Abstract:
:Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene that results in abnormal viscous mucoid secretions in multiple organs and whose main clinical features are pancreatic insufficiency and chronic endobronchial infection. Although it was initially defined and diagnosed based on clinical features and sweat chloride measurement, an in vivo method of assessing CFTR function, the discovery of the CFTR gene in 1989 revealed a broad spectrum of CF phenotypes associated with specific CFTR gene mutations. In this article, we will review the indications for sweat testing, alternative techniques to diagnose CF, and the approach to patients with an ambiguous or indeterminate diagnosis of CF.
journal_name
Clin Rev Allergy Immunoljournal_title
Clinical reviews in allergy & immunologyauthors
Voter KZ,Ren CLdoi
10.1007/s12016-008-8078-xsubject
Has Abstractpub_date
2008-12-01 00:00:00pages
100-6issue
3eissn
1080-0549issn
1559-0267journal_volume
35pub_type
杂志文章,评审abstract::Food allergy is a potentially life-threatening condition with no approved curative therapy. A number of food allergen immunotherapies are being investigated in phase II/III trials; however, these are limited in their ability to restore immune tolerance to food allergens and often result in high rates of allergic side ...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-018-8694-z
更新日期:2018-10-01 00:00:00
abstract::Bronchiolitis is a common, acute, contagious lower respiratory tract illness of infants and young children. The majority of cases are secondary to respiratory syncytial virus. There are a number of risk factors for severe disease, including children less than six weeks of age and patients with atopy and/or asthma. The...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1385/CRIAI:25:3:249
更新日期:2003-12-01 00:00:00
abstract::Patients with primary immunodeficiencies are prone to develop enteropathy of unknown pathogenesis. We hypothesize that ineffective clearance of gastrointestinal pathogens, particularly viruses, in combination with defective immune regulation may cause inflammatory enteropathy in certain immunodeficient hosts. We revie...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-011-8292-9
更新日期:2012-02-01 00:00:00
abstract::Bronchial hyperresponsiveness (BHR) is an essential part of the definition of asthma. Although our understanding of the allergic inflammatory and immunologic mechanisms of asthma have markedly increased, the mechanism of BHR remains to be elucidated. Increased BHR is associated temporally with exposure to allergens, c...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1385/CRIAI:24:1:85
更新日期:2003-02-01 00:00:00
abstract::Beta-agonists have clearly demonstrated benefits for the treatment of both acute and chronic asthma. Therefore, it is perhaps surprising that many of the articles in this issue have focused on concerns about their safety. Much of this concern can be traced back to the "beta-agonist controversy"--the association of hig...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章
doi:10.1385/criai:31:2:279
更新日期:2006-10-01 00:00:00
abstract::Airway remodeling is an established feature of asthma. Histologic examination is essential in the assessment of remodeling that is a pathologic concept. Examinations of autopsied or resected lung have enabled detailed morphologic and morphometric studies and have provided fundamental knowledge of airway remodeling in ...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1385/CRIAI:27:1:045
更新日期:2004-08-01 00:00:00
abstract::Tumor necrosis factor-α (TNF-α) is a central regulator of inflammation, and TNF-α antagonists may be effective in treating inflammatory disorders in which TNF-α plays a major pathogenic role. TNF-α has also been associated with inflammatory mechanisms related to implantation, placentation, and pregnancy outcome. TNF-α...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-016-8596-x
更新日期:2017-08-01 00:00:00
abstract::To investigate the prevalence of anti-third generation cyclic citrullinated peptide antibodies (anti-CCP3) in patients with systemic connective tissue diseases, we assembled a training set consisting of 115 patients with rheumatoid arthritis (RA), 52 with Calcinosis, Raynaud's phenomenon, oesophageal dysmotility, scle...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章
doi:10.1007/BF02686081
更新日期:2007-02-01 00:00:00
abstract::Available data on small numbers of patients with refractory and/or relapsing Wegener's granulomatosis support very good overall outcomes with rituximab, an anti-CD20 biotherapy targeting B cells, in combination with ongoing immunosuppressants, apparently independently of antineutrophil cytoplasm antibody (ANCA) status...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章
doi:10.1007/s12016-007-8026-1
更新日期:2008-02-01 00:00:00
abstract::Mixed cryoglobulinemia (MC) syndrome is an immune complex-mediated vasculitis characterized by the clinical triad of purpura, weakness, and arthralgias, the morbidity of which is mainly related to kidney and peripheral nervous system dysfunction as well as to the development of a secondary lymphoma (Ferri et al. Autoi...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-011-8275-x
更新日期:2012-04-01 00:00:00
abstract::Non-alcoholic steatohepatitis (NASH) is the progressive phenotype of non-alcoholic fatty liver disease associated with the metabolic syndrome. The existence of autoimmune features in NASH has been reported, but its significance remains unclear. We herein report the autoantibody profile of 54 patients with histological...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-013-8383-x
更新日期:2013-08-01 00:00:00
abstract::The Zika virus outbreaks highlight the growing importance need for a reliable, specific and rapid diagnostic device to detect Zika virus, as it is often recognized as a mild disease without being identified. Many Zika virus infection cases have been misdiagnosed or underreported because of the non-specific clinical pr...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-016-8554-7
更新日期:2016-12-01 00:00:00
abstract::Through the last decade, clinical immunology has witnessed a considerable progress in understanding the role of the innate immunity in human host defense, with Toll-like receptors (TLRs) being the most extensively innate immune receptors investigated. Growing literature documents the relevance of TLR signaling pathway...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-009-8135-0
更新日期:2010-02-01 00:00:00
abstract::What makes an antigen an allergen? This question has been at the core of much research regarding the basic mechanisms of allergy as well as the development of diagnostic and therapeutic allergen products. Great strides have been made since Kjell Aas asked this question in his classic review in Allergy (1), but we appe...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章
doi:10.1385/CRIAI:30:2:71
更新日期:2006-04-01 00:00:00
abstract::Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder with a broad spectrum of clinical presentations and association with multiple immunological abnormalities. Recent research of the Janus kinase (JAK)-signal transducer and activator of transcription (STAT) signaling pathway-revealed aberrant STAT s...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-016-8550-y
更新日期:2017-04-01 00:00:00
abstract::Celiac disease is a complex immune-mediated gluten-sensitive enteropathy with protean clinical manifestations. It is manifest in genetically predisposed individuals who ingest gluten in varying amounts. In broad terms, it is thought to affect 1% of the population in the USA. More specifically, the prevalence increases...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-018-8691-2
更新日期:2019-10-01 00:00:00
abstract::A bulk of literature demonstrated that respiratory allergy, and especially asthma, is prevalent in males during childhood, while it becomes more frequent in females from adolescence, i.e., after menarche, to adulthood. The mechanisms underlying the difference between females and males are the effects on the immune res...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-017-8661-0
更新日期:2019-06-01 00:00:00
abstract::Glatiramer acetate (GA) significantly ameliorates multiple sclerosis and was initially discovered through its effects on the animal model experimental autoimmune encephalomyelitis (EAE). Guillain-Barré syndrome (GBS) is a relatively common demyelinating disease of peripheral nerves for which there is a parallel animal...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章
doi:10.1007/s12016-010-8246-7
更新日期:2012-04-01 00:00:00
abstract::During the last decade, B regulatory cells are appreciated to have a central role in preventing autoimmunity and maintaining self-tolerance. They are characterized by expressing different phenotypic markers and the production of either IL-10 or TGF-β or both. The recent recognition of Fas ligand expressing B regulator...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-016-8572-5
更新日期:2017-06-01 00:00:00
abstract::Intravenous immunoglobulin (IVIg) has been used as an immunomodulatory therapy for the treatment of multiple sclerosis (MS). In the current review, we summarize the up-to-date data related to IVIg clinical trials in MS, and the suggested mechanisms of action by which IVIg modulates the relevant immunological pathways ...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1385/CRIAI:29:3:247
更新日期:2005-12-01 00:00:00
abstract::The number of peer-reviewed articles published during the 2017 solar year and retrieved using the "autoimmunity" key word increased significantly compared to 2016 while maintaining a stable share within the immunology field, following years with alternated fortunes. A detailed arbitrary analysis of the published artic...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-018-8699-7
更新日期:2018-12-01 00:00:00
abstract::Antityrosinase antibody is a newly detected antibody in the sera of patients with melanoma or vitiligo. The serum level of the antibody is measured by enzyme-linked immunosorbent assay (ELISA). The autoantigen is tyrosinase itself, the enzyme that participates in pigment (melanin) formation by both melanocytes and mel...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/BF02737633
更新日期:1998-10-01 00:00:00
abstract::This review includes a variety of extremely rare and unusual hymenoptera sting (HS) circumstances with regard to sting localization, geographic region, massivity of multiple stings, and particularly related to clinical symptoms. Such reactions occur in a temporal relationship to HS (s), differ from typical allergic sy...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-014-8434-y
更新日期:2014-08-01 00:00:00
abstract::The term "mold" is utilized to define the ubiquitous fungal species commonly found in household dust and observed as visible multicellular filaments. Several well-defined human diseases are known to be caused or exacerbated by mold or by exposure to their byproducts. Among these, a solid connection has been establishe...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-009-8175-5
更新日期:2010-04-01 00:00:00
abstract::Systemic vasculitides are a group of rare diseases characterized by inflammation of the arterial or venous vessel wall, causing stenosis or thrombosis. Clinical symptoms may be limited to skin or to other organs or may include multiple manifestations as systemic conditions. The pathogenesis is related to the presence ...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-014-8453-8
更新日期:2015-12-01 00:00:00
abstract::Previously believed to have an exclusive role in the release of cytotoxic mediators in the defense against helminthic infections, eosinophils are now considered key players in inflammatory reaction and regulation of immune response. Through activation of a large variety of membrane receptors and production of various ...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1385/CRIAI:30:1:013
更新日期:2006-02-01 00:00:00
abstract::Eosinophils can regulate local and systemic inflammation, and their presence in higher numbers appears to play an important role in the pathology of various atopic and inflammatory diseases. Eosinophil maturation, recruitment, and survival depend on several cytokine regulators, including interleukin (IL)-5, IL-4, and ...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-014-8444-9
更新日期:2016-04-01 00:00:00
abstract::The relationship between autoimmunity and reproduction has long been recognized. This relationship is bidirectional and includes many diverse issues, but in this review article, we focus on which autoimmune disturbances are the basic cause of recurrent pregnancy losses in patients with autoimmune diseases. The antipho...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-009-8179-1
更新日期:2010-12-01 00:00:00
abstract::The heart is a target organ in antiphospholipid syndrome (APS). Endocardial disease, intracardiac thrombosis, myocardial involvement including coronary heart disease and microvascular thrombosis, as well as pulmonary hypertension have all been described in APS patients. Valvular involvement is the most common manifest...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-007-0013-z
更新日期:2007-04-01 00:00:00
abstract:BACKGROUND:The last 5 years have seen the emergence and establishment of antibodies to citrullinated antigens as the diagnostic marker for rheumatoid arthritis (RA). Initially, these were detected using a synthetic peptide, which has undergone a number of modifications to give a diagnostic test with a sensitivity of 65...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章
doi:10.1007/s12016-007-8016-3
更新日期:2008-02-01 00:00:00