Vernal keratoconjunctivitis: atopy and autoimmunity

BACKGROUND: Vernal Keratoconjunctivitis (VKC) is a rare chronic ocular inflammatory disease and it mainly affects boys in the first decade of life. Although it is a self-limiting disease, patients may present many phases characterized by an exacerbation of inflammatory symptoms with a consequent decline of the quality of life. PURPOSE: define the clinical and immunological profile of patients affected by VKC and investigate their familiar history of autoimmune disorders and their autoimmunity pattern. PATIENTS AND METHODS: 28 children were enrolled (20 males, 71%) aged between 4 and 14 years of life affected by VKC. Family history of allergic and immunological diseases was collected for each patient. In particular, it was asked whether some components of their families were affected by Hashimoto's thyroiditis, type I diabetes, psoriasis or rheumatoid arthritis and Systemic Lupus Erythematosus (SLE). All VKC children underwent a serological evaluation of antinuclear antibodies (ANA). RESULTS: A family history of immunological disorders was found in 46% of patients, 28% of Hashimoto's thyroiditis, 14% of type I diabetes, 14% of psoriasis, and 1 of Systemic Lupus Erythematosus. Furthermore, 35% of patients was ANA positive and they corresponded to patients with a higher ocular score and with the most important clinical symptoms. CONCLUSIONS: the detection of ANA positivity and of a familiar history of autoimmune disorders in a high percentage of children with VKC may help us to better understand the association of this ocular inflammatory disease with systemic autoimmune disorders and atopic condition

SEVERE ASTHMA IS REALLY UNCOMMON

We describe the case of a 10-year-old girl with a history of severe persistent asthma and exercise-induced-asthma, controlled using an appropriate treatment with inhaled corticosteroid-long-acting beta-2 adrenergic agonists (ICS+LABA) and leukotriene receptor antagonists. She was healthy until the age of 8 years, when she presented two episodes of radiologically diagnosed pneumonia. After that, she began to present persistent cough, also nocturnal, stridor, dyspnea and respiratory distress and she was sent by pediatrician to our hospital. She performed a global spirometry which shows an obstructive and restrictive phenotype (FEV1: 75,3% and MEF50: 57,6%), without a significantly dilatation after inhaled salbutamol (400 mcg). She underwent to a systemic therapy with oral corticosteroid, with not benefit. She had no fever neither upper respiratory tract infections. We excluded gastro-oesophageal reflux disease, cystic fibrosis, Mycoplasma and Chlamydia pneumonia. Cardiological examination was negative. During hospitalization, she spontaneously expectorated a thick fibrinous mucoid formation. A chest X ray and a computed tomography (CT) scan showed atelectasis of both lung, widespread hyperlucency, and occlusion of the right main bronchus, compatible with a diagnosis of plastic bronchitis. Plastic bronchitis is a rare disease characterized by the formation of large gelatinous or rigid branching airway casts. The prevalence and etiology of plastic bronchitis are still unknown and the symptoms may also overlap with those of other diseases such as severe asthma, in the severe mucus plugging sometimes seen in allergic bronchopulmonary Aspergillosis (ABPA) or in middle lobe syndrome. In the pathogenesis of the disease the inflammation is usually present and initiates cast formation. Treatment includes bronchodilators, inhaled and oral corticosteroids, mucolytics, airway clearance therapy and antibiotics. Other therapies can include inhaled heparin, urokinase, tissue plasminogen activator (TPA), dornase alfa and oral macrolide antibiotics as mucoregulatory therapy2. Conclusions: The presence of asthmatic symptoms without clinical improvement after appropriate therapy is not always suggestive of severe asthma. Therefore, for the appropriate diagnosis, we have to exclude the other lung diseases and, among the differential diagnoses, is possible to consider also plastic bronchitis

Selective IgA deficiency and the risk of asthma

Body: Introduction:Selective IgA deficiency (IgAD), the most frequent primary immunodeficiency is associated with a wide variety of clinical manifestations including an increased frequency of allergic manifestations such us asthma. Objective: To describe the prevalence of allergies and asthma in a selected population of children with IgAD. Materials and Methods: We included 27 children(15 males)with a diagnosis of IgA deficiency with a mean age of onset of symptoms 37.58 ± 29 months. Each subject was investigate about clinical presentation at the time of the diagnosis and allergic skin tests for food allergens and inhalants were performed. Results: At the beginning children with IgAD presented principally infections of the upper and lower airways, 5/27(18,5%)had pneumonia.16/27(59,3%) had Prick test positivity with associated allergic diseases. Among the allergens house dust mites were the most frequent (22,2%) followed by grass pollens(11,1%).7/27(25,9%) presented asthma symptoms and 6/27(22%) rhinoconjunctivitis and atopic eczema. The prevalence of asthma symptoms is more higher in our population of children with IgAD respect to the general pediatric population, respectively 25.9 vs 7.9 (Tozzi AE Pediatric Allergy Immunol 2011) and the difference is statistically significant (p<0,01%) Conclusions: Children with selective IgA deficiency seem to have an increased risk of having recurrent infections and allergic manifestations respect to general pediatric population. Observations that IgAD is associated with an increased prevalence of atopy suggest a possible role of IgA in asthma pathogenesis. The mucosal antigen exposure seems to be increased in the absence of IgA, causing an increased production of IgE against allergens

Assessing the relationship between serum resistin and nasal obstruction in children with allergic rhinitis

Background: Nasal obstruction has been reported as a "key symptom" of allergic rhinitis (AR) because it is deeply associated with impaired quality of life and it reflects more directly the allergic inflammation in the nasal mucosa. Resistin is known to be involved in inflammatory processes exerting an important role in the regulation of cytokine production even though its effective proinflammatory activity at nasal level has never been fully established. This study investigates the relationship between resistin levels and nasal obstruction assessed by an objective method such as active anterior rhinomanometry. Methods: Fifty-three children between 4 and 10 years of age affected by persistent allergic rhinitis (PAR) were enrolled and subdivided in two groups. Serum resistin levels were detected in all children. The same day patients underwent rhinomanometry, which was considered negative (no nasal obstruction) when the fraction of predicted values (p.v.'s) was between 71 and 100% and it was considered positive when the fraction of p. v. was <= 70%. Results: The serum resistin levels were significantly higher in children with moderate-severe PAR than in patients with mild PAR (p<0.03). Furthermore, serum resistin levels were significantly higher in children with positive rhinomanometry compared with negative rhinomanometry (p<0.03). The fraction of p.v.'s of nasal flows in patients with nasal obstruction had a significant negative correlation with serum resistin levels (p<0.001). Conclusion: This study provides evidence that resistin levels are increased in children with severe nasal obstruction measured by an objective and quantitative approach

SERUM RESISTIN LEVELS IN CHILDREN WITH HABITUAL SNORING

Body: Introduction: Intermittent episodes of hypoxia, and reoxygenation in patients with habitual snoring(HS) might predispose to cell stress trough the activation of a proinflammatory response. Resistin , an adipokine produced by adipocytes, circulating monocytes and macrophages is known to be involved in the inflammatory response regulation. Objectives: To investigate the association between HS severity and serum levels of resistin. Methods: Fifty-three children (35 males; 66%) with HS aged between 4 and 16 years of life (mean age 8 ± 3 years) were selected by a SDB validate questionnaire and underwent nocturnal pulse-oximetry recording at home. Moreover 33 healthy non snorers children with similar age, sex and BMI, were included in the study as a control group. Results: Children with HS had serum resistin levels higher than the control group (4,67 ± 1,91 ng/ml vs 3,80 ± 1,50 ng/ml; p<0.04). Notably the highest resistin levels were found in children with not conclusive and positive pulse-oximetry (5,29 ± 1,91 ng/ml vs 4,20 ± 1,93 ng/ml; p<0.008). Conclusions: Our findings support the evidence of an increased production of pro-inflammatory mediators, such as resistin, in children with HS. These results might be probably due to the intermittent hypoxia and reoxygenation often occurring during sleep in children with Sleep disordered breathing. The presence of HS should be always investigated by pediatricians in order to identify children at risk of the early development of complications

HABITUAL SNORING IN CHILDREN WITH PREVIOUS ALLERGIC SENSITIZATION

Previous studies have reported a high prevalence of allergy in children with Habitual Snoring (HS), but the relationship between allergy in the early years of life and the subsequent development of this Sleep Disordered Breathing (SDB) is yet to be elucidated. The purpose of the present study was to determine the role of early, under 36 months of age, allergic sensitization to food (with or without sensitization to airborne allergens) in determining the development of HS 8-10 years after. One hundred and forty-eight children (10-14 years, mean age 12 years) with a history of food allergy were selected. Under the age of 36 months, atopic status was assessed by skin prick test for a panel of airborne and food allergens. Questionnaires filled in by parents were used to collect information on children's snoring and associated symptoms. HS was defined as snoring three or more times per week. At 1-3 years of age 54 (36.5%) children were positive to food allergens alone, and 94 (63.5%) were positive also to airborne allergens. After 8-10 years of life, when patients were aged between 10 and 14 years, habitual snoring was reported in 37 (25%) children. Furthermore, among the 54 children under three years of age sensitized only to food, 8 (14.8%) became HS while of the 94 children sensitized to both food and inhalants allergens 29 (30.9%) developed HS. The difference between those two groups was statistically significant (p=0.04). We reported a significant risk of developing HS in children with early allergic sensitization. Specifically this risk was higher when food allergy was associated with inhalant allergy. The onset of upper airway inflammation due to allergic triggers in subjects under three years of age may be related to the subsequent development of SDB after 8-10 years

Immunogenetic investigation in vernal keratoconjunctivitis

Vernal keratoconjunctivitis has been considered a multifac- torial condition resulting from complex interactions among genetic, immunologic, and environmental factors. The research of genetic characteristics and the identification of a specific HLA haplotype as a possible predictor of disease may contribute to elucidate the pathogenesis of the disease and to point out the VKC as a complex immunologic disorder rather than as a mere allergic disease. The identification of a specific haplotype associated with VKC might be helpful to identify children predisposed to develop this ocular disease and patients with a specific outcome

Immunogenetic investigation in vernal keratoconjunctivitis

Vernal keratoconjunctivitis has been considered a multifac- torial condition resulting from complex interactions among genetic, immunologic, and environmental factors. The research of genetic characteristics and the identification of a specific HLA haplotype as a possible predictor of disease may contribute to elucidate the pathogenesis of the disease and to point out the VKC as a complex immunologic disorder rather than as a mere allergic disease. The identification of a specific haplotype associated with VKC might be helpful to identify children predisposed to develop this ocular disease and patients with a specific outcome