Adult Care in Cystic Fibrosis.

Abstract:

:Cystic fibrosis (CF) is now more common in adults than children in countries with well-developed health care systems. The number of adults continues to increase and will further increase if the new cystic fibrosis transmembrane conductance regulator (CFTR) modulators are disease modifying. Most of the complex morbidity and almost all the mortality of CF occur in adults and will increasingly follow this pattern even with new effective modulator therapies. Maintaining good quality of life including social functioning and maximizing survival for adults are the key priorities. This requires a highly knowledgeable and adaptable multidisciplinary team, which, though focused on maintaining lung health, requires an increasing range of other disciplines and specialties to maximize well-being. Changes in health care systems will require current models of care to adapt to provide care for the large number of adult patients. With increasing survival and age, many are likely to have both CF morbidities and additional diseases of aging. New models are needed for health care delivery for this expanding population with complex medical conditions.

authors

Elborn JS

doi

10.1055/s-0039-3400289

subject

Has Abstract

pub_date

2019-12-01 00:00:00

pages

857-868

issue

6

eissn

1069-3424

issn

1098-9048

journal_volume

40

pub_type

杂志文章,评审
  • Treatment of sarcoidosis.

    abstract::Sarcoidosis is an immune-mediated systemic syndrome of unknown etiology. The treatment of the granulomatous inflammation in sarcoidosis is thus dependent on nonspecific suppression of the immune response. Although steroid-sparing approaches have moved to front-line therapy for many other immune-mediated inflammatory d...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0030-1262218

    authors: Lazar CA,Culver DA

    更新日期:2010-08-01 00:00:00

  • Aspirin-exacerbated respiratory disease: update on pathogenesis and desensitization.

    abstract::Aspirin-exacerbated respiratory disease (AERD) is a unique syndrome of airway inflammation that frequently occurs in patients with nasal polyposis, chronic sinusitis, and asthma. These patients tend to have progressive and recalcitrant sinus disease requiring frequent surgical intervention and in many cases systemic c...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1055/s-0032-1325618

    authors: White AA,Stevenson DD

    更新日期:2012-12-01 00:00:00

  • The role of atypical pathogens in community-acquired pneumonia.

    abstract::The term atypical pneumonia was first used in 1938, and by the 1970s it was widely used to refer to pneumonia due to Mycoplasma pneumoniae, Legionella pneumophila (or other Legionella species), and Chlamydophila pneumoniae. However, in the purest sense all pneumonias other than the classic bacterial pneumonias are aty...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0032-1315636

    authors: Marrie TJ,Costain N,La Scola B,Patrick W,Forgie S,Xu Z,McNeil SA

    更新日期:2012-06-01 00:00:00

  • Epidemiology of Asthma and Influence of Ethnicity.

    abstract::Asthma is a heterogeneous disorder with a complex etiology. Prevalence rates for asthma have been increasing in many countries over the past few decades. While it is unclear why this increase is occurring, the variation reported in asthma prevalence and severity associated with ethnicity offers some insight into the d...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0037-1618568

    authors: Moraes TJ,Sears MR,Subbarao P

    更新日期:2018-02-01 00:00:00

  • Predictors of Maternal Mortality and Prognostic Models in Obstetric Patients.

    abstract::The use of predictive models has been proposed as a potential tool to reduce maternal morbidity and mortality, by aiding in the timely identification of potential high-risk patients. Prognostic models in critical care have been used to characterize the severity of illness of specific diseases. Physiological changes in...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0037-1602244

    authors: Levinson A,Miranda J,Rojas-Suarez JA

    更新日期:2017-04-01 00:00:00

  • The diagnostic approach to deep venous thrombosis.

    abstract::Accurate diagnosis of deep vein thrombosis is important because untreated deep vein thrombosis can cause death or permanent impairment and because effective treatments are available. The approach to the diagnosis of deep vein thrombosis varies because of differences in local resources and expertise. Duplex ultrasonogr...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1055/s-2000-13187

    authors: Elliott CG

    更新日期:2000-01-01 00:00:00

  • Mycobacterial Lung Disease Complicating HIV Infection.

    abstract::Mycobacterial infections have caused enormous morbidity and mortality in people living with human immunodeficiency virus (HIV) infection. Of these, the most devastating has been tuberculosis (TB), the leading cause of death among HIV-positive persons globally. TB has killed more people living with HIV than any other i...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0036-1572559

    authors: Haas MK,Daley CL

    更新日期:2016-04-01 00:00:00

  • Pulmonary defenses against fungi.

    abstract::Pulmonary immunity to fungal pathogens requires both innate and adaptive immune responses. Alveolar macrophages, dendritic cells, and neutrophils are the phagocytic cells of the lung innate system. These cells produce early inflammatory mediators (i.e., reactive oxygen species, cytokines, and chemokines) in response t...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1055/s-2004-822306

    authors: Hernández Y,Herring AC,Huffnagle GB

    更新日期:2004-02-01 00:00:00

  • Exposure-related diffuse lung disease.

    abstract::Practicing pulmonologists are often faced with the question of whether a lung disease is related to something in the patient's workplace, home, or recreational environment. Recognizing a lung disease as exposure related creates both opportunities and obligations for clinicians. In addition to managing the patient, the...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1055/s-0028-1101272

    authors: Rose CS,Lynch DA,Cool CD

    更新日期:2008-12-01 00:00:00

  • Hepatic Sarcoidosis: Current Concepts and Treatments.

    abstract::Hepatic sarcoidosis is a relatively common manifestation of extrapulmonary sarcoidosis. It occurs in 20 to 30% of cases and is rarely severe. However, a cluster of patients may develop severe complications such as cirrhosis and portal hypertension. In this review, we describe the current knowledge of clinical, biologi...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1055/s-0040-1713799

    authors: Rossi G,Ziol M,Roulot D,Valeyre D,Mahévas M

    更新日期:2020-10-01 00:00:00

  • Thoracic applications of dual energy.

    abstract::Computed tomography (CT) is the core imaging modality for the evaluation of thoracic disorders. With the recently developed dual-energy CT (DECT) technique, the clinical utility of CT in the management of pulmonary diseases can be expanded. The most actively investigated principle of dual energy is material decomposit...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0033-1363452

    authors: Remy-Jardin M,Faivre JB,Pontana F,Molinari F,Tacelli N,Remy J

    更新日期:2014-02-01 00:00:00

  • Pseudomonas aeruginosa: evolution of antimicrobial resistance and implications for therapy.

    abstract::Pseudomonas aeruginosa is a formidable pathogen in the infection arena. It is able to easily adapt to the environment which it inhabits and can also colonize and invade the human host to cause serious infections. In 2011, it was responsible for 7.1% of all health care-associated infection in the United States. The mor...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0034-1396907

    authors: McCarthy K

    更新日期:2015-02-01 00:00:00

  • Who Is Safe to Extubate in the Neuroscience Intensive Care Unit?

    abstract::Patients admitted to the neuroscience intensive care unit (NICU) may have respiratory compromise from either central or peripheral neurological pathology, and may hence require intubation and mechanical ventilation for very diverse reasons. Liberation from invasive ventilation, that is, extubation, at the earliest pos...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0037-1608773

    authors: Bösel J

    更新日期:2017-12-01 00:00:00

  • Pleural interventions: management of acute and chronic pneumothorax.

    abstract::Pneumothorax is a common clinical entity that may present to a wide variety of medical specialties. Primary pneumothorax (in the presence of no known underlying lung disease) and secondary pneumothorax (in known lung disease) are distinct entities with varied etiology, treatment, and prognosis. Diagnosis is usually ba...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-2008-1081285

    authors: Rahman NM,Davies RJ,Gleeson FV

    更新日期:2008-08-01 00:00:00

  • Pleural effusions from congestive heart failure.

    abstract::In heart failure (HF), pleural effusion results from increased interstitial fluid in the lung due to elevated pulmonary capillary pressure. Rarely, pleural effusions may occur in association with isolated right HF. HF-associated effusions are typically bilateral, but if unilateral, they are more commonly seen on the r...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0030-1269828

    authors: Porcel JM

    更新日期:2010-12-01 00:00:00

  • Pulmonary involvement in Sjögren syndrome.

    abstract::Sjögren syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic inflammation of exocrine glands and a variety of extraglandular sites. Lung involvement as defined by symptoms and either pulmonary function testing or radiographic abnormalities occurs in approximately 10 to 20% of patients. Subclinica...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0034-1371529

    authors: Kreider M,Highland K

    更新日期:2014-04-01 00:00:00

  • Bronchoalveolar lavage to diagnose respiratory infections.

    abstract::Respiratory samples obtained by bronchoalveolar lavage (BAL) in infectious processes provide important microbiological and cytological information to manage this type of patient. Most of the clinical and experimental BAL investigations have been done in ventilator-associated pneumonia (VAP) and in immunosuppressed con...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-2007-991524

    authors: Ramírez P,Valencia M,Torres A

    更新日期:2007-10-01 00:00:00

  • Pulmonary manifestations of rheumatoid arthritis.

    abstract::Rheumatoid arthritis (RA) is a systemic disease, characterized by symmetric joint involvement, but it can also affect other organ systems, including the lungs. The better-known pulmonary manifestations of RA are interstitial lung disease, rheumatoid nodules, and pleural effusions. Less common manifestations include br...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-2007-985664

    authors: Gauhar UA,Gaffo AL,Alarcón GS

    更新日期:2007-08-01 00:00:00

  • Diagnostic modalities in sarcoidosis: BAL, EBUS, and PET.

    abstract::Advances have been made in minimally invasive diagnostic procedures in sarcoidosis, including bronchoalveolar lavage (BAL), endobronchial ultrasonography-guided transbronchial needle aspiration (EBUS-TBNA), and positron emission tomography (PET). Several independent groups found almost identical predictive values of t...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1055/s-0030-1262207

    authors: Costabel U,Bonella F,Ohshimo S,Guzman J

    更新日期:2010-08-01 00:00:00

  • Reversal of Oral Anticoagulants for Intracerebral Hemorrhage Patients: Best Strategies.

    abstract::In patients with acute intracerebral hemorrhage (ICH), one of the major concerns is ongoing bleeding or ICH expansion. Anticoagulated patients are at higher risk of ongoing expansion and worse outcome. It may be that rapid anticoagulation reversal can reduce the risk of expansion and improve clinical outcome. For thos...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0037-1607993

    authors: Fuh L,Sin JH,Goldstein JN,Hayes BD

    更新日期:2017-12-01 00:00:00

  • Idiopathic pulmonary arterial hypertension and pulmonary veno-occlusive disease: similarities and differences.

    abstract::Pulmonary veno-occlusive disease (PVOD) is a rare disorder and can be misdiagnosed as idiopathic pulmonary arterial hypertension (iPAH). PVOD and iPAH often share a similar clinical presentation, genetic background, and hemodynamic profile. PVOD accounts for 5 to 10% of cases initially considered as iPAH. When compare...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0029-1233310

    authors: Montani D,Kemp K,Dorfmuller P,Sitbon O,Simonneau G,Humbert M

    更新日期:2009-08-01 00:00:00

  • Hematologic failure.

    abstract::Hematologic factors, in particular platelets and the coagulation system, play an important role in the pathogenesis of organ failure in the intensive care unit. Failure of these hematologic systems is common in intensive care patients and may range from isolated thrombocytopenia or prolonged global clotting tests to c...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0031-1287873

    authors: Levi M,Schultz M

    更新日期:2011-10-01 00:00:00

  • Pulmonary Complications of Rheumatoid Arthritis.

    abstract::Rheumatoid arthritis (RA) is a common chronic autoimmune disorder that characteristically causes joint inflammation and damage. In addition, many patients develop extraarticular manifestations which may cause significant comorbidity and premature mortality.Some respiratory tract involvement of the upper and lower airw...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0039-1683995

    authors: Farquhar H,Vassallo R,Edwards AL,Matteson EL

    更新日期:2019-04-01 00:00:00

  • Optimizing therapy for Acinetobacter baumannii.

    abstract::Acinetobacter baumannii is a highly resilient, gram-negative coccobacillus that thrives within the unique and complex ecological setting of an intensive care unit. This evolving pathogen has now surpassed human capacity to create new antimicrobials, and has led physicians into a concerning era for hospital-acquired in...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-2007-996413

    authors: Peleg AY

    更新日期:2007-12-01 00:00:00

  • Epidemiology of sarcoidosis: recent advances and future prospects.

    abstract::Sarcoidosis is by definition a disease of "unknown causes," but recent epidemiologic advances suggest that the long-standing definition of sarcoidosis may soon need to be amended. The recently completed ACCESS (A Case-Control Etiologic Study of Sarcoidosis) study was not able to definitively identify the "cause" of sa...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-2007-970331

    authors: Rybicki BA,Iannuzzi MC

    更新日期:2007-02-01 00:00:00

  • Genetics of pulmonary fibrosis.

    abstract::Pulmonary fibrosis is a devastating disease that occurs in association with environmental exposures, several inherited diseases, collagen vascular disease, as well as in an idiopathic form. Epithelial cell damage, excessive fibroblast proliferation, upregulation of extracellular matrix production, and attenuated matri...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1055/s-2002-25306

    authors: Wurfel MM,Raghu G

    更新日期:2002-04-01 00:00:00

  • Acute Cellular Rejection: Is It Still Relevant?

    abstract::Despite significant progress in the field of transplant immunology, acute cellular rejection (ACR) remains a very frequent complication after lung transplantation (LTx), with almost 30% of LTx recipients experiencing at least one episode of treated ACR during the first year of follow-up. Most episodes respond to the f...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0037-1617424

    authors: Koutsokera A,Levy L,Pal P,Orchanian-Cheff A,Martinu T

    更新日期:2018-04-01 00:00:00

  • Awake or Sedated: Trends in the Evaluation and Management of Agitation in the Intensive Care Unit.

    abstract::Critically ill intensive care unit (ICU) patients often require sedation to tolerate life-saving interventions such as mechanical ventilation. Pain, anxiety, and delirium all contribute to patient distress and agitation which can interfere with ICU medical care if not addressed and treated appropriately. Sedation prac...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0035-1564875

    authors: DeBiasi EM,Akgün KM,Pisani M

    更新日期:2015-12-01 00:00:00

  • Bronchiolitis obliterans syndrome complicating lung or heart-lung transplantation.

    abstract::Lung transplantation is a therapeutic option for patients with end stage lung diseases, but long-term survival remains poor, primarily due to chronic allograft rejection. Bronchiolitis obliterans (BO), a fibrotic process resulting in progressive narrowing of bronchiolar lumens and airflow obstruction, is a manifestati...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1055/s-2004-815601

    authors: Belperio JA,Lake K,Tazelaar H,Keane MP,Strieter RM,Lynch JP 3rd

    更新日期:2003-10-01 00:00:00

  • Pulmonary cryptococcosis.

    abstract::Cryptococcosis is an invasive fungal infection (IFI), caused predominantly by Cryptococcus neoformans or Cryptococcus gattii, that affects both immunocompromised (IC) and non-IC patients. Although the most serious disease manifestation is meningoencephalitis, cryptococcal pneumonia is underdiagnosed and may disseminat...

    journal_title:Seminars in respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1055/s-0031-1295720

    authors: Brizendine KD,Baddley JW,Pappas PG

    更新日期:2011-12-01 00:00:00