Pediatric Emergency Unit, Department of Medical and Surgical Sciences, IRCCS Azienda Ospedaliero-Universitaria, Polyclinic of St.Orsola, University of Bologna, 40138 Bologna, Italy.
Dr. Laura Andreozzi
Pediatric Emergency Unit, Department of Medical and Surgical Sciences, IRCCS Azienda Ospedaliero-Universitaria, Polyclinic of St.Orsola, University of Bologna, 40138 Bologna, Italy.
A complex web of dynamic relationships between innate and adaptive immunity is now evident for many autoinflammatory and autoimmune disorders, the first deriving from abnormal activation of innate immune system without any conventional danger triggers and the latter from self‐/non‐self‐discrimination loss of tolerance, and systemic inflammation. Due to clinical and pathophysiologic similarities giving a crucial role to the multifunctional cytokine interleukin‐1, the concept of autoinflammation has been expanded to include nonhereditary collagen‐like diseases, idiopathic inflammatory diseases, and metabolic diseases. As more patients are reported to have clinical features of autoinflammation and autoimmunity, the boundary between these two pathologic ends is becoming blurred. An overview of monogenic autoinflammatory disorders, PFAPA syndrome, rheumatoid arthritis, type 2 diabetes mellitus, uveitis, pericarditis, Behçet’s disease, gout, Sjögren’s syndrome, interstitial lung diseases, and Still’s disease is presented to highlight the fundamental points that interleukin‐1 displays in the cryptic interplay between innate and adaptive immune systems.
Gluten-related disorders are very common in pediatric patients. Wheat allergy is triggered by an immunoglobulin E (IgE)-dependent mechanism; its prevalence varies according to the age and region, and in Europe has been estimated to be lower than 1%. Many studies investigated the potential role of several external factors that can influence the risk to developing wheat allergy, but results are still inconclusive. It can be responsible for several clinical manifestations depending on the route of allergen exposure: food-dependent exercise-induced anaphylaxis (FDEIA), occupational rhinitis or asthma (also known as baker’s asthma), and contact urticaria. The prognosis of IgE-mediated wheat allergy in children is generally favorable, with the majority of children becoming tolerant by school age. Patients who experienced an anaphylactic reaction prior to 3 years of age and patients with higher level of wheat- or ω-5 gliadin-specific IgE antibodies seem to be at higher risk of persistent wheat allergy. The current management of patients is dietary avoidance. Nowadays, oral immunotherapy has been proposed for wheat allergy with promising results, even if further studies are necessary to establish the best protocol in order to promote tolerance in wheat-allergic children.
Kawasaki syndrome (KS) is the most relevant cause of heart disease in children living in developed countries. Intravenous immunoglobulin (IVIG) has a preventive function in the formation of coronary artery abnormalities and a poor strictly-curative action in established coronary damage. More than two decades ago, the Harada score was set to assess which children with KS should be subject to administration of IVIG, evaluating retrospectively a large cohort of patients with regard to age, sex and laboratory data. Nowadays, high dose IVIG is administered to all children with a confirmed diagnosis of KS, but a tool for predicting non-responsiveness to the initial infusion of IVIG has not been found. The prediction of IVIG resistance is a crucial issue, as recognising these high-risk patients should consent the administration of an intensified initial treatment in combination with IVIG in order to prevent coronary injuries. Few reports have focused on factors, referring to both clinical parameters and laboratory data at the onset of KS, in order to predict which patients might be IVIG non-responsive. We have analysed three different risk scores which were formulated to predict IVIG resistance in Japanese children with typical KS, but their application in non-Japanese patients or in those with incomplete and atypical patterns of the disease has been studied in a fragmentary way. Overall, our analysis showed that early and definite ascertainment of likely IVIG non-responders who require additional therapies reducing the development of coronary artery involvement in children with KS is still a challenge.
29 November 2018 in European Journal of Pediatrics
The possibility of an allergic reaction or an intolerance to additives is frequently suspected by parents, especially for chronic illness with frequent exacerbations such as atopic dermatitis or chronic urticaria. For more than 50 years, potential adverse reactions to additives have been suggested, but to date data are conflicting. The purpose of this article is to provide the clinicians with general information about additives and adverse reactions to them and to suggest a practical approach to children suspected to have reactions to food additives. We performed an extensive research on all English-language Medline articles, case reports and reviews published online until December 2018. Used search terms were: food additives, food dye, adverse reactions, food allergy, food hypersensitivity, intolerance, drugs, children. There are only few case reports of adverse reactions in childhood with a clear involvement of additives. In this review article we reported the associations between additives and adverse reactions described in literature, in order to inform the pediatrician about the potential clinical manifestations. Prior to suspect an adverse reaction to additives, it is important to rule out other possible causes: the diagnostic process is complicated and rarely conclusive. The gold standard is the double-blind placebo controlled oral challenge after an exclusion diet.