Food-induced immediate response of the esophagus in pediatric eosinophilic esophagitis.

BACKGROUND: Food-induced immediate response of the esophagus (FIRE) is a new phenomenon that has been described in eosinophilic esophagitis (EoE) patients. It is suspected when unpleasant symptoms occur suddenly on contact of the triggering food with the esophageal surface and recur with repeated exposures. It can often be mistaken for pollen-food allergy syndrome (PFAS) and solid food dysphagia. Data on FIRE is limited to one survey study and case reports, and there are no screening studies conducted on either adults or children with EoE. In this study, we aimed to screen children aged ≥7 years old with EoE for FIRE. METHODS: Demographic data were collected from medical records. A questionnaire about FIRE was applied to all participants. Skin prick tests were done on suspected patients to identify the triggering foods. FIRE is defined as suitable clinical symptoms with suspected food allergen exposure. RESULTS: A total of 78 patients (74.4% male, median age: 13.5 years) were included. Unpleasant and recurrent symptoms distinct from dysphagia with specific foods were reported in 16.7% of the patients, all of whom had concomitant allergic rhinitis (AR). The symptoms described by almost all patients were oropharyngeal itching and tingling (PFAS: 15.3%) excluding only one patient reporting retrosternal narrowing and pressure after specific food consumption (FIRE: 1.2%). CONCLUSIONS: Although definitive conclusions regarding the true prevalence of FIRE cannot be made, it does not seem to be common as PFAS. However, it deserves questioning particularly in the presence of concurrent AR and/or PFAS in children with EoE.

Long-Term Experience with Anaphylaxis and Desensitization to Alglucosidase Alfa in Pompe Disease.

BACKGROUND AND OBJECTIVE: Pompe disease (PD) is an inherited lysosomal storage disease that progresses with glycogen accumulation in many tissues, due to the deficiency of the acid-alpha glucosidase enzyme. Recombinant alglucosidase alfa (rhGAA) is the only disease-specific treatment option, in the form of enzyme replacement therapy (ERT). Anaphylaxis can develop with rhGAA. There is no study evaluating anaphylaxis and its management in PD in the long term. We aimed to evaluate the development of anaphylaxis and rapid drug desensitization (RDD) with rhGAA in children with PD. MATERIALS AND METHODS: All children diagnosed and followed up in our institution with PD over 12 years between January 2009 and September 2021 were evaluated for development of anaphylaxis and RDD with rhGAA from medical records. RESULTS: Fourteen patients, 64% of whom were female and diagnosed with PD (1 juvenile, 13 infantile types) during the study period included in the study. The median age at diagnosis was 3.2 months (1-40 months). The median follow-up time of the patients was 20 months (1-129 months). Thirteen patients were given rhGAA, one died before ERT. Four (30.8%) patients developed moderate to severe anaphylaxis, and RDD was applied with rhGAA. A total of 390 RDDs have been performed so far without any serious breakthrough reactions during all RDDs. CONCLUSIONS: Anaphylaxis with rhGAA is not rare and RDD with rhGAA is safe and effective in the long term.

A novel pediatric Eosinophilic Esophagitis Symptoms and Adaptive Behavior Scale for different ages: GaziESAS v2.0.

BACKGROUND: High-quality scales (HQS) suitable that measure symptoms and adaptive behaviors (AB) with proven validity and reliability are needed for different age groups of children with eosinophilic esophagitis (EoE). OBJECTIVE: To develop a high-quality pediatric EoE symptoms and AB scale for different age groups. METHODS: Children (7-11 years), teens (12-18 years), and parents of 2-18-year-old children with EoE were included. A HQS should have encompassed: the identification of domain and item generation; content validity (CnV) and field test for construct validity (CsV) and reliability. Convergent validity (CgV) was examined for CsV. Correlations between the Pediatric Eosinophilic Esophagitis Symptom Score, version 2.0 (PEESS v2.0) and Gazi University Eosinophilic Esophagitis Symptoms and Adaptive Behavior Scale (GaziESAS) version 2.0 (v2.0) were examined for CgV. Reliability was determined through internal consistency (Cronbach-α) and test-retest reliability (intraclass correlation coefficients: ICC). RESULTS: Nineteen children, 42 teens, and 82 parents completed the study. GaziESAS v2.0 was composed of 20 items with two main domains: symptoms (subdomains: dysphagia and nondysphagia) and AB. CnV indexes were excellent for all items. The CgV varied from good to excellent correlation (r = 0.6 to r = 0.9). GaziESAS v2.0 showed good reliability (Cronbach-α >0.7 and ICC >0.6). CONCLUSION: GaziESAS v2.0 is the first pediatric HQS that measures the frequency of symptoms and AB in EoE within the last month with separate forms for children, teens, and parents.

Oropharyngeal Dysphagia in Children with Eosinophilic Esophagitis.

Dysphagia is the most troublesome symptom of eosinophilic esophagitis (EoE). This study aimed to investigate oropharyngeal dysphagia in children with EoE and possible related factors. Children with a definite diagnosis of EoE were included in the study. Medical and feeding histories were recorded. A disease control level was determined for each child. An oral structure examination, the Turkish version of the Mastication and Observation Evaluation (T-MOE), the Pediatric version of the Eating Assessment Tool-10 (PEDI-EAT-10) and the 3-oz water swallow test were applied in screening for oropharyngeal dysphagia. Fifty-two children participated in the study. Oropharyngeal dysphagia took the form of abnormal swallowing (PEDI-EAT-10 score ≥ 4) and increased aspiration risk (PEDI- EAT-10 score ≥ 13) in 51.9% and 25.0% of the children, respectively. Seven children failed the 3-oz water swallow test. Abnormal swallowing and aspiration risk were significantly higher in children with prolonged mealtimes, impaired chewing function, and uncontrolled disease (p < 0.05). Chewing function was the most important risk factor for abnormal swallowing and increased aspiration (R2 = 0.36, R2 = 0.52, p < 0.001, respectively). Oropharyngeal dysphagia is common in children with EoE and associated with increased aspiration risk in a subpopulation. Uncontrolled disease, prolonged mealtimes, and impaired chewing function may provide clues for oropharyngeal dysphagia in EoE.

Is the Pediatric Eosinophilic Esophagitis Symptom Score v2.0 reliable for telemedicine?

BACKGROUND: Eosinophilic esophagitis (EoE) is a chronic immune-mediated disease. Telemedicine is a healthcare technology used when a patient is separated by distance. The reliability of the Pediatric Eosinophilic Esophagitis Symptom Score, version 2.0 (PEESS v2.0) for telemedicine applications, has not been studied yet. Therefore, we aimed to evaluate the reliability of PEESS v2.0 for telemedicine. METHODS: We sent a telesurvey using questionnaires via electronic telecommunication as the telemedicine method. Children with EoE and their parents were asked to complete PEESS v2.0 with the telesurvey method (unsynchronized with the physician) and attend in-person visits one week apart. Intraclass correlation (ICC), Wilcoxon, and Bland-Altman tests were used as reliability analyses. Reliability was defined as a strong agreement between the measurements in ICC ≥ 0.8 and a p-value of ≤0.05 and no statistically significant difference between the scores of the two methods in the Wilcoxon and Bland-Altman analyses, i.e. a p-value of >0.05. RESULTS: The total scores of children and parents were higher in in-person visits than in the telesurvey (Wilcoxon tests, p ≤ 0.05). Bland- Altman analysis showed that the mean difference in total scores between the two methods was significant for both children and parents (p ≤ 0.05). ICC levels for the children and parent scores for the entire group ranged from 0.595 to 0.763 (moderate agreement). DISCUSSION: Unsynchronized telesurvey use of PEESS v2.0 is unreliable both for children and parents. We suggest testing the reliability of chosen telemedicine methods before using them in clinical and research practice.

Primary Eosinophilic Gastrointestinal Diseases Beyond Eosinophilic Esophagitis in Children.

OBJECTIVES: There are many unknowns about primary eosinophilic gastrointestinal disease (EGID) in childhood. The aim of this study is to provide data about the frequency, management, control level, and prognosis of well documented primary EGID in childhood. METHODS: This study was conducted in children who underwent endoscopy and/or colonoscopy at a single center over 10-year period up to August 2018. Primary EGID was diagnosed after exclusion of secondary EGID and classified as eosinophilic gastritis (EG), eosinophilic enteritis (EE), eosinophilic gastroenteritis (EGE: eosinophilic gastritis with eosinophilic enteritis) and eosinophilic colitis (EC) according to histopathological evaluation. The pathological number of eosinophil counts were accepted as >30 hpf for gastric mucosa in 5 hpf area, ≥20/hpf for duodenal, jejunal, and ileal mucosa, >50/hpf for right colonic mucosa, >35/hpf for transverse colonic mucosa, and >25/hpf for left colonic mucosa. Presenting symptoms, signs, management, follow-up, disease control level, and remission were analyzed. Remission is defined if the patient is controlled with all clinical, endoscopic/colonoscopic, and histopathologic parameters without any treatments or diet for at least a year. RESULTS: During the study period, 7457 biopsies were taken in 8262 endoscopy and/or colonoscopy procedures. Primary and secondary EGID frequencies were found 0.23% (n = 17 patients) and 0.1% (n =8 patients) per procedure with biopsy in children, respectively. Endoscopy/colonoscopy procedures were not able to performed in 9 patients because of short follow-up period (n = 6) or patients leaving follow-up (n = 3). Nine of the primary EGID patients had esophageal eosinophilia (EsE) at the time of diagnosis, 5 of them were previously managed as EoE. The median follow-up period of primary EGID patients excluding the ones without a control endoscopy/colonoscopy procedure was 3.35 years (min-max: 1.1-9.0 years). Proton pump inhibitors (PPI) were the most frequently used treatment alone or in combination with diet, systemic and/or topical corticosteroids. Disease control was evaluated in 8 of 17 patients and it was uncontrolled in 4, partially controlled in 1, and controlled in 3 patients. Remission was achieved in 2 patients. CONCLUSIONS: The frequency of primary EGID beyond eosinophilic esophagitis (EoE) in children is low. It may be difficult to achieve control in children with primary EGID in the long-term follow-up.

Toward clinically applicable biomarkers for asthma: An EAACI position paper.

Inflammation, structural, and functional abnormalities within the airways are key features of asthma. Although these processes are well documented, their expression varies across the heterogeneous spectrum of asthma. Type 2 inflammatory responses are characterized by increased levels of eosinophils, FeNO, and type 2 cytokines in blood and/or airways. Presently, type 2 asthma is the best-defined endotype, typically found in patients with allergic asthma, but surprisingly also in nonallergic patients with (severe) asthma. The etiology of asthma with non-type 2 inflammation is less clear. During the past decade, targeted therapies, including biologicals and small molecules, have been increasingly integrated into treatment strategies of severe asthma. These treatments block specific inflammatory pathways or single mediators. Single or composite biomarkers help to identify patients who will benefit from these treatments. So far, only a few inflammatory biomarkers have been validated for clinical application. The European Academy of Allergy & Clinical Immunology Task Force on Biomarkers in Asthma was initiated to review different biomarker sampling methods and to investigate clinical applicability of new and existing inflammatory biomarkers (point-of-care) to support diagnosis, targeted treatment, and monitoring of severe asthma. Subsequently, we discuss existing and novel targeted therapies for asthma as well as applicable biomarkers.

Diagnosis and management of NSAID-Exacerbated Respiratory Disease (N-ERD)-a EAACI position paper.

NSAID-exacerbated respiratory disease (N-ERD) is a chronic eosinophilic, inflammatory disorder of the respiratory tract occurring in patients with asthma and/or chronic rhinosinusitis with nasal polyps (CRSwNP), symptoms of which are exacerbated by NSAIDs, including aspirin. Despite some progress in understanding of the pathophysiology of the syndrome, which affects 1/10 of patients with asthma and rhinosinusitis, it remains a diagnostic and therapeutic challenge. In order to provide evidence-based recommendations for the diagnosis and management of N-ERD, a panel of international experts was called by the EAACI Asthma Section. The document summarizes current knowledge on the pathophysiology and clinical presentation of N-ERD pointing at significant heterogeneity of this syndrome. Critically evaluating the usefulness of diagnostic tools available, the paper offers practical algorithm for the diagnosis of N-ERD. Recommendations for the most effective management of a patient with N-ERD stressing the potential high morbidity and severity of the underlying asthma and rhinosinusitis are discussed and proposed. Newly described sub-phenotypes and emerging sub-endotypes of N-ERD are potentially relevant for new and more specific (eg, biological) treatment modalities. Finally, the document defines major gaps in our knowledge on N-ERD and unmet needs, which should be addressed in the future.

Diagnostic challenges of childhood asthma.

PURPOSE OF REVIEW: Diagnosis of asthma in childhood is challenging. Both underdiagnosis and overdiagnosis of asthma are important issues. The present review gives information about challenging factors for an accurate diagnosis of childhood asthma. RECENT FINDINGS: Although underdiagnosis of asthma in childhood has always been the most important diagnostic problem, overdiagnosis of asthma has also been increasingly recognized. This is probably due to diagnosis of asthma based on symptoms and signs alone. Demonstration of variable airflow obstruction by lung function tests is the most common asthma diagnostic tests used in practice and is therefore strongly recommended in children who can cooperate. Recently, an asthma guideline combining the clinical and economic evidences with sensitivity and specificity of diagnostic procedures was developed to improve accuracy of diagnosis and to avoid overdiagnosis. This guideline provided an algorithmic clinical and cost-effective approach and included fractional exhaled nitric oxide measurement as one of the diagnostic tests in addition to lung function. SUMMARY: Diagnosis of asthma in children should be made by combining relevant history with at least two confirmatory diagnostic tests whenever possible. Diagnosis based on short-period treatment trials should be limited to young children who are unable to cooperate with these tests.

Survey of physicians' attitudes toward the use of magnesium sulfate for acute asthma exacerbations in Turkey.

BACKGROUND: Magnesium sulfate (MgSO4) is considered to be a well-tolerated, inexpensive, and effective medication for severe asthma exacerbations. We surveyed physicians who treat patients with asthma to evaluate the current knowledge and usage of MgSO4. METHODS: A self-administered questionnaire was e-mailed to members of the Turkish National Society of Allergy and Clinical Immunology and was distributed to participants of its annual congress. RESULTS: Of the 456 respondents (mean age: 36.53 ± 9.11 years), 42.3% reported to use MgSO4 in asthma exacerbations in their practice, and 48.7% agreed that MgSO4 was included in asthma guidelines. The best known indication was that it was useful only in patients refractory to bronchodilators and systemic corticosteroids (67.7%). The two most common reasons to use MgSO4 were to shorten hospital stay (94.7%), and prevent admission to intensive care unit (80.3%). The respondents mostly used MgSO4 in the treatment of severe or life-threatening exacerbations. Thirty-nine percent believed that MgSO4 had no effect on discharge period, and 29% of them marked minor side effects. The most common reason for not using MgSO4 was inexperience (36.5%). Having an academic affiliation (OR = 3.20, p < 0.001), the number of asthmatic outpatients seen per month (OR = 1.82, p = 0.007), and more recent graduation from medical school (≤1991) (OR = 0.23, p < 0.001) were associated with the use of MgSO4 in the multivariate analysis. CONCLUSION: Even though the effect of MgSO4 in acute severe asthma is well known, only half of the physicians dealing with asthma patients have used it in their practice. Education and encouragement in this regard are necessary.

Long-term follow-up of re-sting reactions in children with moderate to severe venom hypersensitivity.

UNLABELLED: Few data exists about re-sting reactions and their prognosis in children with moderate to severe venom hypersensitivity. The reasons behind not consenting to or prematurely ending venom immunotherapy (VIT) and the preparedness of children who refused or quit VIT for future moderate-severe systemic reaction (SR) to re-stings have not been studied. Data on children with moderate to severe SR after Hymenoptera stings was collected for a 17-year period using our database. A standardized questionnaire was administered to patients who accepted to be interviewed at the clinic. These patients were evaluated in terms of their preparedness for future moderate-severe SR to re-stings. A total of 55 children, 75 % of whom commenced on VIT, were included in the analysis. Different reasons exist for not consenting to VIT; the most common of which is living at a distance from the allergy center. There were no differences in terms of the number of re-stung patients (27.7 and 27.2 %, respectively) and moderate-severe SR (60 and 16.6 %, respectively) between children who prematurely ended or who did not consent to VIT and children who completed VIT. Sixty-four percent of the children who refused or discontinued VIT were not prepared for future moderate-severe SR to re-stings. CONCLUSION: Long-term prognosis for re-sting reactions is good in children with moderate to severe SR to venoms. Some of the reasons behind refusing or discontinuing VIT may be related to quality of life issues. Preparedness of children who refused or discontinue VIT in emergencies is very low.

Association between thyroid autoimmunity and recurrent angioedema in children.

BACKGROUND: The association between thyroid autoimmunity (TA) and idiopathic isolated angioedema (or angioedema without urticaria) has not been evaluated in either children or in adults up until now. We, therefore, aimed to investigate underlying or concomitant TA and/or autoimmune thyroid disease in children diagnosed with recurrent idiopathic angioedema. METHODS: Children who were consecutively diagnosed with recurrent idiopathic histaminergic acquired angioedema (IH-AAE) between January 2011 and January 2014 constituted the case group. A standard diagnostic and therapeutic algorithm was applied to all the patients with recurrent IH-AAE. Thyroid autoantibodies and thyroid function tests were measured in all the patients with recurrent IH-AAE and in healthy control groups. Prophylaxis with an antihistamine was started for patients with frequently recurrent IH-AAE. RESULTS: Eighty consecutive children with recurrent IH-AAE (49 boys; median age, 8.3 years) and 80 healthy children (39 boys; median, 8 years) were enrolled in this prospective, case-control study (p > 0.05 for age and sex). The IH-AAE group was significantly different than the control group with respect to TA (13.7% versus 2.5%, respectively; p = 0.009) but was similar with respect to autoimmune thyroid disease (3.7% versus 0%, respectively; p = 0.2). The median follow-up of the recurrent IH-AAE group was 34 months (range, 12-45 months). Patients with recurrent IH-AAE with and those without TA were not different with respect to either the need or the duration of antihistamine prophylaxis (p > 0.05 for both). CONCLUSIONS: Recurrent IH-AAE may be related to or associated with TA and/or autoimmune thyroid diseases in some children. However, exploring to see whether this association is a causal link or just an epiphenomenon deserves further investigation and longer follow-ups.

Does self-monitoring by means of symptom diaries improve asthma control in children?

OBJECTIVE: Keeping symptom diaries on a regular basis may facilitate the execution of symptom-based action plans, enhance the patients' adherence to treatment program and finally allow better asthma control. We hypothesize that disease control in children who keep symptom diaries regularly would be better compared to children who do not keep symptom diaries regularly. METHODS: Asthmatic children, aged between 6 and 17 years, who were monitored at least 2 years at our outpatient clinic and examined at least twice within the last year, were enrolled in this study. The patients were assigned to the following two groups: group I included the patients who keep symptom diaries regularly and group II included the patients who do not keep symptom diaries regularly. Asthma control parameters of patients during the last year were investigated. The number of asthma attacks require systemic corticosteroid use, the frequency of emergency department (ED) admissions and the number of attacks requiring hospitalization, forced expiratory volume in 1 s (FEV1) values and asthma control test (ACT) scores were compared. RESULTS: 89 (26.2%) of 340 patients included in the study were identified to keep a symptom diary regularly. Although age (p = 0.20) and sex (p = 0.48) did not differ significantly between the groups, regular use of anti-inflammatory drug was found to be significantly higher in group I (p < 0.001). When all of the study parameters were compared using a multivariate analysis, the number of systemic corticosteroid use, ED visits, attacks requiring hospitalization and ACT scores and FEV1 did not differ significantly between the groups (p > 0.05 in all of the parameters). CONCLUSIONS: Keeping a symptom diary on a regular basis in asthmatic children was shown to have neither beneficial effect on the day-to-day asthma control nor a decrease in the future risk of asthma control.

Pediatricians manage anaphylaxis poorly regardless of episode severity.

BACKGROUND: No information exists on how the knowledge or the practice of pediatricians regarding anaphylaxis episodes vary with episode severity. The aim of this study was to assess and compare pediatrician knowledge on the management of mild and severe anaphylaxis using clinical scenarios and to determine factors that affect their decisions. METHODS: A questionnaire consisting of eight questions on the diagnosis and management of anaphylaxis was distributed at two national congresses. A uniform answer box including possible response choices was given below each question, and respondents were asked to check the answers that they thought appropriate. The management of mild and severe anaphylaxis was examined using two clinical case scenarios involving initial treatment, monitoring, and discharge recommendations. RESULTS: Four hundred and ten questionnaires were analyzed. The percentage of pediatricians who correctly answered all questions on the management of mild and severe anaphylaxis scenarios was 11.3% and 3.2%, respectively. Pediatricians did significantly better with initial treatment, but they were less knowledgeable with respect to observation time and discharge criteria in the mild anaphylaxis case scenario compared with the severe one (both P < 0.001). Multiple logistic regression analysis identified only working in an emergency department or intensive care unit as significantly predicting correct diagnosis of anaphylaxis among pediatricians (P = 0.01, 95% confidence interval: 0.11-0.57). No pediatrician-related factors predicted physician knowledge on the management of anaphylaxis. CONCLUSIONS: Pediatricians have difficulty with different steps in managing mild and severe anaphylaxis. Their deficiencies in management may result in failure to prevent recurrences of mild anaphylaxis and may increase mortality in severe anaphylaxis.

Systemic Reactions to Skin Prick Test with Food Allergens in Children.

OBJECTIVE: Although skin prick tests (SPTs) are generally considered safe, limited studies have specifically evaluated reactions related to SPTs with all allergens. In contrast to these studies, our aim is to exclusively evaluate systemic reaction (SR) occurrences with food allergens during SPTs in children. MATERIALS AND METHODS: All patients who underwent skin prick and/or prick-to-prick (PtoP) tests with food allergens at our clinic between January 2010 and January 2020 were included in the study. The occurrence of SR during SPTs was evaluated based on patient records. RESULTS: The study included 1852 patients, with 57% males and a median age of 31 months (1-210). Skin tests were most commonly conducted for the indication of atopic dermatitis (29.3%). During the study, 11.2% had repeat SPTs for tolerance and a new allergy diagnosis. No local reactions or SRs occurred during SPTs. Among those with PtoP tests, 3 patients (0.16%) experienced SRs-1 had anaphylaxis (0.05%), the rest had angioedema. Legumes and sea bass caused these reactions. In patients with severe index reactions and those who underwent PtoP testing, SR development was significantly higher (P < .001 for both), and anaphylaxis occurrence was significantly higher among those undergoing PtoP testing compared to prick testing alone (P = .03). CONCLUSION: The prevalence of both SR and anaphylaxis due to SPT with food allergens was found to be quite low in children. However, it is important to consider the possibility of SR development before conducting SPT with food allergens, especially in patients who will undergo the PtoP test and those with severe index reactions. Cite this article as: Karkin G, Ertoy Karagol HI, Polat Terece S, Köken G, Yapar D, Bakirtas A. Systemic reactions to skin prick test with food allergens in children. Turk Arch Pediatr. 2024;59(1):54-59.

A real-life study on acquired skills from using an adrenaline autoinjector.

BACKGROUND: Training programs performed by allergists have increased the ability of patients' recognition and management of anaphylaxis. We aim to investigate the permanence of effect of an anaphylaxis training program and to determine the factors affecting it beyond training given by allergists. METHODS: Children and/or their caregivers who had been prescribed an adrenaline autoinjector at least 1 year before were invited to take part in the study. The knowledge about anaphylaxis was assessed using a questionnaire and the skills were practically tested. RESULTS: Sixty-four (50 caregivers/14 children >12 years of age) of 80 patients who accepted the invitation were included in the study. Fifty-nine patients obtained the autoinjector after initial prescription. Among them, 42 (71%) still had the device at the time of the study. The most common reason for not having the autoinjector was no longer feeling it was necessary (54.6%). Of the cases, 39.4% were competent in autoinjector use. There was a significant relation between adrenaline autoinjector competency and regular allergy visits (p = 0.010), believing that it is necessary (p = 0.04), having an adrenaline autoinjector (p = 0.003), and previous history of severe anaphylaxis (p = 0.010). Autoinjector competency score decreased as time elapsed from the last visit (rho = -0.382; p = 0.002) and the first instruction (rho = -0.317; p = 0.01). Regular visits (p = 0.009) and history of severe anaphylaxis (p = 0.007) were found as independent factors having an effect on adrenaline autoinjector competency. CONCLUSIONS: Training of patients/caregivers by allergists does not guarantee the permanence of acquired skills on anaphylaxis in the long run. Regular follow-up visits should be fostered.

Angioedema without urticaria in childhood.

BACKGROUND: There has been no separate study investigating angioedema without urticaria (Aw/oU) exclusively in children so far. The purpose of this study was to investigate the frequency, clinical presentation, etiology, management and follow-up of Aw/oU in children. METHODS: This is a prospective study that included all consecutive patients with a history of Aw/oU referred to our clinic between January 2011 and May 2012. A standard diagnostic and therapeutic algorithm was applied to all patients. RESULTS: The frequency of Aw/oU was found to be 1.6% during the study period. An etiological factor could be found in only 45 patients (49%). The causes of Aw/oU were infection (21%), allergy (14%), thyroid autoimmunity (TA)-related (8%) and nonsteroid anti-inflammatory drug hypersensitivity (6%), and idiopathic angioedema (51%). There was no hereditary type I, II or acquired type of angioedema or rare syndromes associated with Aw/oU. The median follow-up was 16 months (range: 12-30 months). Antihistamine prophylaxis was initiated at therapeutic doses in 20 patients with frequently recurrent angioedema due to idiopathic and euthyroid TA-related Aw/oU for 3 months. These patients responded to antihistamine prophylaxis for 3 months. Four patients relapsed after cessation of prophylaxis at the end of 3 months. Antihistamine prophylaxis was prolonged to 6 months in three patients and to 9 months in one patient. CONCLUSIONS: Angioedema without urticaria in children is a rare condition and no etiology can be identified in half of them. Antihistamine treatment alone is sufficient, and prognosis is good in recurrent non hereditary cases in a short-term follow-up period.

Management preferences of pediatricians in moderate and severe acute asthma.

OBJECTIVE: To assess and compare management preferences of physicians for moderate and severe acute asthma based on case scenarios and to determine the factors influencing their decisions. METHODS: A questionnaire based on the Global Initiative on Asthma (GINA) guideline and comprising eight questions on management of acute asthma was delivered to participants of two national pediatric congresses. Management of moderate and severe acute asthma cases was evaluated by two clinical case scenarios for estimation of acute attack severity, initial treatment, treatment after 1h, and discharge recommendations. A uniform answer box comprising the possible choices was provided just below the questions, and respondents were requested to tick the answers they thought was appropriate. RESULTS: Four-hundred and eighteen questionnaires were analyzed. All questions regarding moderate and severe acute asthma case scenarios were answered accurately by 15.8% and 17.0% of physicians, respectively. The initial treatment of moderate and severe cases was known by 100.0% and 78.2% of physicians, respectively. Knowledge of the appropriate plan for treatment after 1h was low both for moderate (45.0%) and severe attacks (35.4%). Discharge recommendations were adequate in 32.5% and 70.8% of physicians for moderate and severe attacks, respectively. Multiple logistic regression analysis revealed that working at a hospital with a continuing medical education program was the only significant predictor of a correct response to all questions regarding severe attacks (p = .04; 95%CI, 1.02-3.21). No predictors were found for information on moderate attacks. CONCLUSIONS: Pediatricians have difficulty in planning treatment after 1 hour both for moderate and severe asthma attacks. Postgraduate education programs that target physicians in hospitals without continuing medical education facilities may improve guideline adherence.

Anaphylaxis in infancy compared with older children.

Although there has been increasing data on pediatric anaphylaxis, information about anaphylaxis in the 1st year of life is scarce. This study provides detailed information on clinical signs and symptoms of anaphylaxis in the 1st year of life. A retrospective review was performed of our pediatric allergy database between 2007 and 2011. Children who met the diagnostic criteria of anaphylaxis were included. They were categorized as "infant" if they were ≤12 months of age at the time of anaphylactic reaction and "children" if >12 months. There were 104 patients (60 male and 44 female subjects) who met the diagnosis criteria of anaphylaxis. From the 104 cases of anaphylaxis, 23 (22.1%) were infants. Boys (p = 0.043), atopic eczema (p = 0.049), and history of food allergy (p < 0.001) were significantly higher in infants than in children with anaphylaxis. Severe anaphylaxis was less frequent in infants than in children (p = 0.04). There was no significant difference between infants and children considering cutaneous and respiratory symptoms (p > 0.05 for both) but persistent vomiting was (p = 0.023). Irritability, persistent crying, and somnolence are the signs which are difficult to interpret in infants with anaphylaxis. Within these signs, irritability, persistent crying, and somnolence were present in 69.6, 43.5, and 26.1% of infants, respectively. Blood pressure was measured in 5 infants (21.7%) compared with 44 children (54.3%; p = 0.005). Four children (4.9%) required more than one epinephrine treatment, but no infant did. Median observation periods were 4 hours in both groups (p = 0.087) and no biphasic reactions occurred in either. Food (p < 0.001) was significantly more and drugs (p = 0.015) were a less frequent cause of anaphylaxis in infants than in children. Anaphylaxis in infants is not rare but many signs of anaphylaxis are overlooked and still undertreated.

Short-term follow-up of episodic wheeze and predictive factors for persistent wheeze.

Wheezing phenotypes may not be stable, and phenotype transitions may occur over time. This study investigates the natural course of episodic viral wheezing (EVW) and identifies the risk factors that predict persistence of wheezing through short-term follow-up. The medical records of children <3 years of age at hospital admission and classified as having EVW were retrospectively screened by two pediatric allergists. A total of 236 children were classified as having EVW between January 2010 and February 2011. The median follow-up period was 19.5 months. At the end of follow-up, wheezing persisted in 145 patients (61.4%) and changed to multiple-trigger wheeze in 37 patients (15.7%). Factors associated with persistent wheeze were age at initial wheezing <24 months, anti-inflammatory treatment at the time of diagnosis, history of severe episodic wheeze in the previous year, wheezing requiring systemic steroids in the previous year, frequent episodic wheeze, parental asthma, and a positive modified asthma predictive index (mAPI) for major criteria (each, p < 0.05). The logistic regression analysis revealed three independent risk factors: anti-inflammatory treatment at the time of diagnosis (p = 0.03), history of severe episodic wheeze in the previous year (p = 0.02), and a positive mAPI for major criteria (p = 0.02). The initial wheezing phenotype may vary over time. History of severe episodic wheeze in the previous year, anti-inflammatory treatment at the time of diagnosis, and a positive mAPI for major criteria predicts persistent wheeze at short-term follow-up.