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- Aging; inflammatory response; myocardial proteasome; pulmonary challenge; respiratory-distress-syndrome; berlin definition; sepsis; heart; compartmentalization; dysfunction; pneumonia; outcomes; transmigration; mortality; Respiratory System; Feinstein (1)
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Articles 1 - 30 of 30
Full-Text Articles in Pulmonology
A Rare Cause Of Recurrent Wheeze And Seizures., Maryam Hassan,, Saher Quraeshi, Ali Bin Sarwar Zubairi
A Rare Cause Of Recurrent Wheeze And Seizures., Maryam Hassan,, Saher Quraeshi, Ali Bin Sarwar Zubairi
Department of Medicine
A 34-year-old woman presented with recurrent attacks of breathlessness and seizures. The patient's condition worsened during the course of her hospital stay, as a result of which she had to be intubated twice. Radiological studies showed a mass in the trachea and the subsequent biopsy of the mass revealed an infiltrating carcinoma with morphological features of adenoid cystic carcinoma.
A Descriptive Study Of Pulmonary Function Testing And Chest Computed Tomography In Survivors Of Veno-Venous Extracorporeal Membrane Oxygenation, Matthew Miles Do, Dorothea T. Watson Do, Rita Pechulis Md, Fccp
A Descriptive Study Of Pulmonary Function Testing And Chest Computed Tomography In Survivors Of Veno-Venous Extracorporeal Membrane Oxygenation, Matthew Miles Do, Dorothea T. Watson Do, Rita Pechulis Md, Fccp
Department of Medicine
No abstract provided.
Severe Pneumomediastinum Complicating Ebus-Tbna, Yousef Shweihat, James D. Perry Iii, Nancy Munn
Severe Pneumomediastinum Complicating Ebus-Tbna, Yousef Shweihat, James D. Perry Iii, Nancy Munn
Internal Medicine
Pneumomediastinum infrequently complicates diagnostic bronchoscopy. Increased airway or alveolar pressure results in air leaks to the mediastinum through existing or induced defects. Excessive cough, recurrent episodes of increased abdominal pressure, vomiting, or sneezing can all induce spontaneous pneumomediastinum. Less commonly it has been documented with lung or neck infections, esophageal or tracheal tears, and rapid increases in altitude such as during plane flights or scuba diving, with mechanical ventilation, substance abuse, and after bronchoscopy. Pneumomediastinum may be, but is not always, associated with pneumothorax.
Ten-Sational!, Children's Mercy Hospital
Ten-Sational!, Children's Mercy Hospital
Our Story Continues
Children’s Mercy lands top ranking in all 10 specialties from U.S. News and World Report
Idiopathic Pulmonary Hemosiderosis: A Rare Cause Of Hemoptysis In Adults, Michael Kalil Do, B Miller Do, Ryan Rogers Do, Vanessa Ribaudo Kaufman Md
Idiopathic Pulmonary Hemosiderosis: A Rare Cause Of Hemoptysis In Adults, Michael Kalil Do, B Miller Do, Ryan Rogers Do, Vanessa Ribaudo Kaufman Md
Department of Medicine
No abstract provided.
Acute Superior Vena Cava Syndrome In The Setting Of An Intravascular Device, Rachel Kinney Do, Matthew Miles Do, Shannon Davis Do, Jennifer Rovella Do
Acute Superior Vena Cava Syndrome In The Setting Of An Intravascular Device, Rachel Kinney Do, Matthew Miles Do, Shannon Davis Do, Jennifer Rovella Do
Department of Medicine
No abstract provided.
Smokeless Tobacco Use Among Pakistani Women: A Missed Opportunity For Tobacco Control, Tanzil Jamali, Sana Tanzil
Smokeless Tobacco Use Among Pakistani Women: A Missed Opportunity For Tobacco Control, Tanzil Jamali, Sana Tanzil
Community Health Sciences
No abstract provided.
Sarcoidosis Presented As Retroperitoneal And Lung Mass, Nousheen Iqbal, Aamer Mahmood, Muhammad Irfan, Khurram Minhas
Sarcoidosis Presented As Retroperitoneal And Lung Mass, Nousheen Iqbal, Aamer Mahmood, Muhammad Irfan, Khurram Minhas
Section of Pulmonary & Critical Care
Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology characterised pathologically by the presence of non-caseating granulomas in involved organs. Sarcoidosis most frequently involves the lungs followed by eye and skin. Presentation as retroperitoneal and lung mass is rare in sarcoidosis. We describe an unusual case of sarcoidosis where the patient presented with large retroperitoneal and lung masses, and was treated as tuberculosis
Re-Recurrent Dermatofibrosarcomaprotuberans Of The Chest Wall., Niaz Hussain, Mujtaba Zaki Naveed, Ghulam H
Re-Recurrent Dermatofibrosarcomaprotuberans Of The Chest Wall., Niaz Hussain, Mujtaba Zaki Naveed, Ghulam H
Section of Pulmonary & Critical Care
Dermatofibrosarcomaprotuberans is a rare, soft tissue tumour with high rate of recurrence. It is locally aggressive, with a low rate of metastasis. We describe the case of a 42 year old man who presented with a re-recurrent, large tumour situated on the anterior chest wall in the sternal region. We did a wide local excision and covered the resulting defect by using bilateral, pectoralis major myocutaneous flaps. Histopathology and immunohistochemical staining findings were consistent with the diagnosis of Dermatofibrosarcoma Protuberance. Post operatively the patient was treated with chemotherapy and radiotherapy.
Pulmonary Rehabilitation And Exercise In Pulmonary Arterial Hypertension: An Underutilized Intervention, Sonu Sahni, Barbara Capozzi, Asma Iftikhar, Vasiliki Sgouras, Marcin Ojrzanowski, Arunabh Talwar
Pulmonary Rehabilitation And Exercise In Pulmonary Arterial Hypertension: An Underutilized Intervention, Sonu Sahni, Barbara Capozzi, Asma Iftikhar, Vasiliki Sgouras, Marcin Ojrzanowski, Arunabh Talwar
Touro College of Osteopathic Medicine (New York) Publications and Research
Pulmonary arterial hypertension (PAH) is a rare and devastating disease characterized by progressive increases in pulmonary arterial pressure and pulmonary vascular resistance which eventually leads to right ventricular failure and death. Early thought process was that exercise and increased physical activity may be detrimental to PAH patients however many small cohort trials have proven otherwise. In addition to the many pharmaceutical options, exercise and pulmonary rehabilitation have also been shown to increase exercise capacity as well as various aspects of psychosomatic health. As pulmonary and exercise rehabilitation become more widely used as an adjuvant therapy patient outcomes improve and physicians …
Age Influences Inflammatory Responses, Hemodynamics, And Cardiac Proteasome Activation During Acute Lung Injury, H. M. Linge, J. Y. Lee, K. Ochani, K. Koga, N. Kohn, K. Ojamaa, S. R. Powell, E. J. Miller
Age Influences Inflammatory Responses, Hemodynamics, And Cardiac Proteasome Activation During Acute Lung Injury, H. M. Linge, J. Y. Lee, K. Ochani, K. Koga, N. Kohn, K. Ojamaa, S. R. Powell, E. J. Miller
Journal Articles
Background: Acute lung injury (ALI) is a significant source of morbidity and mortality in critically ill patients. Age is a major determinant of clinical outcome in ALI. The increased ALI-associated mortality in the older population suggests that there are age-dependent alterations in the responses to pulmonary challenge. The objective of this observational study was to evaluate age-dependent differences in the acute (within 6 hours) immunological and physiological responses of the heart and lung, to pulmonary challenge, that could result in increased severity. Methods: Male C57Bl/6 mice (young: 2-3 months, old: 18-20 months) were challenged intratracheally with cell wall components from …
Resident Endothelial Cells And Endothelial Progenitor Cells Restore Endothelial Barrier Function After Inflammatory Lung Injury, S. Z. Mao, X. B. Ye, G. Liu, D. M. Song, S. F. Liu
Resident Endothelial Cells And Endothelial Progenitor Cells Restore Endothelial Barrier Function After Inflammatory Lung Injury, S. Z. Mao, X. B. Ye, G. Liu, D. M. Song, S. F. Liu
Journal Articles
Objective Disruption of endothelial barrier integrity is a characteristic of many inflammatory conditions. However, the origin and function of endothelial cells (ECs) restoring endothelial barrier function remain unknown. This study defined the roles of resident ECs (RECs) and bone marrow-derived endothelial progenitor cells (BMDEPCs) in endothelial barrier restoration after endotoxemic lung injury. Approach and Results We generated mice that enable to quantify proliferating RECs or BMDEPCs and also to study the causal link between REC or BMDEPC proliferation and endothelial barrier restoration. Using these mouse models, we showed that endothelial barrier restoration was associated with increased REC and BMDEPC proliferation. …
Respiratory Failure With Hilar Mass: Role Of Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration In The Medical Intensive Care Unit, A. Chichra, K. W. Lama, S. J. Koenig
Respiratory Failure With Hilar Mass: Role Of Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration In The Medical Intensive Care Unit, A. Chichra, K. W. Lama, S. J. Koenig
Journal Articles
We report the case of a 58-year-old man on chronic steroid therapy, who developed a rapidly progressive right upper lobe infiltrate/mass that extended into the right hilum. Respiratory failure necessitated endotracheal intubation. Broad spectrum antibiotics were initiated without clinical improvement and because of his immunosuppressive therapy opportunistic pathogens were considered. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) was performed in the Medical Intensive Care Unit (MICU) with rapid onsite evaluation. Specimens obtained from the right hilar mass revealed organisms suspicious for cryptococcal infection, subsequently confirmed via a culture. No complications occurred during the EBUS procedure despite the patient requiring vasopressor support …
"Saw-Tooth Sign" In Upper Airway Disorders - A Case Report, S. Sahni, A. Blood, S. Paulus, A. Talwar
"Saw-Tooth Sign" In Upper Airway Disorders - A Case Report, S. Sahni, A. Blood, S. Paulus, A. Talwar
Journal Articles
Spirometry flow-volume loop measurement is the screening test of choice to rule out obstructive lung diseases. Flow oscillations occasionally seen on flow volume loops, referred to as a "saw-tooth" sign, are thought to be due to an upper airway obstructive processes associated with upper airway collapsibility. Widely described in obstructive sleep apnea syndrome, flow oscillations have also been linked to many other upper airway pathologies. The mechanism by which flow oscillations occur is centered on the inspiratory and expiratory flow of air. It has been theorized that the mechanism of flow oscillations result from rapid intermittent changes in driving pressure …
Lps Down-Regulates Specificity Protein 1 Activity By Activating Nf-Kappab Pathway In Endotoxemic Mice, X. Ye, H. Liu, Y. S. Gong, S. F. Liu
Lps Down-Regulates Specificity Protein 1 Activity By Activating Nf-Kappab Pathway In Endotoxemic Mice, X. Ye, H. Liu, Y. S. Gong, S. F. Liu
Journal Articles
BACKGROUND: Specificity protein (Sp) 1 mediates the transcription of a large number of constitutive genes encoding physiological mediators. NF-kappaB mediates the expression of hundreds of inducible genes encoding pathological mediators. Crosstalk between Sp1 and NF-kappaB pathways could be pathophysiologically significant, but has not been studied. This study examined the crosstalk between the two pathways and defined the role of NF-kappaB signaling in LPS-induced down-regulation of Sp1 activity. METHODS AND MAIN FINDINGS: Challenge of wild type mice with samonelia enteritidis LPS (10 mg/kg, i.p.) down-regulated Sp1 binding activity in lungs in a time-dependent manner, which was concomitantly associated with an increased …
Non-Small-Cell Lung Cancer Clinicopathologic Features And Survival Outcomes In Asian Pacific Islanders Residing In The United States: A Seer Analysis, M. S. Hamid, R. Shameem, K. Gafoor, J. George, B. Mina, K. Sullivan
Non-Small-Cell Lung Cancer Clinicopathologic Features And Survival Outcomes In Asian Pacific Islanders Residing In The United States: A Seer Analysis, M. S. Hamid, R. Shameem, K. Gafoor, J. George, B. Mina, K. Sullivan
Journal Articles
Background. The objective of our study was to ascertain racial/ethnic disparities in Asian/Pacific Islanders (API) for non-small-cell lung cancer (NSCLC) clinicopathologic features and survival outcomes based on various tumor characteristics and treatment modalities. Method. SEER database identified invasive NSCLC cases from 2004 to 2010. Variables included American Joint Committee on Cancer (AJCC) stage 7, tumor grade, tumor size, histology, age, marital status, radiation, surgery, and reason for no surgery. The Kruskall-Wallis test and the Z test were used to examine differences between races/ethnicities and the referent, non-Hispanic white (NHW). Multivariate Cox proportional analyses were used to establish the weight of …
Pulmonary Rehabilitation And Exercise In Pulmonary Arterial Hypertension: An Underutilized Intervention, S. Sahni, B. Capozzi, A. Iftikhar, V. Sgouras, M. Ojrzanowski, A. Talwar
Pulmonary Rehabilitation And Exercise In Pulmonary Arterial Hypertension: An Underutilized Intervention, S. Sahni, B. Capozzi, A. Iftikhar, V. Sgouras, M. Ojrzanowski, A. Talwar
Journal Articles
Pulmonary arterial hypertension (PAH) is a rare and devastating disease characterized by progressive increases in pulmonary arterial pressure and pulmonary vascular resistance which eventually leads to right ventricular failure and death. Early thought process was that exercise and increased physical activity may be detrimental to PAH patients however many small cohort trials have proven otherwise. In addition to the many pharmaceutical options, exercise and pulmonary rehabilitation have also been shown to increase exercise capacity as well as various aspects of psychosomatic health. As pulmonary and exercise rehabilitation become more widely used as an adjuvant therapy patient outcomes improve and physicians …
Dyspnea, Depression And Health Related Quality Of Life In Pulmonary Arterial Hypertension Patients, A. Talwar, S. Sahni, E. J. Kim, S. Verma, N. Kohn
Dyspnea, Depression And Health Related Quality Of Life In Pulmonary Arterial Hypertension Patients, A. Talwar, S. Sahni, E. J. Kim, S. Verma, N. Kohn
Journal Articles
No abstract provided.
A Case Of Delayed Bacteremia From An Unusual Pseudomonas Species, A. Palkar, A. Budhatoki, P. Singh
A Case Of Delayed Bacteremia From An Unusual Pseudomonas Species, A. Palkar, A. Budhatoki, P. Singh
Journal Articles
No abstract provided.
Case Report: Pulmonary Kaposi Sarcoma In A Non-Hiv Patient, A. Kodra, M. Walczyszyn, C. Grossman, D. Zapata, T. Rambhatla, B. Mina
Case Report: Pulmonary Kaposi Sarcoma In A Non-Hiv Patient, A. Kodra, M. Walczyszyn, C. Grossman, D. Zapata, T. Rambhatla, B. Mina
Journal Articles
Kaposi Sarcoma (KS) is an angioproliferative tumor associated with human herpes virus 8 (HHV-8). Often known as one of the acquired immunodeficiency syndrome (AIDS)-defining skin diseases, pulmonary involvement in KS has only been discussed in a handful of case reports, rarely in a non-HIV patient. Herein we report the case of a 77 year-old- male who presented with a 6-week history of progressive dyspnea on exertion accompanied by productive cough of yellow sputum and intermittent hemoptysis. His past medical history was significant for Non-Hodgkin's Follicular B-Cell Lymphoma (NHL). Patient also had biopsy-confirmed cutaneous KS. His physical exam was notable for …
Clinical Relevance Of The Incidentaloma: A Clinician's Viewpoint, S. Sahni, S. Verma, R.S. Thomas, B. Capozzi, A. Talwar
Clinical Relevance Of The Incidentaloma: A Clinician's Viewpoint, S. Sahni, S. Verma, R.S. Thomas, B. Capozzi, A. Talwar
Journal Articles
No abstract provided.
Comparison Of Cytologic Accuracy Of Endobronchial Ultrasound Transbronchial Needle Aspiration Using Needle Suction Versus No Suction, K. Harris, R. Maroun, K. Attwood, M. Chalhoub
Comparison Of Cytologic Accuracy Of Endobronchial Ultrasound Transbronchial Needle Aspiration Using Needle Suction Versus No Suction, K. Harris, R. Maroun, K. Attwood, M. Chalhoub
Journal Articles
BACKGROUND AND OBJECTIVES: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a relatively new procedure initially used for lung cancer diagnosis, staging and re-staging and extended to benign diseases such as sarcoidosis and other mediastinal lesions. Previously, multiple studies evaluated the use of needle biopsy with no aspiration that did not change the diagnostic accuracy compared with needle biopsy aspiration. MATERIALS AND METHODS: All adult subjects who were scheduled to undergo EBUS-TBNA to sample mediastinal lesions were eligible. We evaluated two methods of sampling mediastinal lesions. The first method was the application of negative pressure syringe for needle suction aspiration. The …
Ultrasound In The Diagnosis And Management Of Pleural Effusions, N. J. Soni, R. Franco, M. I. Velez, D. Schnobrich, R. Dancel, M. I. Restrepo, P. H. Mayo
Ultrasound In The Diagnosis And Management Of Pleural Effusions, N. J. Soni, R. Franco, M. I. Velez, D. Schnobrich, R. Dancel, M. I. Restrepo, P. H. Mayo
Journal Articles
We review the literature on the use of point-of-care ultrasound to evaluate and manage pleural effusions. Point-of-care ultrasound is more sensitive than physical exam and chest radiography to detect pleural effusions, and avoids many negative aspects of computerized tomography. Additionally, point-of-care ultrasound can assess pleural fluid volume and character, revealing possible underlying pathologies and guiding management. Thoracentesis performed with ultrasound guidance has lower risk of pneumothorax and bleeding complications. Future research should focus on the clinical effectiveness of point-of-care ultrasound in the routine management of pleural effusions and how new technologies may expand its clinical utility. Journal of Hospital Medicine …
Obstructive Sleep Apnea As A Risk Factor For Type 2 Diabetes Mellitus, P. Rajan, H. Greenberg
Obstructive Sleep Apnea As A Risk Factor For Type 2 Diabetes Mellitus, P. Rajan, H. Greenberg
Journal Articles
No abstract provided.
Dyspnea Associated With Silicone Breast Implant Rupture, Katherine Rider, Hatice Kaya, Guillermo Gutierrez
Dyspnea Associated With Silicone Breast Implant Rupture, Katherine Rider, Hatice Kaya, Guillermo Gutierrez
Medicine Faculty Publications
We present a case of a 48-year-old woman with a significant history of atopy. She presented with a 1-month history of dyspnea on exertion. Pulmonary function testing was normal, with no obstruction or reversibility post-bronchodilation. Bilateral breast implant rupture was detected on further investigation for a left upper lobe nodule, and the patient underwent bilateral implant removal. There was an improvement in her respiratory symptoms. Systemic symptoms, labeled as “Autoimmune/Inflammatory Syndrome Induced by Adjuvants,” are known to be associated with breast implants, with 14% of women in a cohort developing autoimmune disease secondary to their implants. An improvement in symptoms …
Prevalence Of Tobacco Use Among Women: A Cross Sectional Survey From A Squatter Settlement Of Karachi, Pakistan, Nousheen Iqbal, Muhammad Irfan, Nida Ashraf, Safia Awan, Javaid A. Khan
Prevalence Of Tobacco Use Among Women: A Cross Sectional Survey From A Squatter Settlement Of Karachi, Pakistan, Nousheen Iqbal, Muhammad Irfan, Nida Ashraf, Safia Awan, Javaid A. Khan
Section of Pulmonary & Critical Care
Background: While the prevalence of tobacco use has been slowly declining in the developed countries, rates have been steadily rising in the developing countries. This has led to a rapid rise in tobacco related lung diseases among women.
Objective: Determine the prevalence of tobacco use (both smoking and smokeless) among women in an urban squatter settlement (Orangi Town) in Karachi, Pakistan.
Methods: A cross-sectional survey was conducted on 19,325 females aged between 15 and 80 years in Orangi Town, an urban squatter settlement in Karachi, Pakistan. Modified questionnaire, developed by World Health Organization WHO and Global Adult …
Relaxation Techniques For People With Chronic Obstructive Pulmonary Disease: A Systematic Review And A Meta-Analysis, Eleonora Volpato, Paolo Banfi, Sheena M. Rogers, Francesco Pagnini
Relaxation Techniques For People With Chronic Obstructive Pulmonary Disease: A Systematic Review And A Meta-Analysis, Eleonora Volpato, Paolo Banfi, Sheena M. Rogers, Francesco Pagnini
Dartmouth Scholarship
Chronic Obstructive Pulmonary Disease (COPD) people suffer from severe physical impairments, which often elicit significant psychological distress and impact their quality of life. This meta-analysis aimed to assess evidence from the scientific literature on the effects of relaxation techniques. Methods. We investigated 9 databases to select 25 RCTs. Studies included both inpatients and outpatients with COPD. Both respiratory and psychological outcomes were considered.
The Role Of The Complement System In Cigarette Smoke Induced Emphysema Development And Progression, Sarah E. Stephenson
The Role Of The Complement System In Cigarette Smoke Induced Emphysema Development And Progression, Sarah E. Stephenson
MUSC Theses and Dissertations
Emphysema is a complex inflammatory condition of the respiratory tract with an incompletely elucidated pathogenesis. Due to the complexity of the disease, treatment options are limited for patients and have poor efficacy. Therefore a greater understanding of disease pathogenesis is imperative to expand therapeutic strategies. Central to disease development is the activation of the innate and adaptive immune system, however, targeting blockade of only one side of the immune response, has shown little clinical benefit. The complement system, while regarded as an innate immune mechanism, can modulate both innate and adaptive immune responses. Given these dual modulatory effects, the complement …
Allergen Immunotherapy For Allergic Asthma: Protocol For A Systematic Review., Sangeeta Dhami, Ulugbek Nurmatov, Ioana Agache, Susanne Lau, Antonella Muraro, Marek Jutel, Graham Roberts, Cezmi Akdis, Matteo Bonini, Moises Calderon, Thomas Casale, Ozlem Cavkaytar, Linda Cox, Pascal Demoly, Breda Flood, Eckard Hamelmann, Kenji Izuhara, Ömer Kalayci, Jörg Kleine-Tebbe, Antonio Nieto, Nikolaos Papadopoulos, Oliver Pfaar, Lanny Rosenwasser, Dermot Ryan, Carsten Schmidt-Weber, Stan Szefler, Ulrich Wahn, Roy-Gerth Van Wijk, Jamie Wilkinson, Aziz Sheikh
Allergen Immunotherapy For Allergic Asthma: Protocol For A Systematic Review., Sangeeta Dhami, Ulugbek Nurmatov, Ioana Agache, Susanne Lau, Antonella Muraro, Marek Jutel, Graham Roberts, Cezmi Akdis, Matteo Bonini, Moises Calderon, Thomas Casale, Ozlem Cavkaytar, Linda Cox, Pascal Demoly, Breda Flood, Eckard Hamelmann, Kenji Izuhara, Ömer Kalayci, Jörg Kleine-Tebbe, Antonio Nieto, Nikolaos Papadopoulos, Oliver Pfaar, Lanny Rosenwasser, Dermot Ryan, Carsten Schmidt-Weber, Stan Szefler, Ulrich Wahn, Roy-Gerth Van Wijk, Jamie Wilkinson, Aziz Sheikh
Manuscripts, Articles, Book Chapters and Other Papers
BACKGROUND: The European Academy of Allergy and Clinical Immunology (EAACI) is in the process of developing the EAACI Guidelines for Allergen Immunotherapy (AIT) for Allergic Asthma. We seek to critically assess the effectiveness, cost-effectiveness and safety of AIT in the management of allergic asthma.
METHODS: We will undertake a systematic review, which will involve searching international biomedical databases for published, in progress and unpublished evidence. Studies will be independently screened against pre-defined eligibility criteria and critically appraised using established instruments. Data will be descriptively and, if possible and appropriate, quantitatively synthesised.
DISCUSSION: The findings from this review will be used …
Perceptions Of Social Isolation And Social Support In Alpha-1 Antitrypsin Deficiency And Sarcoidosis: Results Of A Mixed-Methods Study, Susan Krause Flavin
Perceptions Of Social Isolation And Social Support In Alpha-1 Antitrypsin Deficiency And Sarcoidosis: Results Of A Mixed-Methods Study, Susan Krause Flavin
MUSC Theses and Dissertations
Purpose: The purpose of this dissertation was to explore perceptions of social isolation and social support in individuals with one of two rare lung conditions, alpha-1 antitrypsin deficiency (AATD) or sarcoidosis. First, a dimensional concept analysis was conducted to identify key factors contributing to the phenomenon of social isolation. Next, an integrative review of existing instruments to measure social isolation was conducted to identify the most appropriate instrument for the study. Finally, a mixed-methods study was conducted to explore these phenomena in the two populations of interest. Problem: Many rare diseases are chronic, complex and associated with other disabilities (M. …