Where today’s science meets tomorrow’s care TM Rare Lung ...€¦ · Jack Kelly for their...

American Thoracic Society International Conference

May 19-May 24, 2017Washington, DC

conference.thoracic.org

Rare Lung Disease GuideWhere today’s science meets tomorrow’s careTM

1

May 2017

Dear Colleagues:

The scope of the American Thoracic Society is amazingly broad – covering pulmonary, critical care, and sleep medicine. The challenge that we face each year as we put the International Conference program together is that some clinical topics that may be of central importance to our members, conference attendees and patients may not be prominently featured in the program. This is especially the case with some of rare lung diseases.

With each challenge though, comes opportunity. Because of the wealth of scientific and clinical information presented at ATS 2017, these diseases, though uncommon, will be the focus of many presentations over the next several days. The purpose of this Rare Lung Disease Guide is to help you more easily find these presentations.

In his chapter on rare lung diseases in “Breathing in America: Diseases, Progress, and Hope” (2010) Francis X. McCormack, MD noted that research on uncommon respiratory diseases has produced some of the most exciting discoveries in pulmonary medicine, stating that “Insights gained from uncommon lung diseases often shed light on the more common ones.”

The definition of a rare disease by its prevalence varies by country. In the European Union, for instance, it is defined as a disease that affects fewer than 1 in 2,000 while in the United States it is a disease that affects fewer than 1 in 200,000. This guide was compiled by a small group of ATS Members who studied the ATS 2017 abstracts – authors requested to be considered for inclusion. Many thanks to Dr. Frank McCormack, Dr. Lisa Young, Dr. Jonathan Kropski, Dr. Nishant Gupta and Mr. Jack Kelly for their assistance with this resource.

There are a number of patient advocacy groups that are exhibiting at ATS 2017. In addition to attending the presentations noted within, I encourage you to visit with them to learn more about these rare conditions. Groups listed in BOLD are members of the ATS Public Advisory Roundtable and can all be found in PAR ROW, near the ATS Center in the Exhibit Hall. Learn more about this group and its many contributions to the Society and its work at: http://www.thoracic.org/patients/par/.

Sincerely,

Zea Borok, MD

Zea Borok, MDChair, ATS International Conference Committee

Alpha-1 Foundation – Booth 1054ARDS Foundation- Booth 1356Asthma and Allergy Foundation of America – Booth 957Children’s Interstitial Lung Disease Foundation, Inc. (chILD) – Booth 1257Foundation for Sarcoidosis Research – Booth 1156The LAM Foundation – Booth 1456Lung Transplant Foundation – Booth 1055

Lymphangiomatosis and Gorham’s Disease Alliance – Booth 1454NTM Info & Research, Inc. – Booth 1255Primary Ciliary Dyskinesia Foundation (PCD) – Booth 955Pulmonary Fibrosis Foundation – Booth 1207Pulmonary Hypertension Association – Booth 1154Relapsing Polychondritis Awareness and Support Foundation, Inc. – Booth 1363Scleroderma Foundation – Booth 1354Tuberous Sclerosis Alliance –Booth 1355

DISCLAIMER

The purpose of this guide is to help the American Thoracic Society International Conference attendees navigate the viewing of posters for disorders that typically get less attention at the conference because of their rarity. During abstract submission, authors were instructed to check the box for consideration in the Rare Lung Disease Guide; the abstracts on that list were then further narrowed down. Conditions represented by less than 50 abstracts were considered for inclusion. We regret that not all rare diseases could be represented, and welcome suggestions for additional categories for listing in future editions. Please email us with any suggestions: [email protected].

3

Rare Disease Term

Date/Day Board Number/Time Abstract Title Session ID and Title Location Acute Eosinophilic Pneumonia

Sunday, May 21, 2017

P924 9:15 am-4:15 pm; Poster Viewing 11:15 am-1:00 pm

Smoking-Related Acute Eosinophilic Pneumonia Compared to Idiopathic and Drug-Related Forms

A42: New Developments in ILD Research Area J, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Monday, May 22, 2017

P945 9:15 am-4:15 pm;Poster Viewing 11:15 am-1:00 pm

Acute Eosinophilic Pneumonia and Tracheitis Following Recent Smoking

B42: Interstitial Lung Disease: A Potpourri of Cases

Area J, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Tuesday, May 23, 2017


Daptomycin-Induced Acute Eosinophilic Pneumonia: A Treatable Serious Side Effect

C43: Drug Induced Lung Disease: Case Reports

Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


A Case of Systemic Hypereosinophilia Associated with Doxycycline Administration




Rare Case of Acute Eosinophilic Pneumonia C56: Critical Care Case Reports: ARDS Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


A Rare Cause of “Antibiotic Resistant” Pneumonia C66: Pediatric Case Reports: Diffuse Lung Diseases

Area F, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Acute Fibrinous and Organizing Pneumonia



Acute Fibrinous Organizing Pneumonia in a Patient with Cryoglobulinemia and Hepatitis C

A39: Autoimmune Lung Disease: Case Reports

Area C, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Acute Fibrinous and Organizing Pneumonia: One Case Report and Literature Review

A43: Non-IPF ILD: Clinical Studies Area J, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



Acute Fibrinous and Organizing Pneumonia in a Lung Transplant Patient Managed Without Calcineurin Inhibitors

B46: Transplantation: Case Reports and Clinical Studies

Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Acute Interstitial Pneumonia



Acute Interstitial Pneumonia Versus Acute Respiratory Distress Syndrome: Is There a Difference?

C56: Critical Care Case Reports: ARDS Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Alpha-1 Antitrypsin Deficiency



Focal Bronchiectasis Due to Alpha-1 Antitrypsin Deficiency Complicated by Mycobacterium Avium-Intracellulare Infection

A40: Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

Area K, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

4

Rare Disease Term

Date/Day Board Number/Time Abstract Title Session ID and Title Location



Production and Preliminary In Vitro Evaluation of a Plant-Made, Oxidation Resistant Alpha-1 Antitrypsin

A73: Beyond Cigarette Smoke in COPD Area L, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


A Novel Role for Alpha-1 Antitrypsin in Protecting the Lung from Destruction by MMP-13



Next Generation Sequencing of Alpha-1 Antitrypsin Deficiency MZ Individuals Shows Frequent Bi-Allelic Mutations




The Effect of PiZ Genotype and Augmentation Therapy on Bronchoalveolar Lavage (BAL) and Peripheral Blood Mononuclear Cell (PBMC) Transcriptomes in Alpha-1 Antitrypsin Deficiency

B34: Genetics and Genomics of Lung Disease

Area E, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


812 9:15 am-11:15 am; Poster Discusion 10:00 am-11:15 am Poster Viewing: 9:15 am-10:00 am

Stereological Analysis of the Peripheral Lung Anatomy in Patients with Panlobular and Centrilobular Emphysema Using MDCT and microCT

C29: Advances in Pulmonary Imaging to Study Lung Structure and Function

Marquis Ballroom 1-2 (Level M2), Marriott Marquis Washington


Aα-Val541 a Novel Biomarker of Proteinase 3 Activity C33: Novel Biomarkers in Lung Disease Area N, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Alpha 1-Antitrypsin Pi MZ vs Pi ZZ: Ramifications of a Mistaken Identity

C48: COPD: Case Reports I Area L, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Alpha 1 Antitrypsin Deficiency with Normal Levels Presenting as a Diagnostic Challenge



Protein Z Deficiency: An Uncommon Cause of Diffuse Thrombotic Disease

C70: Call My Name: Case Reports in Pulmonary Vascular Disease



The Release of PAD2 and PAD4 from Neutrophils by Primary Granule Degranulation: A Link Between Alpha-1 Antitrypsin Deficiency and the Anti-CCP Autoimmune Response

C73: New Mechanisms and Therapies in COPD

Area L, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


The Biological Effects of Double Dose Augmentation Therapy for Subjects with Alpha-1 Antitrypsin Deficiency

C74: Advances in Translational COPD Area L, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Can Urinary Peptides Discriminate Between COPD Patients with and Without Inherited Alpha1-Antitrypsin Deficiency?



Alpha 1 Antitrypsin (A1AT) Increases Macrophages Efferocytosis Via Mannose Binding Lectin (MBL) - Mannose Receptor (MAR) Signaling


5

Rare Disease Term




Augmentation Therapy Increases Hydrogen Peroxide Accumulation in Peripheral Blood Mononuclear Cells of ZZ Alpha-1 Antitrypsin Deficiency Patients



Alpha1-Antitrypsin Reduces Endoplasmic Reticulum Stress and Release of Inflammatory Cytokines in Cystic Fibrosis Primary Bronchial Epithelial Cells In Vitro



Alpha-1 Antitrypsin Augmentation Therapy Modulates Neutrophil Lipid Raft Structure and Cell Adhesion in Patients with Alpha-1 Antitrypsin Deficiency



Inhaled Alpha-1-Antitrypsin Restores Lower Respiratory Tract Protease- Anti-protease Homeostasis and Reduces Inflammation in Alpha-1 Antitrypsin Deficient Individuals:Â A Phase 2 Clinical Study Using Inhaled Kamada-API



Comparison of the Forced Oscillation Technique and Spirometry in COPD and Alpha-1-Antitrypsin Deficient Patients

C80-B: Multi-Modality Assessment of COPD, Asthma, and Asthma-COPD Overlap Syndrome


Wednesday, May 24, 2017

417 1:30-3:30 pmPoster Discussion 2:00-3:30 pmPoster Viewing 1:30-2:00 pm

Non-Typeable Haemophilus Influenzae Is Associated with Rapid Lung Function Decline and Poor Macrophage and Neutrophil Phagocytosis in Patients with Alpha-1 Anti-Trypsin Deficiency

D101: Mechanistic and Translational Studies in COPD

Room 151 B (Middle Building, Street Level), Walter E. Washington Convention Center


Demographics and Clinical Profile of Patients with Alpha-1 Antitrypsin Deficiency PiMZ and PiMS Genotypes Started on Augmentation Therapy

D103: Alpha 1-Antitrypsin Deficiency: 50 Years of Progress

Room 144 A-C (Middle Building, Street Level), Walter E. Washington Convention Center


Multicomponent Intervention Improves BMI in a Randomized Trial of Alpha-1 Antitrypsin Deficient Patients




Increase in Exercise Activities in Alpha-1 Antitrypsin Deficient Patients: Results of a Randomized Trial




Factors Associated with FEV1 Decline in Alpha-1 Antitrypsin Deficient Patients



6

Rare Disease Term




Higher C-Reactive Protein Levels Are Associated with Less Symptoms Among a Pi (MZ) Alpha-1 Antitrypsin Phenotype Cohort




A Re-Analysis of FEV1 Decline and Augmentation Effects Using Stricter Slope Measurements from the NIH Alpha 1-Antitrypsin Deficiency




The Effect of Alpha1-Proteinase Inhibitor (A1-PI) Therapy on Changes in Regional Lung Density: Post-Hoc Analysis of the RAPID/RAPID Extension Trial




Identification of Genetic Variants in the SERPINA1 Gene that Influence A1AT Levels




Comorbidity Patterns in Alpha-1-Antitrypsin Deficiency Depend on Natural History and Substitution Therapy




Decline in FEV1 in Patients with PiZZ Alpha-1-Antitrypsin Deficiency Depends on the Exacerbation Rate




CT Evident Emphysema in MZ and SZ Alpha 1 Antitrypsin Deficiency: Smoking as a Phenotype-by-Environment Effect




Chronic Oxidative Stress Leads to Accelerated Telomere Shortening in Children with Alpha-1 Antitrypsin Deficiency




Results of a Large Scale Gene-Based Screening Program for Alpha-1-Antitrypsin Deficiency in the USA




The Irish National Alpha-1 Antitrypsin Deficiency Targeted Detection Programme



7

Rare Disease Term




Improving Testing for Alpha-1 Antitrypsin Deficiency Using the Pulmonary Function Laboratory




Real Life Treatment Benefit of Intravenous Augmentation Therapy for Severe Alpha-1 Antitrypsin Deficiency



9:15-11:15 amOral Presentation 9:45-10:00 am

Human Alveolar Epithelial Cells Derived from Gene-Edited Induced Pluripotent Stem Cells for Alpha-1 Antitrypsin Deficiency Lung Disease Modelling

D18: Who Does What in COPD: The Cellular Origins

Room 204 A-C (South Building, Level 2), Walter E. Washington Convention Center

824 9:15-11:15 amPoster Discussion 10:00-11:15 amPoster Viewing 9:15-10:00 am

Comprehensive Gene Resequencing of SERPINA1 in the SPIROMICS Multi-Ethnic Population

D28: The Web of Omics: DNA, RNA, and Protein Modifications


Antisynthetase Syndrome


P933 9:15 am-4:15 pm Poster Viewing 11:15 am-1:00 pm

Characterization of MDA5-Antibody Associated Interstitial Lung Disease Phenotypes




A Peculiar Case of Auto-Antibody Negative Anti-Synthetase Syndrome

B41: Antisynthetase Syndrome and Autoimmune Myositis Associated ILD: Cases from the Wards



A Unique Manifestation of Anti-Synthetase Syndrome in an Indian Male




A Mechanic’s Hands: An Unusual Presentation of Anti-PL-7 Anti-Synthetase Syndrome Diagnosed with Chest X-Ray




Amyopathic Dermatomyositis and Interstitial Lung Disease Associated with Anti-NXP2 Autoantibody




Atypical Presentation of Anti-Jo-1 Positive Interstitial Lung Disease B41: Antisynthetase Syndrome and Autoimmune Myositis Associated ILD: Cases from the Wards


8

Rare Disease Term




With ChILD- A Case of Antisynthetase Syndrome in Pregnancy B41: Antisynthetase Syndrome and Autoimmune Myositis Associated ILD: Cases from the Wards



Bamboo Nodes Reveal Antisynthetase Syndrome as Cause of Interstitial Lung Disease




Severe Pulmonary Hypertension and Myocarditis in a Patient with Overlapping Sjogren and Antisynthetase Syndrome





Anti-Synthetase Syndrome (ASS) Presenting as Acute Respiratory Distress Syndrome (ARDS) in a Patient Without Myositis Features



703 9:15 am-11:15 pm Poster Discussion 10:00-11:15 am Poster Viewing 9:15 am-10:00 am

Cyclophosphamide for Antisynthetase Syndrome-Associated Interstitial Lung Disease

D23: Autoimmune Lung Disease: Clinical Presentation, Prognosis, and Treatment


Benign Metastatic Leiomyoma



Benign Metastatic Leiomyoma in the Absence of Uterine Leiomyoma C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Bilateral Phrenic Nerve Paralysis



Under the Radar: Bilateral Phrenic Nerve Palsy as a Cause of Progressive Dyspnea



Birt-Hogg-Dube Syndrome



A Female Patient with Birt-Hogg-Dube Syndrome Whose Computed Tomography of the Chest Showed Numerous Pulmonary Cysts Similar to Lymphangioleiomyomatosis

A41: LAM and Other Cystic Lung Disease: Case Reports



More Than Skin Deep: A Case of Birt-Hogg-Dube Syndrome A41: LAM and Other Cystic Lung Disease: Case Reports



Characterization of CT Scan in Birt-Hogg-Dube Syndrome Compared with Non-BHD Diffuse Cysts Lung Diseases in Chinese Patients


9

Rare Disease Term




Birt-Hogg-Dube Syndrome Prospectively Detected by Review of Chest Computed Tomography Scans


MondayMay 22, 2017


Unilateral Recurrent Pneumothoraces in a Teenager with Pleurodesis

B67: Interesting and Unusual Pediatric Case Reports


Bronchiectasis



A STAT1 Gain-of-Function Mutation in Multifactorial Bronchiectasis in a Five Year-Old

C65: Pediatric Case Reports: Bronchiectasis, Cystic Fibrosis, and Lung Infections



1:30-3:30 pm Oral Presentation 2:00-2:15 pm

United States Bronchiectasis Registry Longitudinal Follow Up at Two Years

D94: Advances in Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

Room 207 A-B (South Building, Level 2), Walter E. Washington Convention Center

Bronchiolitis Obliterans



Sporadic Obliterative Bronchiolitis: A Case Series and Literature Review



Spirometry Trends and Utility of Fractional Exhaled Nitric Oxide Measurement in Bronchiolitis Obliterans Syndrome in Hematopoietic Stem Cell Transplant Recipients

A76: What’s in the Tool Box to Assess Lung Function




Role of Alpha 1-Antitrypsin and Human Neutrophil Elastase in BALf of Lung Transplant Recipients: Preliminary Data

B35: Translational Studies in Lung Transplantation



Workers Exposed to Styrene Who Died from Respiratory Diseases Suspicious for Possible Obliterative Bronchiolitis

B58: Occupational Lung Disease: Case Studies, Epidemiology, and Mechanisms

Area A, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


EGFR Ligands Increase MEK-Dependent Hyaluronan Synthesis in Naïve Rat Lung Fibroblasts




“Money Is the Root of All Evil” – A Case of Constrictive Bronchiolitis Secondary to Money Exposure




Bronchiolitis Obliterans in a Civilian Survivor of a Chemical Warfare Attack

B59: Chemical Exposures Area A, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Lung Epithelial Injury and Aberrant Repair After Sulfur Mustard Inhalation in Rats

B59: Chemical Exposures Area A, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



A Case of Constrictive Bronchiolitis from Burn-Pit Inhalational Exposure in Iraq Treated with Tacrolimus

C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

10

Rare Disease Term




Proteomics of Bronchoalveolar Lavage (BAL) Fluid in Bronchiolitis Obliterans Syndrome (BOS)

D92: Basic Mechanisms of Lung Allograft Rejection

Room 202 B (South Building, Level 2), Walter E. Washington Convention Center


LOXL-2 Contributes to the Development of Obliterative Bronchiolitis (OB) in Humans

D92: Basic Mechanisms of Lung Allograft Rejection

Room 202 B (South Building, Level 2), Walter E. Washington Convention Center


Loss of Multiciliated Epithelium and Impaired Mucosal Immunity in Lungs of Soldiers with Constrictive Bronchiolitis

D98: Insights into Environmental Exposures in Asthma, COPD, and Constrictive Bronchiolitis

Room 145 A-B (Middle Building, Street Level), Walter E. Washington Convention Center

Bronchocentric Granulomatosis



Bronchocentric Granulomatosis in a Previolusly Healthy Woman B42: Interstitial Lung Disease: A Potpourri of Cases


Carcinoid


924 2:15-4:15 pm Poster Discussion 2:45-4:15 pmPoster Viewing 2:15-2:45 pm

Evaluation of the Prognostic Significance of AJCC Staging Guidelines in Lung Carcinoid Tumors

B110: Pulmonary Nodules and Thoracic Surgery: Working Across the Aisle




Cherry Pit Extraction Leading to Incidental Finding of Carcinoid Tumor Treated with Argon Plasma Coagulation Laser

C45: EBUS and Airway Interventions: IP Case Reports

Area M, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Can Two Be in One? Infected Bronchogenic Cyst and Carcinoid Tumorlets

C62: Case Reports: Pulmonary Infections – Miscellaneous



Mammogram Leading to Diagnosis of Lung Neoplasm C80-G: Thoracic Oncology Case Reports I



A Rare Case of Papillary Carcinoid Tumor of the Lung C80-H: Thoracic Oncology Case Reports II




An Atypical Case of Typical Carcinoid C80-I: Thoracic Oncology Case Reports III



Diarrhea and Flushing After Endobronchial Mass Biopsy: An Unusual Clue Toward Diagnosis

C80-I: Thoracic Oncology Case Reports III



A Tale of Carcinoid in a Veteran C80-J: Thoracic Oncology Case Reports IV


11

Rare Disease Term

Date/Day Board Number/Time Abstract Title Session ID and Title LocationCastleman’s



A Rare Case Report: Idiopathic Multicentric Castleman Disease Complicated with Acute Respiratory Distress Syndrome Under Extracorporeal Membrane Oxygenation Support

C57: Critical Care Case Reports: Hematology, Oncology, Rheumatology, and Immunology


Catamenial Pneumothorax



Non Catamenial Endometriosis Related Pneumothorax B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


A Case of Recurrent Pneumothorax B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Thoracic Endometriosis Presenting as Catamenial Hemopneumothorax

B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Chronic Eosinophilic Pneumonia



Steroid Dependent Chronic Eosinophilic Pneumonia Treated Successfully with Azathioprine; a Novel Therapy Reported on Two Patients





Eosinophilic Pneumonia: A Multiple Cause Scenario C66: Pediatric Case Reports: Diffuse Lung Diseases



Atypical Presentation of an Uncommon Disease: A Case of Idiopathic Chronic Eosinophilic Pneumonia?

C66: Pediatric Case Reports: Diffuse Lung Diseases


Chronic Granulomatous Disease



Myeloid Derived Suppressor Cells Limit Inflammation and Lung Injury in Response to Aspergillus Via a Functional NADPH Oxidase

C93: Host Defense Against Pathogen Invasion

Independence Ballroom A-D (Level M4), Marriott Marquis Washington

Chylothorax



A Case of Recurrent Chylothorax in Renal Cell Carcinoma B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Chylothorax: Where Is the Obstruction? B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Markedly Delayed Occurrence of Chylothorax Following Thoracic Surgery


Rare Disease Term


12



Chylothorax Caused by Cirrhosis: A Common Cause of Transudative Chylothorax




Dasatinib-Induced Chylothorax in Chronic Myeloid Leukemia C43: Drug Induced Lung Disease: Case Reports



Management and Outcomes of Congenital Chylothorax in the NICU: A Case Series

C64: Pediatric Case Reports: BPD and Congenital Lung Diseases


Coatomer Subunit Alpha Mutations


325 9:15-11:15 am Poster Discussion 9:45-11:15 am Poster Viewing 9:15-9:45 am

A Recurrent De Novo Mutation in COPA Causes Follicular Bronchiolitis in an Icelandic Family

C21: Omics in Lung Disease Room 151 A (Middle Building, Street Level), Walter E. Washington Convention Center

P680 9:15 am-4:15 pm Poster Viewing 11:15 am-1:00pm

COPA Mutation Causing Interstitial Lung Disease in an Infant C66: Pediatric Case Reports: Diffuse Lung Diseases


Congenital Central Hypoventilation Syndrome



Congenital Central Hypoventilation Syndrome: Co-Occurrence of a PHOX2B Polyalanine Repeat Expansion Mutation (PARM) and a Non-PARM (NPARM) in Newborn with Asymptomatic Parents Carrying the PHOX2B Mutations

A65: Pediatric Case Reports: Sleep and Respiratory Physiology




An Unusual Cause of Hypercarbia in Premature Twins C64: Pediatric Case Reports: BPD and Congenital Lung Diseases


Congenital Malformations



Massive Hemoptysis Due to an Iatrogenic Pseudoaneurysm of an Aberrant Bronchial Artery




When Infertility, Sinusitis, and Bronchiectasis Are Not Cystic Fibrosis: A Case of Recurrent Pneumonia in a Patient with Mayer-Rokitansky-Kuster-Hauser Syndrome




Asymptomatic Adult with Congenital Cystic Adenomatoid Malformation of the Lungs




Azygos Continuation of the IVC (ACIV) Mimicking a Mediastinal Mass A44: Interventional Pulmonary: Case Reports

Area B, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Lung Hypoplasia in a Genetic Mouse Model of Down Syndrome A96: Best of Pediatrics Room 202 A (South Building, Level 2), Walter E. Washington Convention Center

13

Rare Disease Term




A Rare Association with Aspergilloma: Intralobar Pulmonary Sequestration

A63: Case Reports: Fungal Area D, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



Azygous Lobe Contributing to Recurrent Pneumothorax Refractory to Pleurodesis



Congenital Lobar Emphysema Intervention Decision in Infant with Complex Congenital Heart Disease




Congenital Heart Disease Associated Trisomy 21 with Complete Tracheal Rings




Persistent Cough and Post Obstructive Bronchitis Due to a Paratracheal Bronchogenic Cyst




Pulmonary Hypertension Due to Partial Anomalous Pulmonary Venous Return

B70: My Name is ... Pulmonary Vascular Disease Case Reports



Subpleural Cysts on Chest Tomography in Seven Months Old Baby with Tachypnea




Holt-Oram Syndrome and Pulmonary Artery Sling with Tracheal Stenosis: A Rare Combination of Two Rare Congenital Disorders




Hiding in Plain Sight C70: Call My Name: Case Reports in Pulmonary Vascular Disease



A Persistent Cough that Won’t Go Away: An Unusual Case of Tracheal Diverticulum

B39: Cough: Rare and Common Area B, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Cough Due to a Cyst: Congenital Abnormalities in Adults B39: Cough: Rare and Common Area B, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Crack Lung



Crack Lung: A Rare but Potentially Fatal Complication of Cocaine Use



Cryptogenic Organizing Pneumonia



Retrospective Analysis of 40 Patients with Cryptogenic Organizing Pneumonia Treated with Clarithromycine and 22 Patients Treated with Prednisone


14

Rare Disease Term




Cryptogenic Organizing Pneumonia After a Motor Vehicle Accident B42: Interstitial Lung Disease: A Potpourri of Cases



A Rare Fulminant and Fatal Case of Cryptogenic Organizing Pneumonia




Organizing Pneumonia Secondary to Hiatal Hernia B42: Interstitial Lung Disease: A Potpourri of Cases




Bronchiolitis Obliterans Organizing Pneumonia in Association with Common Variable Immunodeficiency Syndrome

C44: Immunodeficiency: Case Reports Area M, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Desquamative Interstitial Pneumonitis



Respiratory Bronchiolitis Associated Interstitial Lung Disease (RBILD)/Desquamative Interstitial Pneumonia (DIP) Presenting as Cystic Lung Disease





Interstitial Lung Disease in a Chronic Smoker B42: Interstitial Lung Disease: A Potpourri of Cases


Diffuse Alveolar Hemorrhage



Diffuse Alveolar Hemorrhage as a Rare Manifestation of Rheumatoid Arthritis




Up in Smoke: An Unusual Cause of Diffuse Alveolar Hemorrhage A60: Adverse Effects of Tobacco, Marijuana, and Recreational Drugs

Area N, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


P916 9:15 am-4:15 pmPoster Viewing 11:15 am-1:00 pm

Therapeutic Dilemma in a Patient with a History of Recurrent Diffuse Alveolar Hemorrhage Due to Idiopathic Pulmonary Hemosiderosis Requiring Anticoagulation





Diffuse Alveolar Haemorrhage (DAH): A Rare Complication of Anticoagulant Therapy with Warfarin




Diffuse Alveolar Hemorrhage as a Result of Seronegative Pulmonary Renal Syndrome



Sevoflurane-Induced Diffuse Alveolar Hemorrhage in a Child C66: Pediatric Case Reports: Diffuse Lung Diseases


15

Rare Disease Term

Date/Day Board Number/Time Abstract Title Session ID and Title LocationDiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH)



A Case of DIPNECH Developing True Midgut Carcinoid with Disease Arrest After Resection of the Carcinoid and Institution of Long Term Azithromycin Treatment. A Ten Year Clinical and Radiological Follow-Up




The Various Clinical Faces of the Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH)

B80-L: General Thoracic Oncology Area N, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



Atypical Carcinoid Originating in the Bronchus and Accompanied by Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Case Report




Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): A Case Presentation and Overview of Documented Cases

C80-J: Thoracic Oncology Case Reports IV


Diffuse Pulmonary Meningotheliomatosis



A Rare Case of Diffuse Pulmonary Meningotheliomatosis B42: Interstitial Lung Disease: A Potpourri of Cases



Diffuse Pulmonary Meningotheliomatosis: A Scary, Yet Benign Picture





Minute Pulmonary Meningothelial Like Pulmonary Nodules C80-I: Thoracic Oncology Case Reports III


Duchene Muscular Dystrophy (DMD)


2:15-4:15 pmOral Presentation 2:45-3:00 pm

Respiratory Function in Eteplirsen-Treated Duchenne Muscular Dystrophy (DMD) Patients Compared to Natural History

A96: Best of Pediatrics Room 202 A (South Building, Level 2), Walter E. Washington Convention Center



Changes in Surface Ventilation Following Mechanical Insufflation:Exsufflation Therapy in Patients with Duchenne’s Muscular Dystrophy

B50: Critical Care: Beyond the Ventilator - Non-Invasive Ventilation, High Flow Oxygen, and Non-Conventional Ventilation



Correlation Between Volume- and Flow-Related Pulmonary Function Outcomes in Patients with DMD

B69: When Kids Cannot Sleep (or Breathe)




Effect of Idebenone on Bronchopulmonary Adverse Events and Hospitalizations in Patients with Duchene Muscular Dystrophy (DMD)

C105: Disorders of Respiratory Physiology and Sleep in Children

Room 201 (South Building, Level 2), Walter E. Washington Convention Center

16

Rare Disease Term




Steroid Use Delays but Does Not Prevent Loss of Pulmonary Function in Patients with Duchene Muscular Dystrophy (DMD)




Longitudinal Pulmonary Function in Children with Neuromuscular Disorders



Dyskeratosis Congenita



Bone Marrow Failure Secondary to Short Telomere Syndrome After Lung Transplantation




Hepatopulmonary Syndrome and Telomerase (TERT) Mutation: A Rare Association





Dyskeratosis Congenita Presenting with Pulmonary Arteriovenous Malformation (PAVMs): A Case Report



Embolism-Other



Chronic Pulmonary Silicone Embolism Is Not a Common Finding in Explanted Lungs from Women with Prior Breast Augmentation



Emphysema-Secondary



A Case of Cutis Laxa with Juvenile Emphysema Arising from Frameshift Mutations in Exon 30 of the Elastin Gene (ELN)


Endobronchial Histoplasmosis



Endobronchial Histoplasmosis Presenting as Respiratory Distress in an 8 Month Old Infant



Eosinophilic Granulomatosis with Polyangiitis



Intrapulmonary EGPA Subtype Identified by Clinical Parameters Clustering

A32: Asthma and Allergy Clinical Studies Area C, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Incidental Pulmonary Nodules and Unusual Eosinophilic Granulomatous Lymphadenitis: An Under-Recognized Manifestation of Systemic Vasculitis



17

Rare Disease Term

Date/Day Board Number/Time Abstract Title Session ID and Title LocationEpithelioid Hemangioendothelioma



A Rare Malignancy in an Active Smoker C80-H: Thoracic Oncology Case Reports II



An Unusual Case of Solitary Pulmonary Nodule in a Patient with History of Epithelial Ovarian Cancer

C80-H: Thoracic Oncology Case Reports II


Erdheim Chester Disease



A Rare Case of Diffuse Cystic Lung Disease A41: LAM and Other Cystic Lung Disease: Case Reports



Lung Involvement in Erdheim-Chester Disease: An Independent Prognostic Factor?




Pulmonary Erdheim-Chester Disease; a Long-Term Success of Treatment with Oral and Inhaled Corticosteroid



Familial Interstitial Pneumonia



Early Interstitial Changes in Family Members of Patients with Familial Interstitial Pneumonia

C75: Fibrosis: Current and Future Approaches



Predicting Undiagnosed Early Pulmonary Fibrosis in Familial Interstitial Pneumonia Relatives Using Peripheral Blood Mononuclear Cell Gene Expression



Fibrosing Mediastinitis



Fibrosing Mediastinitis: A Rare Complication of Histoplasmosis A63: Case Reports: Fungal Area D, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



A Genome-Wide Association Study of 58 Patients with Fibrosing Mediastinitis

B34: Genetics and Genomics of Lung Disease




Fibrosing Mediastinitis Induced Pulmonary Hypertension and Respiratory Failure Successfully Treated with Pulmonary Venous Stent and Rituximab Therapy




714 9:15 am-11:15 pm Poster Discussion 10:00-11:15 am Poster Viewing 9:15-10:00 am

Rituximab in the Management of Complicated Fibrosing Mediastinitis: The Mayo Clinic Experience



18

Rare Disease Term

Date/Day Board Number/Time Abstract Title Session ID and Title LocationGood Syndrome



What is so Bad in Good Syndrome? A Case Report and Pulmonary Images



Goodpasture Syndrome



What Triggered Massive Hemoptysis in Goodpasture Syndrome with Isolated Pulmonary Involvement?

C68: Pediatric Case Reports: Autoimmune Diseases and Immunodeficiencies


Granulomatosis with Polyangititis



Physical Signs and Radiographic Manifestation of ANCA Associated Granulomatosis with Polyangiitis





Resolution of Acute Respiratory Failure Due to Granulomatosis with Polyangitis While on Extracorporeal Membrane Oxygenation as a Bridge to Transplantation

C58: Critical Care Case Reports: Notable Causes and Complications in Acute Respiratory Failure



713 9:15 am-11:15 pm Poster Discussion 10:00-11:15 am Poster Viewing 9:15-10:00 am

Rituximab for Refractory Granulomatosis with Polyangiitis and for Eosinophilic Granulomatosis with Polyangiitis



Granulomatous and Lymphocytic Interstitial Lung Disease


121 2:15-4:15 pm Poster Discussion 3:00-4:15 pm Poster Viewing 2:15-3:00 pm

BLF/UKPIN Consensus Statement on the Definition, Diagnosis and Management of Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) in Common Variable Immunodeficiency Disorders (CVID)

B103: Clinical Studies in Bronchiectasis, Immunodeficiency, and Drug Induced Lung Disease




Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency: A Case Report




Mistaken Identity: Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) and Common Variable Immune Deficiency (CVID) Initially Diagnosed as Sarcoidosis



An Unusual Mimicker of Asthma in an Active Duty Army Physician C44: Immunodeficiency: Case Reports Area M, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Granulomatous Lymphocytic Interstitial Lung Disease in a Pediatric Case of Common Variable Immunodeficiency



19

Rare Disease Term

Date/Day Board Number/Time Abstract Title Session ID and Title LocationHemangiopericytoma



Hemangiopericytoma Masquerading as a Cardiac Murmur C80-H: Thoracic Oncology Case Reports II


Hereditary Hemorrhagic Telangiectasia



C.1309C/T Endoglin (ENG) Gene Mutation Associated Multiple Pulmonary Arteriovenous Malformations Successfully Treated by Endovascular Coiling and Vascular Plugs

A44: Interventional Pulmonary: Case Reports




Pulmonary Arterovenous Malformations in Hereditary Hemorrhagic Telangiectasia: Cross Sectional Study of 398 Patients from Argentina

B72: Others Here With Us: Clinical Features of Pulmonary Vascular Disease I




A Young Female with Difficult-to-Control Asthma C36: Asthma and Allergy: Case Series and Reports



Elective Vs. Non-Elective Hospitalizations in Patients with Hereditary Hemorrhagic Telangiectasia (i.e. HHT, Osler-Weber-Rendu Disease)

C51: Critical Care: More Non-Pulmonary Critical Care Problems

Area D, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



Geographical Distribution of Hereditary Haemorrhagic Telangiectasia in Ireland

D107: Diamonds and Pearls: Pulmonary Hypertension Phenotypes


9:15-11:15 am Oral Presentation 11:00-11:15 am

Prevalence of Pulmonary Hypertension in a Large Cohort of Hereditary Hemorrhagic Telangiectasia Patients

D17: Sign “O” The Times: Diagnosis and Risk Stratification of Pulmonary Hypertension

Liberty Ballroom I-L (Level M4), Marriott Marquis Washington

Hermansky-Pudlak Syndrome



Clinical Issues Associated with Lung Transplantation in Hermansky-Pudlak Syndrome Pulmonary Fibrosis

A102: Transplantation: Advances and Challenges

Room 150 B (Middle Building, Street Level), Walter E. Washington Convention Center



Successful Single Lung Transplantation for Hermansky-Pudlak Syndrome with Pulmonary Fibrosis




Acute Exacerbation of Pulmonary Fibrosis in Hermansky-Pudlak Syndrome



20

Rare Disease Term




Plasma Biomarkers of Diffuse Parenchymal Lung Disease in Hermansky-Pudlak Syndrome

C101: IPF: Clinical Studies, Therapeutics, and More II



Generation of Mouse Lung Epithelial Cell Models of Hermansky-Pudlak Syndrome (HPS) Via CRISPR-Cas9 Mediated Mutation in MLE15

C101: IPF: Clinical Studies, Therapeutics, and More II



Hermansky-Pudlak Syndrome 2 Mutations Alter Mitochondrial Biogenesis and Function in the Alveolar Epithelium

C107: Mitochondria and Metabolic Alterations in Lung Disease



The Endocannabinoid/CB1R System Is Overactive in Hermansky-Pudlak Syndrome Associated Pulmonary Fibrosis in Humans



High Altitude Pulmonary Edema



An Unorthodox Treatment Regimen to Prevent Recurrence of High Altitude Pulmonary Edema (HAPE) in a Young Skier

A65: Pediatric Case Reports: Sleep and Respiratory Physiology




What the HAPE Is Going On: High Altitude Pulmonary Edema at Children’s Hospital Colorado



Hot Tub Lung


124 9:15-11:15 pmPoster Discussion 10:00-11:15 amPoster Viewing 9:15-10:00 am

Hot Tub Lung: A Diagnostic Challenge D25: Unusual Infections: Case Reports Room 144 A-C (Middle Building, Street Level), Walter E. Washington Convention Center

Hyper IgE Syndrome



A Case of Positive Pressure Ventilation in Bullous Lung Disease A80-B: Case Reports in Sleep Medicine Area H, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention

Hypersensitivity Pneumonitis



Hypersensitivity Pneumonitis Associated Mortality in the US A42: New Developments in ILD Research Area J, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Sepsis and Pneumonitis Due to Intravesical BCG C43: Drug Induced Lung Disease: Case Reports


21

Rare Disease Term




The Basement Flight – Bird Fancier’s Lung in a Teenager C66: Pediatric Case Reports: Diffuse Lung Diseases



An Unusual Case of Dyspnea and Hypoxemia in a Teenager C68: Pediatric Case Reports: Autoimmune Diseases and Immunodeficiencies



Telomere Length in Chronic Hypersensitivity Pneumonitis Explant Lungs





The MUC5b Promoter Polymorphism and Shorter Telomere Length Are Associated with Idiopathic Pulmonary Fibrosis-Like Features in Chronic Hypersensitivity Pneumonitis

D104: Advances in Interstitial Lung Disease


Idiopathic Capillary Leak Syndrome



Leaking to Death: A Possible Case of Clarkson Syndrome (Idiopathic Capillary Leak Syndrome)

C58: Critical Care Case Reports: Notable Causes and Complications in Acute Respiratory Failure


IgG4 Related Disease



Organizing Pneumonia Masking the Diagnosis of IgG4 Related Disease: A Case of a 79 Year Old Man with Myelodysplastic Syndrome





Clinical Features of IgG4-Related Disease Associated with Lung Involvement




Immunoglobulin G4-Related Lung Disease Mimicking Pulmonary Langerhans Cell Histiocytosis on Imaging



IgG4-Related Pulmonary Nodules Mimicking Malignancy C44: Immunodeficiency: Case Reports Area M, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Recurrent Respiratory Failure and Bilateral Pleural Effusions in a Patient with IgG4-Related Disease

C46: Pleural Disease: Case Reports II Area M, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Blush or Flush? A Preventable Case of Carcinoid Crisis C57: Critical Care Case Reports: Hematology, Oncology, Rheumatology, and Immunology



Toddler by Nomenclature, Veteran by Pathology C57: Critical Care Case Reports: Hematology, Oncology, Rheumatology, and Immunology


22

Rare Disease Term

Date/Day Board Number/Time Abstract Title Session ID and Title LocationInflammatory Bowel Disease



Lung Activity of Crohn’s Disease: A Case Report B42: Interstitial Lung Disease: A Potpourri of Cases


Inhalational Diseases



Acute Inhalational Metallic Nickel Poisoning: Report of 4 Cases C43: Drug Induced Lung Disease: Case Reports


Kaposi’s Sarcoma



Primary Pulmonary Kaposi’s Sarcoma as the Initial Presentation of HIV Infection


Kartagener’s Syndrome


P668 P1351 9:15 am-4:15 pm Poster Viewing 11:15 am-1:00 pm

A Case of Pulmonary Nodules and Opacities in a Young Adult Female with Primary Ciliary Dyskinesia



Lane-Hamilton Syndrome


P285 P1351 9:15 am-4:15 pm Poster Viewing 11:15 am-1:00 pm

A Woman with Celiac Disease and Diffuse Alveolar Hemorrhage A39: Autoimmune Lung Disease: Case Reports


Langerhan’s Cell Histiocytosis



An Intriguing Case of Pulmonary Langerhans Cell Histiocytosis Following Treatment for Chronic Lymphocytic Leukemia




A Rare Case of Pulmonary Non-Langerhans Cell Histiocytosis A41: LAM and Other Cystic Lung Disease: Case Reports



Management of Spontaneous Pneumothorax and Safety of Air Travel in Patients with Pulmonary Langerhans Cell Histiocytosis



A Novel Approach Leads to the Resolution of a Persistent Pneumothorax in a Young Male with Pulmonary Langerhans Cell Histiocytosis



23

Rare Disease Term




Lung Involvement in Childhood Langerhans Cell Histiocytosis, A Multi-Institutional Study from the French LCH Study Group

B108: Cystic Fibrosis, Primary Ciliary Dyskinesia, and ILD



Not a Usual Smoker’s Cough B42: Interstitial Lung Disease: A Potpourri of Cases




Pulmonary Langerhans Cells Histiocytosis; an Important Differential in Patients with Human Immunodeficiency Virus




Smoking Teenager with Diffuse Cystic Lung Disease C66: Pediatric Case Reports: Diffuse Lung Diseases



Pulmonary Crystal-Storing Histiocytosis: A Case Report C80-I: Thoracic Oncology Case Reports III




Molecular and Cellular Mechanisms of Pulmonary Langerhans Cell Histiocytosis



Laryngeal Lichen Planus With Glottic Stenosis



Laryngeal Lichen Planus with Glottic Stenosis C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Lemierre’s Syndrome



Atypical Complications of an Unexpected Case of Lemierre’s Disease

A52: Critical Care Case Reports: Adult and Pediatric Sepsis and ICU Infections




Septic Thrombophlebitis of Internal Jugular Vein and Septic Lung Emboli as Rare Complication of Acute Tonsillitis

B66: Case Reports: Bacterial Infections Area L, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


The Forgotten Disease: The Case of a Young Man with Lemierre’s Syndrome

B66: Case Reports: Bacterial Infections Area L, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Leukemic Infiltration



Leukemic Bronchopulmonary Infiltrates in Patients with Chronic Lymphocytic Leukemia: The Culprit or a Bystander?


24

Rare Disease Term


Lipid Storage Disorders



Respiratory and Airway Smooth Muscle pathology in a Pompe disease mouse model

B75: Current Updates in Disease Modeling




Insidious Respiratory Failure in a 54-Year-Old Woman: The Clue to Diagnose a Family with Pompe Disease


Lipoid Pneumonia



Massive Lung Hemorrhage Induced by Exogenous Lipoid Pneumonia Solely Treated with Bronchial Artery Embolization Using Platinum Coil





Chronic Cough: A Case of Obfuscation B39: Cough: Rare and Common Area B, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


18F Fluorodeoxyglucose (FDG) Positron Emission Tomography (PET) in Exogenous Lipoid Pneumonia (ELP) Emulating Metastatic Pancreatic Cancer to Lungs





Hypoxemia in Lipoid Pneumonia: Role of Intrapulmonary Bronchopulmonary Anastomoses



Lymphangioleiomyomatosis



Recurrence of Lymphangioleiomyomatosis After Double Lung Transplantation in a Patient with LAM




A 45 Year Old Lady Presenting with Bilateral Pneumothoraces A41: LAM and Other Cystic Lung Disease: Case Reports



A Rare Case Report of Lymphangioleiomyomatosis A41: LAM and Other Cystic Lung Disease: Case Reports



When Cysts Leak: A Case of Recurrent Pneumothorax in Pregnancy A41: LAM and Other Cystic Lung Disease: Case Reports



Pulmonary Lymphangioleiomyomatosis: A Case Report A41: LAM and Other Cystic Lung Disease: Case Reports



The Etiology of Diffuse Cystic Lung Diseases A41: LAM and Other Cystic Lung Disease: Case Reports


25

Rare Disease Term




Efficacy and Safety of Low Dose Sirolimus in Lymphangioleiomyomatosis



Risk Factors for Stomatitis in Patients with Lymphangioleiomyomatosis During Treatment with Sirolimus: A Multicenter Investigator-Initiated Prospective Study



Effects of Culture Medium and Glutaminase Inhibition on Proliferation of Normal Human Airway Smooth Muscle Cells and Pulmonary LAM Patient-Derived Cells

A74: Regulatory Mechanisms of the Molecular Pathways in Fibrosis




Metabolomic Profiling Identifies mTOR Dependent and Independent Pathway Activation in Lymphangioleiomyomatosis

B29: Autophagy: Mechanisms and Therapeutic Opportunities



Skim Milk Chylothorax! B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Oxidized Regenerated Cellulose Induces Pleural Thickening in Patients with Pneumothorax: Possible Involvement of Mesothelial Mesenchymal Transition

B45: Pleural Disease: Clinical Studies Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



Vitamin D Binding Protein and Alpha-1-Acid Glycoprotein Levels in Serum Are Associated with Lymphangioleiomyomatosis

C80-A: Everything You Need to Know About Bronchopulmonary Dysplasia, Cystic Fibrosis and Diffuse Parenchymal Lung Diseases



Hyperpolarized 129Xe Ventilation and Diffusion MRI of Lymphangioleiomyomatosis




PD-L1 in Pulmonary Lymphangioleiomyomatosis (LAM): A Potential Therapeutic Target for Advanced Disease with Loss of TSC1/2



26

Rare Disease Term




Tankyrase Inhibitors: A Potential Novel Therapy for LAM/TSC D104: Advances in Interstitial Lung Disease



TSC2-Null Cell-Fibroblast Interactions Enhance Protease Activity by Extracellular Acidification in Lymphangioleiomyomatosis (LAM)




Tumorigenic Cells of Lymphangioleiomyomatosis Are of a Neural Crest Origin and Susceptible to Tyrosine Kinase Inhibitors




Changes in Lung Function During Pregnancy in Patients with Lymphangioleiomyomatosis




NKG2D Ligand Expression in Lymphangioleiomyomatosis D104: Advances in Interstitial Lung Disease



Lymphangioleiomyomatosis (LAM) Cell Survival Is Driven by IGF2-Induced STAT3 Activation

D108: Cell Fate Decisions: Senescence, Repair, and Regeneration


Lymphatic Anomalies: Generalized Lymphatic Anomaly (GLA), Kaposiform Lymphangiomatosis (KLA), Lymphangiectasia



Disseminated Pulmonary Lymphangiomatosis with Bone Involvement in an Adult Patient




Diagnosis and Treatment of Non-Traumatic Chylothorax Using Advanced Lymphatic Imaging and Percutaneous Lymphatic Embolization




Lymphangiography as Treatment for Pulmonary Lymphangiectasia in a Patient with Noonan Syndrome





Primary Pulmonary Lymphangiectasis in a Male Infant with Transposition of Great Arteries




Congenital Pulmonary Lymphangiectasia with CD4 Lymphopenia Presenting in Adulthood



27

Rare Disease Term




Kaposiform Pulmonary Lymphangiomatosis: A Systemic Disease Amenable to Mechanical Manipulation?




Late Presentation of Lymphatic Abnormalities in a Child with Noonan Syndrome and a R1T1 Mutation



Lymphoma



A Rare Case of Primary Effusion Lymphoma Post Cardiac Transplant with HIV Negative and HHV8 Positive Serology




Typical Presentation of Atypical Disease: A Case of Angio-Immunoblastic Lymphoma

C80-G: Thoracic Oncology Case Reports I


Lymphoma-BALT/MALT



Bronchus Associated Lymph Tissue (BALT) Lymphoma Masquerading as Pneumonia




An Unusual Cause of Lung Mass C80-G: Thoracic Oncology Case Reports I



Synchronous Pulmonary MALT Lymphoma and Cryptosporidium Infection: Coincidence or Causal Connection?




A Case of MALT Lymphoma as an Insidious Lung Mass C80-J: Thoracic Oncology Case Reports IV


Lymphoma-B-Cell



Broadening the Differential: EBV-Associated Diffuse Large B-Cell Lymphoma of the Elderly




Primary Pulmonary Intravascular Large B-Cell Lymphoma: A Report of Three Cases and Literature Review




Primary Pulmonary Diffuse Large B-Cell Lymphoma Mimicking Cannon Ball Metastases




A Very Rare Case of Multifocal Endobronchial Diffuse B-Cell Lymphoma



28

Rare Disease Term


Lymphoma-Hodgkins



A Teenager with Chronic Cough and Multiple Cavitary Lung Lesions B67: Interesting and Unusual Pediatric Case Reports


Lymphoma-Marginal Zone



Primary Pulmonary Non-Hodgkin’s Lymphoma: Expanding the Ground-Glass Opacification Differential




Killing Two Birds with One Stone: A Tale of Pulmonary Lymphoma and Hepatitis C



Lymphoma-Primary Pulmonary



Clinical and Misdiagnosed Analysis of Primary Pulmonary Lymphoma: A Retrospective Study


Lymphoma-T-Cell



Why Does this Patient Have Pneumocystis? A Challenging Case of Granulomatous Pneumocystis and Rash as the Initial Presentation of Common Variable Immunodeficiency in the Context of Peripheral T-Cell Lymphoma



Airway Exchange Catheter Used in a Lymphoma Patient with Acute Respiratory Failure Who Failed to Be Mechanically Ventilated




Needle in a Haystack: A Rare Pulmonary Presentation of Peripheral T Cell Lymphoma



Mediastinal Fibrosis



Extrinsic Airway Compression Secondary to Histoplasma Mediastinal Granuloma

A63: Case Reports: Fungal Area D, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



Mediastinal Granulomatous Inflammation Secondary to Ruptured Silicone Breast Implants



Mounier Kuhn



Mounier-Kuhn Disease, a Rare Cause of Dyspnea Associated with Pulmonary Nocardiasis, Restrictive Lung Physiology, and IgM Deficiency



29

Rare Disease Term



108 9:15-11:15 pmPoster Discussion 10:00-11:15 amPoster Viewing 9:15-10:00 am

Mounier- Kuhn Syndrome: An Overlooked Cause of Recurrent Lower Respiratory Tract Infections

D25: Unusual Infections: Case Reports Room 144 A-C (Middle Building, Street Level), Walter E. Washington Convention Center

Myositis



Necrotizing Myositis Leading to Respiratory Failure and Death A52: Critical Care Case Reports: Adult and Pediatric Sepsis and ICU Infections




Treatment of Rapidly Progressive Interstitial Lung Disease with Positive Melanoma Differentiation Associated Gene 5 with Amyopathic Dermatomyositis Using Lung Rest Ventilation with Extracorporeal Membrane Oxygenation with Corticosteroids, Rituximab, and Cellcept





Not Every Orthopnea Means Heart Failure C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Neuroendocrine Cell Hyperplasia of Infancy



Neuroendocrine Cell Hyperplasia of Infancy (NEHI): Does Failure to Thrive Predict or Influence Pulmonary Morbidity?



Neuromuscular



An Unusual Case of Neuromuscular Restrictive Lung Disease C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Ossification



Dendriform Pulmonary Ossification A39: Autoimmune Lung Disease: Case Reports



Dendriform Pulmonary Ossification in Interstitial Lung Disease A43: Non-IPF ILD: Clinical Studies Area J, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Papillomatosis



Solitary Bronchial Papilloma; A Rare Differential for Bronchial Obstruction with Malignant Potential




Recurrent Respiratory Papillomatosis C45: EBUS and Airway Interventions: IP Case Reports


30

Rare Disease Term




Tracheal Papillomatosis C45: EBUS and Airway Interventions: IP Case Reports



Multiple Malignant Tracheal Papillomatosis C80-G: Thoracic Oncology Case Reports I


Pemphigoid



Best Treatment for a Rare Cause of Airway Obstruction? A Case of Tracheal Pemphigoid Responsive to Cyclophosphamide and Prednisone



Plastic Bronchitis



Study Design: Phase II Safety and Efficacy of Inhaled Tissue Plasminogen Activator (Activase) for the Treatment of Acute Pediatric Plastic Bronchitis

A64: Bronchopulmonary Dysplasia Area F, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Platypnea/Ortho



A Novel Cause of Platypnea Orthodeoxia Syndrome B70: My Name is ... Pulmonary Vascular Disease Case Reports


Pleuropulmonary Fibroelastosis



Lung Transplantation for a Chinese Young Man with Idiopathic Pleuroparenchymal Fibroelastosis




Alveolar Fibroelastosis in Bronchiolitis Obliterans After Lung and Stem Cell Transplantation: A Comparative Analysis of Morphological and Molecular Motifs

B76: Drivers of Cell Fate, Senescence, and Phenotype




Chemotherapy-Related Pleuroparenchymal Fibroeleastosis in a Patient Referred for Lung Transplant




Idiopathic Pleuroparenchymal Fibroelastosis in Adolescent Male C66: Pediatric Case Reports: Diffuse Lung Diseases


Plueuropulmonary Blastoma



A Pleural Effusion Hiding a Congenital Lung Lesion: A Congenital Lung Lesion Hiding a Rare Malignancy



31

Rare Disease Term


Post-Transplant Lymphoproliferative Disorder



A Unique Case of Gradual Visual Loss Post Lung Transplant Secondary to Post Transplant Lymphoproliferative Disease



Primary Ciliary Dyskinesia



Genetic Testing, a Valuable Tool in the Diagnosis of Primary Ciliary Dyskinesia





Increased Oxidative Stress in Nasal Epithelial Cells of Primary Ciliary Dyskinesia Patients




Children with Primary Ciliary Dyskinesia and Situs Ambiguus Have Worse Clinical Outcomes




Pulmonary Radio-Aerosol Mucociliary Scan to Evaluate Primary Ciliary Dyskinesia in Children with Bronchiectasis





A 13-Year-Old Boy with Primary Ciliary Dyskinesia and Stubbornly Persistent Worsening Lung Function




Comparison Between Lung Function in Children with Cystic Fibrosis and Primary Ciliary Dyskinesia

C67: Suppurative Lung Diseases in Children



Cytoplasmic Ciliary Inclusions in Isolation Are Not Sufficient for the Diagnosis of Primary Ciliary Dyskinesia in Children

C67: Suppurative Lung Diseases in Children



Motile Cilia Preassembly Occurs in a Biphasic Pattern C77: Advancements in the Biology of BPD and Other Congenital Pediatric Lung Diseases



A Physiological Rationale for Epithelial Sodium Channel Inhibition and Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Therapy (Ivacaftor) in Primary Ciliary Dyskinesia

C79: Advancing Understanding of Obstructive Lung Disease: Medications and Mechanisms of Delivery

Area L, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Pulmonary Alveolar Microlithiasis



Pulmonary Alveolar Microlithiasis Caused By Two Homozygous Mutations



32

Rare Disease Term




A Caucasian Woman with PAM Due to a Rare Mutation B42: Interstitial Lung Disease: A Potpourri of Cases



Inhibition of miR-21 Suppresses miR-223 in Estrogen-Treated Experimental Pulmonary Hypertension

B80-E: From Bench to Bedside: The Right Ventricle, Pulmonary Vessels, and Their Disorders




Preclinical Studies Reveal Low Phosphate Diet as a Potential Therapy for Pulmonary Alveolar Microlithiasis



Pulmonary Alveolar Proteinosis



Anti-GMCSF Monoclonal Gammopathy-Induced Pulmonary Alveolar Proteinosis – Individualized Medicine Using Mass Spectrometry




Unusual Co-Occurrence of Polymyositis-Interstitial Lung Disease and Pulmonary Alveolar Proteinosis




The Price of the Perfect Smile: A Dental Ceramist’s Journey from Respiratory Collapse and Back




Peripheral Alveolar Nitric Oxide Concentration Reflects Macrophage Inflammation of Auto-Immune Pulmonary Alveolar Proteinosis



Characteristics of Iranian Patients with Pulmonary Alveolar Proteinosis (PAP): A Single Center 11 Year Retrospective Study



Utility of Therapeutic Lobar Lung Lavage in Pulmonary Alveolar Proteinosis – an Indian Experience

A45: Interventional Pulmonary: Clinical Studies



Pulmonary Alveolar Proteinosis Complicated by Blastomycosis A63: Case Reports: Fungal Area D, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



Development and Validation of Csf2ra Gene-Deficient Mice as a Clinically Relevant Model of Children with Hereditary Pulmonary Alveolar Proteinosis




Pulmonary Macrophage Transplantation Therapy in Csf2ra Gene-Ablated Mice: A Novel Model of Hereditary Pulmonary Alveolar Proteinosis in Children




A Rare Case of Secondary Pulmonary Alveolar Proteinaceous Disease in a Patient with Solid Organ Malignancy

B40: Sarcoidosis: Illustrative Cases and Clinical Series


33

Rare Disease Term




Alveolar Proteinosis: A Case Report B42: Interstitial Lung Disease: A Potpourri of Cases



Spontaneus Improvement of Pulmonary Alveolar Proteinosis in a High Altitude Resident After Descent to Sea Level




Acute on Chronic Respiratory Failure as a Result of Pulmonary Alveolar Proteinosis




Pulmonary Alveolar Proteinosis Diagnosed Via Cryobiopsy: A Case Report




Pulmonary Alveolar Proteinosis: A Rarely Suspected Etiology of Respiratory Distress in an Adolescent Patient with Hematologic Malignancy




Change in Distribution of Pulmonary Blood During Whole Lung Lavage in Patients with Autoimmune Pulmonary Alveolar Proteinosis: A Simulation-Based Study




ABCG1 Plays an Important Role in Surfactant Metabolism in Pulmonary Alveolar Proteinosis

B79: Role of Immune Cells in Acute Lung Injury




Molecular Alterations Across Multiple Tissues in Pulmonary Alveolar Proteinosis Patients Share Commonalities with Immune Related Lung and Autoimmune Disorders

C33: Novel Biomarkers in Lung Disease Area N, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Disease Activity in Autoimmune Pulmonary Alveolar Proteinosis Analyzed by a Multiplex Immunoassay System

C39: Biomarkers and Prognostic Factors in ILD



Serial Lobar Lavage as an Alternative to Whole Lung Lavage for the Treatment of Symptomatic Pulmonary Alveolar Proteinosis



A Diamond in the Rough: Pulmonary Alveolar Proteinosis in Myelodysplastic Syndrome



Pulmonary Alveolar Proteinosis: Not a Common Cause of Dyspnea C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Pulmonary Alveolar Proteinosis Presenting as Dyspnea and Thrush C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Pulmonary Alveolar Proteinosis (PAP) - a Rare Case of Adult Onset Congenital PAP


34

Rare Disease Term




Pulmonary Alveolar Proteinosis and Disseminated Mycobacteriosis: A Terminal Complication of Myelodysplastic Syndrome



Diffuse Tree-in-Bud Opacities Due to Pulmonary Alveolar Proteinosis Secondary to GATA2 Deficiency



Rubinstein-Taybi Syndrome and Unusual Lung Biopsy Finding of Pulmonary Alveolar Proteinosis and Severe Pulmonary Injury




A Pediatric Case of Refractory Autoimmune Pulmonary Alveolar Proteinosis




Validating a Novel Dried Blood Spot Card Test for the Diagnosis of Autoimmune Pulmonary Alveolar Proteinosis (Autoimmune PAP) Within the National PAP Registry




Chronic Inhalation of Recombinant Human Granulocyte/Macrophage Colony Stimulating Factor (GM-CSF) and GM-CSF Antibody in Nonhuman Primates



Pulmonary Amyloidosis



Serial Changes in Pulmonary Function Testing in a Patient with Tracheobronchial Amyloidosis: Relevance for Early Diagnosis




A Case of Pulmonary Amyloidosis B42: Interstitial Lung Disease: A Potpourri of Cases




Pulmonary Amyloid: A Rare Cause of Dyspnea and Hemoptysis C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Dyspnea, Hypoxia, Chest Pain, and Sub-Pleural Cavitary Lesions C80-I: Thoracic Oncology Case Reports III


Pulmonary Capillary Hemangiomatosis



The Inception Unraveled: The Rare Within the Rare C70: Call My Name: Case Reports in Pulmonary Vascular Disease



A Rare Cause and Complex Presentation of Pulmonary Hypertension: Pulmonary Capillary Hemangiomatosis



35

Rare Disease Term




Late Presentation of Pulmonary Capillary Hemangiomatosis and Successful Treatment with Doxycycline



Pulmonary Interstitial Glycogenosis



Persistent Pulmonary Hypertension as a Result of Pulmonary Interstitial Glycogenosis



Pulmonary Malinosculation



Could There Be an Autosomal Dominant Syndrome of Pulmonary Malinosculation



Pulmonary Veno Occlusive Disease



Pulmonary Vein Thrombosis Due to IVIG Use in Guillain-Barre Syndrome




The PE that Never Was: A Rare Case of Combined PVOD and Pulmonary Vein Thrombosis





Pulmonary Veno-Occlusive Disease Masquerading as Chronic Thrombo Embolic Pulmonary Hypertension





Patterns of Pulmonary Vascular Remodeling and Expression of Serine-Threonine Kinase GCN2 in Pulmonary Veno-Occlusive Disease: A Comparative Analysis

D107: Diamonds and Pearls: Pulmonary Hypertension Phenotypes


9:15-11:15 am Oral Presentation 9:15-9:30 am

Clinical Phenotypes and Outcomes of Heritable and Sporadic Pulmonary Veno-Occlusive Disease

D17: Sign “O” The Times: Diagnosis and Risk Stratification of Pulmonary Hypertension

Liberty Ballroom I-L (Level M4), Marriott Marquis Washington

Rare Genetic Abnormalities



MECP2 Duplication Syndrome: A Rare Cause of Immune Dysfunction and Recurrent Lower Respiratory Tract Infections



Rare Pleural Disease



Pleural Fibromatosis and Hydroxyurea Therapy C46: Pleural Disease: Case Reports II Area M, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

36

Rare Disease Term




An Unusual Case of Bilateral Pleural Effusion B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Urinothorax: A Case of a Right-Sided Pleural Effusion with a Contralateral Left-Sided Ureteral Stricture



An Uncanny Connection! Bilothorax B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


A Novel Effusion: A Case of Thoracolithiasis Associated Recurrent Pleural Effusion



Demons-Meigs Syndrome: A Rare Cause of Pleural Effusion B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Uremic Pleuritis: An Unusual Case of Recurrent Pleural Effusions in a Renal Transplant Adolescent





Chest Pain and Dyspnea Secondary to a Rare Complication of Pancreatitis



A Leaky Situation: Diagnosing Pancreaticopleural Fistulas C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



Pancreaticopleural Fistula – A Rare Complication of Pancreatitis C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Rare but Real: Pleural Effusion Associated with Bosutinib Therapy for Chronic Myeloid Leukemia (CML)



Rare Pulmonary Tumors: Hamartoma



Giant Pulmonary Hamartoma: Serial Imaging of a Rapidly Growing Central Pulmonary Mass



Rare Tumors: Embolic



Microscopic Tumor Emboli: A Rare Case of Hypoxemia C80-G: Thoracic Oncology Case Reports I


Rare Tumors: Airway



Primary Tracheal Malignancies: A SEER Database Analysis B80-L: General Thoracic Oncology Area N, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

37

Rare Disease Term


Rare Tumors: Chondrosarcoma



Pulmonary Gestational Choriocarcinoma: A Rare Lung Cancer Doppelganger




The First Case of Primary Pulmonary Choriocarcinoma Reported from East Africa



Rare Tumors: Dendritic



Interdigitating Dendritic Cell Sarcoma Presenting as Solitary Pulmonary Nodule



Rare Tumors: Germ Cell



Primary Germ Cell Tumor Presenting as Anterior Mediastinal Mass – a Rare Entity!



Rare Tumors: Glomus Cell



Non-Resolving Pneumonia as the Presentation of Pulmonary Glomus Cell Tumor



Rare Tumors: Lymphangitic



Symptomatic Bronchogenic Cyst with Vascular Compression and Actinomycosis Superinfection Treated with Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration (EBUS-FNA)




Rare Case Report of Pulmonary Lymphangitic Spread in Rectal Cancer



Rare Tumors: Mediastinal



Anesthesia Challenges Facing Giant Mediastinal Mass C80-H: Thoracic Oncology Case Reports II


Rare Tumors: Melanoma



Non-Cutaneous Solitary Pleural Based Primary Pulmonary Malignant Melanoma




Primary Pulmonary Melanoma with Rapid Progression C80-I: Thoracic Oncology Case Reports III


38

Rare Disease Term


Rare Tumors: Metastatic Angiosarcoma



Massive Spontaneous Hemothorax Secondary to Metastatic Angiosarcoma


Rare Tumors: Metastatic Basal Cell



Metastatic Basal Cell Cancer to the Lungs C80-G: Thoracic Oncology Case Reports I


Rare Tumors: Metastatic Melanoma



An Unusual Recurrence of Remote Cutaneous Malignant Melanoma as Metastatic Endobronchial Melanoma



Rare Tumors: Metastatic Osteosarcoma



A Rare Case of Metastatic Pulmonary Osteosarcoma in Association with Non-Malignant Pleural and Pericardial Effusion



Rare Tumors: Metastatic Thyroid



Incidental Thyroid Cancer Found Post Pneumonectomy for Lung Adenocarcinoma: A Case Report



Rare Tumors: Midline Carcinoma



A Tough NUT to Crack C80-I: Thoracic Oncology Case Reports III


Rare Tumors: Mucoepidermoid



Mucoepidermoid Carcinoma of the Lung: A Case Report C80-H: Thoracic Oncology Case Reports II



Pulmonary Mucoepidermoid Carcinoma: A Rare Primary Lung Tumor C80-J: Thoracic Oncology Case Reports IV


Rare Tumors: Myofibroblastic



Novel Approach to Inflammatory Myofibroblastic Bronchial Tumor: A Case Report




Inflammatory Myofibroblastic Tumor of the Lung Presenting as an Incidental Pulmonary Nodule in a Wheezy Infant



39

Rare Disease Term




Not All that Wheezes Is Asthma C80-I: Thoracic Oncology Case Reports III



Contralateral Recurrence of Inflammatory Myofibroblastic Tumour of the Lung Ten Years After Pneumonectomy




Is Inflammatory Myo-Fibroblastic Tumor a Cancer or an Infection?: A Case Report and Review of Literature



Rare Tumors: Neuroectodermal



Rare Case of Primary Pleural Primitive Neuroectodermal Tumour in an Adult Presenting as Hemothorax



Rare Tumors: Other



Exercise Capacity Among Survivors of High-Risk Neuroblastoma B69: When Kids Cannot Sleep (or Breathe)




Pulmonary Adenofibroma: A Rare Benign Lung Lesion C80-H: Thoracic Oncology Case Reports II



Needle Tract Recurrence After CT-Guided Lung Biopsy: A Report of a Case in an Adjacent Lobe



Rare Tumors: Plasmacytoma



A Rare Posterior Mediastinal Mass Diagnosed by EBUS C45: EBUS and Airway Interventions: IP Case Reports


Rare Tumors: Pleural



A Rare Cause of Pleural Effusion B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


A Rare Case of Pleural Angiosarcoma Presenting with Massive Hemothorax




A Rare Case of Extraskeletal Mesenchymal Chondrosarcoma of the Pleura

C46: Pleural Disease: Case Reports II Area M, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


A Case of Metastatic Solitary Fibrous Tumor of the Lung C80-G: Thoracic Oncology Case Reports I



A Rare Pleural Pearl C80-H: Thoracic Oncology Case Reports II


40

Rare Disease Term




A Case Report of a Malignant Solitary Fibrous Tumor of the Pleura with Recurrence




Abdominal Pain and Malignant Melanoma Pleural Effusion C80-H: Thoracic Oncology Case Reports II



The Silent Giant: A Rare Case of a Pleural Hemangiopericytoma C80-I: Thoracic Oncology Case Reports III



Pleuro-Pulmonary Angiosarcoma Presenting as Spontaneous Hemothorax and Bilateral Pulmonary Nodules



Rare Tumors: Primary Airway



Not All Coughs Are Due to Upper Airway Cough Syndrome: An Unusual Manisfestation of Endobronchial Hamartoma



Primary Endobronchial Leiomyoma: A Rare Benign Pulmonary Neoplasm



Recurrent Left-Sided Lung Consolidation and Pneumonia B67: Interesting and Unusual Pediatric Case Reports




Endobronchial Leiomyoma in Pregnancy: Successful Therapeutic Bronchoscopic Resection




A Middle-Aged Man Presenting with Synchronous Endobronchial Lesions




A Rare Case of Papillary Endobronchial Squamous Cell Carcinoma C80-G: Thoracic Oncology Case Reports I



Unusual Presentation of Endobronchial Leiomyoma C80-G: Thoracic Oncology Case Reports I



Lung Mass - Is It Really Cancer? Primary Epidermoid Tumor of Lung C80-H: Thoracic Oncology Case Reports II



Mucoepidermoid Carcinoma Presenting as an Endobronchial Lesion C80-H: Thoracic Oncology Case Reports II



Adenoid Cystic Carcinoma: The Uncommonly Common Primary Tracheal Tumor



41

Rare Disease Term




A Rare Case of an Endobronchial Lesion: Adenoid Cystic Carcinoma C80-H: Thoracic Oncology Case Reports II



Granular Cell Tumor Causing Right Middle Lobe Atelectasis and Recurrent Pneumonia




Endobronchial Granular Cell Tumor: A Rare, Can’t-Miss Diagnosis C80-I: Thoracic Oncology Case Reports III



Pulmonary Granular Cell Tumor Presenting with Post-Obstructive Pneumonia




A Case of Benign Solitary Fibrous Tumor Becoming Malignant C80-J: Thoracic Oncology Case Reports IV


Rare Tumors: Sarcoma



Rare and Rarer: Pediatric Post-Obstructive Pneumonia from Primary Pulmonary Ewing Sarcoma




Primary Pulmonary Artery Angiosarcoma, Case Report, ROL and CT, TEE, MRI, PET Combo. Can We Improve Diagnostic Yield, so Improve Survival?





Myeloid Sarcoma Presenting as an Endobronchial Lesion Causing Recurrent Post-Obstructive Pneumonia




Fatal Pulmonary Kaposi Sarcoma in an AIDS Patient C80-H: Thoracic Oncology Case Reports II



Use of EBUS-TBNA in the Diagnosis of Primary Pulmonary Artery Sarcoma



Rare Tumors: Sarcoma, Synovial



Primary Pulmonary Synovial Sarcoma: A Rare Malignancy C80-G: Thoracic Oncology Case Reports I


Rare Tumors: Schwannoma



Primary Endobronchial Schwannoma Successfully Removed Via Rigid Bronchoscopy on Veno-Venous Extracorporeal Membrane Oxygenation (VV-ECMO) Support



42

Rare Disease Term


Rare Tumors: Seminoma



Diagnosis of Mediastinal Recurrence of Seminoma with Endobronchial Ultrasound



Rare Tumors: Teratoma



A Mediastinal Giant Mature Cystic Teratoma Revealed After Pleural Effusion Treated Twice as Tuberculosis



Rare-Tumors: Sarcoma



Pulmonary Leiomyosarcoma: A Rare Occurrence C80-H: Thoracic Oncology Case Reports II


Relapsing Polychondritis



A Case of Relapsing Polychondritis Solely Presenting with Chronic Cough: A Rare Presentation of a Rare Disease





Respiratory Symptoms in Relapsing Polychondritis: Data from an International Survey

C40: Dyspnea: Clinical Studies Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



A Rare Case of Relapsing Polychondritis Presenting with Life Threatening Airway Collapse



18F-FDG PET/CT Plays a Valuable Role in Assessing Almost Cartilages and Detecting Early or Atypical Relapsing Polychondritis

C80-C: Imaging Methodology and Application to Lung Disease

Area N, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Sequestration



Incidental Finding of Bronchopulmonary Sequestration in Elderly Woman


Shrinking Lung



Shrinking Lung Syndrome with Hypercapneic Respiratory Failure Responsive to Mycophenolate Mofetil and Systemic Steroids




Acute Lupus Pneumonitis as a Precursor to Shrinking Lung Syndrome





Giant Bulla with Hilar and Paratracheal Lymphadenopathy in a Young Male Smoker: A Rare Case Report


43

Rare Disease Term


Sickle Cell Lung


9:15-11:15 amOral Presentation 9:30-9:45 am

Absence of Longitudinal Decline in Lung Volume in Children with Sickle Cell Anemia: SAC Multi-Center Prospective Cohort

B18: Pediatric Asthma Ballroom B (South Building, Level 3), Walter E. Washington Convention Center

Sjögren’s



Interstitial Lung Disease in Patients with Primary Sjogren’s Syndrome: Correlation of Radiology, Pathology, and Clinical Phenotype


Splenosis



An Unusual Case of Pulmonary Nodules C46: Pleural Disease: Case Reports II Area M, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

STAT3 Deficiency



Pulmonary Manifestations in Adult Patients with STAT3 Deficiency: A French Nationwide Registry-Based Cohort Study

B103: Clinical Studies in Bronchiectasis, Immunodeficiency, and Drug Induced Lung Disease


Stiff Person Syndrome



Respiratory Manifestations of Stiff Person Syndrome (SPS) C40: Dyspnea: Clinical Studies Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Subglottic Stenosis



Idiopathic Subglottic Stenosis Presenting in Pregnancy C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Surfactant Mutations - Other



The Classification of ATP-Binding Cassette Subfamily a Member 3 (ABCA3) Mutations Using the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Classification System

C77: Advancements in the Biology of BPD and Other Congenital Pediatric Lung Diseases



Heterogeneity of Lung Disease Associated with NK2 Homeobox 1 Mutations



44

Rare Disease Term



P620 9:15-11:15 amPoster Discussion 9:45-11:15 amPoster Viewing 9:15-9:45 am

A Mouse Model of Surfactant Dysregulation and Aberrant Lung Remodeling Induced by Modulation of the Abca3 Gene

D21: Immune Pathways in Acute Lung Injury And Fibrosis


Surfactant Mutations-SP-C



Deriving Alveolar Type 2 Cells from Pluripotent Stem Cells to Model Surfactant Protein C (SP-C) Mutations In Vitro

B76: Drivers of Cell Fate, Senescence, and Phenotype




Spontaneous Alveolitis and Lung Injury Following Induction of Expression of the Surfactant Protein C Mutant SP-CI73T in the Alveolar Epithelium: An Acute Exacerbation of IPF in Mice?

C28: From Targets to Therapies in Fibrosis



Building a Better Mousetrap: Tamoxifen Induced Upregulation of the Surfactant Protein C (SFTPC) Mutant SP-CI73T Produces an Idiopathic Pulmonary Fibrosis Lung Phenotype In Vivo

C28: From Targets to Therapies in Fibrosis


Swyer-James



Pulmonary Hypertension Associated with Swyer-James MacLeod Syndrome: A Case Report



Thoracic Endometriosis



Recurrent Unexplained Pleural Effusion in Young Female? Think Outside the Box



Surgical Outcome After Video Assisted Thoracoscopic Resection of Thoracic Endometriosis and Total Visceral Covering Technique in Patients of Refractory Catamenial Pneumothorax

B45: Pleural Disease: Clinical Studies Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center



A Different Kind of Premenstrual Syndrome C46: Pleural Disease: Case Reports II Area M, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


A Rare Cause of Recurrent Hemoptysis C66: Pediatric Case Reports: Diffuse Lung Diseases


Tracheobronchomalacia



Expiratory Central Airway Collapse and Medical Management: Outcome Analysis

A101: Advances in Cough and Dyspnea Room 206 (South Building, Level 2), Walter E. Washington Convention Center

45

Rare Disease Term




Bacterial Colonization During Stent Trial in Excessive Central Airway Collapse

B61: Bacterial Respiratory Infections Area L, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Tracheobronchopathia Osteochondroplastica



Tracheobronchopathica Osteochondroplastica Presenting with Epistaxis and Atrophic Rhinitis




Experimental Endobronchial Use of Pneumatic Lithotripter in a Symptomatic Case of Tracheobronchopathia Osteochondroplastica (TO)




Tracheobronchopathia Osteochondroplastica: A Consideration for Association with Malignancy Versus Radiation Exposure





Isolated Left Upper Lobe Trachobronchopathia Osteochondroplastica B39: Cough: Rare and Common Area B, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Vascular Anomalies



Primary Racemose Hemangioma of the Right Bronchial Artery with Thrombus and Slow Meandering Blood Flow

A57: Critical Care Case Reports: Cardiovascular Disease II




Down Syndrome and Subclavian Aberrant Artery: A Case Report B67: Interesting and Unusual Pediatric Case Reports



33 Year Old Man with Hemoptysis: A Deeper Look into Unilateral Atresia of Pulmonary Artery





Unilateral Pulmonary Hypoplasia in Adulthood - A Case Report C42: Dyspnea: Case Reports Area G, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center


Not All Endobronchial Lesions Should Be Biopsied: A Case of Massive Hemoptysis and Endobronchial Varices




Cauliflower Lung: A Pediatric Pulmonary Presentation of Cutaneovisceral Angiomatosis with Thrombocytopenia (CAT)




Hemoptysis: A Case Of Bronchial Dieulafoy C70: Call My Name: Case Reports in Pulmonary Vascular Disease



Unilateral Pulmonary Artery Agenesis, An Unusual Cause of Unilateral Acute Respiratory Distress Syndrome (ARDS)



46

Rare Disease Term




Pulmonary Venous Varix: An Uncommon Pulmonary Vascular Abnormality as a Cause of Ischemic Stroke




Bilateral Multiple Sclerosing Hemangioma: A Clinical and Imagistic Diagnostic Challenge



Vasculitis: Idiopathic Pulmonary Capillaritis



Clinical and Pathologic Assessment of Isolated and Immune-Mediated Pulmonary Capillaritis in Children





Remission in Pediatric Patient with Idiopathic Pulmonary Capillaritis Treated with Pulse Steroids, Rituximab and IVIG



Vasculitis: Other



Don’t Judge a Lung by It’s Cover A39: Autoimmune Lung Disease: Case Reports


Vasculitis: Takayasu



Pulmonary Artery Manifestations in Takayasu Arteritis: A Retrospective Study of China



Vasculitis-Behcet’s

Monday, May 22, 201


Chylothorax as the Initial Presentation of Behcet’s Disease B44: Pleural Disease: Case Reports I Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

Vasculitis-Churg Strauss



Assessment of Tissue Eosinophilia by Immunohistochemistry in Video-Assisted Thoracoscopic Surgery (VATS) Biopsies from Severe Asthma Patients with or Without Asthmatic Granulomatosis

B31: Pre-Clinical and Translational Studies in Asthma


Vasculitis-Churg Strauss Syndrome



Mepolizumab for Refractory Asthma in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

C36: Asthma and Allergy: Case Series and Reports


47

Rare Disease Term


Vasculitis-Hypocomplementemic



Hypocomplementemic Urticarial Vasculitis Syndrome: A Rare Potentially Lethal Disease



Vasculitis-Pauci Immune



Isolated Pauci-Immune Pulmonary Capillaritis Presenting as Diffuse Alveolar Hemorrhage




Case of Puci-Immune Pulmonary Capillaritis Treated with Rituximab A39: Autoimmune Lung Disease: Case Reports


Vasculitis-Rheumatoid



A Window into the Soul: A Case of Transmural Ulceration of the Tracheobronchial Tree



Xanthogranulomatous Inflammation



Xanthogranulomatous Inflammation in the Lung C62: Case Reports: Pulmonary Infections – Miscellaneous




Pulmonary Empyema with Thoracic Compression resulting from Direct Invasion of Xanthogranulomatous Pyleonephritis

D25: Unusual Infections: Case Reports Room 144 A-C (Middle Building, Street Level), Walter E. Washington Convention Center

Zika Virus



Hypoxemic Respiratory Failure Secondary to Zika Virus Infection C56: Critical Care Case Reports: ARDS Area O, Hall B-C (Middle Building, Lower Level), Walter E. Washington Convention Center

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