Abstract:
:Inhibitors to factor VIII (FVIII) are alloantibodies directed against epitopes able to neutralise FVIII procoagulant activity. They may render FVIII replacement therapy ineffective. They represent the most severe complication of haemophilia A. At least three mechanisms of FVIII neutralisation activity by anti-FVIII antibodies have been described: (1) steric hindrance; (2) recognition of neo-epitopes and (3) catalytic activity. The Nijmegen modification of the Bethesda is the recommended method for inhibitor surveillance. The occurrence of inhibitors is a relatively frequent and early event in previously untreated patients. Conversely, it is rare in previously treated patients. Therapeutic strategies for managing inhibitors include: inhibitor eradication, haemostatic management of bleeding episodes and/or surgery and supportive care. For high responding inhibitors, immune tolerance induction (ITI) is the strategy for achieving antigen-specific tolerance to FVIII. ITI success rate ranges commonly between 60% and 80%. For treatment of patients with high-titre, high-responding inhibitors, 'by-pass' therapy is generally recommended. Activated prothrombin complex concentrates represent the historically primary 'by-pass' treatment. Recombinant factor VIIa has also been widely used as a by-passing agent. Considering the small patient population, it has to be considered that full immunogenicity data cannot be collected premarketing authorisation. Thus, stringent follow-up of patients in the post-authorisation phase is required.
journal_name
Clin Rev Allergy Immunoljournal_title
Clinical reviews in allergy & immunologyauthors
Tellier Z,André MH,Polack Bdoi
10.1007/s12016-009-8115-4subject
Has Abstractpub_date
2009-10-01 00:00:00pages
125-34issue
2eissn
1080-0549issn
1559-0267journal_volume
37pub_type
杂志文章,评审abstract::Atherosclerosis is a disease of the vascular wall, which predominantly affects large and medium-sized arteries. It represents a leading cause of morbidity and mortality in the Western world. In the last few decades, it has been clearly shown that immune system plays a relevant role in atherogenesis. The effectors of b...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-008-8098-6
更新日期:2009-08-01 00:00:00
abstract::It has been shown that many hereditary motor neuron diseases are caused by mutation of RNA processing enzymes. Survival of motor neuron 1 (SMN1) is well-known as a causative gene for spinal muscular atrophy (SMA) and mutations of glycyl- and tyrosyl-tRNA synthetases are identified as a cause of distal SMA and Charcot-...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-011-8286-7
更新日期:2012-02-01 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::As summarized in Table 1, the literature consistently supports the hypothesis that allergic asthmatic patients have seasonal BHR changes that parallel allergen exposure. These seasonal changes appear to be preventable by treatment with corticosteroids (systemic, inhaled, or nasal), disodium cromoglycate, and immunothe...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/BF02826585
更新日期:1997-07-01 00:00:00
abstract::Pruritus, fatigue, and metabolic bone disease are frequent complications of cholestatic liver diseases, which can be quite distressing for the patient and can considerably reduce the quality of life. The molecular pathogenesis of these extrahepatic manifestations of cholestasis is poorly understood, and hypotheses to ...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1385/CRIAI:28:2:147
更新日期:2005-04-01 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::Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic disease of the liver and bile ducts that is associated with inflammatory bowel disease, generally leads to end-stage liver disease, and is complicated by malignancies of the biliary tree and the large intestine. The pathogenesis of PSC remains enigmati...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-008-8085-y
更新日期:2009-02-01 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::Sarcoidosis is a systemic granulomatous disease that frequently involves the lung. Although classically thought of as a restrictive lung disease, airway obstruction has become a recognized feature of the disease in the past years. Sarcoidosis can affect the airway at any level and when the involvement includes small a...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1385/CRIAI:25:2:115
更新日期:2003-10-01 00:00:00
abstract::Prognosis of several autoimmune diseases, especially rheumatoid arthritis (RA), ankylosing spondylitis, Crohn's disease (CD), and psoriasis, usually refractory to conventional treatment improved considerably with the introduction of tumor necrosis factor alpha (TNF-alpha) antagonistic agents, which is now available (i...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-009-8140-3
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::Atherosclerosis is an inflammatory disease, and several antigens have been shown to activate the immune response and affect the development of atherogenesis. This suggests that modulation of the immune system could represent a useful approach to prevent and/or treat this disorder. A vaccination approach might be a use...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-009-8145-y
更新日期:2010-04-01 00:00:00
abstract::Patients presenting to the emergency department (ED) or clinic with acute exacerbation of asthma (AEA) can be very challenging varying in both severity and response to therapy. High-dose, frequent or continuous nebulized short-acting beta2 agonist (SABA) therapy that can be combined with a short-acting muscarinic anta...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-014-8448-5
更新日期:2015-02-01 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::Chronic hyperplastic eosinophilic sinusitis (CHES) is a T-helper (Th)2-like, lymphocyteinitiated, eosinophil-rich inflammatory disease. The complex immune interactions required to orchestrate these processes begin with the presentation of antigen by mature dendritic cells to Th lymphocytes that display the appropriate...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1385/CRIAI:30:3:195
更新日期:2006-06-01 00:00:00
abstract::Therapy of atopic dermatitis should comprise emollients, topical glucocorticosteroids, or calcineurin inhibitors, phototherapies, immunosuppressants like cyclosporin A, and other treatments. All these treatments should be improved, thanks to research. But new therapeutic perspectives should be given by topical anti-in...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-010-8226-y
更新日期:2011-12-01 00:00:00
abstract::Over the years, the commonly used term to describe juvenile idiopathic arthritis (JIA) has changed. By definition, JIA includes all types of arthritis with no apparent cause, lasting more than 6 weeks, in patients aged less than 16 years at onset. JIA pathogenesis is still poorly understood: the interaction between en...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-014-8460-9
更新日期:2015-10-01 00:00:00
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 i...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-008-8078-x
更新日期:2008-12-01 00:00:00
abstract::Autoimmune diseases are immune disorders characterized by T cell hyperactivity and B cell overstimulation leading to overproduction of autoantibodies. Although the pathogenesis of various autoimmune diseases remains to be elucidated, environmental factors have been thought to contribute to the initiation and maintenan...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-013-8402-y
更新日期:2014-10-01 00:00:00
abstract::Systemic lupus erythmatosus (SLE) is a multi-system autoimmune disease characterized by auto-reactive cells and auto-antibodies, which can potentially affect all organ systems. Typical organ systems that are affected include the heart, lungs, skin, kidneys, and central nervous system. Its expression is believed to be ...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1385/CRIAI:25:1:41
更新日期:2003-08-01 00:00:00
abstract::The expert panel report version 2 (EPR-2) is a comprehensive set of recommendations for the evaluation and treatment of asthma. However, physicians demonstrate scant adoption of many of the key evaluation and treatment components of the EPR-2, with primary care physicians being less likely to adopt EPR-2 recommendatio...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1385/CRIAI:27:2:065
更新日期:2004-10-01 00:00:00
abstract::One of the important factors and consequences in persistent asthma is the change in the vasculature of the airways and lung parenchyma. These changes could contribute to worsening asthma control and predispose asthmatics to critical asthma syndromes. For many years, the contribution of vasculature to severe asthma was...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-014-8420-4
更新日期:2015-02-01 00:00:00
abstract::Contact dermatitis accounts for 95% of occupational skin disorders. Irritant contact dermatitis (ICD) is often caused by cumulative exposure to weak irritants, accounting for 80% of all cases of contact dermatitis. ICD can co-exist with atopic dermatitis (AD) and allergic contact dermatitis (ACD). Patients with AD and...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-018-8713-0
更新日期:2019-02-01 00:00:00
abstract::Reduced bone mineral density is frequently found especially in adult celiac disease (CD) and dietary guidelines favor vitamin D supplementation in adults and children with CD. Vitamin D serum levels were investigated in CD populations in order to challenge its routine supplementation. Israeli (61), Spanish (59), CD ch...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章
doi:10.1007/s12016-010-8237-8
更新日期:2012-06-01 00:00:00
abstract::Rheumatoid arthritis and spondyloarthritis are inflammatory joint disorders with an autoimmune pathogenesis and systemic involvement. The skin is one of the most frequently affected extraarticular sites with a number of manifestations or distinct diseases, including common conditions, such as rheumatoid nodules and ps...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-017-8629-0
更新日期:2018-04-01 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::The diagnosis of celiac disease (CD) remains a clinical challenge based on the incomplete specificity and sensitivity rates of current non-invasive tests. Furthermore, histological assessments fail to identify all overt cases and, in particular, do not manifest pathognomonic alterations in silent cases. Accordingly, t...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-009-8160-z
更新日期:2010-04-01 00:00:00
abstract::Legumes belonging to Fabaceae family of the order Fabales are a rich and important source of proteins and many essential elements. Due to its nutritious elements, these are preferably included in human diet in most part of the world. But, unfortunately, IgE binding proteins have been identified in majority of legumes,...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-012-8310-6
更新日期:2013-08-01 00:00:00
abstract::Skin tests by prick technique offer considerable guidance in the diagnosis of food allergy. Negative prick skin tests are powerful evidence against food allergy. Positive food skin tests are slightly to moderately predictive of reaction to a food on DBPCFC. Oral food challenge is necessary for confirmation of food all...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/BF02737614
更新日期:1999-10-01 00:00:00
abstract::The hyper IgM syndromes are a group of rare inherited immune deficiency disorders characterized by impairment of immunoglobulin isotype switching resulting from defects in the CD40 ligand/CD40 signaling pathway. X-linked forms of hyper IgM are caused by defects in the CD40 ligand gene or NF-κB essential modulator, whi...
journal_title:Clinical reviews in allergy & immunology
pub_type: 杂志文章,评审
doi:10.1007/s12016-013-8378-7
更新日期:2014-04-01 00:00:00