Search

The immune system is divided ilito two major branches: innate and cidaptive. The iniiate immune system is the body's first line of defense and rapidly responds in a nonspecific manner to various microorganisms, foreign materials, or injuries. Phagocytes, which include tilacrophages, nionocytes, and netttrophils, are iminte immune cells tliat con surround and kill microorganisins, ingest foreign material, at:id remove dead cells. They also indirectly boost both innate and adaptive immune responses through Various activation signals. Plingocytic defects characteristically lead to filligal aild bacterial infections of the respiratory tract, lymph nodes, skill, and other organ systems, mid they are commonly associated with iii.flammatory bowel disease. This prinier-will review high-yield innate defects of phagocytic cells, inchiding defects of respiratory (oxidative) burst, defects of neutrophil migration, cyclic and severe congenital neutropenias and associated disorders, and other phagocyte defect disorders. [ABSTRACT FROM AUTHOR]

Background: The normally acidic skin pH changes in atopic dermatitis (AD) to alkaline, which contributes to the associated skin-barrier dysfunction. Hence, acidic cleansers would be preferred, but such information is scarce. Objective: Guiding health-care providers and patients on selecting skin cleansers with a pH optimal for AD. Methods: A total of 250 products were tested: 37 soaps (32 bars, 5 liquid) and 213 syndets (14 bars, 199 liquid); 10% solutions were tested for pH by using a pH meter; pH values 6.65–7.35 were considered neutral. Results: The pH of the tested skin cleansers varied widely (3.59–10.83). All 37 soaps were highly alkaline. In the 14 syndet bars, the pH was neutral in 6, alkaline in 8, and acidic in none. In the 199 syndet liquids, the pH was acidic in 84.9%, neutral in 11.1%, and alkaline in 4.0%. The product’s pH was disclosed in none of the 37 soaps and in only 32 syndets (15%), of which 9 bars were labeled “balanced,” whose measured pH was neutral in 6 and alkaline in 3. Of the other 23 syndets, the labeled pH was referred to as “balanced” in 20 whose measured pH was neutral in 2 (6.80, 6.88) and acidic in 18 (3.59–6.59). The pH in the other three syndets was 4.25–6.00. Conclusion: All tested soaps had undesirable pH, whereas 84.9% of the liquid syndets were acidic (which is desirable) and 11.1% were neutral (which could be acceptable). Only 12.8% of the products disclosed the pH, an issue in need of improvement [ABSTRACT FROM AUTHOR]

Biallelic mutations in the ACP5 gene cause spondyloenchondrodysplasia with immune dysregulation (SPENCDI). SPENCDI is characterized by the phenotypic triad of skeletal dysplasia, innate and adaptive immune dysfunction, and variable neurologic findings ranging from asymptomatic brain calcifications to severe developmental delay with spasticity. Immune dysregulation in SPENCDI is often refractory to standard immunosuppressive treatments. Here, we present the cases of two patients with SPENCDI and recalcitrant autoimmune cytopenias who demonstrated a favorable clinical response to targeted JAK inhibition over a period of more than 3 years. One of the patients exhibited steadily rising IgG levels and a bone marrow biopsy revealed smoldering multiple myeloma. A review of the literature uncovered that approximately half of the SPENCDI patients reported to date exhibited increased IgG levels. Screening for multiple myeloma in SPENCDI patients with rising IgG levels should therefore be considered. [ABSTRACT FROM AUTHOR]

Background: Hymenoptera venom anaphylaxis (HVA) is reported in up to 3% of stings and accounts for approximately 40 US deaths annually. HVA patients require immediate availability of epinephrine and Allergist referrals for consideration of venom immunotherapy. Data regarding epinephrine autoinjector prescriptions, Allergist referral rates, and potential racial disparities are limited. Objective: The primary objective was to determine if there were statistically significant differences in epinephrine autoinjector prescriptions and Allergist referrals between white and African American patients. The secondary objectives were to determine if there were statistically significant differences between adult and pediatric patients and to determine if there were significant differences between epinephrine prescriptions between patients with and without Allergist referrals. Method: This study is a retrospective, descriptive chart review analyzing patients seen between January 01, 2019 and December 31, 2021. Data were obtained utilizing the Epic Systems (Verona, WI) application Slicer Dicer. Individual chart review was performed for age, race, epinephrine autoinjector prescription, and Allergist referral. Results: 342 patients were identified as having HVA. White patients (60 out of 219; 27.4%) were more likely to get epinephrine autoinjector prescriptions than African American patients (17 out of 109; 15.6%) (p = 0.018). Adult patients (25 out of 314; 8.0%) were less likely than pediatric patients (8 out of 28; 28.6%) to have Allergist referrals (p = 0.004). Patients with Allergist referrals (25 out of 32; 78.1%) were more likely to be prescribed an epinephrine autoinjector than patient without Allergist referrals (54 out of 310; 17.4%) (p < 0.00001). Conclusion: Epinephrine autoinjector prescriptions and Allergist referrals are low overall in HVA. Racial disparities were identified with African American patients being significantly less likely to receive epinephrine autoinjector prescriptions. Additionally, adult patients, who may be at increased risk, were less likely to receive Allergist referrals. [ABSTRACT FROM AUTHOR]