IPEX syndrome is an X-linked inborn error of immunity clinically characterized by the triad of: enteropathy, polyendocrinopathy and eczema. However many other clinical presentations lacking the triad above described have been reported what underpin the need of careful clinical suspicion, immunological evaluation and genetic sequencing. Here we report a case of a Brazilian boy...
Genetic defects in the development, maturation, and/or function of the immune cells can lead to Inborn errors of immunity (IEI) which may predispose patients to malignancies. The overall risk for cancer in children with IEI ranges from 4 to 25% and the type of malignancy is highly dependent on the specific mutant gene underlying IEI. We investigated 3056 IEI patients registered...
The antigen avoidance has been used in the diagnosis and treatment of hypersensitivity pneumonitis (HP); however, its usefulness in stable fibrotic HP is controversial. To investigate the usefulness of the antigen avoidance test in patients with fibrotic HP in stable phase. The antigen avoidance test was conducted during a 2-week hospitalization comparing clinical parameters at...
To date, little consideration has been given to access to allergy-related care, despite the fact that food allergy affects a considerable proportion of children. As such, the current study aimed to describe access to food allergy-related services in Canada and the United States (US). Participants were recruited via social media from March-July 2021 and were asked to complete an...
In anaphylaxis, the dosing of injectable epinephrine in medical settings has been arbitrarily recommended to be 0.01 mg/kg of body weight. For ethical reasons, there have been no dose–response studies or double-blind studies performed on patients with active anaphylaxis. Intramuscular delivery of epinephrine has been the standard. Auto-injectors for use in the treatment of...
Long non-coding RNAs (lncRNAs) have been extensively reported to play critical roles in the pathogenesis of various disease, especially in cancer. However, little is known about the role of lncRNAs in the pathogenesis of pediatric allergic asthma. High-throughput sequencing analysis was performed to identify differentially expressed mRNAs and lncRNAs in peripheral blood...
Sensitization to Ara h 2 has been proposed as a promising biological marker for the severity of peanut allergy and may reduce the need for oral food challenges. This study aimed to evaluate whether peanut oral food challenge is still a useful diagnostic tool for children with suspected peanut allergy and an elevated level of Ara h 2-specific IgE. Additionally, we assessed whether...
Self-administered subcutaneous immunoglobulin G (SCIg) reduces nursing time and eliminates the need for treatment at ambulatory care clinics, as compared with clinic-based intravenously administered IgG (IVIg), and are therapeutically equivalent. Estimating the economic impact of self-administered SCIg versus clinic-administered IVIg therapy may guide treatment recommendations. A...
Risk factors for the pathogenesis of chronic rhinosinusitis (CRS) remain largely undetermined, which is likely due to the heterogeneity of the disease. White blood cell counts have been largely unexplored as a risk factor for CRS even though different types of white blood cells are involved in the inflammatory process of CRS. To investigate causal associations between different...
In this study, it was aimed to investigate the prevalence of type 1 hypersensitivity reaction under tuberculosis treatment and the management of hypersensitivity. The study is a case series. All of the patients who were hospitalized between 01.02.2015–01.05.2021 were examined. All patients who developed a drug-induced type 1 immediate hypersensitivity reaction were included...
Allergic rhinitis (AR) is frequent in children and adolescents and can severely affect their lives. This article describes the development and validation of a questionnaire to assess treatment needs and benefits in children and adolescents, the PBI-AR-K, in a sample of patients receiving grass pollen sublingual immunotherapy. The PBI-AR-K was developed based on an open survey...
Childhood adenoid hypertrophy (AH) is common and is often associated with allergic asthma, resulting in complications like obstructive sleep apnea syndrome (OSAS). Management of the disease and its complications is often challenging. We report here a case of a 10-year-old boy who suffered from severe allergic asthma and rhinitis and was treated with omalizumab. Before the...
Idiopathic pulmonary fibrosis (IPF) is a fatal fibrotic lung disease with limited treatment options. Plumbagin (PL) is an herbal extract with diverse pharmacological effects that have been recently used to treat various types of cancer. This study aims to explore the anti-fibrotic effect of PL and possible underlying mechanisms in IPF. We used a bleomycin-induced experimental...
The effects of a delayed diagnosis of asthma on lung function in children have not been well investigated. Therefore, a retrospective cohort study was conducted in a children’s hospital to analyse the effect of delayed diagnosis time on lung function in children with asthma. We conducted a retrospective cohort study in Jinan Children's Hospital from January 1, 2010, to December...
Since the first reported case of COVID-19, infections due to the virus have ranged from mild to severe. Patients with inborn errors of immunity are thought to be at increased risk for infections such as COVID-19 due to the nature of their disease and being immunocompromised. Similarly, pregnant women by nature of physiological changes in immunity are susceptible to infections and...
The lymphocyte transformation test (LTT) is an in vitro assay used to diagnose drug induced hypersensitivity reactions by detecting the activation and expansion of drug-specific memory T cells to the suspected implicated drug. Traditionally radiolabelled thymidine (3H-thymidine) has been used but requires the handling and disposal of radioactive materials. To examine safe...
The proliferation of airway smooth muscle cells (ASMCs) contributes to the contractility and inflammation in the pathophysiology of asthma. This intrigued us to clarify the effect of microRNA (miR)-224-5p on biological characteristics of ASMCs in mice with asthma-like airway inflammation and responses through the FHL1-dependent MAPK pathway. An ovalbumin (OVA)-induced asthma...
Dupilumab-induced ocular surface disease (DIOSD) has been reported in patients with atopic dermatitis treated with dupilumab, and has been recognized as an adverse event of dupilumab. Our objective was to describe two cases of DIOSD with alterations in eotaxin-2 and interleukin (IL)-8 messenger ribonucleic acid (mRNA) expression on the ocular surface. In the ocular surface test...
Fecal calprotectin (FC) is a noninvasive biomarker for assessing the inflammatory status of the gastrointestinal tract. The aim of this study was to determine the association between FC levels and Eosinophilic colitis (EC) before and after treatment in pediatrics. In this cross-sectional study, 330 patients with rectorrhagia and FC levels > 200 μg/g were included in the study...
Immunoglobulin replacement therapy is the standard-of-care treatment for patients with primary immunodeficiency diseases who have impaired antibody production and function. Clinicians and patients may consider intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (SCIG) options, and each route may offer different benefits for the individual. IVIG requires fewer...
The COVID-19 pandemic has highlighted disparities in healthcare, particularly in the United States, even though disparities have existed since the organization of the modern healthcare system. Recruitment of patients from racial and ethnic minority groups is often minimal in phase 3 clinical trials, and is further exacerbated in the case of trials for rare diseases such as...
Reports of allergic reactions to the COVID-19 vaccines have been documented, which may also contribute to hesitancy. Despite the low likelihood that the COVID-19 vaccine will trigger an allergic reaction, we and others have reported that families with allergy remain vaccine hesitant due to concerns of COVID-19-vaccine-triggered anaphylaxis. To present our scoping review protocol...
Hereditary angioedema (HAE) is an inherited disease characterized by recurrent angioedema without urticaria or pruritus. The most common types of HAE are caused by deficiency or dysfunction in C1 esterase inhibitor (C1-INH-HAE). The association between C1-INH-HAE and systemic lupus erythematosus (SLE) is known; however, variations in the underlying pathophysiology, disease course...
COVID-19 presents with a variable clinical course from asymptomatic to severe respiratory distress with nearly 25% mortality in mechanically-ventilated patients. As such, there is uncertainty regarding how host factors modulate the disease course. This report examines these factors in two geriatric patients with multiple comorbid conditions who were residents of the long-term...
Asthma, allergic rhinitis and eczema have been identified as the most prevalent childhood allergic diseases. However, the prevalence of these allergic diseases can vary in different regions within a country and in the world. The objective of the study was to estimate the prevalence of asthma, allergic rhinitis and eczema in schoolchildren in the Kandy and Anuradhapura districts...