Omalizumab in the treatment of Morbihan syndrome in an adolescent girl - case report and literature review.

Morbihan syndrome (MS) is characterized by solid facial edema, usually related to rosacea or acne vulgaris. The facial edema deforms the patient's features, can impair peripheral vision, and affects quality of life. Its pathophysiology remains unclear. The disease usually has a slow and chronic course. MS most commonly affects middle-aged Caucasian men with rosacea and is rare in people below 20 years of age. MS is a diagnosis of exclusion. There is no standard treatment for MS, though systemic isotretinoin and antihistamines are mainly used. We present the case of an adolescent girl with MS nonresponding to 19 months of isotretinoin treatment with add-on antihistamines. Therapy with monthly administration of omalizumab (anti-IgE) for 6 months was an effective therapeutic option, improving the quality of life. Our case is the second description of omalizumab use in Morbihan syndrome, the first in an adolescent.

Atopic dermatitis and gestational age - is there an association between them? A review of the literature and an analysis of pathology.

Introduction: The connection between prematurity and atopic dermatitis (AD) is an intensively investigated topic with existing knowledge gaps. The last review with a meta-analysis in this field was published in 2018. Since then, there have been great advances in the comprehension of AD pathophysiology. Aim: To update the knowledge and to discuss the recent findings in the field of AD and its association with prematurity in light of the newest publications. Material and methods: An electronic search of Medline was conducted, limited to the last eleven years. The screening of the full version of English articles was performed to ensure the fulfilment of the selection criteria. Results: Thirteen articles met the inclusion criteria, with a total of over 4 million participants. In the majority of the studies (n = 8), prematurity was associated with a lower risk of atopic dermatitis, although there were also publications (n = 5) that did not find an association between these factors. Conclusions: According to this study, prematurity is associated with a lower risk of atopic dermatitis.

Stinging insect identification in children with Hymenoptera venom allergy and their parents.

Introduction: The treatment of Hymenoptera venom allergy (HVA) is based among others on the proper identification of the culprit insect. Aim: To assess the accuracy in identifying stinging insects by children with HVA and their parents. Material and methods: Participants were recruited from a paediatric medical centre. The data on their demographics, sting history and ability of insect identification (based on pictures) were obtained using a questionnaire. The study sample consisted of 102 children with HVA and their parents as well as 98 children without HVA and their parents. Results: The rates of subjects correctly identifying insects in the groups were 91.2%, 92.5%, 78.8%, 82.4%, respectively. When compared to children with HVA, those without HVA were less likely to correctly identify the bee, bumblebee and hoverfly. In this group, the correct identification of the wasp was more common among children living in the countryside. The correct identification of the bee and bumblebee by children without HVA was more common among children living in the city. Conclusions: Some children with HVA and their parents cannot correctly identify stinging insects despite previous life-threatening allergic reactions. The ability to identify stinging insects may depend on the HVA diagnosis and place of residence.

The 2020 update on anaphylaxis in paediatric population.

The objective of the review is to present recent updates on anaphylaxis in paediatric population worldwide. The article summarizes the results of epidemiological studies, diagnostic methods and treatments. We present a new WAO definition of anaphylaxis (2019), which broader criteria excluding dermal symptoms should facilitate faster life-saving adrenaline use. Adrenaline remains the best treatment to manage severe symptoms and to prevent biphasic reactions. There is ongoing effort to increase adrenaline use, such as modified autoinjectors, individual training, and diversified dosing. There are five independent risk factors of lethal anaphylaxis in children, including history of asthma, almost immediate onset of symptoms, unwell appearance, tachycardia and hypotension. We also report improvements in diagnostics, like component-resolved diagnostics, and novel therapies stimulating immunotolerance. We signal the development of ICD-11 with updated coding of anaphylaxis, which corresponds better to clinical observations.

Peanut-induced anaphylaxis in children and adolescents: Data from the European Anaphylaxis Registry.

BACKGROUND: Peanut allergy has a rising prevalence in high-income countries, affecting 0.5%-1.4% of children. This study aimed to better understand peanut anaphylaxis in comparison to anaphylaxis to other food triggers in European children and adolescents. METHODS: Data was sourced from the European Anaphylaxis Registry via an online questionnaire, after in-depth review of food-induced anaphylaxis cases in a tertiary paediatric allergy centre. RESULTS: 3514 cases of food anaphylaxis were reported between July 2007 - March 2018, 56% in patients younger than 18 years. Peanut anaphylaxis was recorded in 459 children and adolescents (85% of all peanut anaphylaxis cases). Previous reactions (42% vs. 38%; p = .001), asthma comorbidity (47% vs. 35%; p < .001), relevant cofactors (29% vs. 22%; p = .004) and biphasic reactions (10% vs. 4%; p = .001) were more commonly reported in peanut anaphylaxis. Most cases were labelled as severe anaphylaxis (Ring&Messmer grade III 65% vs. 56% and grade IV 1.1% vs. 0.9%; p = .001). Self-administration of intramuscular adrenaline was low (17% vs. 15%), professional adrenaline administration was higher in non-peanut food anaphylaxis (34% vs. 26%; p = .003). Hospitalization was higher for peanut anaphylaxis (67% vs. 54%; p = .004). CONCLUSIONS: The European Anaphylaxis Registry data confirmed peanut as one of the major causes of severe, potentially life-threatening allergic reactions in European children, with some characteristic features e.g., presence of asthma comorbidity and increased rate of biphasic reactions. Usage of intramuscular adrenaline as first-line treatment is low and needs to be improved. The Registry, designed as the largest database on anaphylaxis, allows continuous assessment of this condition.

Current practices in diagnosis of Hymenoptera venom allergy in Poland.

Introduction: Hymenoptera venom allergy (HVA) is associated with a high risk of anaphylaxis. Effective treatment of HVA patients requires allergologists' familiarity with the latest HVA recommendations. Aim: Evaluation of current practices in HVA diagnosis in Poland. Material and methods: A survey questionnaire was conducted in 32 HVA centres in Poland. Results: The response rate was 97%. There were 1829 patients evaluated due to HVA in 2015. Sixty six percent (n = 21) of the centres used skin prick tests, out which 90% (n = 19) used 100 µg/ml of the venom extract as the highest concentration. All the centres performed intradermal tests (IDT) and serum specific IgE (sIgE), an initial diagnostic tool in 91% (n = 29). The highest venom concentration in IDT was 1 µg/ml in 75% (n = 24), 0.1 µg/ml in 16% (n = 5), 0.01 µg/ml in 3% (n = 1) and 10 µg/ml in 6% (n = 2). Baseline serum tryptase was assessed in 84% of the centres (n = 27), out of which 53% (n = 17) tested all their patients, whereas 31% (n = 10) checked only those with life-threatening reactions. In case of negative IDT/sIgE, 59% of the centres (n = 19) performed components evaluation, while 19% (n = 6) did the basophil activation test. In case of no identification of the culprit insect and sensitization to both venoms, VIT employed venom with higher sIgE. Conclusions: Most allergology centres in Poland follow HVA guidelines. We identified two inaccuracies in their HVA management including non-adequate venom concentration in IDT and a false belief in correspondence between sIgE concentration and severity of allergic reactions.

Is the clinical manifestation of anaphylaxis in children influenced by the trigger of reaction?

INTRODUCTION: The number of anaphylaxis diagnoses in children is rising, being still based on the clinical picture. AIM: To determine whether triggers of anaphylaxis influence its clinical characteristics in children and adolescents. MATERIAL AND METHODS: The study group included 114 children (5 months-17 years, mean age: 8.0 ±4.8 years), (66%: boys) with the episode of anaphylaxis up to 1 year back. Medical data were entered to the NORA Registry by means of a validated structured on-line questionnaire. RESULTS: Three most frequent triggers of anaphylaxis were: insect venom (47.4%), food (35.1%), drugs (5.3%), with a predominance of food (egg white, cow's milk, nuts and peanuts) in the 0-6 years age group, while insect venom (bee predominance) in the 7-17 years age group (p = 0.016). Clinical manifestations differed between food vs. venom allergic reactions and presented as gastro-intestinal (GI) (61.4%) (p = 0.004), respiratory (RS) (93.9%) (p = 0.036), and cardiovascular (CVS) (74.6%) (p = 0.022) symptoms. Among objective symptoms, vomiting was the most common symptom in the 0-2 years age group (47.1%) (p = 0.006), while hypotension in those aged 7-12 years (40%) (p = 0.010). Severity of symptoms evaluated as Mueller's grade (IV - 74.5%) and as Ring and Messmer's grade (III - 65.8%), depended on the trigger (p = 0.028, p = 0.029, respectively). Life-threatening symptoms occurred in 26 children (fall of the blood pressure - 22%, loss of consciousness - 4.4%). CONCLUSIONS: The clinical manifestation of anaphylaxis in children is both trigger and age dependent, irrespective of the gender. A typical patient with food anaphylaxis was younger, presenting predominantly GI symptoms, while a typical patient with venom anaphylaxis was older, with mostly cardiovascular symptoms.

Intervention in anaphylaxis: the experience of one paediatric centre based on NORA reports.

Introduction: Intramuscular adrenaline administration is the primary intervention in anaphylaxis. Aim: To analyse the data on intervention in children admitted due to anaphylaxis to the tertiary paediatric centre and compare them to the data from the Network for Online-Registration of Anaphylaxis. Material and methods: A validated structured on-line questionnaire was used to collect data concerning the first and second-line intervention in anaphylaxis. The study was conducted in cooperation with the European Anaphylaxis Registry. Results: The study group comprised 114 children (76 boys, 66.87%) aged 5 months-17 years with the predominance of moderate-to-severe anaphylaxis (grade III in Ring and Messmer's, and grade IV in Mueller's scale). In 103 (90.4%) children the first line of medical intervention was provided by medical staff. In the first-line intervention 39 (34.8%) children were given adrenaline. Five (4.4%) children were given the second dose of adrenaline and were admitted to the intensive care unit. In the second-line intervention adrenaline was given to 12 (15.6%) children. In one third it was at least the second reaction to the same trigger. Children treated with adrenaline were older (9.3 ±4.8 years), in comparison to those not treated (7.3 ±4.6 years, p = 0.034). Directly after the episode of anaphylaxis the children got the prescription for the adrenaline autoinjector in 35.1%, emergency training in 7.9%, and counselling on the avoidance of the anaphylaxis trigger in 30.7%. Grade III R&M reaction increased 3-fold the odds of AAI prescription (95% CI: 1.08-8.15). Conclusions: There is a strong need to continue education on proper management of anaphylaxis in children.

Secondary prevention measures in anaphylaxis patients: Data from the anaphylaxis registry.

BACKGROUND: Patients with a history of anaphylaxis are at risk of future anaphylactic reactions. Thus, secondary prevention measures are recommended for these patients to prevent or attenuate the next reaction. METHODS: Data from the Anaphylaxis Registry were analyzed to identify secondary prevention measures offered to patients who experienced anaphylaxis. Our analysis included 7788 cases from 10 European countries and Brazil. RESULTS: The secondary prevention measures offered varied across the elicitors. A remarkable discrepancy was observed between prevention measures offered in specialized allergy centers (84% of patients were prescribed adrenaline autoinjectors following EAACI guidelines) and outside the centers: Here, EAACI guideline adherence was only 37%. In the multivariate analysis, the elicitor of the reaction, age of the patient, mastocytosis as comorbidity, severity of the reaction, and reimbursement/availability of the autoinjector influence physician's decision to prescribe one. CONCLUSIONS: Based on the low implementation of guidelines concerning secondary prevention measures outside of specialized allergy centers, our findings highlight the importance of these specialized centers and the requirement of better education for primary healthcare and emergency physicians.

Venom allergy treatment practices in Poland in comparison to guidelines: next edition of the national audit.

INTRODUCTION: Venom immunotherapy treatment (VIT) is the only causal treatment of hymenoptera venom anaphylaxis, which aims to provide long-lasting immunoprotection against severe reactions to subsequent stings. AIM: To reassess the compliance of VIT procedures in the Polish allergy centres with the European guidelines. MATERIAL AND METHODS: A structured questionnaire survey conducted in all 33 VIT-centres. The response rate was 94%. RESULTS: The ultrarush initial protocol was the most common protocol (71%, n = 22), usually lasting for 3.5 h (50%, n = 7). The most frequent (36%, n = 11) time interval from the initial to the first maintenance dose (MD) was 14 days, ranging from 7 to 35 days. All centres used an MD of 100 µg. The most frequent time interval between subsequent MDs was 4 weeks (58%, n = 18). Five years' of VIT was declared by 71% (n = 22). Before the termination of treatment, more than half of the centres (58%, n = 18) performed sIgE and almost half (42%, n = 13) performed skin tests. To confirm VIT efficacy, few centres (26%, n = 8) conducted the sting challenge. About half of centres provided the patients with an adrenalin auto-injector both at the time of initial diagnostics and at the end of treatment. More than half (55%, n = 17) used antihistamines in all patients. Almost half (45%, n = 14) declared to stop treatment with ß-blockers and almost one fourth (23%, n = 7) discontinued angiotensin-converting-enzyme inhibitors. CONCLUSIONS: In the most important procedures, there is a very high compliance with the guidelines. In the areas where the guidelines are not precise, we observed a large spread of results.

[Chronic cough in children].

Coughing is one of the most common patient complaints at physicians' office. The majority of children experience 5 to 8 episodes of cough lasting about a week throughout the year. Episodes of cough which last longer than 4 weeks, defined as a chronic cough, result in serious parental concern, impaired quality of life, increased number of medical consultations and the adverse effects of inappropriately used medications. Overall, a chronic cough is not only a serious health problem, but also a social one. The article presented below summarizes our current knowledge on the pathophysiology of chronic cough, the latest diagnostics and most recent measurement and monitoring methods as well as recommendations for therapeutic proceedings. In order to emphasize the distinct pathophysiology of chronic cough we use a new term: cough hypersensitivity syndrome. We point out the necessity of the concurrent implementation of more than one cough monitoring method for its more adequate evaluation. This article in addition presents the diagnostic and therapeutic algorithms in the treatment of a chronic cough which shorten the time to make a proper diagnosis, enable the introduction of adequate treatment, and ultimately improve the patients' quality of life. We present new therapeutic strategies, which are based on regulating the activity of vagal afferent nerves and modifying the neurotransmiters' transmission in the brainstem and midbrain.

Simplification of intradermal skin testing in Hymenoptera venom allergic children.

BACKGROUND: The direct comparison between children and adults with Hymenoptera venom anaphylaxis (HVA) has never been extensively reported. Severe HVA with IgE-documented mechanism is the recommendation for venom immunotherapy, regardless of age. OBJECTIVE: To determine the differences in the basic diagnostic profile between children and adults with severe HVA and its practical implications. METHODS: We reviewed the medical records of 91 children and 121 adults. RESULTS: Bee venom allergy was exposure dependent, regardless of age (P < .001). Atopy was more common in children (P = .01), whereas cardiovascular comorbidities were present almost exclusively in adults (P = .001). In the bee venom allergic group, specific IgE levels were significantly higher in children (29.5 kUA/L; interquartile range, 11.30-66.30 kUA/L) compared with adults (5.10 kUA/L; interquartile range, 2.03-8.30 kUA/L) (P < .001). Specific IgE levels for culprit insect venom were higher in bee venom allergic children compared with the wasp venom allergic children (P < .001). In adults, intradermal tests revealed higher sensitivity, accompanied by larger area of skin reactions, regardless of type of venom. At concentrations lower than 0.1 µg/mL, 16% of wasp venom allergic children and 39% of bee venom allergic children had positive intradermal test results. The median tryptase level was significantly higher in adults than in children for the entire study group (P = .002), as well as in bee (P = .002) and wasp venom allergic groups (P = .049). CONCLUSION: The basic diagnostic profile in severe HVA reactors is age dependent. Lower skin test reactivity to culprit venom in children may have practical application in starting the intradermal test procedure with higher venom concentrations.

Types of laryngomalacia in children: interrelationship between clinical course and comorbid conditions.

The aim of this study was to: (1) find out whether laryngomalacia (LM) types are related to clinical course; (2) which patients with LM are at higher risk of other airway malacia [tracheomalacia (TM) and/or bronchomalacia (BM)]; and (3) evaluate the prevalence of LM in our region. Patients with established LM diagnosis and complete clinical and endoscopy records were enrolled. They were classified into different LM types according to classification based on the side of supraglottic obstruction. One hundred ten children were included. The most common LM appearance was type I-58 children, followed by combine types (I + II and I + III)-38. The other airway malacia were found in 47 patients: TM in 31, BM in 10, and TM with BM in 6. Other comorbidities (cardiac, neurological, and genetic disorders) were identified in 30 children. Patients with combine types of LM differ from those with single type of LM in terms of prematurity (13 vs 31 %, p = 0.04) and higher weight on the examination day (p = 0.006). Patients with other airway malacia differ from children with isolated LM in terms of prematurity (40 vs 13 %, p = 0.008), comorbidities (38 vs 19 %, p = 0.024), and lower weight on the examination day (p = 0.014). The prevalence of clinically relevant LM was one in 2600-3100 newborns. Clinical course of LM cannot be anticipated on the basis of solely endoscopic evaluation of the larynx. Comorbidities and prematurity increase the risk of other airway malacia. The prevalence of LM is relatively high in the middle-south part of Poland.

Natural history of Hymenoptera venom allergy in children not treated with immunotherapy.

BACKGROUND: Differences in treatment approach still exist for children after systemic sting reactions. In addition, there are still some doubts about when systemic reactors should be treated with venom immunotherapy (VIT). OBJECTIVE: To determine the rate of sting recurrence and natural history of Hymenoptera venom allergy (HVA) in children not treated with VIT. METHODS: A total of 219 children diagnosed as having HVA who were not treated with VIT were identified in 3 pediatric allergology centers. Survey by telephone or mail with the use of a standardized questionnaire was conducted. The number of field re-stings, subsequent symptoms, and provided treatment were analyzed. RESULTS: A total of 130 of the 219 patients responded to the survey, for a response rate of 59.4%. During the median follow-up period of 72 months (interquartile range, 52-85 months), 44 children (77% boys) were stung 62 times. Normal reactions were most common, occurring in 27 patients (62%). Severe systemic reactions (SSRs) occurred in 8 (18%) of those who were re-stung. The subsequent reaction was significantly milder (P < 0.001), especially in the case of patients re-stung by the same insect (P < .001). None of the children with prediagnostic large local reactions and negative test results for venom specific IgE developed SSRs after re-sting by the culprit insect (P = .03). In children with SSRs, median time from diagnosis to re-sting was 2 times longer than that in those with large local reactions and normal reactions (P = .007). CONCLUSIONS: Most children with HVA not treated with VIT reported milder reactions after a re-sting. Probability of SSR to re-sting increases along with the severity of initial reaction.

Development of Parents' of Children with Hymenoptera Venom Allergy Quality of Life Scale (PoCHVAQoLS).

INTRODUCTION: Venom allergy in children, as a potentially life-threatening disease, may have a considerable impact on the quality of life of the parents of the affected patients. AIM: To present development of the quality of life scale for such parents. MATERIAL AND METHODS: The study sample included 70 parents of children with a history of insect sting reaction, referred for consultations to the allergy centre of the University Children's Hospital of Krakow, Krakow, Poland, in 2000-2010. An initial pool of 56 items divided into 6 domains was prepared. The items with intercorrelations higher than 0.7 were removed from each domain and principal component analysis was conducted for each domain separately to provide a one-dimensional subscale for each domain. Reliability of the subscales was assessed using the Cronbach α coefficient in terms of the Classical Test Theory and with the rho coefficient in terms of the Item Response Theory. The multidimensionality of the scale was tested using multitrait scaling. RESULTS: Two to four items from each domain were selected to constitute five subscales. Both the rho and α coefficients for all the subscales were 0.75 or higher. The multitrait method showed that almost all the items indicated stronger correlations with their own subscale than with other subscales. Correlations between subscales were lower than 0.5. CONCLUSIONS: The presented scale consists of high validity and reliability subscales measuring the quality of life of parents of Hymenoptera venom allergic children. As their quality of life is strongly related to the health of their children, such information may be helpful in everyday clinical practice.

Skin prick tests are not useful for the qualification for venom immunotherapy in children.

Background: The basis for qualification for venom immunotherapy (VIT) is the fulfilment of both the clinical and immunological criteria. Diagnostic tests that confirm the immunological criterion of an IgE-mediated sensitization include skin prick tests (SPT), intradermal tests (IDT), and serum specific IgE (sIgE) for the culprit venom. Objective: This study aimed to assess the usefulness of SPT as the immunological marker in the diagnosis of insect venom sensitization in children with history of systemic reaction (SR) to insect sting evaluated by means of I-IV-grades Mueller's scale. There are no such studies in children. Methods: This cross-sectional study sample consisted of 416 children aged 3-18 years (mean age 10.6 ± 3.8), 76% males, all with the history of a systemic reaction (SR) after a Hymenoptera sting (48% of grade III/IV according to Mueller scale), diagnosed between 1999 and 2019 in the tertiary referral centre. The standard diagnostic tests were used. Specificity, sensitivity, and positive and negative predictive values were computed to assess the diagnostic properties of the clinical tests to distinguish between mild and severe SR. To assess the relative value of an individual test in predicting the qualification to VIT we incorporated the Shapley value (SV). Results: Positive SPT results were found in up to no more than 3% of children; among them less than 1% had only positive SPT and were negative for sIgE and IDT. Approximately 85% of the children had detectable venom sIgE, followed by positive IDT (75%). Almost 70% of children had positive both sIgE and IDT results. In children with grade III/IV reaction, about 80% of children had positive results of both of these tests. sIgE and IDT had sensitivity >0.80, whereas SPT had high specificity (>0.97) in differentiating between mild and severe SR. Relative value of diagnostic tests in predicting qualification to VIT varied between venoms. Bee venom IDT had higher SV (0.052) than sIgE (0.041). In contrast, wasp venom sIgE had higher SV (0.075) than IDT (0.035). Conclusion: SPTs are not an useful immunological marker of venom sensitization in children, and eliminating SPT does not result in a loss of diagnostic accuracy. Limiting diagnostics to venom sIgE and IDT would shorten the procedure and reduce costs. Future studies are needed to determine if venom sIgE as the first line diagnostic test, with IDT added only if the venom sIgE is undetectable, is an optimal diagnostic process.

Cow's milk and hen's egg anaphylaxis: A comprehensive data analysis from the European Anaphylaxis Registry.

BACKGROUND: Cow's milk (CM) and hen's egg (HE) are leading triggers of anaphylaxis in early childhood. The aim of this study was to identify clinical phenotypes and therapeutic measures for CM anaphylaxis (CMA) compared to HE anaphylaxis (HEA) in children up to 12 years of age, based on a large pan-European dataset from the European Anaphylaxis Registry. METHODS: Data from 2007 to 2020 on clinical phenotypes and treatment from 10 European countries, as well as Brazil, were analysed. The two-step cluster analysis was used to identify the most frequent phenotypes. For each trigger, three clusters were extracted based on sex, age, and existence of symptoms in four vitally important systems. RESULTS: Altogether 284 children with CMA and 200 children with HEA were identified. They were characterised as male (69% vs. 64%), infants (65% vs. 61%), with a most frequent grade III of Ring&Messmer classification (62% vs. 64%), in CMA versus HEA, respectively. Respiratory symptoms occurred more often in CMA (91% vs. 83%, p = 0.010), especially in infants (89% vs. 79%, p = 0.008). Cardiovascular symptoms were less frequent in CMA (30% vs. 44%, p = 0.002), in both infants (33% vs. 46%, p = 0.027), and older children (25% vs. 42%, p = 0.021). The clusters extracted in the CMA group were characterised as: (1) mild dermal infants with severe GI (40%), 2. severe dermal (35%), 3. respiratory (25%). While in HEA group: 1. infants with severe GI and/or reduction of alertness (40%), (2) conjunctival (16%), (3) mild GI without conjunctivitis (44%). The severity of the reaction was independent from the amount of ingested allergen protein, regardless of trigger. The first-line adrenaline application differed between the countries (0%-92%, as well as the reasons for not administering adrenaline, p < 0.001). CONCLUSIONS: Despite the similarity of their age, sex, and severity grade, the clinical profiles differed between the CMA and HEA children. Adrenaline was underused, and its administration was country dependent. Further studies are needed to assess to what extent the differences in the clinical profiles are related to matrix and/or absorption effects, and/or the allergen itself.

Food-Induced Anaphylaxis: Data From the European Anaphylaxis Registry.

BACKGROUND: Food is one of the most common elicitors of anaphylaxis, with an increasing incidence over recent years. OBJECTIVES: To characterize elicitor-specific phenotypes and identify factors enhancing the risk or severity of food-induced anaphylaxis (FIA). METHODS: We analyzed data from the European Anaphylaxis Registry applying an age- and sex-matched analysis of associations (Cramer's V) for single food triggers and calculated odds ratios (ORs) for severe FIA. RESULTS: We identified 3,427 cases of confirmed FIA showing an age-dependent elicitor ranking (for children: peanut, cow's milk, cashew, and hen's egg; and for adults: wheat flour, shellfish, hazelnut, and soy). The age- and sex-matched analysis revealed defined symptom patterns for wheat and cashew. Wheat-induced anaphylaxis was more frequently associated with cardiovascular symptoms (75.7%; Cramer's V = 0.28) and cashew-induced anaphylaxis with gastrointestinal symptoms (73.9%; Cramer's V = 0.20). Furthermore, concomitant atopic dermatitis was slightly associated with anaphylaxis to hen's egg (Cramer's V = 0.19) and exercise was strongly associated with anaphylaxis to wheat (Cramer's V = 0.56). Additional factors influencing the severity were alcohol intake in wheat anaphylaxis (OR = 3.23; CI, 1.31-8.83) and exercise in peanut anaphylaxis (OR = 1.78; CI, 1.09-2.95). CONCLUSIONS: Our data show that FIA is age-dependent. In adults, the range of elicitors inducing FIA is broader. For some elicitors, the severity of FIA seems to be related to the elicitor. These data require confirmation in future studies considering a clear differentiation between augmentation and risk factors in FIA.

Hymenoptera venom allergy in humans.

Reactions to Hymenoptera stings may appear as local or systemic responses. According to European data, the incidence of systemic reactions to Hymenoptera stings in the general population is 0.3-7.5%, with the value being 0.3-0.8% in children and 14-43% in beekeepers. The most common systemic allergic (anaphylactic) reactions are caused by honeybees (Apis mellifera), and certain species of wasps in the family Vespidae. Severe generalized immediate-type allergic (anaphylactic) reactions to insect stings are of the highest clinical importance. They affect skin, gastrointestinal tract, respiratory and cardiovascular system. The classification of severity of anaphylactic reaction following insect stings is based on the 4-grade Mueller scale. Crucial in patomechanism of anaphylaxis are specific IgE antibodies directed against the components of the venom, which mediate the activation of mast cells, the main effector cells of anaphylaxis. Therapeutic management in insect venom allergy should be considered in the context of prophylaxis, intervention in case symptoms develop, prevention in the form of venom specific immunotherapy (VIT). There are two steps of VIT 1. Initial dose venom immunotherapy (given according to four protocols which differ the time to reach the maintenance dose) 2. Maintenance dose VIT, usually equal 100 µg. Standard treatment time should span 3-5 years. The main mechanisms of immune tolerance that are initiated by VIT are associated with: 1. a decreased reactivity of effector cells, 2. expansion of T regulatory lymphocytes with IL-10 expression. Therapeutic effectiveness amounts to 90-100% in wasp venom allergy and approximately 80% in bee venom allergy.

[Primary ciliary dyskinesia in highlights of consensus statement. Presentation of pediatric cases]. / Pierwotna dyskinezja rzesek w swietle rekomendacji. Opis przypadków klinicznych u dzieci.

UNLABELLED: Cilia are evolutionarily conserved structures that play a role in diverse cell types. Motile cilia are involved in the most prominent ciliopathy called primary ciliary dyskinesia (PCD), which combines oto-sino-pulmonary symptoms (impaired mucociliary clearance that is important innate defense mechanism), male infertility and in nearly 50% cases situs inversus. Disease is usually inherited as autosomal recessive disorder, concerning mainly outer and/or inner dynein arms of cilia. Diagnosis of PCD requires the presence of characteristic clinical phenotype and confirmation the diagnosis by either identification of specific defect in electron microscopy or other evidence of abnormal ciliary function. The diagnosis of PCD may be delayed, missed or made incorrectly. The first ERS consensus statement which formulates recommendations regarding diagnostic as well as therapeutic approaches to children with PCD, is a very helpful tool in the management of this patients. We present our own experience with three children with PCD diagnosed in our Department. CONCLUSION: in children with clinical symptoms suggesting PCD, even with negative screening tests, the estimation of specific cilia defect in electron microscopy is indicated.