spirometry, 6MWT done at baseline 3, 6, 9 and 12 months after. Spirometric parameters walk distance and O2 saturationbefore and after exercise at first and the end of treatment were measured.Results:Four patients had TSC-LAM while the other 2 patients had S-LAM. The mean level of sirolimus was 13 ng/ml after one-year treatment. Mean FEV1 at enrollment and end of study was 1000cc (33% predict) and 1228cc (42% predict) respectively (P=0.674). The mean FVC at baseline and end of study was 1648cc (49% predict) and 1866cc (55% predict) (P=0.996). The mean FEV1/FVC at enrollment and the end of treatment was 58% and 62% respectively (P=0.753). The mean FEF25-75 at first and at the end of treatment was 16% and 26%, respectively (P=0.028). The mean walk distance in 6MWT at first and at the end of study was 315 meters (P=0.9). The mean percentage of O2 saturation at rest was 84% and 92% at first and at the end of study (P=0.104).Conclusion:In LAM patients, sirolimus has been shown stabilizeto or improve lung function, rest and exertional O2 saturation. Sirolimus was effective in LAM patients who had severe or very severe physiological disorders.

Pharmacological and genetic reappraisals of protease and oxidative stress pathways in a mouse model of obstructive lung diseases.

Protease-antiprotease imbalance and oxidative stress are considered to be major pathophysiological hallmarks of severe obstructive lung diseases including chronic obstructive pulmonary disease (COPD) and cystic fibrosis (CF), but limited information is available on their direct roles in the regulation of pulmonary phenotypes. Here, we utilized βENaC-transgenic (Tg) mice, the previously established mouse model of severe obstructive lung diseases, to produce lower-mortality but pathophysiologically highly useful mouse model by backcrossing the original line with C57/BL6J mice. C57/BL6J-βENaC-Tg mice showed higher survival rates and key pulmonary abnormalities of COPD/CF, including mucous hypersecretion, inflammatory and emphysematous phenotypes and pulmonary dysfunction. DNA microarray analysis confirmed that protease- and oxidative stress-dependent pathways are activated in the lung tissue of C57/BL6J-βENaC-Tg mice. Treatments of C57/BL6J-βENaC-Tg mice with a serine protease inhibitor ONO-3403, a derivative of camostat methylate (CM), but not CM, and with an anti-oxidant N-acetylcystein significantly improved pulmonary emphysema and dysfunction. Moreover, depletion of a murine endogenous antioxidant vitamin C (VC), by genetic disruption of VC-synthesizing enzyme SMP30 in C57/BL6J-βENaC-Tg mice, exaggerated pulmonary phenotypes. Thus, these assessments clarified that protease-antiprotease imbalance and oxidative stress are critical pathways that exacerbate the pulmonary phenotypes of C57/BL6J-βENaC-Tg mice, consistent with the characteristics of human COPD/CF.

Flavorings in electronic cigarettes: an unrecognized respiratory health hazard?

Flavorings in electronic cigarettes: an unrecognized respiratory health hazard?

P208 Assessment Of Curvilinearity (curv) And Phase Iii Analysis Of Multiple Breath Washout (mbw)

Introduction and Hypothesis In cystic fibrosis (CF), but not in PCD (Am J Respir Crit Care Med. 2013;188:545–549), lung clearance index (LCI) and spirometry are correlated. The difference may be related to differences in small airway disease. To explore this further, the novel MBW analyses Curv, S cond* and S acin* were calculated in PCD and CF (Eur Respir J. 2013;42(suppl 2):380–388). Curv assesses specific ventilation inhomogeneity calculated as the ratio of the slope of the first half to the second half of the washout, and unlike LCI is not sensitive to deadspace effects. S cond and S acin are not useful in severe obstructive lung disease; S cond* and S acin* are recalculations corresponding respectively to VI in the conducting airways and the acinar region. (S cond* is measured from the slopes of the increase in phase III modified to include the 0–3 lung turnovers and S acin* * is phase III over the first breath of the washout, minus the contribution of S cond* ). We hypothesised that these novel indices will differ in PCD compared to CF due to differences in small airways disease. Methods 38 PCD (14 male, group mean (range) age 21.8 (7.2–59.1) years, FEV1 Z score -3.18 ((-6–0.17)) and CF (14 male, group mean (range) age 10.9 (6.8–19.1) years, FEV1 Z score -2.72 ((-5.4–0.9)) patients matched for P. aeruginosa status and 24 healthy controls recorded spirometry and MBW. LCI, Curv, Scond* and Sacin* were calculated. Results There was no difference in LCI, FEV 1 and Curv between the patient groups. LCI was correlated with S cond* (CF p = 0.0006, r = 0.5, PCD, p = 0.03 r = 0.3), S acin* (CF p acin (CF p cond was not. There was no difference in S acin* * between the groups, but S cond* was significantly lower in PCD, approaching that of healthy controls. Conclusions Curv is similarly impaired in PCD and CF. S cond* is nearly normal in PCD but not CF, supporting the hypothesis that there are differences in distal airway disease between these conditions. Finally, the results suggest that the new indices may be better discriminators between diseases in severe obstructive lung disease.

Safety of carbon dioxide insufflation during gastric endoscopic submucosal dissection in patients with pulmonary dysfunction under conscious sedation.

Carbon dioxide (CO2) insufflation is effective for gastric endoscopic submucosal dissection (ESD). However, its safety is unknown in patients with pulmonary dysfunction. This study aimed to investigate the safety of CO2 insufflation during gastric ESD in patients with pulmonary dysfunction under conscious sedation. We analyzed 322 consecutive patients undergoing ESD using CO2 insufflation (1.4 L/min) for gastric lesions. Pulmonary dysfunction was defined as a forced expiratory volume in 1.0 s/forced vital capacity (FEV1.0%) <70% or vital capacity <80%. Transcutaneous partial pressure of CO2 (PtcCO2) was recorded before, during, and after ESD. In total, 127 patients (39%) had pulmonary dysfunction. There were no significant differences in baseline PtcCO2 before ESD, peak PtcCO2 during ESD, and median PtcCO2 after ESD between the pulmonary dysfunction group and normal group. There was a significant correlation between PtcCO2 elevation from baseline and ESD procedure time (r = 0.22, P < 0.05) only in the pulmonary dysfunction group. In patients with FEV1.0% <60%, the correlation was much stronger (r = 0.39, P < 0.05). Neither the complication incidences nor the hospital stay differed between the two groups. CO2 narcosis or gas embolism was not reported in either group. CO2 insufflation during gastric ESD in patients with pulmonary dysfunction under conscious sedation is safe with regard to complication risk and hospital stay. However, in patients with severe obstructive lung disease, especially in those with FEV1.0% <60%, longer procedure time may induce CO2 retention, thus requiring CO2 monitoring.

Balloon Dilatation of Pediatric Subglottic Laryngeal Stenosis during the Artificial Apneic Pause: Experience in 5 Children

Introduction. Balloon dilatation is a method of choice for treatment of laryngeal stenosis in children. The aim of procedure in apneic pause is to avoid new insertion of tracheostomy cannula. Patients and Methods. The authors performed balloon dilatation of subglottic laryngeal strictures (SGS) in 5 children (3 girls and 2 boys) without tracheotomy. Two of them with traumatic and inflammatory SGS had a tracheal cannula removed in the past. The other 3 children with postintubation SGS had never had a tracheostomy before. The need for tracheostomy due to worsening stridor was imminent for all of them. Results. The total of seven laryngeal dilatations by balloon esophagoplasty catheter in apneic pause was performed in the 5 children. The procedure averted the need for tracheostomy placement in 4 of them (80%). Failure of dilatation in girl with traumatic stenosis and concomitant severe obstructive lung disease led to repeated tracheostomy. Conclusion. Balloon dilatation of laryngeal stricture could be done in the absence of tracheostomy in apneic pause. Dilatation averted threatening tracheostomy in all except one case. Early complication after the procedure seems to be a negative prognostic factor for the outcome of balloon dilatation.

Severe obstructive disease: Similarities and differences between smoker and non-smoker patients with COPD and/or bronchiectasis

IntroductionPoorly reversible airflow obstruction may or may not be related to smoking. ObjectivesTo describe patients with severe obstructive lung disease including etiology, imaging, functional aspects, systemic manifestations, and the pattern of bronchodilator response. MethodsSixty-eight patients (age 55.9±13.7 years, FEV1 [forced expiratory volume in one second] 31.9±10.2% predicted) underwent spirometry, evaluation of body mass composition, 6-minute walk test, X-ray, thorax high-resolution CT scanning, and clinical evaluation. ResultsOf 68 patients enrolled, 37 had chronic obstructive pulmonary disease (COPD) and 31, extensive bronchiectasis. Among COPD patients the CT scans showed emphysema in 78.4%, and bronchiectasis in 48.6%. There were no significant differences between smokers and non-smokers, except for vital capacity, significantly smaller in non-smokers (p<0.001). We found 29 and 20 volume responders (VR) according to Paré et al. (FEV1/FVC>1=flow responder or <1=VR) and ATS/ERS criteria, respectively. According to Paré et al. criteria, there were 18 patients with FEV1<30% predicted among 29 VR, and 12 with FEV1<30% predicted among 39 without volume response (p=0.0101). ConclusionsIn patients with severe obstruction, smoking does not appear to be relevant in determining functional or systemic differences, and Paré et al. criteria can detect more VR. Bronchiectasis is a common finding in severe COPD.

Sobreposição de asma brônquica e DPOC

Immunoglobulin quantification showed an increase in total IgE levels (671 IU/mL), with no other abnormalities. Alpha-1 antitrypsin levels were normal. Respiratory function test results revealed moderately severe obstructive lung disease accompanied by inflation, moderately reduced alveolar-capillary diffusion, and mild type I respiratory failure. The patient had a negative bronchodilator response (Table 1). Her electrocardiogram was normal, and the results of the microbiological study of sputum were negative. The final diagnosis was uncontrolled bronchial asthma, pulmonary emphysema, diffuse bronchiectasis, and gastroesophageal reflux disease (GERD). The patient attributed her hypopharyngeal pain to the use of inhaled corticosteroids and therefore decided to discontinue the medication, having consequently shown improvement. She was started on esomeprazole, and salmeterol was replaced by indacaterol. Although this improved her pharyngeal and epigastric pain, she still had To the Editor:

Abdominal Compartment Syndrome and Acute Kidney Injury Due to Excessive Auto–Positive End-Expiratory Pressure

Abdominal compartment syndrome is an under-recognized cause of acute kidney injury in critically ill patients. We report a case of a patient with severe obstructive lung disease who, while intubated for respiratory failure, developed abdominal compartment syndrome and oliguric acute kidney injury due to air-trapping and excessive auto-positive end-expiratory pressure (auto-PEEP; also known as intrinsic PEEP). When chemical paralysis was initiated and the auto-PEEP resolved, the patient's intra-abdominal hypertension rapidly improved and kidney function recovered immediately. Abdominal compartment syndrome secondary to excessive auto-PEEP appears to be unreported in the literature; however, any process that significantly increases intrathoracic pressure conceivably could cause increased pressure to be transmitted to the abdominal compartment, resulting in organ failure. Patients undergoing mechanical ventilation, which puts them at risk of airflow obstruction and the development of intra-abdominal hypertension, should be evaluated for air-trapping and excessive auto-PEEP.

Focus on Diagnosis: Spirometry

1. Kamakshya P. Patra, MD 1. Department of Pediatrics, Mayo Clinic, Rochester, MN. * Abbreviations: FEF25%–75%: : forced expiratory flow, midexpiratory phase FEV1: : forced expiratory volume fraction in 1 second FVC: : forced vital capacity PEFR: : peak expiratory flow rate Office spirometry, traditionally done by the pulmonary specialist, has become commonplace in the pediatric primary care office. The National Asthma Education and Prevention Program guidelines recommend routine use of spirometry for the initial diagnosis and ongoing management of asthma. A nationwide survey demonstrated that only 21% of physicians used spirometry for the management of children with asthma, as per the guidelines. And, approximately one half of the physicians were unable to interpret the results of spirometry. Office spirometry has been shown to result in change in asthma management in 15% of children. In 45% of cases, the severity of asthma was underrated by the providers in the absence of spirometry. A peak flow meter, which measures peak expiratory flow rate (PEFR), is a simple, inexpensive, and portable device that may be helpful in the management of asthma. However, PEFR is effort-dependent and does not reflect small airway function. The average cost of a good spirometer is in the $1,500 to $2,000 range. There must be training of the office personnel who perform the spirometry, and the spirometer should be calibrated at least daily. In general, patients must be at least age 4 to 4.5 years to perform reliable forced vital capacity (FVC) maneuvers required for spirometry. A 7-year-old girl has experienced wheezing since age 3 years and has been diagnosed as having asthma. Her symptoms did not improve despite therapy with albuterol, corticosteroids, salmeterol, and monteleukast. She was referred to the pediatric pulmonology clinic for persistent symptomatology. Spirometry was performed, which revealed a fixed obstruction. Flow volume loops revealed truncation of inspiratory and expiratory limbs (Fig 1). Computed tomography scan of the …

Focus on Diagnosis

Focus on Diagnosis

Severe obstructive disease: Similarities and differences between smoker and non-smoker patients with COPD and/or bronchiectasis

IntroductionPoorly reversible airflow obstruction may or may not be related to smoking. ObjectivesTo describe patients with severe obstructive lung disease including etiology, imaging, functional aspects, systemic manifestations, and the pattern of bronchodilator response. MethodsSixty-eight patients (age 55.9±13.7 years, FEV1 [forced expiratory volume in one second] 31.9±10.2% predicted) underwent spirometry, evaluation of body mass composition, 6-minute walk test, X-ray, thorax high-resolution CT scanning, and clinical evaluation. ResultsOf 68 patients enrolled, 37 had chronic obstructive pulmonary disease (COPD) and 31, extensive bronchiectasis. Among COPD patients the CT scans showed emphysema in 78.4%, and bronchiectasis in 48.6%. There were no significant differences between smokers and non-smokers, except for vital capacity, significantly smaller in non-smokers (p<0.001). We found 29 and 20 volume responders (VR) according to Paré et al. (FEV1/FVC>1=flow responder or <1=VR) and ATS/ERS criteria, respectively. According to Paré et al. criteria, there were 18 patients with FEV1<30% predicted among 29 VR, and 12 with FEV1<30% predicted among 39 without volume response (p=0.0101). ConclusionsIn patients with severe obstruction, smoking does not appear to be relevant in determining functional or systemic differences, and Paré et al. criteria can detect more VR. Bronchiectasis is a common finding in severe COPD.

Vanishing Lung Syndrome and HIV Infection

Persons with HIV infection have a higher risk of infectious pulmonary complications, chronic obstructive pulmonary disease, lung cancer, pulmonary hypertension, and pulmonary fibrosis than individuals not infected with HIV. Herein, we describe the clinical course of a patient with longstanding and well-controlled HIV infection and multiple previous pneumothoraces who presented to medical attention with insidious onset of shortness of breath and was diagnosed with vanishing lung syndrome (VLS). The VLS or giant bullous emphysema is a distinct clinical syndrome characterized by large bullae, predominantly in the upper lobes, occupying at least one third of the hemithorax and compressing surrounding normal lung parenchyma. It is a progressive disorder that typically occurs in young men, the majority of whom are smokers. As people with HIV/AIDS are now surviving well into middle age and beyond, clinicians are more likely to encounter VLS and severe obstructive lung disease, which are potentially fatal but preventable conditions.