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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. Patients 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. Results 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.

Background Limited data are available regarding C1 inhibitor (C1-INH) administration and anti–C1-INH antibodies. Objective To assess the incidence of antibody formation during treatment with pasteurized, nanofiltered plasma-derived C1-INH (pnfC1-INH) in patients with hereditary angioedema with C1-INH deficiency (C1-INH-HAE) and the comparative efficacy of pnfC1-INH in patients with and without antibodies. Methods In this multicenter, open-label study, patients with C1-INH-HAE (≥12 years of age) were given 20 IU/kg of pnfC1-INH per HAE attack that required treatment and followed up for 9 months. Blood samples were taken at baseline (day of first attack) and months 3, 6, and 9 and analyzed for inhibitory anti–C1-INH antibody (iC1-INH-Ab) and noninhibitory anti–C1-INH antibodies (niC1-INH-Abs). Results The study included 46 patients (69.6% female; mean age, 38.9 years; all white) who received 221 on-site pnfC1-INH infusions; most patients received 6 or fewer infusions. No patient tested positive (titer ≥1:50) for iC1-INH-Ab at any time during the study. Thirteen patients (28.2%) had detectable niC1-INH-Abs in 1 or more samples. Nine patients (19.6%) had detectable niC1-INH-Abs at baseline; 3 of these had no detectable antibodies after baseline. Of 10 patients (21.7%) with 1 or more detectable result for niC1-INH-Abs after baseline, 6 had detectable niC1-INH-Abs at baseline. Mean times to symptom relief onset and complete symptom resolution per patient were similar for those with or without anti–niC1-INH-Abs. Conclusion Administration of pnfC1-INH was not associated with iC1-INH-Ab formation in this population. Noninhibitory antibodies were detected in some patients but fluctuated during the study independently of pnfC1-INH administration and appeared to have no effect on pnfC1-INH efficacy. Trial Registration clinicaltrials.gov Identifier: NCT01467947.

The severity of allergic symptoms in patients with atopic dermatitis (AD) intensifies when the number of colonies patient’s of Staphylococcus aureus on patents’ skin increases. The basic feature determining the quality of any diagnostic test for S. aureus is its credibility. Performing a test always carries the risk of obtaining false positive and/or false negative results. Furthermore, producing material for microbiological analysis of internal body cavities is sometimes difficult. Therefore, in our study, we compared the results of three tests to determine if their results were mutually compatible and if they confirmed whether S. aureus was present in patients with AD and what was its role in the development of the disease in those patients. Infection with S. aureus was tested in patients with AD and healthy volunteers using the API Staph system. The specific IgE antibodies for staphylococcal enterotoxin A (SEA) and B (SEB) were measured using the UniCAP system. The secretion of IFN-γ, IL-2, IL-13 by peripheral blood mononuclear cells (PBMCs) after stimulation with SEA and SEB were studied with Elispot assay. We found that only certain patients with AD and S. aureus produced antibodies against SEA and SEB in the acute phase of AD. The secretion of IFN-γ was low in patients with exacerbated AD and S. aureus. Testing for the presence of S. aureus in the mucous membrane of the nasal vestibule and skin lesions is not sufficient for complex diagnosis of the role of S. aureus in the pathomechanism of AD. Measuring the presence of antibodies against bacterial components in patients’ serum and the reactivity of patients’ immune cells against these bacterial components is required in order to accurately diagnose this role of S. aureus in a patient.

Acquired angioedema is a rare disease caused by a deficiency of C1 esterase inhibitor with recurrent swelling symptoms. It may occur in the course of lymphoproliferative disorders or autoimmune diseases. Symptoms resemble hereditary angioedema, and the only differentiating features is negative family history, late onset of symptoms and accompanying lymphoproliferative disorder. The aim of the study was to analyze the cases of acquired angioedema. The retrospective analysis of 341 patients from the registry of patients with C1 inhibitor deficiency. Results: We identified 4 patients among 119 with HAE (3.57%) diagnosed in this same period of time 2012-2016 who fulfilled the criteria of acquired edema. In two cases the primary reason of angioedema was lymphoproliferive disease, in two monoclonal gammapathy of unknown reason. We analyzed also the results of laboratory tests C4, C1 inhibitor, C1q. In all cases the face was dominated localization. After the treatment of primary lymphoproliferive disease, in two cases, we observed total remission of angioedema. Only one patient with gammapathy require treatment with C1 inhibitor during the attacks. In these case we observed both plasma deriver, and recombinant C1 inhibitor were effective.

SERPING1 genotyping of subjects suspicious for hereditary angioedema due to C1-INH deficiency (C1-INH-HAE) is important for clinical practice as well as for research reasons. Conventional approaches towards the detection of C1-INH-HAE-associated SERPING1 variants are cumbersome and time-demanding with many pitfalls. To take advantage of the benefits of next-generation sequencing (NGS) technology, we developed and validated a custom NGS platform that, by targeting the entire SERPING1 gene, facilitates genetic testing of C1-INH-HAE patients in clinical practice. In total, 135 different C1-INH-HAE-associated SERPING1 variants, out of the approximately 450 reported, along with 115 negative controls and 95 randomly selected DNA samples from affected family members of C1-INH-HAE index patients, were included in the forward and reverse validation processes of this platform. Our platform's performance, i.e. analytical sensitivity of 98.96%, a false negative rate of 1.05%, analytical specificity 100%, a false positive rate equal to zero, accuracy of 99.35%, and repeatability of 100% recommends its implementation as a first line approach for the genetic testing of C1-INH-HAE patients or as a confirmatory method. A noteworthy advantage of our platform is the concomitant detection of single nucleotide variants and copy number variations throughout the whole length of the SERPING1 gene, moreover providing information about the size and the localization of the latter. During our study, 15 novel C1-INH-HAE-related SERPING1 variants were detected.

[b]Abstract Introduction and objective[/b]. 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. [b]Materials and method.[/b] 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. [b]Results[/b]. 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. Conclusion [/b] 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.

Background: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) may affect health-related quality of life (HRQoL). A specific HRQoL questionnaire for adult patients with C1-INH-HAE, the HAE-QoL, has recently been developed in Spain. Objective: The objective of this study was to perform a cross-cultural validation and psychometric study of the HAE-QoL in an international setting. Methods: Cross-cultural adaptation of the Spanish HAE-QoL draft version and an international rating phase with experts were performed. The resultant version of the HAE-QoL, a clinical questionnaire, and Short Form 36-item Health Survey Version 2.0 (SF-36v2) were pilot tested internationally. Item reduction was based on both descriptive and exploratory factor analysis. Psychometric properties were assessed. Results: Cross-cultural adaptation of the HAE-QoL was performed in 18 countries. The draft version of the HAE-QoL was pilot tested in 332 patients, and accurate data were obtained from 290 patients from 11 countries. The reduction process resulted in a new version with 25 items and 7 dimensions (treatment difficulties, physical functioning and health, disease-related stigma, emotional role and social functioning, concern about offspring, perceived control over illness, and mental health). Strong psychometric properties were observed (Cronbach´s α 0.92; test-retest reliability 0.87). Convergent validity showed mild to moderate correlations with SF-36v2 physical and mental component summaries (0.45 and 0.64, respectively) and with SF-36v2 dimensions (P < .004). HAE-QoL scores discriminated significantly among severity groups (median: asymptomatic 133.5 vs severe 84.0; P < .001); between patients with and without long-term prophylaxis (median: 101 vs 90; P = .001); and between patients with and without psychiatric and/or psychological care (median: 74 vs 103; P ≤ .001). Conclusions: The HAE-QoL, currently available in 18 languages, showed good reliability and validity evidence. Fil: Prior, Nieves. Hospital Universitario Severo Ochoa; España Fil: Remor, Eduardo. Universidad Autónoma de Madrid; España Fil: Pérez Fernández, Elia. Fundacion Hospital Alcorcon; España Fil: Caminoa, Magdalena. Instituto de Investigacion Hospital Universitario la Paz ; España Fil: Gómez-Traseira, Carmen. Instituto de Investigacion Hospital Universitario la Paz ; España Fil: Gayá, Francisco. Instituto de Investigacion Hospital Universitario la Paz ; España Fil: Aabom, Anne. Odense University Hospital; Dinamarca Fil: Aberer, Werner. Medical University; Austria Fil: Betschel, Stephen. Saint Michael's Hospital; Canadá Fil: Boccon Gibod, Isabelle. Joseph Fourier University; Francia. Grenoble University Hospital; Francia Fil: Bouillet, Laurence. Joseph Fourier University; Francia. Grenoble University Hospital; Francia Fil: Bygum, Anette. Odense University Hospital; Dinamarca Fil: Csuka, Dorottya. Semmelweis University; Hungría Fil: Farkas, Henriette. Semmelweis University; Hungría Fil: Gomide, Maria. Universidade de Sao Paulo; Brasil Fil: Grumach, Anete. Universidade de Sao Paulo; Brasil Fil: Leibovich, Iris. Chaim Sheba Medical Center; Israel Fil: Malbrán, Alejandro. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud "Dr. C. G. Malbrán"; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina Fil: Moldovan, Dumitru. Mures County Hospital; Rumania Fil: Mihaly, Eniko. Mures County Hospital; Rumania Fil: Obtulowicz, Krystyna. Jagiellonian University; Polonia Fil: Perpén, Cecilia. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud "Dr. C. G. Malbrán"; Argentina Fil: Peveling Oberhag, Adriane. University Medical Center Mainz; Alemania Fil: Porebski, Grzegorz. Jagiellonian University; Polonia Fil: Chavannes, Celine Rayonne. Saint Michael's Hospital; Canadá Fil: Reshef, Avner. Chaim Sheba Medical Center; Israel Fil: Staubach, Petra. University Medical Center Mainz; Alemania Fil: Wiednig, Michaela. Medizinische University; Austria Fil: Caballero, Teresa. Instituto de Investigacion Hospital Universitario la Paz ; España

Gluten is the product of a chemical bond of wheat prolamin proteins (glia- dins and glutenins) in an aqueous me- dium. IgE mediated gluten allergy can be induced either by gluten as an in- gredient in foods or wheat prolamines present in the air. The aim of the study was clinical analysis of 13 patients, who demonstrated elevated levels of gluten specific IgE and identification of the most allergenic protein fractions from several samples of wheat using serum of examined subjects. Clinical analysis showed the occupational allergy to gluten in the form of rhinitis, asthma and airborne dermatistis in 9 subjects, whose symptoms disappeared during isolation from occupational exposure despite the use of a normal diet. In case of 4 patients with severe forms of chronic urticaria and atopic dermatitis, who are also allergic to grass pollen at the same time, the introduction of a gluten-free diet resulted in improvement of health conditions. The study of wheat protein fractions revealed a significant polymorphism dependent on the wheat sample. In the protein fractions, low and high molecular glutenin fractions, and alpha, beta, gamma, and omega-gliadins were separated. It has been shown that the strongest immunogenic effect causes omega-5 gliadin fraction. The removal of this fraction resulted in reduction of skin reactivity evaluated by skin prick test in the studied patients.

Nasal mucosa cytology is an additional examination useful to differentiate chronic rhinitis in allergological and laryngological diagnostics, including chronic sinusitis. The aim of the study was to estimate the cytological picture in a group of patients with the highest percentage of squamous epithelia, suggesting the atrophic rhinitis. The analysis was carried out on the basis of cytological results performed in 3055 patients diagnosed because of chronic rhinitis. Among these patients, in 31 individuals the higher percentage (30% - 76%) of squamous cells was found. In six patients, the clear predominance of squamous cells over ciliated and goblet cells (3:1) was reported. Only in three patients the increased percentage in eosinophils was stressed (3%, 5% and 9% respectively). No basic and metaplastic cells were observed in the studied cytograms. The squamous cells occurred probably as a result of normal mucosa damages during the different inflammatory changes, both allergic and non-allergic, the impact of toxic agents, irritants (including nasal drops). The higher percentage of squamous cells in the nasal cytogram could have explained the low effectiveness of local inflammatory treatment in these patients.

Background We analyzed the clinical response of pediatric and adolescent hereditary angioedema (HAE) patients to pdC1-INH in the International Multicenter Prospective Angioedema C1-INH Trials (I.M.P.A.C.T.) 1 and 2. Methods Patients included in this post hoc analysis of prospectively collected data were between 10 and 18 yr old with type I or II HAE and a documented history of abdominal or facial attacks. Patients received a single injection of pdC1-INH concentrate (Berinert®, CSL Behring, Marburg, Germany) 20 U/kg. Efficacy end-points were time from the administration of study drug to onset of symptom relief and time to complete relief of all symptoms. Results Seven pediatric patients were included in I.M.P.A.C.T.1 with only 1 attack analyzed per patient. Median time to onset of relief was 0.42 h and to complete resolution was 8.08 h. No patient experienced a worsening of symptoms during the 0–4-h assessment period. Nine patients who experienced a total of 115 attacks were included in the analysis of I.M.P.A.C.T.2. Abdominal attacks were rated as ‘severe’ more frequently than were other types of attacks. The number of attacks per patient ranged from 2 to 42, and study participation ranged from 1 to 38 months. Median times to onset of symptom relief and to complete symptom resolution were 0.49 h and 14.1 h, respectively. Of 4 treatment-emergent adverse events in both studies, only 2 were considered related to treatment. Conclusions Study results showed that outcomes with pdC1-INH treatment of HAE in pediatric patients are comparable with outcomes in adults.

Background Hereditary angioedema caused by C1 esterase inhibitor deficiency is a rare disorder. Objective To compare the efficacy of pasteurized C1 esterase inhibitor concentrate (Berinert, CSL Behring) at intravenous doses of 10 or 20 U/kg body weight with placebo in the treatment of single, acute abdominal or facial attacks in patients with hereditary angioedema. Methods This was a randomized, double-blind, placebo-controlled study in 125 patients with type I or II hereditary angioedema. The primary outcome was time from start of treatment to onset of symptom relief. Secondary outcomes were time to complete resolution, proportion of patients with worsened intensity of angioedema symptoms between 2 and 4hours after treatment, and number of vomiting episodes within 4 hours. Results Median time to onset of relief was significantly shorter with C1 esterase inhibitor concentrate at a dose of 20 U/kg than with placebo (0.5 vs 1.5 hours; P = .0025), whereas with 10 U/kg, the time to onset of relief was only slightly shorter than with placebo (1.2 vs 1.5 hours; P = .2731). Compared with placebo, the reduction in time to onset of relief was greatest for severe attacks (0.5 vs 13.5 hours). The secondary outcomes consistently supported the efficacy of the 20 U/kg dose. C1 esterase inhibitor concentrate was safe and well tolerated. No seroconversions were observed for HIV, hepatitis virus, or human B19 virus. Conclusion C1 esterase inhibitor concentrate given intravenously at a dose of 20 U/kg is an effective and safe treatment for acute abdominal and facial attacks in patients with hereditary angioedema, with a rapid onset of relief.

Gliadins and glutenins--the main components of wheat gluten--are highly complex and polymorphic proteins of wheat kernels. They are also allergenic proteins causing a range of food allergies. We hypothesized that the diversity of chemical structures and properties may relate to the diversification of immunoreactive properties of various subunits and fractions of gluten proteins. In the present study we used IgE sera, obtained from patients manifesting different symptoms of wheat allergies, for immunobloting analysis, to prove the specificity of immunological reaction between IgE antibodies and individual gliadins and glutenins, separated by SDS-PAGE. The results suggest that patients have different characteristics of IgE binding to the separated protein subunits and fractions. Sera of two patients showed strong binding of omega-gliadins, while alpha-gliadins and LMW glutenin subunits of MW = 43 and 45 kDa were highly allergenic for two other subjects in the test group of patients.

Nasal exfoliative cytology is a complementary tool in diagnostics of allergic (AR) and non-allergic (NAR) rhinitis. The aim of the study was to determine the usefulness of the nasal cytology in patients sensitive to common inhalant allergens with positive SPT(+) and negative SPT(-) (Skin Prick Tests) depending on the symptoms of intermittent and persistent rhinitis. The study was performed in a group of 285 patients treated in the Department of Allergology University Hospital in Krakow, suspected on AR in 2008-2010. The patients were made a smear test of inferior nasal concha. The samples were stained using the eosin-hematoxylin method and examined under a light microscope (1000x). Patients were divided into two groups: SPT(+) (144 patients) and SPT(-) (141 patients). Depending on the percentage of obtained eosinophils each group was divided into three subgroups: 0-2%, 3-20%,20%. In the most percentage of patients with 3-20% of eosinophils in nasal cytology were found, in both studied groups (SPT)(+) and (SPT)(-), while the highest percentage of eosinophils (20%) was observed in the bigger group of patients with SPT(+), than with SPT(-). The number of patients with eosinophils20% in the SPT(+) group was higher in patients with persistent symptoms (NS differences), while in the SPT(-) group, the number of patients with intermittent symptoms in the subgroup20% of eosinophils statistically prevailed (p0.001). The mean percentage of eosinophils in both groups was comparable, while the statistically significant differences were found considering the distinguished subgroups. In intermittent SPT(+) group the most sensitizing allergens were pollen grains (birch or grass pollen), while the patients with persistent AR symptoms were mainly sensitive the house dust mites. The mean percentage of eosinophils in an exfoliative cytology correlated significantly with allergic rhinitis symptoms and SPT results, the most evident relationship was found between higher level of eosinophils and the patients with confirmed AR diagnosis on the basis of positive SPT, manifesting the intermittent symptoms.

The symptoms of pollen allergy in the European population occur in a period of increased pollen precipitation, and take the form of allergic rhinitis and conjunctivitis, bronchial asthma, contact urticaria, and food allergy. Diagnosis in addition to medical history, takes into account the positive results of skin tests and elevated allergen-specific IgE antibodies (specific IgE) in serum. These studies are considered to be objective diagnostic tests confirming the diagnosis of pollen allergy. Not in every case there is a correspondence of symptoms and results of diagnostic tests, which puts into question the accuracy of the diagnosis of pollen allergy. The aim of this study was to test the characteristics of patients with oral allergy syndrome on the background of all patients with pollen allergy and evaluation of the diagnostic value of history, skin tests and specific IgE levels in the diagnosis of patients with pollen allergy and oral allergy syndrome. A retrospective analysis of the cases of 85 patients with a diagnosis of pollen allergy and the 30 patients with OAS was performed. In our study the most common sensitizing allergen in patients with OAS was birch pollen, while patients showing no symptoms of OAS were equally sensitive to timothy and birch pollen. The main food responsible for the presence of the OAS in the mechanism of cross-allergy to pollen was an apple. Among patients with OAS we did not show significantly higher incidence of polyvalent allergies. It was shown, however, that there is a tendency that the maximum concentration of allergen-specific IgE causing clinically significant symptoms, ie in line with the pollen season, is higher in the OAS patients than in the absence of OAS. Further research is needed using new diagnostic methods, which would predict future symptoms after eating certain foods in particularly endangered patients with pollen allergy.

Background Hereditary angioedema (HAE) is a disease characterized by recurrent tissue swelling affecting various body locations. Recent literature shows that patients with frequent attacks may benefit from long-term prophylaxis. This study evaluated the safety and prophylactic effect of weekly administrations of recombinant C1INH (rhC1INH). Methods Patients with a history of HAE attacks occurring ≥every 2 weeks received a once weekly administration of 50 U/kg rhC1INH. Hereditary angioedema attack history was collected at screening. Breakthrough attacks during the study were recorded at each visit. Following a 2-week run-in period, HAE patients received 8 weekly rhC1INH administrations and were followed-up for an additional 6 weeks. Efficacy was evaluated by comparing the HAE attack incidence during the treatment period to the historical attacks over the previous 2 years. Safety evaluation was based on clinical laboratory and adverse events (AEs) reports. Results The 25 participants reported a mean of 0.9 attacks/week over the past 2 years. The mean breakthrough attack rate during the treatment period was 0.4 attacks/week (95% CI 0.28–0.56). A total of 30 treatment-emergent-AEs were reported in 13 patients, all mild to moderate. One patient died from a laryngeal attack 25 days after last study drug administration. The only possible drug related AEs reported were dry mouth, dizziness and anxiety in one patient and hypotension in another. There were no allergic AEs and no neutralizing antibodies observed. Conclusions Weekly administrations of 50 U/kg rhC1INH appeared to reduce the frequency of HAE attacks and were generally safe and well tolerated.

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.

Contact allergy is the most frequent type of allergy, affecting 26-40% of all adults and 21-36% children. The gold standard in the diagnosis of contact allergy is patch test.To study the influence of the range and composition of patch test series on the efficacy of the diagnostic procedure.Retrospective analysis of the frequency of positive reactions among patients diagnosed with patch tests at our Department during 2 periods: From December 2003 to March 2005, patients were tested with a series of 9 substances plus white petrolatum as the negative control. From April 2005 to July 2008, the series was expanded to 21 substances, while petrolatum was removed.In the analyzed period, 1379 patients were tested with 9 substances plus petrolatum (group referred to as "G9") and 682 patients with 21 substances ("G21"). In G9, at least one positive reaction was observed in 343 (24.9%, 95% CI: 22.6-27.2%) patients, as compared to 376 (55.1%; 95% CI: 51.4-58.7%) in G21 (p0.0001). The increase in the number of tested substances from 9 to 21 led to significant increase in the mean number of positive reactions per one patient (0.34 in G9 versus 0.90 in G21; p0.0001). We have not observed any positive reaction to white petrolatum.Patch testing with more extensive test series increases the chance for the detection of patient's sensitizations. As we have not observed any positive reaction to white petrolatum, using the vehicle as negative control does not seem to offer any advantage.

Background: Patch testing is an essential procedure in the investigation of eczema in children. Objectives: To analyse the frequency of contact hypersensitivity and allergic contact dermatitis among Polish children with eczema. Patients/methods: During an allergy screening programme involving 9320 children aged 7 and 16 years, 12.6% reported symptoms of chronic/recurrent eczema. From this group, a representative sample of 229 eczema children underwent patch testing: 96 children aged 7 years and 133 teenagers aged 16 years. Patch testing was with 10 allergens: methylchloroisothiazolinone/methylisothiazolinone (MCI/MI), nickel sulfate, mercury ammonium chloride, thimerosal, cobalt chloride, potassium dichromate, lanolin, fragrance mix I, Myroxylon pereirae (balsam of Peru), and colophonium. Results: 49.4% tested children were found patch test (PT) positive. 43.8% of 7 year olds with eczema were PT positive, with sensitization to nickel sulfate (30.2%), thimerosal (10.4%), cobalt chloride (8.3%), fragrance mix I (7.3%), MCI/MI (6.3%), potassium dichromate (6.3%), M. pereirae (3.1%), mercury ammonium chloride (2.3%), and colophonium (1.0%). 52.6% teenagers were PT positive, with sensitization to nickel sulfate (23.3%), thimerosal (27.8%), cobalt chloride (10.5%), potassium dichromate (6.0%), mercury ammonium chloride (2.3%), M. pereirae (1.5%), and MCI/MI (0.8%). The final diagnosis of allergic contact dermatitis was confirmed in 36% of 7 year olds and 26% of 16 year olds. Conclusions: Every second child with eczema is PT positive, whereas every third child is finally diagnosed with allergic contact dermatitis.

Introduction Hereditary angioedema (HAE) is caused by mutations in the C1inh gene, leading to dysfunction of the C1-esterase inhibitor (C1-INH). C1-INH interacts with MASP-1 and MASP-2 proteases, participating in the mannan-binding lectin (MBL) pathway of complement activation. The aim of the study was to investigate the contribution of possible changes in MBL/MASP-2 complex activity and Helicobacter pylori, hepatitis B virus (HBV), and hepatitis C virus (HCV) infections to the severity and frequency of clinical symptoms of HAE. Materials and Methods The study was performed in 65 patients with HAE and 113 healthy persons. The parameters measured were C1-INH, C4, MBL concentration and MBL/MASP-2 complex activity, and serological markers of H. pylori, HBV, and HCV infection. Scores for the frequency and severity of HAE symptoms were determined. Results HAE scores were significantly higher in patients whose C1-INH activity did not exceed 10% than in patients with activity of 10-52% (p=0.016). No significant differences were found in the median levels of MBL concentration and MBL/MASP-2 complex activity between patients and the control group. There was a slight association between contact with H. pylori in patients and HAE symptom score (p=0.052, not significant). Adult patients showed a 2.6-times higher frequency of anti-HBc than the general population. HBV DNA was negative in anti-HBc(+) patients. Conclusions These results suggest that the MBL complement activation pathway itself does not contribute to the frequency of angioedema attacks. Infections with H. pylori and HBV may slightly influence the disease score (not significant).

Nickel is knows as the most common cause of allergic contact dermatitis, as well as diffuse eczema, allergic rhinitis and bronchial asthma. The mechanism of contact allergy to nickel is well known. In spite of numerous investigations, the mechanism of systemic allergy to nickel is still not clear. 22 patients with positive patch tests to nickel were analyzed. On basis of clinical symptoms the patients were divided into two groups: 1. with contact allergy dermatitis to nickel--8 patients 2. with systemic allergy to nickel (allergic rhinitis and/or diffuse eczema--14 patients. The control group included non-atopic patients with negative patch test to nickel--6 patients. 10 ml of blood were taken from each patient and peripheral mononuclear blood cells (PMBC) were isolated. In PBMC culture, NiSO4 and PHA were stimulated. The control group was non-stimulated cells. The supernatants were collected after 3 and 6 days of culture and the levels of cytokines IL-5, 4 and IFNgamma were measured (ELISA). The concentration of IFNgamma in supernatants from stimulated as well as non-stimulated cells from patients with contact allergy to nickel was higher in comparison to the control group. The concentration of IL-5 in this group was low. There was an increase in the production of IFNgamma and IL-5 after NiSO4 stimulation in patients with systemic allergy to nickel. The higher concentration of IFNgamma in the same groups of patients investigated was in supernatants from the third day of PBMC culture were compared to the sixth day. After 3 and 6 days of culture, the concentration of IL-4 (ELISA) was below detection level in all supernatants analyzed.IFNgamma plays an essential role in the mechanism of developing of contact allergy to nickel; and IFNgamma as well as IL-5 play a role in the mechanism of developing systemic allergy to nickel. The third day of PBMC culture is more reliable for IFNgamma estimation.

Background: Patients with hereditary C1-inhibitor deficiency have recurrent attacks of angioedema, preferably treated with C1-inhibitor concentrate. A recombinant human C1-inhibitor (rHuC1INH) was developed, derived from milk from transgenic rabbits. This study was undertaken to investigate the effects of rHuC1INH in the treatment of acute angioedema attacks in patients with a hereditary C1-inhibitor deficiency. Study Design and Methods: Patients with hereditary C1-inhibitor deficiency were treated with rHuC1INH (at a dose of 100 U/kg) within 8 hours after onset of an acute attack. Time to initiation of relief and time to minimal symptoms were reported by both the patient and the treating physician. Results: Thirteen severe angioedema attacks in 9 patients with hereditary C1-inhibitor deficiency were treated with rHuC1INH. There was rapid improvement of clinical conditions for all attacks, with approximately 80 percent of treated patients experiencing symptom relief within 2 hours and minimal symptoms within 12 hours. There were no drug-related adverse events or immunogenic reactions to C1-inhibitor or rabbit proteins. Conclusion: The transgenically produced rHuC1INH was successfully used in the treatment of acute angioedema attacks in patients with hereditary C1-inhibitor deficiency.

Eosinophilia in peripheral blood occurs in allergic as well as non-allergic diseases. In the research there were 26 patients with eosinophil level above 5% encountered in leukocyte smear of peripheral blood encountered. Patients were split into 3 groups: patients with allergic atopic disease (ANN), patients with non-atopic (contact dermatitis) disease and patients with upper airways infection and/or parasitic disease. In the researched groups there were no statistically significant differences of ECP level in serum and of ECP/Eo ratio detected. There were also correlations between ECP and ECP/Eo ratio for every researched group analyzed. The highest correlation was observed in patients with contact dermatitis and in non-atopic patients in general. Initial results show uselessness of applied research methods in the routine differential diagnosis of eosinophilia.

Hereditary angioedema (HAE) is a rare condition, resting on attacks of edema in various localizations, potentially life-threatening if in facial, laryngeal, pharyngeal or gastrointestinal area. The disease is caused by deficiency or impaired activity of C1 inhibitor, therefore C1 inhibitor infusion is the the essential treatment and the only efficient method in an acute attack of HAE. Nowadays C1 inhibitor applied in our patients is obtained from human plasma, what restricts the availability of the drug and carries relevant risks. After many years of research it came to the synthesis of the recombinant protein with features of human C1 inhibitor. Its first usage in Poland took place-in two HAE patients with severe edema in 2004. The course and efficiency of this treatment is reported in the paper. Recombinant human C1 inhibitor occurred efficient and safe in presented case of severe angioedema.

The study is aimed to determine the importance of type I and type IV allergy in eczema caused by allergy to nickel. The study was performed at 55 patients (42 women, 13 men, aged 16-58 yrs) suffering from hand dermatitis (19 cases), disseminated eczema (22 cases) and atopic dermatitis (14 cases) with positive skin patch test to 2.5% nickel sulphate. In each patients history of illness was analyzed, total serum IgE level (tIgE) was estimated and specific IgE (sIgE) for nickel and also absolute blood eosinophils and basophils counts were estimated for the evaluation of the atopy features. In each patient patch skin test with different nickel sulphate dilutions were performed as well as skin prick tests with different dilutions of nickel sulphate. The following oral provocation tests were carried out with the nickel sulphate in doses 0.56 mg, 1.12 mg, 2.24 mg, 5.6 mg and 11.2 mg. The test was stopped at the dose provoking the symptoms of illness. Positive family history, the increased tIgE serum level as well as absolute counts of eosinophils and basophils were present in some patients with atopic and contact dermatitis and they were not useful in differential diagnosis of this forms of skin allergy. Skin patch test with different concentrations of nickel sulphate was helpful to establish the degree of contact sensitivity in all patients. The oral provocation test with different dose of nickel sulphate also provoked symptoms in some patients in each observed groups, but the reaction to the lowest dose was observed only in patients with atopic dermatitis. Specific IgE to nickel as well as skin prick testing also with different dilutions of nickel sulphate are not useful in the diagnosis of nickel allergy. In the all examined patients they were negative. It seems that both types of allergy (type I and IV) may take part in the patho-mechanism of atopic and contact skin allergy with alternate prevalence of one of its depending on patient condition.

The aim of the study was estimation of the IgE value, circulating immunocomplexes and the activity of complement system in allergic contact as well as atopic dermatitis in patients with skin nickel hypersensitivity. The study was done in 30 patients in the age range of 18-53 yrs suffering from allergic contact dermatitis (15 patients) and atopic dermatitis (15 patients) with positive skin patch test to nickel. The concentration of total IgE as well as specific IgE to mites, Phleum, Betula, Artemisia and the concentration of circulating complexes in the serum of patients were estimated. The activity of C1inh and CH50 was estimated in plasma of the patients. The results of the study indicated an increase of concentration of circulating immuno-complexes in 80% of the patients of both groups, an increase of total IgE in 50% of patients with atopic dermatitis and in 25% of the patients with contact dermatitis. There were no changes in the activity of C1inh and the value of CH50 in both groups of patients.

The studies were performed in 32 patients in the age range of 17-58 years suffering from bronchial asthma in the exacerbation period of their illness. 16 patients suffered from IgE related/atopic and 16 from IgE non IgE related/nonatopic bronchial asthma. The complement haemolytic activity (CH50) and the activity of C1 inhibitor (C1 INH) were measured in the plasma. In 60% of patients with IgE related asthma and in 45% of patients with non IgE related asthma we stated decrease of CH50. The activity of C1INH also was decreased in 80% of patients with atopic asthma and in 40% of patients with nonatopic asthma. The observed changes were not related to aspirin tolerance as well as to IgE mediation of their illness. Control examinations of these parameters repeated during remission of asthma symptoms in 11 patients with low values during the exacerbation of the illness (in 5 patients with atopic and in 6 with non-atopic asthma) revealed an increase of their values; however without features of normalisation.