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Abstract Oral immunotherapy is proposed as the only active intervention to modify allergies and decrease the risk of severe reactions. However, it is crucial to note that oral immunotherapy still presents a notable failure rate and potential for severe, life-threatening outcomes. Notably, patients who discontinue oral immunotherapy may face an increased risk of fatal reactions. Omalizumab could be a viable option for patients with failed oral immunotherapy.

Abstract Background Erythropoietic protoporphyria is a rare disorder which represents an important health problem in children, causing painful photosensitivity. Little is known on the correlation between genetic profile and clinical manifestations. The standard of care for Erythropoietic protoporphyria is based on avoiding sun and using sun protections, but recent literature has suggested that cimetidine may have a role in improving sun sensitivity. Herein we report our case series describing the successful use of cimetidine and analyzing potential phenotype-genotype correlations. Case presentation This case series describes five patients presented to our Rheumatology Service complaining sun sensitivity. Blood exams and genetic analysis were consistent with the diagnosis of erythropoietic protoporphyria. Four of 5 patients received cimetidine in addition to standard therapies and the effect of treatment was evaluated by Erythropoietic Protoporphyria - Quality of Life questionnaire. Conclusions Erythropoietic protoporphyria usually manifests in early childhood after a short sun exposure. Skin manifestations are the main reason for investigations, although sometimes they can be more subtle, leading to a significant diagnostic delay. Skin diseases in children can have profound effects on their family and social relationships. A treatment with cimetidine appears to be an excellent therapeutic option in children with Erythropoietic protoporphyria.

Introduction

Abstract Currently, there are a few detailed guidelines on the overall management of children and adolescents with moderate-severe atopic dermatitis. AD ​​is a complex disease presenting with different clinical phenotypes, which require an individualized and multidisciplinary approach. Therefore, appropriate interaction between primary care pediatricians, pediatric allergists, and pediatric dermatologists is crucial to finding the best management strategy. In this manuscript, members of the Italian Society of Pediatric Allergology and Immunology (SIAIP), the Italian Society of Pediatric Dermatology (SIDerP), and the Italian Society of Pediatrics (SIP) with expertise in the management of moderate-severe atopic dermatitis have reviewed the latest scientific evidence in the field. This narrative review aims to define a pathway to appropriately managing children and adolescents with moderate-severe atopic dermatitis.

Abstract Background Some studies addressed the issue of omalizumab (OML) effectiveness in children starting their first oral immunotherapy (OIT) attempt but no study investigated the possible role of OML in the setting of patients with persisting milk allergy after a failed OIT attempt. Methods Single‐center, prospective, observational study in a selected group of patients with a persisting and severe cow milk (CM) allergy associated with moderate allergic asthma, in which a previous OIT attempt had already failed. We performed an open oral food challenge (OFC) to identify patients who tolerated less than 173 mg of cow's milk protein. At the end of the recruitment, we have found four patients with a mean age of 16.25 years (8–24) who had suspended a previous OIT attempt and still reacted to an amount of CM equal or below 173 mg. Enrolled patients, after an 8‐week course of OML along with a CM avoiding diet, underwent again an open OFC with CM to re‐evaluate their threshold. Eventually, a new OIT course was started using the same OIT protocol of the previous attempt, maintaining cotreatment with OML for the first 12 months. For each patient, we documented: the threshold of CM at OFC, level of specific immunoglobulin E (IgE) and IgG4 for milk, and quality of life (QoL). Results During OIT the four patients experienced no reactions or extremely mild ones (oral itching, transient mild abdominal pain). All increased their threshold of CM in OML if compared with the baseline and maintained it long after that biologic therapy had discontinued. Specific milk proteins IgG4 levels significantly increased in all. Conclusion In this series, OML was effective in patients with severe CM allergy who had previously failed OIT, allowing milk intake without adverse reactions and improving the QoL.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease with a multifactorial aetiology that involves a strict interplay between genetic factors, immune dysregulation and lifestyle. Familial forms represent around 40% of total HS cases and show an autosomal dominant mode of inheritance of the disease. In this study, we conducted a whole-exome sequence analysis on an Italian family of 4 members encompassing a vertical transmission of HS. Focusing on rare damaging variants, we identified a rare insertion of one nucleotide (c.225dupA:p.A76Sfs*21) in the DCD gene encoding for the antimicrobial peptide dermcidin (DCD) that was shared by the proband, his affected father and his 11-years old daughter. Since several transcriptome studies have shown a significantly decreased expression of DCD in HS skin, we hypothesised that the identified frameshift insertion was a loss-of-function mutation that might be associated with HS susceptibility in this family. We thus confirmed by mass spectrometry that DCD levels were diminished in the affected members and showed that the antimicrobial activity of a synthetic DCD peptide resulting from the frameshift mutation was impaired. In order to define the consequences related to a decrease in DCD activity, skin microbiome analyses of different body sites were performed by comparing DCD mutant and wild type samples, and results highlighted significant differences between the groins of mutated and wild type groups. Starting from genetic analysis conducted on an HS family, our findings showed, confirming previous transcriptome results, the potential role of the antimicrobial DCD peptide as an actor playing a crucial part in the etio-pathogenesis of HS and in the maintenance of the skin’s physiological microbiome composition; so, we can hypothesise that DCD could be used as a novel target for personalised therapeutic approach.

Abstract Introduction Early introduction oral immunotherapy (E‐OIT) in the first year of life can be a safe treatment for infants with cow's milk allergy (CMA). Once the protocol is completed, doubts remain whether children achieve tolerance or remain desensitized. According to current guidelines, this is determined by an avoidance period followed by a re‐exposure to the food allergen during an in‐hospital oral food challenge (OFC). In real life, this approach can be complicated, time‐consuming, and anxiety‐provoking for parents. We assessed the long‐term safety of E‐OIT for CMA in a cohort of children who switched to an unrestricted diet without testing the achievement of tolerance at the end of the OIT protocol. Materials and Methods We performed a descriptive analysis of the clinical follow‐up of a cohort of children diagnosed with IgE‐mediated CMA and undergoing E‐OIT protocol in their first year of life. In a previous publication, the same cohort of patients had been studied to assess the feasibility of E‐OIT for CMA. In the present study, we reported the results of a telephone survey, carried out through a questionnaire to their families enquiring about milk consumption and other ongoing atopic conditions of children. Results After an average of 4 years from the start of E‐OIT, 62/73 patients (85% of the historical cohort) participated in the survey. Among them, all 56 patients who had previously successfully completed the protocol reported an unrestricted cow's milk intake. Ninety–three percent of these children did not experience any further allergic reactions, while the remaining 7% described only mild and transitory reactions until the 6‐month period after the end of the protocol. Conclusions This study confirmed the long‐term safety of E‐OIT for CMA and challenged the paradigm of the need for allergen food withdrawal to discern between desensitization and tolerance. It could be a starting point for planning future trials on this issue.

Abstract Background There are no universally accepted criteria for discontinuing milk oral immunotherapy (MOIT) in patients with persistent cow milk allergy (CMA) and little data are available on predictive risk factors for dropping out from oral immunotherapy (OIT), due to allergic reactions or other reasons. Methods We retrospectively reviewed clinical records of patients with persistent severe CMA undergoing MOIT in a tertiary care center hospital to investigate risk factors associated with discontinuation of OIT. Persistent and severe allergy was defined as the history of systemic reactions and any milk protein‐specific IgE level >85 kU/ml. All patients were first admitted for an in‐hospital rush phase eventually followed by an at‐home dose increase. We evaluated the effect of various factors on two primary outcomes: the highest dose of milk ingested during the in‐hospital rush phase and during the home OIT phase. Results We identified 391 patients, of whom 131 met the inclusion criteria for the retrospective study, 54 females and 77 males. Data of the home OIT phase were available for 104 patients (27%). Regarding the home OIT outcome, an association for having a cow milk avoiding diet was found with reaching a dose below 10 ml during the in‐hospital rush phase (relative risks [RR]: 2.33, confidence interval [CI]: 0.85; 6.42), an age above than 10 years from the time of admission (RR: 3.29, CI: 0.85; 12.73), and a higher total number of reactions occurred during the hospitalization (RR: 1.54, CI: 1.02; 2.32), whereas the presence of respiratory reactions with wheezing (RR: 1.93, CI: 0.49; 7.61) and an IM adrenaline use was related to a higher risk of having an OIT still in progress (RR: 5.47, CI: 0.33; 7.73). Conclusions In this cohort of children with persistent CMA undergoing OIT who presented with respiratory reactions with wheezing, the development of anaphylaxis with the need for IM adrenaline, and age above 10 years were predictors of poor long‐term outcome.

Abstract Background Oral immunotherapy (OIT) is a promising therapeutic approach for children with persistent IgE‐mediated cow's milk allergy (CMA) but data are still limited. Objective To analyze the prevalence of life‐threatening anaphylaxis in children with persistent CMA undergoing OIT and to evaluate potential risk factors. Methods This is a retrospective cohort study among children with persistent CMA undergoing OIT over a 20‐year period, following a specific Oral Tolerance Induction protocol. Adverse reactions during the whole period and data on long‐term outcome were registered. Descriptive and nondescriptive statistics were used to describe data. Results Three hundred forty‐two children were evaluated. During OIT, 12 children (3.5%) presented severe anaphylactic reactions that needed an adrenaline injection. None required intubation, intensive care unit (ICU) admission, or showed a fatal outcome. Five of them abandoned OIT, five reached unrestricted diet and the others are still undergoing OIT. As far as outcome is concerned, 51.2% reached an unrestricted diet; 13.5% are at the build‐up stage; and 28.0% (97 patients) stopped the OIT. Among these 96 children, 6.3% experienced a severe reaction induced by accidental ingestion of milk with two fatal outcomes. Conclusions The risk of life‐threatening reactions was nearly two times lower (3.5% vs. 6.3%) among patients assuming milk during OIT than in those who stopped the protocol. A trend in favor of more severe reactions, requiring ICU admission, or fatal, was shown in patients who stopped OIT.

Abstract Background We reported the case of a two-old-year boy with a painful acute hemorrhagic edema. This is a self-limited benign condition: usually, affected children are well appearing and this strongly support the diagnosis. In the opposite, in our case, we observed a painful presentation of the edema. Therefore, we demonstrated that rarely, this condition could have also a painful presentation. Conclusions This case report helps clinician to know that also acute hemorrhagic edema could have a painful presentation, so we must considered it in the differential diagnosis with sepsis, sickle cell crisis and child abuse. We believe that these findings will be of interest to pediatricians.

Pyogenic granuloma (PG) is an acquired benign vascular hyperplasia. Even though PG is not so rare, its etiology is still unclear. Assuredly, an association between vascular anomalies and PG is an extremely rare finding, and a proper management of these cases is not well standardized to date. Herein, we report our experience with a giant PG arising spontaneously within a port-wine stain of the thorax. Previous cases reported in the pediatric literature and strategies of treatment are also discussed.

Abstract Background Cold-induced urticaria is a kind of physical urticaria characterized by the appearance of wheals after exposure to cold. The atypical form is a rare sub-type characterized by appearance of hives even in areas not directly exposed to the cold and by a negative cold stimulation test. Its diagnosis is often challenging because of the lack of specific tests and it is usually based on the patient’s clinical history. Hypotension due to generalized exposure to the cold is described both in the typical and the atypical forms. Case presentation We describe a 9-year-old boy who, at the beginning of the summer after the first swim in the sea, developed generalized urticaria, dyspnea, conjunctival hyperemia, blurred vision and loss of strength. The child was treated with intramuscular steroid and intravenous antihistamine, and the symptoms quickly resolved. Insect bite, contact with fish and drug ingestion were denied, and no unusual food had been eaten before the swim. A tentative diagnosis was made of either aquagenic urticaria or cold urticaria, but the specific tests were negative. Although the cause was unknown, prophylactic treatment with antihistamines was prescribed but in spite of this, wheals developed all over the body, after every swim in the sea. The child then came to our attention and relying on clinical history a diagnosis of atypical cold urticaria was made: development of hives even in areas not directly exposed to cold and a negative response to the cold stimulation test, are the characteristic features of this rare form of cold urticaria. Conclusion Atypical cold urticaria should be suspected in all cases of anaphylaxis related to cold exposure (i.e. contact with water) with a negative cold stimulation test.

Abstract Background Despite not being licensed for the treatment of infantile hemangiomas (IH) in infants younger than 5 weeks or older than 5 months, propranolol is often used in these age groups to prevent or to treat potentially severe complications. The objective of the present study was to review the experience of 8 Italian pediatric and dermatologic centers regarding propranolol treatment for IH started before 5 weeks or after 5 months of age. Methods We retrospectively reviewed the records of patients followed up for IH, on propranolol treatment started before 5 weeks or after 5 months of age, and collected information on sociodemographic data, treatment indications, IH involution, IH relapse, and treatment side effects. Results A total of 343 patients were enrolled; 15 were started on propranolol before 5 weeks (group 1), 328 were started after 5 months of age (group 2). The most frequent indications were permanent aesthetical disfigurement (91.8%) and function threatening complications (42.6%). In most cases, the treatment was effective. The involution was partial in 67.7% of patients. In 11.8% of cases a relapse was observed. No relapse was observed in group 1. Treatment complications were reported in 15.8% of children, most frequently sleep disorders (6.6%), followed by irritability (5.1%) and diarrhea (2.2%). Only a case of mild constipation was observed in group 1. Conclusion The safety and effectiveness profile of propranolol in infants younger than 5 weeks or older than 5 months may be acceptable. Taking in account propranolol's potential in preventing severe complications, further studies should assess the acceptability of propranolol treatment, especially in the

Il contributo affronta la questione del finanziamento e del costo dei decreti pubblici e si propone di ricostruire, sulla base dell’ analisi dei casi esemplari di Atene, Delo e Cos, i fattori economici che determinavano la formazione del prezzo di una stele pubblica. A questo scopo si analizzano i processi di pubblicazione e produzione, dallo stanziamento dei fondi e dal bando dei contratti, al reperimento di materiale e mano d’opera, all’organizzazione della bottega, fino al trasporto e alla collocazione del prodotto finito. This paper analyzes the production of a marble stele and its costs from the public decision to publish an inscription to the erection of the final product. On the basis of the case studies of Athens, Cos and Delos, the paper discusses the supply of materials, the work of the mason, the labor cost and the letting out of the contracts for the production.