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This study investigated the production of poly(lactic acid) (PLA) nanofibers containing cypress (CUP) essential oil (EO) via electrospinning. The nanofibers were produced from polymer solution prepared with different percentages of cypress EO. Cypress EO-containing PLA nanofibers were characterized and some mechanical and thermal properties were examined using thermogravimetric analysis, scanning electron microscopy, Fourier-transform infrared spectroscopy, and dynamic mechanical analysis. The thermal stability of the nanofibers was reduced depending on the percentage of the cypress EO. As the ratio of the cypress EO to polymer matrices was increased, it was observed that the glassy transition temperatures of the nanofibers decreased and their flexibility increased. The T g value was determined to be 53.74°C for the neat PLA nanofiber, while 51.83°C for the PLA-CUP nanofiber (containing 15% cypress EO). According to the results of releasing trial, the increased amount of cypress EO resulted in less cypress EO releasing from polymer matrices. The nanofibers were observed to exhibit antibacterial activity against Escherichia coli and Staphylococcus aureus. The inhibition zone diameter of the nanofibers containing 10% cypress EO was 20 mm for S. aureus and 16 mm for E. coli, while 10 mm in the presence of Kanamycin.

Callosobruchus maculatus (Fabricius), also known as the cowpea beetle or pulse beetle, is the most important pest of stored cowpea. The beetle causes both qualitative and quantitative losses in the grains, all of which result in low crop yields. The objective of this work was to investigate the insecticidal effect of rose essential oil (REO) on adult Callosobruchus maculatus in terms of mortality and oviposition. In addition, the viable mesophilic aerobic bacteria of C. maculatus were evaluated, and surface molecular electrostatic potential (MEP) maps were examined to determine the interaction domains of the bioactive components of REO. The interaction mechanism between the AChE enzyme (PDB ID: 6XXY, 4EY6, 4EY7 and 6O4W) and the REO components was investigated using the molecular docking method. The highest mortality of 92.40 ± 0.03% was obtained at 16 μL within 72 h of exposure. Higher concentrations of the essential oil decreased the laying performance of C. maculatus. A significant decrease of 91.33% in the number of eggs laid was observed. Gas chromatography–mass spectrometry (GC/MS) results showed that the major constituents of Rosa damascena Miller essential oil (REO) were citronellol (46.15%), geraniol (16.19%), nonadecane (8.80%) and nerol (7.43%). Treatments of 4, 8, 12 and 16 μL of REO significantly reduced the viable mesophilic aerobic bacteria of C. maculatus samples compared with the control. It was found that the most effective binding was between the REO components and the 4EY7 protein, with binding energies ranging from −6.9 to −8.9 kcal/mol. Although the major component of REO was citronellol (46.15%), the best interaction was observed with farnesol isomer B (0.80%). Overall, these results suggest that REO has the potential to control C. maculatus as an environmentally friendly alternative to synthetic pesticides. [ABSTRACT FROM AUTHOR]

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Major histocompatibility complex class II (MHC‐II) deficiency or bare lymphocyte syndrome (BLS) is a rare, early‐onset, autosomal recessive, and life‐threatening inborn error of immunity. We aimed to assess the demographic, clinical, laboratory, follow‐up, and treatment characteristics of patients with MHC‐II deficiency, together with their survival. We retrospectively investigated 21 patients with MHC‐II deficiency. Female/male ratio was 1.63. The median age at diagnosis was 16.3 months (5 months–9.7 years). Nineteen patients (90.5%) had parental consanguinity. Pulmonary diseases (pneumonia, chronic lung disease) (81%), diarrhoea (47.6%), and candidiasis (28.6%) were common. Four (19%) had autoimmunity, two developed septic arthritis, and three (14%) developed bronchiectasis in the follow‐up. Three patients (14%) had CMV viraemia, one with bilateral CMV retinitis. Eight (38.1%) had lymphocytopenia, and four (19%) had neutropenia. Serum IgM, IgA, and IgG levels were low in 18 (85.7%), 15 (71.4%), and 11 (52.4%) patients, respectively. CD4+ lymphocytopenia, a reversed CD4+/CD8+ ratio, and absent/low HLA‐DR expressions were detected in 93.3%, 86.7%, and 100% of the patients, respectively. Haematopoietic stem cell transplantation (HSCT) was performed on nine patients, and four died of septicaemia and ARDS after HSCT. The present median age of patients survived is 14 years (1–31 years). Genetic analysis was performed in 10 patients. RFX5 homozygous gene defect was found in three patients (P1, P4 and P8), and RFXANK (P2 and P14) and RFXAP (P18 and P19) heterozygous gene defects were found in each two patients, respectively. This large cohort showed that BLS patients have severe combined immunodeficiency (SCID)‐like clinical findings. Flow cytometric MHC‐II expression study is crucial for the diagnosis, differential diagnosis with SCID, early haematopoietic stem cell transplantation (HSCT), and post‐HSCT follow‐up. Genetic studies are required first for matched family donor evaluation before HSCT and then for genetic counselling. [ABSTRACT FROM AUTHOR]

This scientific letter explores the limitations of next-generation sequencing (NGS) techniques in diagnosing inborn errors of immunity (IEI). The authors present a case study of a patient with LRBA deficiency who could not be diagnosed using NGS panel and whole-exome sequencing. They emphasize the importance of clinical suspicion and the need for additional diagnostic techniques, such as Sanger sequencing. The authors also discuss a study that compares the levels of biological defects in patients with LRBA deficiency and CTLA-4 insufficiency, using whole-genome sequencing to analyze a cohort of individuals with primary immunodeficiency. The findings shed light on the genetic factors and mechanisms involved in these conditions. [Extracted from the article]

Background. A clinical presentation similar to severe combined immunodeficiency (SCID) with defective T cell activation but normal lymphocyte development occurs due to certain molecule defects including ORAI1- and STIM1. Case. A four-month-old girl sufferd from fever, restlessness, diarrhea, and poor weight gain following the neonatal period. There was consanguinity and a positive family history. She had hypotonia and spontaneous opisthotonic posture. Refractory and extensive CMV infections were detected; immunological investigations revealed normal quantitative immunoglobulins and low numbers of CD3+, CD4+, and CD8+ cells. The next generation sequencing analysis revealed a mutation in the ORAI1 gene. Conclusions. The present patient's history of refractory and widespread CMV infections shows a clinically substantial reduction in resistance against opportunistic microorganisms. This case emphasizes the importance of considering STIM1 and ORAI1 defects in patients with SCID phenotype and neurologic involvement, such as hypotonia. [ABSTRACT FROM AUTHOR]

Objective: Chronic mucocutaneous candidiasis leads to persistent or recurrent fungal infections of the nail, skin, oral, and genital mucosa. Impaired interleukin 17-mediated immunity is a cause of chronic mucocutaneous candidiasis. We aimed to show the pathogenicity of a novel interleukin 17 receptor A mutation through functional studies. Materials and Methods: After next-generation sequencing analysis showed the interleukin 17 receptor A variant, we confirmed the variant by Sanger sequencing and functional validation of the variant by flow cytometry. Results: We present the case of a 6-year-old male patient who presented with recurrent oral and genital Candida infections and eczema. He had staphylococcal skin lesions, fungal susceptibility, and eczema. The patient carried a novel homozygous nonsense [(c.787C> T) (p.Arg263Ter)] mutation in the interleukin 17 receptor A gene. Sanger sequencing confirmed the variant and revealed the segregation of the variant in the family. We used flow cytometry to detect interleukin 17 receptor A protein expression in peripheral blood mononuclear cells from patients and measured Th17 cell percentage. We observed low interleukin 17 receptor A protein expression in patient peripheral blood mononuclear cells, decreased CD4+ interleukin 17+ cell percentage, and decreased interleukin 17F expression in CD4+ cells compared to healthy controls. Conclusions: Innate immune defects may lead to chronic recurrent fungal and bacterial infections of the skin, mucosa, and nails. Generally, genetic and functional analysis is needed in addition to basic immunological tests. [ABSTRACT FROM AUTHOR]

Background/aim: Reducing lymphedema-associated burden and disability in the pediatric setting requires improved awareness and understanding clinical properties of the lymphedema. The aim of this study was to evaluate the clinical and demographic characteristics of patients with pediatric lymphedema presented to different lymphedema centers in Turkey. Materials and methods: The socio-demographic and clinical characteristics of the children including age, gender, presence of genetic syndromes, duration of edema, site and stage of lymphedema and the received therapies were determined. Parental and children education on self-management techniques were recorded. Results: A total of 122 children (female: 66, male: 56) with a mean age of 120.7 ± 71.2 months were included from 7 centers. Of them; 92% had primary, 8% had secondary lymphedema mostly due to infection and trauma. Lymphedema was part of a syndrome in 18% of the children. The most common site of involvement was the lower extremity, followed by upper extremity and genital involvement. Lymphedema was complicated in 17 % of children, mainly with a clinical picture of cellulitis, infection, and pain. The median duration of lymphedema was 41 (5-216) months. Although most of the children had stage 2 lymphedema, only 40% of them received treatment. The most commonly received treatment was compression therapy. No family or child was educated for self- care management before. Conclusion: In conclusion, pediatric lymphedema has a comparable gender distribution and usually involves the lower extremities. Although most of the children had advanced disease, more than half of the patients did not receive any treatment indicating the unmet need for management of lymphedema. The education of patients and/or children about self-management methods were lacking. We suggest educational activities for both families of children with lymphedema and health care providers, in order to facilitate early reference to lymphedema units and to receive prompt preventive and therapeutic approaches for this suffering condition. [ABSTRACT FROM AUTHOR]