Hereditary angioedema (HAE) in patients with C1 inhibitor deficiency : Cracow register

102 patients suffering from hereditary angioedema (HAE) belonging to 41 families have been studied. 96 patients (95.1%) had type I HAE characterized by low antigenic level and low functional activity of C1 inhibitor. 6 patients (4.9%) had type II HAE related to low functional activity of C1 inhibitor. The swelling of subcutaneous tissue was observed in 74 patients and the bowel muccose in 38 patients. The infusions of C1 inhibitor (Berinert P) were very effective and good tolerated in the therapy of 50 sever angioedema attacks. 15 patients were given long prophylactic treatment with Danazol with full success but side effects were very often in many patients especially in women

Systemic action of nickel

Nickel is the most common substance in our environment. It is known as commonest couse of allergic contact dermatitis. Similar to chromium, cobalt and zinc, nickel may also be a cause of allergy in the upper and lower airways. It may lead to diseases described as "hard metal asthma" and "hard metal rhinitis". The study aimed to determine the prevelance of systemic nickel induced reaction in patients sensitized to nickel and suffering from atopic dermatitis, allergic contact dermatitis and urticaria. In all patients during remission of symptoms were performed patch tests with European Standard contact allergens, serial dilution patch tests and oral provocation test with increasing doses of nickel sulphate. Our studies indicate that oral nickel challenge can provoke symptoms of skin allergy type I and type IV

Bradykinin and oxidative stress in patients with hereditary angioedema due to C1 inhibitor deficiency

Introduction Hereditary angioedema (HAE) is a rare autosomal dominant disease caused by genetic dysfunction of C1 inhibitor (C1‑INH) due to mutations in the SERPING1 gene. The disorder is mediated mainly by bradykinin. The clinical course of the disease is varied and not related to genetic changes. Objectives We aimed to evaluate redox homeostasis of peripheral blood mononuclear cells (PBMCs) in patients with HAE due to C1‑INH deficiency (C1‑INH‑HAE) by measuring the levels of reactive oxygen species (ROS) of PBMCs as well as plasma advanced glycation end products (AGEs) and advanced oxidation protein products (AOPPs). We also aimed to assess the effect of bradykinin on ROS levels. Patient s and methods We enrolled 30 adults with C1‑INH‑HAE and 15 healthy individuals. The levels of ROS were measured by flow cytometry, while the plasma levels of AGEs and AOPPs were determined spectrophotometrically by enzyme‑linked immunosorbent assays. Result s Basal and hydrogen peroxide (H2O2)–induced ROS levels were higher in patients with HAE when compared with controls (P = 0.002 and P = 0.001, respectively), indicating abnormalities in redox homeostasis. Plasma AOPP and AGE levels were similar in both groups. Bradykinin reduced basal and H2O2‑induced ROS generation in PBMCs only in patients with HAE (P = 0.03). Conclusions The higher basal and H2O2‑induced ROS levels in patients with C1‑INH‑HAE indicate redox imbalance. However, by reducing basal and H2O2‑induced ROS levels, bradykinin shows antioxidant action in this disorder

Influence of environment exposures on the frequency of contact allergies in children and adolescents

Contact allergy is detected in every second child with the symptoms of chronic or recurrent eczema, and in every third child the final diagnosis is allergic contact dermatitis. Haptens responsible for the majority of contact sensitizations in children are substances ubiquitous in our environment, e.g. metals, preservatives, fragrances, propolis, and balsam of Peru. Much concern is provoked by the higher rates of sensitization to fragrances in younger children, compared to adolescents, which may be attributed to the higher exposure nowadays of infants and children to fragrant products. On the other hand, a limitation of exposure to the preservatives thimerosal and Kathon CG has resulted in decreased rates of sensitization to these haptens. Altogether, these observations demonstrate that the rates of contact sensitizations in children reflect changes in their environment, and limitations imposed on the use of haptens with strong sensitizing properties, may be an effective tool in the prevention of contact allergy

Konestat alfa, ludzki inhibitor C1 esterazy oraz ikatybant w leczeniu ostrych ataków obrzęku u osób dorosłych z dziedzicznym obrzękiem naczynioruchowym spowodowanym niedoborem inhibitora C1 esterazy. Porównanie opcji terapeutycznych na podstawie przeglądu systematycznego

 INTRODUCTION: Hereditary angioedema (HAE) is a genetic disease caused by C1-esterase inhibitor deficiency, characterized by recurrent attacks of intense, massive, localized subcutaneous oedema that can involve all parts of the body. The aim of this study is a comparison of the clinical effectiveness of conestat alfa, human C1 esterase inhibitor (C1INH), and icatibant in the treatment of acute angioedema attacks in adults with HAE.MATERIALS AND METHODS: A systematic review of literature published up to May 2012 was performed to assess the efficacy and safety of conestat alfa, C1INH, and icatibant in the treatment of acute angioedema attacks in adults with HAE. Databases were searched at MEDLINE (PubMed), EMBASE, and Cochrane. The general search structure was designed as a combination of keywords or synonyms: (hereditary angioedema) AND (conestat alfa OR human C1 esterase inhibitor concentrate OR synonyms OR icatibant). Only randomized clinical studies were selected.RESULTS: Systematic review yielded no clinical trials directly comparing the therapeutic options mentioned. Two randomized clinical trials were found which compared each of the following: conestat alfa, C1INH, and icatibant with placebo. Based on the gathered evidence it was demonstrated that taking any of the medicinal substances mentioned in the treatment of acute angioedema attack results in shorter time to beginning of relief of symptoms, time to minimal symptoms, the probability of the treatment response after 4 hours is increased, and the safety profile is comparable to placebo.CONCLUSIONS: Due to significant heterogeneity of identified trials, the scientific evidence available was insufficient to point out the most effective therapeutic option in the treatment of acute oedemas in HAE.   WSTĘP: Dziedziczny obrzęk naczynioruchowy (HAE) jest chorobą uwarunkowaną genetycznie, spowodowaną niedoborem inhibitora C1 esterazy, charakteryzującą się szybko nasilającymi się nawracającymi obrzękami tkanki podskórnej, które mogą obejmować wszystkie części ciała. Celem niniejszej pracy było porównanie efektywności klinicznej konestatu alfa, naturalnego inhibitora C1 esterazy oraz ikatybantu w leczeniu ostrych napadów obrzęku u osób dorosłych z HAE. MATERIAŁ I METODY: Dokonano systematycznego przeglądu literatury medycznej z zastosowaniem słów kluczowych dotyczących problemu zdrowotnego oraz analizowanych leków, opublikowanej do 8 maja 2012 roku, indeksowanej w bazach danych: MEDLINE (PubMed), EMBASE i Cochrane. Strategia wyszukiwania obejmowała zastosowanie kombinacji słów kluczowych oraz ich synonimów połączonych operatorami logicznymi: (hereditary angioedema) AND (conestat alfa OR human C1 esterase inhibitor concentrate OR synonyms OR icatibant). Spośród odnalezionych tytułów wyselekcjonowano wszystkie randomizowane badania kliniczne. WYNIKI: W wyniku przeglądu systematycznego nie odnaleziono badań klinicznych, w których bezpośrednio porównywano zastosowanie powyższych opcji terapeutycznych. Odnaleziono natomiast po 2 badania, w których porównywano zastosowanie konestatu alfa, naturalnego inhibitora C1 esterazy oraz ikatybantu z placebo. Na podstawie zgromadzonych dowodów wykazano, że podanie każdej z powyższych substancji w terapii ostrego ataku obrzęku skraca czas do rozpoczęcia ustępowania objawów, czas do prawie całkowitego ustąpienia objawów oraz zwiększa prawdopodobieństwo odpowiedzi na leczenie po 4 godzinach od podania, a profil bezpieczeństwa leków jest porównywalny z placebo. WNIOSKI: Ze względu na dużą heterogeniczność badań nie było możliwe wskazanie najskuteczniejszej opcji terapeutycznej w leczeniu ataków obrzęku w HAE na podstawie dostępnych dowodów naukowych.

Antibody reactivity in patients with IgE-mediated wheat allergy to various subunits and fractions of gluten and non-gluten proteins from ω-gliadin-free wheat genotypes

Introduction and objective Gluten proteins (gliadins and glutenins) are polymorphic wheat storage proteins of allergenic properties. Significant differences in chemical composition between both protein groups allow to expect highly specific immunological response of individual subunits and fractions in reactions with IgE sera of people allergic to wheat. The aim of these studies was to identify and characterize the most allergenic gluten proteins (GP) and nongluten proteins (NGP) occurred in two closely related wheat hybrid genotypes. Material and Methods 3xC and 3xN wheat hybrids, which differ strongly in regard of gliadin composition, were analyzed. Seven people manifesting different symptoms of wheat allergy donated sera for the experiment. The technique of immunoblotting after SDS-PAGE was used for identification of allergenic subunits and fractions among GP and NGP. Immunologically active protein bands were visualized by chemiluminescence. Results Great variation of immunodetection spectra was observed. Results of immunoblotting showed LMW glutenins to be of highest, gliadins of medium, while NGP of lowest allergenicity for selected patients. The 43-kDa and 47-kDa LMW glutenin subunits, 40-kDa and 43-kDa γ-gliadin fractions and 49-kDa NGP can be considered as the most immunoreactive among all protein bands [b]separated by SDS-PAGE. Conclusions The observed differentiation of immunodetection spectra allows to model highly specific IgE-binding profiles of allergenic wheat proteins attributed to individual patients with symptoms of gluten intolerance. Highly immunoreactive subunits and fractions among GP and NGP were identified. The observed immunoreactivity of 49 kDa NGP is worth to emphasize, as it has never been reported as wheat allergenic protein before