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.

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.

Neuropsychiatric adverse drug reactions induced by montelukast impair the quality of life in children with asthma.

INTRODUCTION: Montelukast-induced neuropsychiatric adverse drug reactions (ADRs) have been reported in retrospective studies. This study aimed to reveal the neuropsychiatric ADRs triggered in patients taking montelukast due to asthma in real time, and to evaluate the effect of these ADRs on quality of life (QoL). METHODS: Patients, ages 3-18 years, taking montelukast for the first time and their parents were included. Ages 3-7 years were defined as the preschool and ages 8-18 years as the school-age group. At the beginning of the study and at the end of the second week of treatment, the neuropsychiatric complaint assessment questionnaire and the KINDL QoL scale were administered to patients and their parents. The effect of ADRs on the decrease in QoL was evaluated by multivariable logistic regression. RESULTS: Neuropsychiatric ADRs were reported in 78 (62.4%) of 125 patients, who recovered when the drug was discontinued. Temperamental behavior, nightmares and sleep disorders occurred significantly more often in both groups compared with pretreatment (p < 0.001 for each). In both groups, except in the child-reported family relationships subscale in the school-age group, significant decreases were found in both child and parent proxy-reported QoL total/sub-scores compared with pretreatment (p˂0.001 for each). It was found in the evaluation that the overall QoL of those experiencing ADRs in both age groups was more affected. (Child-reported QoL ORpreschool age=2.66, p = 0.048; ORschool-age=5.95, p = 0.027; parent-proxy QoL ORpreschool age =3.52, p = 0.010, ORschool-age=6.43, p = 0.027). CONCLUSIONS: Montelukast-induced neuropsychiatric ADRs are more frequent than reported in the literature and negatively impact children's QoL.

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.

The effect of the COVID-19 pandemic on long-term treatment compliance and disease control in children with persistent asthma.

BACKGROUND: No long-term data exists on asthma treatment compliances (ATC), exacerbations (AE), and control (AC) during the COVID-19 pandemic in children. This study aimed to evaluate ATC, AE, AC and the related factors among children with persistent asthma (PA) within the first year of the pandemic Methods. Children aged 6-18 years with PA who were under regular inhaled corticosteroid treatment for at least a year prior to the first COVID-19 case in Türkiye were included. Data on AE and AC were collected from medical files. Factors affecting ATC and AC as well as COVID-19 history were assessed by means of a questionnaire. RESULTS: The study included 247 cases. COVID-19 was detected in 14.5% of them. In the first year of the pandemic, ATC decreased to 56.7% and the most common reason was the absence of asthma symptoms. There was a significant improvement in AC (p < 0.001). The number of upper respiratory tract infections (URTI) and AE were significantly decreased during the first year of the pandemic (p < 0.001). COVID-19 infection, smoking in the household, school attendance, a family member working outside the home, house dust mite sensitization or allergic rhinitis had no significant effect on AC (p > 0.05). Regression analysis determined that children who did not have any URTI had 2.4 times better AC compared to those who had (p= 0.02; %95 CI: 1.1-5.4). CONCLUSIONS: Although ATC decreased significantly in the long-term in the first year of the pandemic, significant improvement was observed in AE and AC compared to the previous year, which was related only to not having URTI.

First successful concomitant therapy of immune tolerance induction therapy and desensitization in a CRIM-negative infantile Pompe patient.

OBJECTIVES: Enzyme replacement therapy (ERT) with alglucosidase alfa (rhGAA) has changed the fatal course of infantile Pompe disease, however, development of anti rhGAA antibodies and infusion-associated reactions (IAR) restrict the tolerability and effectiveness of the treatment. CASE PRESENTATION: We describe a successful concomitant immune tolerance induction (ITI) and desensitization protocols in a cross-reactive immunologic material (CRIM) negative 7-month-old male patient. At the age of 5 months and eighth dose of the ERT, the patient developed IAR and his rhGAA specific IgE was negative however, his rhGAA specific IgG titer was as high as 12,800. ITI therapy to suppress antibody formation and a desensitization protocol was devised to be given concomitantly. At the end of 5-week therapy, his fatigue and weakness improved profoundly and a control antidrug antibody level decreased at 800. At the time of the patient's follow up, he was still on ERT with desensitization at the age of 15 months without any reactions. CONCLUSIONS: This is the first report in the literature applying concomitant ITI and desensitization protocols in a CRIM negative infantile-onset Pompe disease patient successfully, hence the importance of the case.

Cyclopentolate eye drops-induced anaphylaxis in an infant.

BACKGROUND: Cyclopentolate is frequently used as a mydriatic agent during ophthalmological examinations in childhood and hypersensitivity reactions associated with this drug are rare. We aim to report an infant who experienced anaphylaxis due to cyclopentolate eye drops. CASE: A nine-month-old girl, who was being followed up with a diagnosis of retinoblastoma, presented for consultation for urticaria, cough, stridor, and dyspnea that developed after the administration of topical cyclopentolate to the eyes. The patient was diagnosed with anaphylaxis and treated with adrenaline. During the follow-up, tropicamide was used safely as an alternative drug. CONCLUSIONS: In children, hypersensitivity reactions due to cyclopentolate are very rare. Only four pediatric patients were reported in the literature to have developed an allergic reaction after the administration of cyclopentolate eye drops. We present here the youngest patient who developed anaphylaxis with cyclopentolate eye drops. Anaphylaxis due to cyclopentolate should be kept in mind, rapidly recognized, and treated when a reaction develops.

New Perspectives in the Management of Mild to Moderate Asthma in Children.

Mild to moderate asthma makes up the greatest proportion of all asthma severities in childhood. Children who are treated with steps 1-2 are defined as having mild asthma and step 3 as having moderate asthma, according to the guidelines. Although many studies focused on the management of severe asthma over the last decade, there have also been important changes and improvements in the management of mild to moderate asthma. In this article, new perspectives in the management of children with mild to moderate asthma will be reviewed and compared according to the two major guidelines.

Anaphylaxis to levetiracetam in an adolescent: a very rare occurence.

BACKGROUND: Antiepileptic drugs (AEDs) are among the most common causes of severe delayed-type hypersensitivity reactions such as Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) and Drug Reaction with Eosinophilia and Systemic Symptoms(DRESS) in children. These reactions are more commonly seen with aromatic AEDs such as phenytoin and carbamazepine than the non-aromatic or new generation AEDs. However immediate-type hypersensitivity reactions such as urticaria/angioedema, anaphylaxis are very rare with AEDs. CASE: Levetiracetam is an increasingly used new non-aromatic antiepileptic drug and reported to have a better safety profile in daily practice. We present the first adolescent case who developed an anaphylactic reaction with intravenous levetiracetam, not reported in this age group before in the literature. CONCLUSION: Hypersensitivity reactions in the form of anaphylaxis can be rarely observed with new generation AEDs. Therefore, when any antiepileptic drug is started on any patient, immediate type serious reactions such as anaphylaxis should be kept in mind, not only focusing on delayed reactions such as SJS, TEN,or DRESS.

Reliability and Validity Study of Turkish Version of Pediatric Eosinophilic Esophagitis Symptom Scores® (Tr-PEESS v2.0) Led to Development of a New Pediatric Eosinophilic Esophagitis Scale: GaziESAS.

BACKGROUND/AIMS: There has been no valid and reliable Turkish scale that measures symptoms in children with eosinohilic esophagitis (EoE). The aim of the study is test the validity and reliability of Turkish version of Pediatric Eosinophilic Esophagitis Symptom Scores® (Tr-PEESS v2.0) Materials and Methods: Relevant forms of Tr-PEESS v2.0 were applied to 2-18 years old children with EoE and to their parents. KINDL QoL patient and parent questionnaires and GaziESAS scale which was developed in this study were used to test convergent validity of Tr-PEESS v2.0. Discriminant validity was evaluated among three EoE treatment groups: under treatment, off treatment due to remission and uncompliant with treatment. Reliability was evaluated by internal consistency, test-retest reliability, and item analysis. RESULTS: Fiftytwo children/teens (mean age 130.2±60.3 months) and 84 parents were interviewed twice one week apart. Mean duration of EoE was 47.2±35.6 months. Tr-PEESS v2.0 reports correlated with GaziESAS (range,0.361-0.855) and KINDL QoL questionnaires (range,-0.316-0.413). Parent report of Tr-PESS v2.0 discriminated children uncompliant with treatment from the ones off treatment and undertreatment. Cronbach's α values and intraclass correlation coeffcients (ICC) values of Tr-PEESS v2.0 ranged from 0.614 to 0. 895 and 0.646 to 0.910, respectively. CONCLUSION: Tr-PEESS v2.0 is a valid and reliable tool to use in Turkish children. GaziESAS is a new parent-proxy pediatric EoE scale with additional adaptive behaviour domain that passed scale developmental stages successfully for Turkish children with EoE.

Nonsteroidal anti-inflammatory drugs-exacerbated respiratory disease in adolescents.

BACKGROUND: There has been no study investigating nonsteroidal anti-inflammatory drugs (NSAIDs)-exacerbated respiratory disease (NERD) exclusively in childhood. Therefore, in the current study, the authors aimed to evaluate the diagnostic features, clinical characteristics, and follow-up of adolescents diagnosed with NERD. METHODS: The patients who were consecutively diagnosed with NERD between January 2011, and November 2013, included in the study. Oral provocation test (OPT) with at least 2 different NSAIDs was used to confirm NSAID hypersensitivity in patients with underlying asthma/chronic rhinosinusitis/nasal polyps. All patients were followed regularly in 3-month intervals by the Pediatric Allergy and Otorhinolaryngology Department for asthma, allergic rhinitis, or chronic rhinosinusitis with or without nasal polyps. RESULTS: A total of 10 adolescents with NERD were included in the study. The mean age of the patients at the time of diagnosis was 14.9 ± 1.5 years. Hives or angioedema accompanied respiratory complaints induced by NSAIDs. The mean duration of follow-up was 28.9 ± 12.4 months. All patients had asthma except 1 with asymptomatic bronchial hyperreactivity. Asthma of patients was well-controlled with moderate dose of inhaled corticosteroids. Chronic rhinosinusitis with or without nasal polyps developed in 2 patients. Aspirin desensitization was required in these 2 patients with recurrent nasal polyps. CONCLUSION: NERD in childhood has much more favorable clinical characteristics and course than in adulthood. Few adolescents with NERD refer with typical chronic upper respiratory tract complaints. The asthma component seems to be mild and is well controlled in the short-term follow-up.

Children with chronic nonspecific isolated cough.

BACKGROUND: This study observed children with chronic nonspecific isolated cough (NIC) to investigate clinical differences between children whose symptoms resolved spontaneously and those who eventually developed asthma and then explored the differences among the children who eventually developed asthma in terms of their time of response to a trial of inhaled corticosteroid (ICS). METHODS: Children with chronic NIC were managed either with a wait-and-review approach or with a 2-week trial with 400 µg/d inhaled budesonide according to the preference of their parents. Responses were monitored with a validated cough score. Treatment was prolonged to 8 weeks in the case of partial responders. All children were followed up at 3-month intervals. RESULTS: A total of 109 children (median [interquartile range] age, 5 [3.5-9] years; cough duration, [8-16] weeks]) were followed for a mean (± SD) time of 21(± 5) months. Cough did not recur in 71% (spontaneous resolution) but relapsed in 28% of the children who later responded to ICS treatment again (asthma). Aeroallergen sensitization (relative risk, 2.86; 95% CI, 1.17-6.99) and previous history of chronic cough (relative risk, 2.68; 95% CI, 1.10-6.49) increased the risk of asthma. Cough duration, the cough score, the family history of asthma, and serum eosinophilia were not found discriminative for the final diagnosis. There were no differences among children who eventually developed asthma and responded to either the 2-week or 8-week trial in terms of the study parameters. CONCLUSIONS: Chronic NIC does not recur in the majority of children. Initial response to the ICS trial may be misleading but the trial may be preferred for children who have atopic sensitization, a previous history of chronic cough, or both .