Clinical and immunological phenotypes of selective IgM deficiency in children: Results from a multicenter study

background: a few studies assessed the clinical and immunological features of selective IgM deficiency (SIgMD), especially in the pediatric age. we aimed to characterize the clinical and immunological phenotypes of a cohort of pediatric patients with SIgMD according to the different diagnostic criteria available. methods: In this multicenter study, we evaluated pediatric SIgMD patients diagnosed at the pediatric clinic in pavia, Italy, or through the Italian primary Immunodeficiency NETwork (IPINET) and monitored changes in their diagnosis over a time frame that ranges from several months to several years. results: forty-eight patients with SIgMD were included (mean serum IgM: 33 mg/dL). the most common clinical manifestations were recurrent infections (67%) and allergies (48%). subgroup analysis according to SIgMD definition criteria of the european society for Immunodeficiencies (ESID) showed no significant difference in clinical manifestations, also considering the group with additional immunological abnormalities. sixteen patients had long-term follow-up, during which 87% preserved their SIgMD diagnosis, while two patients showed a reduction in IgA in addition to low IgM. conclusions: our data suggest that the identification of a reduction in serum IgM in children should lead to a complete immunological work-up to obtain a comprehensive clinical and immunological characterization of the patient. the follow-up of these patients is fundamental to define the disease evolution and appropriate management

An Italian Multicenter Study on the Epidemiology of Respiratory Syncytial Virus During SARS-CoV-2 Pandemic in Hospitalized Children

Since the beginning of 2020, a remarkably low incidence of respiratory virus hospitalizations has been reported worldwide. We prospectively evaluated 587 children, aged <12 years, admitted for respiratory tract infections from 1 September 2021 to 15 March 2022 in four Italian pediatric hospitals to assess the burden of respiratory viruses during the COVID-19 pandemic in Italy. At admission, a Clinical Respiratory Score was assigned and nasopharyngeal or nasal washing samples were collected and tested for respiratory viruses. Total admissions increased from the second half of October 2021 to the first half of December 2021 with a peak in early November 2021. The respiratory syncytial virus (RSV) incidence curve coincided with the total hospitalizations curve, occurred earlier than in the pre-pandemic years, and showed an opposite trend with respect to the incidence rate of SARS-CoV-2. Our results demonstrated an early peak in pediatric hospitalizations for RSV. SARS-CoV-2 may exhibit a competitive pressure on other respiratory viruses, most notably RSV

The impact of allergic rhinitis on bronchial asthma: What therapy?

Childhood asthma constitutes a significant healthcare burden due to its chronic nature and prevalence, affecting approximately 5–10 % of the pediatric population. Asthma is often heterogeneous and characterized by chronic airway inflammation. It is classified into two main phenotypes based on airway inflammation: T2-high (eosinophilic) and T2-low (neutrophilic/pauci-granulocytic) phenotype. Allergic sensitization is commonly associated with T2-high asthma, involving T helper 2 and innate immunity cells and type 2-associated cytokines, including interleuikin-4 (IL-4), IL-5, and IL-13. Type 2 inflammation is characterized by eosinophilic infiltrate. Asthma and allergic rhinitis share common mechanisms, and their association, known as united airways disease, highlights the close interconnection between upper and lower airways. Effective allergic rhinitis control positively impacts asthma, emphasizing the importance of early identification and personalized therapeutic strategies. One is allergen immunotherapy, a disease-modifying approach demonstrating preventive and therapeutic efficacy in children. However, more research is needed to optimize pediatric allergic rhinitis management

Pd-Promoted Homocoupling Reactions of Unsaturated Silanes in Aqueous Micelles (Eur. J. Org. Chem. 12/2010)

The cover picture shows a Pd-promoted homocoupling reaction of polyenylsilanes carried out in water containing nonionic amphiphiles. The reaction in the “green” aqueous environment affords extended conjugated polyenes with higher yields relative to those obtained when the same process is performed in organic solvents. Details are discussed in the article by G. M. Farinola et al. on p. 2275 ff. The background shows a nice view of Polignano a Mare, a lovely small town few miles away from Bari, located on a sea cliff along the Adriatic coast of Puglia

A Combinated Theoretical and Experimental Investigation on the Enantioselective Oxidation of Aryl Benzyl Sulfides in the Presence of a Chiral Titanium Catalyst

An experimental investigation of the enantioselective oxidation of aryl benzyl sulfides by tert-butyl hydroperoxide in the presence of a titanium/ hydrobenzoin catalyst has shown that these sulfides are ideal substrates for this catalytic system, with negligible interference by the substituents on the aryl groups. A reaction mechanism based on DFT computations has been proposed. The DFT MPWB1K functional was used in the theoretical investigation to account for weak hydrogenbonding and p interactions. The computed reaction profile explains the experimentally observed enantioselectivity, which is determined by the thermodynamics of the first phase of the reaction. A detailed discussion of the hydrogen- bonding and p interactions that drive the reaction along the observed stereochemical path is give

Eosinophilic Gastrointestinal Diseases in Inborn Errors of Immunity

Inborn errors of immunity (IEI) are disorders mostly caused by mutations in genes involved in host defense and immune regulation. Different degrees of gastrointestinal (GI) involvement have been described in IEI, and for some IEI the GI manifestations represent the main and characteristic clinical feature. IEI also carry an increased risk for atopic manifestations. Eosinophilic gastrointestinal diseases (EGIDs) are emerging disorders characterized by a chronic/remittent and prevalent eosinophilic inflammation affecting the GI tract from the esophagus to the anus in the absence of secondary causes of intestinal eosinophilia. Data from the U.S. Immunodeficiency Network (USIDNET) reported that EGIDs are more commonly found in patients with IEI. Considering this element, it is reasonable to highlight the importance of an accurate differential diagnosis in patients with IEI associated with mucosal eosinophilia to avoid potential misdiagnosis. For this reason, we provide a potential algorithm to suspect an EGID in patients with IEI or an IEI in individuals with a diagnosis of primary EGID. The early diagnosis and detection of suspicious symptoms of both conditions are fundamental to prevent clinically relevant complications

Targeted therapies and complete responses in first line treatment of metastatic renal cell carcinoma. A meta-analysis of published trials

Antiangiogenic agents (AAs) have reported grater efficacy compared to interferon. Despite these advances, radiological complete response to therapy is rare. We meta-analyzed the incidence of complete response in patients treated with AAs and in controls in main randomized clinical trials for first-line therapy in metastatic renal cell carcinoma.PubMed was reviewed for phase II-III randomized clinical trials with AAs vs. non-AAs in patients with good or intermediate prognosis. We calculated the relative risk of events in patients assigned to AAs compared to control.Five RCTs were found; four were phase III and one was phase II. A total of 2747 patients was valuable for final analysis and randomized to receive AAs or control. Patients in the control-group had interferon (85%) or placebo (15%); patients in the AAs-group received bevacizumab (48%), sunitinib (26%), pazopanib (20%) or sorafenib (6%).The incidence of complete response in patients treated with AAs was 2.0% (95% CI, 1.2-2.8) compared to 1.4% (95% CI, 0.7-2.1) in the control arm. Comparing the different type of AAs, the incidence of complete response was 2.5% (95% CI, 1.2-3.8) in the bevacizumab group and 1.6% (95% CI, 0.1-2.5) in the TKIs group. The relative risk to have a complete response was 1.52 (95% CI, 0.85-2.73; p=0.16) in patients treated with AAs compared to controls; this was found higher in patients treated with TKIs compared to bevacizumab.The complete response is a rare event in metastatic kidney tumor, even if AAs reported greater efficacy in terms of progression-free survival and of overall response rate, they did not increase the curative rate of metastatic disease. Probably, some biologic factors other than angiogenesis may influence the complete response in this disease. © 2013 Elsevier Ltd

Optimizing Patient Surgical Management Using WhatsApp Application in the Italian Healthcare System

Introduction: Smartphones changed the method by which doctors communicate with each other, offer modern functionalities sensitive to the context of use, and can represent a valuable ally in the healthcare system. Studies have shown that WhatsApp\u2122 application can facilitate communication within the healthcare team and provide the attending physician a constant oversight of activities performed by junior team members. The aim of the study was to use WhatsApp between two distant surgical teams involved in a program of elective surgery to verify if it facilitates communication, enhances learning, and improves patient care preserving their privacy. Methods: We conducted a focused group of surgeons over a 28-month period (from March 2013 to July 2015), and from September 2014 to July 2015, a group of selected specialists communicated healthcare matters through the newly founded "WhatsApp Surgery Group." Each patient enrolled in the study signed a consent form to let the team communicate his/her clinical data using WhatsApp. Communication between team members, response times, and types of messages were evaluated. Results: Forty six (n = 46) patients were enrolled in the study. A total of 1,053 images were used with an average of 78 images for each patient (range 41-143). 125 h of communication were recorded, generating 354 communication events. The expert surgeon had received the highest number of questions (P, 0.001), while the residents asked clinical questions (P, 0.001) and were the fastest responders to communications (P, 0.001). Conclusion: Our study investigated how two distant clinical teams may exploit such a communication system and quantifies both the direction and type of communication between surgeons. WhatsApp is a low cost, secure, and fast technology and it offers the opportunity to facilitate clinical and nonclinical communications, enhance learning, and improve patient care preserving their privacy

Successful treatment with Omalizumab of a child affected by Systemic Mastocytosis: clinical and biological implications

Abstract Background Pediatric Mastocytosis is a rare and heterogeneous disease, characterized by accumulation of mast cells in the skin (Cutaneous Mastocytosis) and/or, less frequently, in other organs, mainly liver, spleen, bone marrow, lymph nodes and gastrointestinal tract (Systemic Mastocytosis). Patients affected by Systemic Mastocytosis show symptoms caused by  a massive release of mast cell mediators: itching, flushing, abdominal pain, generalized weakness, fatigue and neuropsychiatric disorders. Moreover, children with Systemic Mastocytosis are at greater risk of anaphylactic/anaphylactoid reactions, often poorly controlled by the conventional therapy with antihistamines, mast cells stabilizers and steroids. As a result, children affected by Systemic Mastocytosis have a poor quality of life and suffer the consequence of prolonged steroidal treatment. Case presentation A child with Systemic Mastocytosis and severe symptoms, refractory to symptomatic and steroidal therapy, has been successfully treated with Omalizumab, an anti-IgE monoclonal antibody usually employed in allergic patients with severe asthma and orticaria. The onset of clinical benefit of Omalizumab therapy was extraordinarily rapid, but proved to be strictly dependent on drug administration. The child has become completely and steadily asymptomatic. No other anaphylactic episodes have been reported. Steroid treatment could be definitively withdrawn after the second dose of Omalizumab, and all the other medications were later reduced. Twenty months after beginning, Omalizumab therapy is still ongoing with good symptomatology control; no side effects have been observed so far. Conclusions In our experience, Omalizumab is an effective treatment for children affected by Systemic Mastocytosis not responding to conventional medical treatments. The main strengths of this therapy are its rapid and extraordinary efficacy to control the severe mast cells mediator-related symptoms, the lack of side effects and its steroid-sparing effect. However, more extensive and controlled studies in pediatric patients affected by Systemic Mastocytosis are needed to substantiate these promising findings