Insights into Patient Experiences with Facilitated Subcutaneous Immunoglobulin Therapy in Primary Immune Deficiency: A Prospective Observational Cohort.

BACKGROUND: Immunoglobulin G replacement therapy (IgRT), intravenous (IV) and subcutaneous (SC) routes, is pivotal in treatment of primary immunodeficiencies (PID). In recent years, facilitated subcutaneous immunoglobulin (fSCIG), a combination of rHuPH20 and 10% IgG has emerged as a delivery method to combine advantages of both IV and SC. METHOD: In an observational prospective cohort, we investigated patient experience with fSCIG in PID patients from 5 PID centers for up to 12 months. We assessed the efficacy and safety of this treatment with patient/caregiver- and physician-reported indicators. Additionally, we analyzed patient treatment satisfaction (TSQM-9) and quality of life (QoL). RESULTS: We enrolled 29 patients (22 pediatric and 7 adults; 14 females and 15 males; (median: 15, min-max: 2-40.9 years) who initiated fSCIG as IgRT-naive (n = 1), switched from conventional rapid-push 10% SCIG (n = 6) or IVIG (n = 22). Among the participants, 19 (65%) exhibited antibody deficiencies, 8 (27%) combined immunodeficiencies, and 2 (7%) immune dysregulations. Remarkably, targeted trough immunoglobulin G levels were achieved under all previous IgRTs as well as fSCIG. No severe systemic adverse drug reactions were documented, despite prevalent local (%86.45) and mild systemic (%26.45) adverse reactions were noted with fSCIG. Due to mild systemic symptoms, 2 patients switched from fSCIG to 10% SCIG. The patient satisfaction survey revealed a notable increase at 2-4th (p = 0.102); 5-8th (p = 0.006) and 9-12th (p < 0.001) months compared to the baseline. No significant trends were observed in QoL surveys. CONCLUSION: fSCIG demonstrates admissable tolerability and efficacy in managing PIDs in addition to notable increase of patients' drug satisfaction with IgRT. The identified benefits support the continuation of this therapy despite the local reactions.

COVID-19 and vaccination in hereditary angioedema: Single center experience.

Like many microbial agents, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and its vaccination may increase the frequency and/or severity of attacks. We aimed to observe/evaluate patients with hereditary angioedema (HAE) followed by Sakarya University Research/Training Hospital pediatric allergy unit, Sakarya, Türkiye, for the effects of SARS-CoV-2 infection and COVID-19 vaccination. Ten HAE patients-3 males and 7 females-were evaluated retrospectively. Their mean age was 31.80 ± 19.15 (min. 12 - max. 66) years. Four of 10 patients were diagnosed with type 1 HAE and 6 with type 2 HAE. Two out of 6 patients (mother and daughter) diagnosed with type 2 HAE had angioedema attacks during COVID-19 disease. Six out of 10 HAE patients received different COVID-19 vaccines available in Türkiye up to third and fourth doses. There was no increase in COVID-19 vaccine-related attacks during, after 72 hours, and up to the year after vaccination. As a result, we consider it safe to administer inactivated and/or mRNA vaccines in our patients with HAE. In addition, catching SARS-CoV-2 infection was not always associated with disease exacerbation or activation.

COVID-19 frequency and clinical course in children with asthma.

BACKGROUND: The epidemic of severe acute respiratory syndrome coronavirus 2 infection, known as the coronavirus disease 2019 (COVID-19), has caused a global health concern. Since its emergence, numerous studies have focused on various clinical manifestations and outcomes in different populations. However, studies are ongoing as the consequences and impact of COVID-19 in children with chronic diseases such as asthma are controversial. AIM: To fill this research gap by retrospectively evaluating the course, laboratory, and clinical findings of COVID-19 among 414 asthmatic children followed up from the pediatric allergy outpatient clinic and known to have had COVID-19. METHODS: The data of 5510 patients over the age of 5 diagnosed with asthma in our hospital's data were retrospectively scanned with specific parameters using protocol numbers from the hospital filing system. The data included retrospective evaluation of pulmonary function test results before and after COVID-19, routine hematological and biochemical parameters, sensitization states (total IgE, specific IgE, and skin prick test results), and radiological (computed tomography) findings. To inquire about the course and symptoms of COVID-19, asthma patients or their parents were then called and evaluated with a questionnaire. RESULTS: As a result of retrospectively scanning the data of 5510 asthma patients over the age of 5, it was determined that 414 (7.5%) patients had COVID-19. The mean age of 414 patients was 17.18 ± 4.08 (min: 6; max: 28) years. Two hundred and three of our 414 patients are male, and 211 are female. When their vaccination status was questioned, 21.5% were vaccinated. When the symptoms of our 290 patients were questioned, it was stated that 59.0% had fever symptoms. The rate of using regular prophylactic asthma medications was 19%. The rate of using salbutamol in asthma was found to be 22%. The rate of patients using methylprednisolone was 1%. Emergency service admission was 17.2%, and hospitalization was found to be 4.8%. Leukopenia (< 4000) was found in 14.1% of patients, and 8.08% of our patients had neutropenia (< 1500). Lymphopenia (< 1500) was detected in 44.4% of patients, and lymphocytosis (> 4000) was found in 5.05% of patients. In 65% of our patients, the C-reactive protein value was elevated. A high aspartate aminotransferase and alanine aminotransferase value was detected in 3.2% and 5.4% of patients were found, respectively. 31% of patients had an elevated lactate dehydrogenase value. Typical radiological findings for COVID-19 were detected in 3/309 of patients. CONCLUSION: According to our study, there is a correlation between the severity of COVID-19 and asthma symptoms and the course of the disease. However, it is worth noting that the retrospective nature of the study and the differences in sample size, age, and demographic characteristics between the two groups do not allow for an optimal comparison. Therefore, further investigation is needed to explore the relationship between COVID-19 and asthma, and it can be suggested that COVID-19 may trigger asthma attacks and asthma may impact the course of COVID-19.

Mast Cell's Role in Cytokine Release Syndrome and Related Manifestations of COVID-19 Disease.

In this narrative review, firstly, we describe the characteristics of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and the pathogenesis of its infection in humans. Later, the importance of mast cells in SARS-CoV-2 infection and their role in Coronavirus Disease 2019 (COVID-19) will be discussed. SARS-CoV-2 is a transmissible agent frequently detected in some mammalian species and also in humans. Literature data published in PubMed that covered mast cells' role in cytokine release syndrome and related manifestations of COVID-19 disease were reviewed by the authors independently and collectively. Recommendations for the management of cytokine release syndrome and related manifestations were made by the authors. Mast cells are concentrated in environments where they encounter viruses, bacteria, and toxins, especially in the skin, nasal mucosa, lungs, airways, gastrointestinal tract, and meninges, to prevent their entry into the human body. Once SARS-CoV-2 enters the host, it stimulates one of the mast cells, together with pre-existing innate immune cells that form a defensive barrier in the submucosa of the respiratory tract and nasal cavities against pathogenic microorganisms. The roles of mast cells in SARS-CoV-2-induced hyperinflammation and cytokine storms have recently been one of the hot topics in the literature. Physicians should keep in mind the mast cells' role in cytokine release syndrome and related manifestations of COVID-19 disease. Mast cell-targeting therapies (e.g., H1 and H2 receptor antagonists) can reduce the severity and course of the disease when used after complications associated with COVID-19 are suspected or seen.