Fiberoptic Endoscopic Evaluation of Swallowing Findings in Children With Rett Syndrome.

Feeding abnormalities, swallowing dysfunction, and gastrointestinal issues cause poor weight gain, oral motor dysfunction, and air swallowing in children with Rett syndrome (RTT). Pneumonia is the leading cause of death. Our study describes fiberoptic endoscopic swallowing findings in 11 female RTT children. Each patient was evaluated using the 8-point Penetration/Aspiration Scale (PAS). The average age was 7 years. All patients had tongue dyskinesis and prolonged oral stage. Eight girls exhibited liquid entering the airway without coughing, whereas 6 did well with pureed meal. Three girls had pneumonia. Age was not correlated with pneumonia episodes (P = .18). Pureed material was related with pneumonia (P = .006), whereas liquids were not. Pureed PAS was positively correlated with Liquid PAS (P = .008) and age (P = .004). All aspiration/penetration incidents occurred before the pharyngeal phase. No patient under 7 years experienced pneumonia episodes. Silent aspiration can occur early in infancy, although pneumonia episodes can occur later.

A toddler diagnosed with severe postinfectious bronchiolitis obliterans and COVID-19 infection.

During the COVID-19 pandemic, management of SARS-CoV-2 infection in children with underlying chronic lung disease has been challenging. There are limited studies in children with respiratory comorbidities, apart from asthma, presumably due to low morbidity of SARS-CoV-2 infection in the general pediatric population along with the low incidence of certain pulmonary conditions. Compassionate use of remdesivir has been shown to reduce time to clinical improvement in adults and has been retrospectively studied in small pediatric cohorts with promising results. Whether children with underlying respiratory conditions may benefit from antiviral treatment in the context of different pathophysiologic backgrounds and unknown drug safety and efficacy needs to be further evaluated. We present a case of COVID-19 infection in a 3-year old toddler with severe postinfectious bronchiolitis obliterans, who received compassionate treatment with 5-day-course of remdesivir, and recovered with favourable outcome.

Atypical food protein-induced enterocolitis syndrome in children: Is IgE sensitisation an issue longitudinally?

BACKGROUND: Food Protein-Induced Enterocolitis Syndrome (FPIES) is a clinically well-characterised, non-Immunoglobulin E (IgE)-mediated food allergy syndrome, yet its rare atypical presentation remains poorly understood. OBJECTIVE: Aim of this study was to present the 10-year experience of a referral centre highlighting the atypical FPIES cases and their long-term outcome. METHODS: FPIES cases were prospectively evaluated longitudinally in respect of food outgrowth and developing other allergic diseases with or without concomitant IgE sensitisation. RESULTS: One hundred subjects out of a total of 14,188 referrals (0.7%) were identified. At presentation, 15 patients were found sensitised to the offending food. Fish was the most frequent eliciting food, followed by cow's milk and egg. Tolerance acquisition was earlier for cow's milk, followed by egg and fish, while found not to be protracted in atypical cases. Resolution was not achieved in half of the fish subjects during the 10-year follow-up time. Sensitisation to food was not related to infantile eczema or culprit food, but was related to sensitisation to aeroallergens. In the long-term evaluation, persistence of the FPIES or aeroallergen sensitisation was significantly associated with an increased hazard risk of developing early asthma symptoms. CONCLUSION: Sensitisation to food was related neither to eczema or culprit food nor to tolerance acquisition but rather to the development of allergic asthma through aeroallergen sensitisation. In addition to an IgE profile at an early age, FPIES persistence may also trigger mechanisms switching FPIES cases to a T-helper 2 cells immune response later in life, predisposing to atopic respiratory symptoms; albeit further research is required.

Atypical food protein-induced enterocolitis syndrome in children: Is IgE sensitisation an issue longitudinally?

Background: Food Protein-Induced Enterocolitis Syndrome (FPIES) is a clinically well-characterised, non-Immunoglobulin E (IgE)-mediated food allergy syndrome, yet its rare atypical presentation remains poorly understood. Objective: Aim of this study was to present the 10-year experience of a referral centre highlighting the atypical FPIES cases and their long-term outcome. Methods: FPIES cases were prospectively evaluated longitudinally in respect of food outgrowth and developing other allergic diseases with or without concomitant IgE sensitisation. Results: One hundred subjects out of a total of 14,188 referrals (0.7%) were identified. At presentation, 15 patients were found sensitised to the offending food. Fish was the most frequent eliciting food, followed by cow’s milk and egg. Tolerance acquisition was earlier for cow’s milk, followed by egg and fish, while found not to be protracted in atypical cases. Resolution was not achieved in half of the fish subjects during the 10-year follow-up time. Sensitisation to food was not related to infantile eczema or culprit food, but was related to sensitisation to aeroallergens. In the long-term evaluation, persistence of the FPIES or aeroallergen sensitisation was significantly associated with an increased hazard risk of developing early asthma symptoms. Conclusion: Sensitisation to food was related neither to eczema or culprit food nor to tolerance acquisition but rather to the development of allergic asthma through aeroallergen sensitisation. In addition to an IgE profile at an early age, FPIES persistence may also trigger mechanisms switching FPIES cases to a T-helper 2 cells immune response later in life, predisposing to atopic respiratory symptoms; albeit further research is required (AU)

Review suggests that the immunoregulatory and anti-inflammatory properties of allergenic foods can provoke oral tolerance if introduced early to infants' diets.

For years, the advice was to postpone introducing allergenic foods, in order to prevent food allergies. However, food allergies have escalated rather than declined and our review of the latest epidemiological, immunological and nutritional research suggests that early weaning practices may be beneficial. The most allergenic foods, such as fish, eggs and peanuts, have proved to be inherently rich in tolerogenic substances that can play a significant role in preventing allergies. CONCLUSION: We found evidence that the immunoregulatory and anti-inflammatory properties of allergenic foods can provoke oral tolerance if introduced early to both low-risk and high-risk infants.

Asthma symptoms and bronchial reactivity in school children sensitized to food allergens in infancy.

Food allergy in infancy usually disappears but is followed primarily by respiratory allergy. We hypothesized that children allergic to common food allergens in infancy are at increased risk of wheezing illness and bronchial hyperresponsiveness during school age. In a case-control study 69 children 7.2 to 13.3 years of age allergic to egg (N = 60) and/or fish (N = 29) in early life (first 3 years) who attended our allergy outpatient clinic were recruited. They received follow-up for 1 year and were evaluated by parental questionnaire, skin prick testing, spirometry, and metacholine bronchial challenge. Another 154 children (70 sensitized to inhaled allergens) recruited selectively from a general population sample with no history of food allergy during their first 3 years served as control subjects. Twenty-three children (38.3%) maintained their sensitization to egg and 19 (65.5%) to fish; the prevalence of sensitization to > or = 1 inhaled allergen(s) increased from 59.4% to 71% during childhood. Current asthma symptoms were reported more frequently in the study group than in either control groups, sensitized to inhaled allergens and non-sensitized. Children of the study group showed a significantly increased frequency of positive response to metacholine bronchial challenge compared to the control group as a whole; the difference was statistically indicative when study groups separately were compared to the sensitized control subjects. Multivariate logistic regression analysis showed that bronchial hyperresponsiveness, as well as reported current asthma symptoms were associated with early wheezing and early sensitization to inhaled allergens but not with atopic dermatitis in infancy or persistence of egg or fish allergy. Children allergic to egg or fish in infancy are at increased risk for wheezing illness and hyperactive airways in school age; asthma and bronchial hyperresponsiveness development is mostly determined by wheezing and senzitization to inhaled allergens in early life regardless of atopic dermatitis in infancy or retention of food allergy.

Bronchial hyperresponsiveness, atopy, and bronchoalveolar lavage eosinophils in persistent middle lobe syndrome.

Most cases of middle lobe syndrome (MLS) in children are considered to be due to asthma and may recover spontaneously; however, in persistent MLS, repeated episodes of infection often institute a vicious cycle that may lead to persistent symptoms and bronchial hyperresponsiveness (BHR). The present study was undertaken to investigate whether asthma, as an underlying diagnosis, is predictive of a favorable outcome of children with persistent MLS. We evaluated 53 children with MLS who underwent an aggressive management protocol that included fiberoptic bronchoscopy (FOB) and bronchoalveolar lavage (BAL). These patients were compared to two other groups: one consisting of children with current asthma but no evidence of MLS (N = 40) and another of non-asthmatic controls (N = 42), matched for age and sex. Prevalence of sensitization (>or=1 aeroallergen) did not differ between patients with MLS and "non-asthmatics" but was significantly lower than that of "current asthmatics." A positive response to methacholine bronchial challenge was observed with increased frequency among children with MLS when compared to "current asthmatic" and non-asthmatic children. Multivariate logistic regression analysis revealed a positive correlation between an increased number of eosinophils in the BAL fluid (BALF) and a favorable outcome, whereas no correlation was detected between sensitization or BHR and BAL cellular components. In conclusion, children with MLS have an increased prevalence of BHR, even when compared to asthmatics, but exhibit prevalence of atopy similar to that of non-asthmatics. An increased eosinophilic BALF count is predictive of symptomatic but not radiographic improvement of MLS patients after aggressive anti-asthma management.

The role of timely intervention in middle lobe syndrome in children.

STUDY OBJECTIVES: Middle lobe syndrome (MLS) in children is characterized by a spectrum of clinical and radiographic presentations, from persistent or recurrent atelectasis to pneumonitis and bronchiectasis of the right middle lobe (RML) and/or lingula. This study was undertaken to evaluate the effect of early intervention, including fiberoptic bronchoscopy (FOB), in the development of bronchiectasis in MLS. DESIGN: Children with atelectasis of the RML and/or lingula persisting for > 1 month or recurring two or more times despite conventional treatment underwent high-resolution CT (HRCT) scanning and FOB. Appropriate treatment and follow-up were provided, and the effect of the duration of symptoms on clinical outcome and the development of bronchiectasis was investigated. The patient cohort was retrospectively reviewed. PATIENTS: We evaluated 55 children with MLS. The median age at diagnosis, duration of symptoms, and duration of clinical deterioration before diagnosis were 5.5 years (range, 3 months to 12 years), 14.5 months (range, 3 to 48 months), and 8 months (range, 3 to 36 months), respectively. MEASUREMENTS AND RESULTS: FOB revealed marked obstruction in two children (ie, a foreign body and an endobronchial tumor) and positive findings for a culture of BAL fluid in 49.1% of patients. The remaining 53 patients were followed up for a median duration of 24 months (range, 5 to 96 months). The clinical outcome was "cure" in 60.4% of patients, "improvement" in 32.1% of patients, and "no change" in the remaining patients. Bronchiectasis was documented prior to FOB by HRCT scan in 15 patients (27.3%). The duration of the deterioration of symptoms prior to presentation positively correlated with the development of bronchiectasis (p = 0.03) and an unfavorable clinical outcome (ie, improvement or no change) [p = 0.02]; a positive correlation was also found between the duration of symptoms and the development of bronchiectasis (p = 0.04). CONCLUSIONS: Timely medical intervention in patients with MLS that includes FOB with BAL prevents bronchiectasis that may be responsible for an ultimately unfavorable outcome.

Bronchial hyper-responsiveness in selective IgA deficiency.

Secretory IgA in mucosal secretions has a broad protective function. The insufficient protection provided by the respiratory mucosa in children with selective IgA deficiency (sIgAD) might facilitate the development of bronchial hyper-responsiveness (BHR) and consequently asthma symptoms. This study was conducted to clarify the prevalence of BHR in sIgAD children and the relationship with atopic status. A cohort of 20 children (group A) aged 6.4-20.1 yr (median: 12.6) with sIgAD (serum IgA <6 mg/dl) were evaluated for BHR using inhaled hypertonic saline test as well as for atopy by skin prick testing (SPT) to eight common aero-allergens. Seventy other children with normal levels of serum IgA, but sensitized to aero-allergens (group B) and 102 with normal IgA and negative SPTs (group C) were also evaluated. Baseline spirometry demonstrated that forced vital capacity (FVC) values in group A were significantly lower than in C. Forced expiratory volume in 1 s values were similar in all groups, but impairment of the forced expiratory flow over the middle half of the FVC was detected in group B. The prevalence of BHR was similar among group A (30.0%) and group B (35.7%) (p = 0.79) but they differed from group C (5.9%) (p = 0.005). An association between BHR and reported current (p = 0.001) but not lifetime asthma symptoms among group A was also observed. There was no association between atopy and BHR in group A but only to mites' sensitization (p = 0.03). In conclusion, these results indicate that sIgAD constitutes a risk factor for development of BHR but it appears to be related to sensitization to mites.