Abstract:
INTRODUCTION:Hereditary angioedema (HAE) with C1 inhibitor (C1-INH) deficiency is a rare autosomal dominant disease. Although the first symptoms can appear in childhood, the diagnosis's delay has a strong impact on the patient's quality of life. We analyzed clinical and laboratory characteristics and the drug therapy of pediatric patients with HAE in Brazil. METHODS:Medical records from 18 reference centers of HAE patients under 18 years of age were evaluated after confirmed diagnosis was performed by quantitative and/or functional C1-INH. RESULTS:A total of 95 participants (51 M:44 F; mean age: 7 years old) out of 17 centers were included; 15 asymptomatic cases were identified through family history and genetic screening. Angioedema attacks affected the extremities (73.5%), gastrointestinal tract (57%), face (50%), lips (42.5%), eyelids (23.7%), genitals (23.7%), upper airways (10%), and tongue (6.3%). Family history was present in 84% of patients, and the mean delay in the diagnosis was 3.9 years. Long-term prophylaxis (51/80) was performed with tranexamic acid (39/80) and androgens (13/80); and short-term prophylaxis (9/80) was performed with tranexamic acid (6/80) and danazol (3/80). On-demand therapy (35/80) was prescribed: icatibant in 7/35, fresh frozen plasma in 16/35, C1-INH plasma-derived in 11/35, and tranexamic acid in 12/35 patients. CONCLUSIONS:This is the first study on HAE pediatric patients in Latin America. Clinical manifestations were similar to adults. Drugs such as androgens and tranexamic acid were indicated off-label, probably due to restricted access to specific drugs. Educational programs should address pediatricians to reduce late diagnosis and tailored child therapy.
journal_name
Int Arch Allergy Immunoljournal_title
International archives of allergy and immunologyauthors
Araújo-Simões J,Boanova AGP,Constantino-Silva RN,Fragnan NTML,Pinto JA,Minafra FG,Gonçalves RF,Valle SRD,Alonso MLO,Dortas SD,Goudouris ES,Rêgo-Silva AM,Marques MM,Serpa FS,Chong-Neto HJ,Nelson RF,Mansour E,Moreira IF,doi
10.1159/000512944subject
Has Abstractpub_date
2021-01-28 00:00:00pages
1-7eissn
1018-2438issn
1423-0097pii
000512944pub_type
杂志文章abstract:BACKGROUND:The human IgE response is associated with allergy and with host defence against parasitic worms. A response to Sm22.6, the dominant IgE antigen in adult Schistosoma mansoni worms, correlates with resistance to re-infection after treatment. Sm22.6 is one of a family of EF-hand containing parasite proteins wit...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000095997
更新日期:2007-01-01 00:00:00
abstract:BACKGROUND:Protective acquired immunity against helminths and allergic sensitisation are both characterised by high IgE antibody levels. Levels of IgE antibodies are naturally tightly regulated by several mechanisms including binding of the CD23 receptor. Following observations that helminth infections and allergic sen...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000346545
更新日期:2013-01-01 00:00:00
abstract::Factors are discussed as potential diagnostic parameters of atopic disorders. The amounts of sCD23 in sera from newborn children (n = 4,329) were determined by radioimmunoassay with monoclonal antibodies specific for CD23. The sCD23 levels ranged between 0 and 81.5 ng/ml of CD23-specific mAb. Furthermore, the sera of ...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000236346
更新日期:1992-01-01 00:00:00
abstract::The peripheral blood mononuclear cells (PBMC) from humans allergic to grass pollens (GR+ subjects) show strong in vitro proliferative responses to purified allergens from Lolium perenne pollen Lol p 1, and to a lesser extent to Lol p 2 and Lol p 3. By contrast, PBMC from grass allergic patients undergoing immunotherap...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000237452
更新日期:1997-02-01 00:00:00
abstract:BACKGROUND:Sensitivity to the mite Blomia tropicalis is related to asthma in tropical climates, but correlates of sensitivity to B. tropicalis and its relationship to Dermatophagoides pteronyssinus sensitivity have not been widely examined in families with asthma. The main objective of this study was to determine preva...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000070927
更新日期:2003-06-01 00:00:00
abstract::Human foamy virus (HFV) is a recently characterized member of the retrovirus subfamily Spumaretrovirinae. HFV has a complex structure: it encodes the classical three retroviral structural genes gag, pol and env, but also possesses additional regulatory, so-called bel sequences. Foamy viruses have been discussed occasi...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000236350
更新日期:1992-01-01 00:00:00
abstract:BACKGROUND:Atopic dermatitis (AD) is increasing among the elderly. Staphylococcus aureus colonization is one of the most important aggravating factors in AD. OBJECTIVE:This study analyses the clinical features of AD in elderly patients and determines the pathogenic roles of staphylococcal superantigens in AD with low ...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000329150
更新日期:2012-01-01 00:00:00
abstract:BACKGROUND:Egg allergy is one of the most common food allergies in children. The standard therapy for egg allergy is strict avoidance. Yet, there is considerable clinical and scientific interest in primary or secondary prevention. A major drawback of oral tolerance (OT) induction protocols, however, is the possibility ...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000363110
更新日期:2014-01-01 00:00:00
abstract:BACKGROUND:Transforming growth factor-β1 (TGF-β1)-in-duced proliferation of airway smooth muscle cells plays critical roles in the development of airway remodeling. Six1 (sine oculis homeobox homolog 1) has been demonstrated to be involved in airway inflammation and remodeling in asthmatic mice. OBJECTIVES:The aim of ...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000505064
更新日期:2020-01-01 00:00:00
abstract:BACKGROUND:In a subgroup of patients with atopic eczema (AE), aeroallergens are relevant eliciting factors. The atopy patch test (APT) was proposed as inflammation model for AE. OBJECTIVE:It was the aim of this study to investigate the effect of pretreatment with 1% pimecrolimus cream (Elidel) on the APT and skin pric...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章,随机对照试验
doi:10.1159/000093249
更新日期:2006-01-01 00:00:00
abstract::In all mammalian species investigated so far, mast cells and basophils are the only cells that synthesize histamine and express plasma membrane receptors that bind IgE with high affinity (Fc epsilonRI). Human basophils and mast cells derive from distinct precursors that originate in the bone marrow and fetal liver and...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章,评审
doi:10.1159/000237670
更新日期:1997-11-01 00:00:00
abstract:BACKGROUND:Partially hydrolyzed cow's milk protein-based formula (pHF) possesses low allergenicity. Here, we investigate the safety and efficacy of oral immunotherapy using pHF for children with cow's milk protein allergy (CMPA). OBJECTIVES:A randomized, double-blind, controlled single-center trial was conducted to ev...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章,随机对照试验
doi:10.1159/000490804
更新日期:2018-01-01 00:00:00
abstract::Kimura disease is a rare disorder of unknown etiology, characterized by the presence of benign subcutaneous granuloma, marked peripheral blood eosinophilia and elevation of the immunglobulin E (IgE) serum level. Here, we present a case of a 12-year-old boy with Kimura disease who had a history of repeated severe influ...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000337777
更新日期:2012-01-01 00:00:00
abstract::Drug hypersensitivity reactions (DHR) are clinically and functionally heterogeneous. Different subclassifications based on timing of symptom appearance or type of immune mechanism have been proposed. Here, we show that the mode of action of drugs leading to immune/inflammatory cell stimulation is a further decisive fa...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章,评审
doi:10.1159/000453265
更新日期:2016-01-01 00:00:00
abstract:BACKGROUND:Microbial components in the environment are potent activators of the immune system with capacity to shift the active immune response towards priming of Th1 and/or Th2 cells. Lipopolysaccharide (LPS), a cell-wall component of Gram-negative bacteria, is extensively present in food products like cow's milk. It ...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000081306
更新日期:2004-11-01 00:00:00
abstract:BACKGROUND:omega-5 gliadin is a major allergen in exercise-induced wheat allergy (EIWA), but it is also implicated in immediate-type reactions to wheat. An ImmunoCAP assay to measure omega-5 gliadin-specific IgE has become available. This study aimed to evaluate this new biological test in wheat allergy diagnosis and t...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000176309
更新日期:2009-01-01 00:00:00
abstract::Serum antibodies reactive with streptococcal cell wall peptidoglycan (PG) and its peptide subunit (synthetic tetra-D-alanine) were measured by enzyme-linked immunosorbent assay (ELISA) in patients with rheumatoid arthritis (RA), juvenile rheumatoid arthritis (JRA), osteoarthritis and acute rheumatic fever (RF) compare...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000236137
更新日期:1992-01-01 00:00:00
abstract:BACKGROUND:Matrix metalloproteinase (MMP)-2, MMP-9 and MMP-13 can degrade type IV collagen which is the major component of the basement membrane zone (BMZ). In bullous pemphigoid (BP), the separation occurs within the BMZ. OBJECTIVE:To evaluate the involvement of MMPs in the pathogenesis of BP, we examined the express...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000090385
更新日期:2006-01-01 00:00:00
abstract:BACKGROUND:Docosahexaenoic acid (DHA) found in fish oil is known to depress inflammation-related mediators. We investigated a novel delivery method of tridocosahexaenoyl-glycerol (DHA-TG). METHODS:BALB/c mice (6-8 weeks old) were primed intraperitoneally with ovalbumin (OVA) and Al(OH)(3) on days 0 and 7, and with aer...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000053645
更新日期:2000-12-01 00:00:00
abstract::Thymulin is a nonapeptide hormone isolated from the thymus gland. It has immunomodulatory effects which have not yet been well defined. Its major actions have been shown to be on T-cells and their immature precursors. In this study, thymulin was tested in vitro for its effect on the release of IL-1 alpha, IL-2, IL-6 a...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000236509
更新日期:1993-01-01 00:00:00
abstract::We report a clinical study comparing the skin test reactivity to recombinant Aspergillus fumigatus allergen 1 (rAsp fI/a) in patients with atopic dermatitis and A. fumigatus sensitisation (n = 15), A. fumigatus-allergic patients with asthma (n = 10) and healthy control subjects (n = 10). All patients sensitised to A. ...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000237123
更新日期:1995-09-01 00:00:00
abstract::The relationship between the IgE load on mast cells and their secretory capacity when challenged with anti-IgE was studied in peritoneal cells obtained from rats of three different strains, Hooded Lister (HL), Wistar Kyoto (WKY) and Sprague-Dawley (SD). IgE was determined cytofluorometrically after labelling with FITC...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000236849
更新日期:1995-03-01 00:00:00
abstract:BACKGROUND:There seems to be an association between paracetamol consumption during late pregnancy and the prevalence of wheezing in infancy and childhood. The aim of the present study is to determine whether the aforementioned association is modified by the presence of asthma in the mother. METHODS:A total of 1,741 ch...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000176304
更新日期:2009-01-01 00:00:00
abstract:BACKGROUND:Anti-inflammatory therapy is a logical approach to slowing the inevitable lung function deterioration in cystic fibrosis (CF) patients. This study's aim was to evaluate inflammatory markers and disease progression in paediatric CF patients chronically treated with azithromycin or low-dose prednisolone. METH...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000453451
更新日期:2017-01-01 00:00:00
abstract:BACKGROUND:Studies have shown that serum total immunoglobulin E (IgE) levels are higher in asthmatics. However, the role of the serum total IgE level, independently from atopy, in adult asthma is not understood. We studied the associations between serum total IgE, the number of sensitizations and the sum of specific Ig...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000342464
更新日期:2013-01-01 00:00:00
abstract::It has been well documented that environmental factors such as antigen-presenting cells and related cytokines can affect the development of T-helper cells. The purpose of this study was to investigate the role of different antigen-presenting cells on T-cell development. Ovalbumin (OVA) combined with complete Freud's a...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000237394
更新日期:1996-12-01 00:00:00
abstract:BACKGROUND:Pre-clinical and clinical studies have evaluated the efficacy of probiotics in allergy. However, predictive in vitro systems for rational strain selection are still missing. METHODS:We developed a novel in vitro screening system for the characterization of probiotics with anti-allergic potential. In this mo...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000343703
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:Exposure to the house dust mite Blomia tropicalis is increasingly being implicated as a major risk factor for asthma exacerbations in sensitized individuals. The objective of this study is to clone and characterize B. Tropicalis allergens in order to better define their role in allergic asthma. METHODS:A la...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章
doi:10.1159/000068375
更新日期:2003-01-01 00:00:00
abstract:BACKGROUND:Reports on oral immunotherapy (OIT) for anaphylactic food allergy are lacking. We investigated the efficacy and safety of peanut OIT for anaphylactic patients. METHODS:We enrolled 22 peanut anaphylactic patients who underwent OIT between 2011 and 2013, all of whom demonstrated anaphylaxis during a baseline ...
journal_title:International archives of allergy and immunology
pub_type: 临床试验,杂志文章
doi:10.1159/000486310
更新日期:2018-01-01 00:00:00
abstract::Toll-like receptors have a critical role in innate immunity and host defence. However their role in allergic disease has not been studied in great detail. The presence of these receptors on mast cells opens up new possibilities concerning the role of Toll-like receptors in the pathogenesis of asthma and atopic dermati...
journal_title:International archives of allergy and immunology
pub_type: 杂志文章,评审
doi:10.1159/000073709
更新日期:2003-10-01 00:00:00