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Cystic Fibrosis: An autosomal recessive genetic disease of the Exocrine glands. It is caused by mutations in the gene encoding the Cystic fibrosis transmembrane conductance regulator expressed in several organs including the Lung, the Pancreas, the Biliary system, and the Sweat glands. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in Airway obstruction; chronic Respiratory infections; Pancreatic insufficiency; maldigestion; salt depletion; and Heat prostration.

Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients

1Foundation Hubrecht Organoid Technology, 2Department of Pediatric Pulmonology, Regenerative Medicine Centre Utrecht, Wilhelmina Children's Hospital, University Medical Centre Utrecht, 3Department of Stem Cells and Cancer, Walter and Eliza Hall Institute of Medical Research, 4Hubrecht Institute for Developmental Biology and Stem Cell Research, University Medical Centre Utrecht

JoVE 55159


 Medicine

Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD)

1Department of Medicine and the Gregory Fleming James Cystic Fibrosis Center, University of Alabama at Birmingham, 2Department of Pulmonology and Tuberculosis, University Medical Center Utrecht, 3Center for Experimental Medicine, Queens University, Northern Ireland, 4Hadassah Hebrew University Medical Center, Jerusalem, 5Centro Fibrosi Cistica, Azienda Ospedaliera Universitaria Integrata Verona, Italy, 6Service de Pneumologie et Allergologie Pédiatriques and Center de Ressources et de Compétence de la Mucoviscidose, Hôpital Necker Enfants Malades, 7INSERM U 1151, Institut Necker Enfants Malades

Video Coming Soon

JoVE 57006


 JoVE In-Press

Replication of the Ordered, Nonredundant Library of Pseudomonas aeruginosa strain PA14 Transposon Insertion Mutants

1Department of Pediatrics, Mucosal Immunology and Biology Research Center, Massachusetts General Hospital, 2Department of Molecular Biology, Massachusetts General Hospital, 3Department of Genetics, Harvard Medical School, 4Department of Pediatrics, Harvard Medical School

JoVE 57298


 Immunology and Infection

An IL-8 Transiently Transgenized Mouse Model for the In Vivo Long-term Monitoring of Inflammatory Responses

1Department of Medicine, General Pathology Division, Cystic Fibrosis Translational Research Laboratory "D. Lissandrini", University of Verona, 2Corporate Preclinical R&D, Chiesi Farmaceutici S.p.A., 3Department of Diagnostic and Public Health, Microbiology Division, University of Verona, 4Dipartimento di Scienze Medico-Veterinarie, University of Parma, 5Department of Biomedical Biotechnological and Translational Sciences, University of Parma, 6Department of Computer Science, University of Verona, 7Department of Neurological, Biomedical and Movement Sciences, University of Verona, 8Cystic Fibrosis Regional Center (CFC), AOUI Verona

JoVE 55499


 Immunology and Infection

Deciphering and Imaging Pathogenesis and Cording of Mycobacterium abscessus in Zebrafish Embryos

1Dynamique des Interactions Membranaires Normales et Pathologiques, CNRS, UMR 535, Université Montpellier, 2Centre d'études d'agents Pathogènes et Biotechnologies pour la Santé, CNRS, FRE 3689, Université Montpellier, 3Unité de Formation et de Recherche des Sciences de la Santé, EA3647-EPIM, Université Versailles St Quentin

JoVE 53130


 Immunology and Infection

A Protein Microarray Assay for Serological Determination of Antigen-specific Antibody Responses Following Clostridium difficile Infection

1Breast Surgery Group, Division of Medical Sciences and Graduate Entry Medicine, School of Medicine, Queen's Medical Centre, University of Nottingham, 2Medical Microbiology and Immunology Department, Faculty of Medicine, Mansoura University, 3Nottingham Digestive Diseases Centre and NIHR Nottingham Digestive Diseases Biomedical Research Centre, School of Medicine, Queen's Medical Centre, University of Nottingham

JoVE 57399


 Immunology and Infection

Genetic Engineering of Primary Mouse Intestinal Organoids Using Magnetic Nanoparticle Transduction Viral Vectors for Frozen Sectioning

1Department of Medicine, Division of Hematology, Johns Hopkins University School of Medicine, 2Department of Cell Biology, Johns Hopkins University School of Medicine, 3Department of Oncology, Johns Hopkins University School of Medicine, 4Department of Pathology, Johns Hopkins University School of Medicine, 5Institute for Cell Engineering, Johns Hopkins University School of Medicine

Video Coming Soon

JoVE 57040


 JoVE In-Press

Phenotyping Mouse Pulmonary Function In Vivo with the Lung Diffusing Capacity

1Department of Environmental Health Sciences, Johns Hopkins University Bloomberg School of Public Health, 2Department of International Health, Johns Hopkins University Bloomberg School of Public Health, 3Department of Medicine, Johns Hopkins University School of Medicine

JoVE 52216


 Biology

A Novel Surgical Approach for Intratracheal Administration of Bioactive Agents in a Fetal Mouse Model

1Molecular Virology and Gene Therapy, KU Leuven, 2Department of Woman and Child, KU Leuven, 3Neurobiology and Gene Therapy, KU Leuven, 4Division of Nuclear Medicine, KU Leuven, 5Biomedical NMR Unit/ MoSAIC, KU Leuven

JoVE 4219


 Medicine

Respiratory Exam I: Inspection and Palpation

JoVE 10028

Source: Suneel Dhand, MD, Attending Physician, Internal Medicine, Beth Israel Deaconess Medical Center

Disorders of the respiratory system with a chief complaint of shortness of breath are among the most common reasons for both outpatient and inpatient evaluation. The most obvious visible clue to a respiratory problem will be whether the patient is displaying any signs of respiratory distress, such as fast respiratory rate and/or cyanosis. In a clinical situation, this will always require emergent attention and oxygen therapy. Unlike pathology in other body systems, many pulmonary disorders, including chronic obstructive pulmonary disease (COPD), asthma, and pneumonia, can be diagnosed by careful clinical examination alone. This starts with a comprehensive inspection and palpation. Keep in mind that in non-emergency situations the patient's complete history will have been taken already, gaining important insight into exposure histories (e.g., smoking), which could give rise to specific lung diseases. This history can then confirm physical findings as the examination is performed.


 Physical Examinations I

Preparing and Administering Inhaled Medications

JoVE 10390

Source: Madeline Lassche, MSNEd, RN and Katie Baraki, MSN, RN, College of Nursing, University of Utah, UT

Inhaled medications are prescribed for conditions affecting the bronchi, which branch off of the trachea, and bronchioles, which are progressively smaller conducting airways spread throughout the lung tissue. These conditions can be classified as acute (i.e., temporary, with quick onset) or chronic (i.e., persistent and/or recurrent symptoms lasting months to years). Common acute conditions requiring inhaled medications include acute bronchitis, pneumonia, tuberculosis, pulmonary edema, and acute respiratory distress syndrome. Chronic conditions requiring inhaled medications encompass those classified as COPD (i.e., asthma, chronic bronchitis, and emphysema), as well as other chronic conditions, including cystic fibrosis, lung cancer, and pneumoconiosis. These conditions often require medications to open airways, decrease airway inflammation, and promote airflow. The delivery of medications directly into the airways allows for a faster response when compared to systemically administered medications and decreases the impact of systemic side effects. Inhaled medications come in different forms and delivery devices. Common inhaled medications include short- and


 Nursing Skills

Culturing of Human Nasal Epithelial Cells at the Air Liquid Interface

1Center for Environmental Medicine, Asthma, and Lung Biology, The University of North Carolina at Chapel Hill, 2Department of Pediatrics, The University of North Carolina at Chapel Hill, 3Pulmonary Diseases and Critical Care, The University of North Carolina at Chapel Hill, 4Curriculum in Toxicology, The University of North Carolina at Chapel Hill

JoVE 50646


 Biology

A Detailed Protocol for Perspiration Monitoring Using a Novel, Small, Wireless Device

1Wellness Promotion Science Center, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, 2Advanced Research Center for Human Sciences, Waseda University, 3Department of Clinical Laboratory Science, Graduate School of Medical Science, Kanazawa University, 4Asanogawa General Hospital

JoVE 54837


 Medicine

Label-free Neutrophil Enrichment from Patient-derived Airway Secretion Using Closed-loop Inertial Microfluidics

1Research Laboratory of Electronics, Massachusetts Institute of Technology, 2Department of Electrical Engineering and Computer Science, Massachusetts Institute of Technology, 3Department of Medicine, University of Pittsburgh, 4Department of Biological Engineering, Massachusetts Institute of Technology, 5Vascular Medicine Institute, University of Pittsburgh

JoVE 57673


 Immunology and Infection

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