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Fruits ofHeracleum crenatifoliumBoiss.,Heracleum sphondyliumL. subsp. ternatum (Velen.) Brummitt, andHeracleum platytaeniumBoiss. (Umbelliferae) were hydrodistilled to obtain essential oils that were then analyzed by GC and GC/MS. The major component was identified as octyl acetate (93.7, 87.6 and 31.6% respectively). Octyl butyrate was also characterized as the main component inH. platytaeniumoil. Furthermore, anticandidal activity of the oils was evaluated using the microdilution broth method. All the oils showed good inhibitory effects againstC. glabrata. [ABSTRACT FROM AUTHOR]

Background: The increasing prevalence of carbapenem-resistant Enterobacteriaceae (CRE) infections among children represents a significant global concern, leading to elevated mortality rates. The aim of this study was to evaluate the risk factors, outcomes, 30-day mortality rates and contributing factors in children with CRE bloodstream infections (CRE-BSIs)., Methods: Data regarding demographic characteristics, treatment approaches and outcomes of hospitalized children aged 0-18 years diagnosed with CRE-BSIs between January 2018 and December 2022 were extracted from medical records. Mortality within 30 days of diagnosis and the predictive factors were analysed., Results: A total of 114 children, with a median age of 11 months (range: 6-69.5), were included. All cases of CRE-BSIs were either healthcare associated or hospital acquired and presented with at least one underlying comorbidity. A previous history of CRE colonization or infection rate was 48.2% (55/114). Klebsiella pneumoniae 87.7% (100/114) was the most frequently isolated microorganism, with a 30-day mortality rate of 14% (16/114). Multivariate analysis identified paediatric intensive care unit admission, invasive mechanical ventilation, inotropic support and thrombocytopenia due to CRE-BSIs as the most discriminative predictors for 30-day mortality (P < 0.001). Central venous catheter (CVC) removal was associated with a reduced mortality rate (P = 0.012). High-dose prolonged infusion of MEM-based or polymyxin-based antibiotic combinations did not impact survival. Lower MEM MIC values were associated with improved survival., Conclusions: The mortality rate of CRE-BSI is notably high in childhood. The use of antibiotic combination strategies did not demonstrate a significant impact on 30-day survival; however, the removal of CVCs was found to lower mortality rates., (© The Author(s) 2024. Published by Oxford University Press on behalf of British Society for Antimicrobial Chemotherapy.)

Purtscher-like retinopathy (PLR) is a rare entity related with retinal ischemia due to endothelial dysfunction and embolization. We present a case of a 17-year-old patient who presented with PLR associated with transplant-associated thrombotic microangiopathy. Visual acuity was finger counting at 1 meter in both eyes. Fundoscopy demonstrated peripapillary cotton-wool spots and hypopigmentation in the middle peripheral retina. Both eyes showed signs of macular edema with intraretinal hemorrhages. Optical coherence tomography and fundus fluorescence angiography was performed to support the diagnosis. After systemic treatments and panretinal photocoagulation, the patient's vision improved and the macular edema resolved. [ Ophthalmic Surg Lasers Imaging Retina 2024;55:603-606.] .

Background: Major histocompatibility complex class II deficiency, a combined immunodeficiency, results from loss of HLA class II expression on antigen-presenting cells. Currently, hematopoietic stem cell transplantation stands as the sole curative approach, although factors influencing patient outcomes remain insufficiently explored., Objectives: To elucidate the clinical, immunologic, and genetic profiles associated with MHC-II deficiency and identify prognostic indicators that affect survival rates., Methods: In this multicenter retrospective analysis, we gathered data from 35 patients with a diagnosis of MHC-II deficiency across 12 centers in Turkey. We recorded infection histories, gene mutations, immune cell subsets, and surface MHC-II expression on blood cells. We conducted survival analyses to evaluate the impact of various factors on patient outcomes., Results: Predominant symptoms observed were pneumonia (n = 29; 82.9%), persistent diarrhea (n = 26; 74.3%), and severe infections (n = 26; 74.3%). The RFXANK gene mutation (n = 9) was the most frequent, followed by mutations in RFX5 (n = 8), CIITA (n = 4), and RFXAP (n = 2) genes. Patients with RFXANK mutations presented with later onset and diagnosis compared with those with RFX5 mutations (P =.0008 and .0006, respectively), alongside a more significant diagnostic delay (P = .020). A notable founder effect was observed in five patients with a specific RFX5 mutation (c.616G>C). The overall survival rate for patients was 28.6% (n = 10), showing a significantly higher proportion in individuals with hematopoietic stem cell transplantation (n = 8; 80%). Early death and higher CD8 + T-cell counts were observed in patients with the RFX5 mutations compared with RFXANK-mutant patients (P = .006 and .009, respectively)., Conclusions: This study delineates the genetic and clinical panorama of MHC-II deficiency, emphasizing the prevalence of specific gene mutations such as RFXANK and RFX5. These insights facilitate early diagnosis and prognosis refinement, significantly contributing to the management of MHC-II deficiency., (Copyright © 2024 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive disorder due to mutations in the TYMP gene. Clinical findings are characterized by neurologic manifestations and severe gastrointestinal dysfunction. The syndrome is usually fatal, the most effective treatment appears to be hematopoietic stem cell transplantation (HSCT)., Procedure: In this retrospective study, we evaluated HSCT that was performed using a reduced toxicity myeloablative conditioning regimen in patients with MNGIE at our center., Results: A total of six allogeneic transplant procedures were performed in four patients. Three patients had fully matched donors, and one patient had a haploidentical donor. Treosulfan-based myeloablative conditioning regimen was applied in five of six transplants. Bone marrow was used as a stem cell source. One patient is being followed up in the 4th year of posttransplant with full chimeric and without graft versus host disease (GVHD). One patient died of acute stage IV gastrointestinal system GVHD. Two patients underwent second transplantation due to engraftment failure, one of which was the patient who had a haploidentical transplant., Conclusions: Treosulfan-based regimen is well tolerated, although engraftment failure with this conditioning regimen can be a significant problem. We share our haploidentical transplant experience, which will be the first reported case in the literature., (© 2023 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Introduction: The increasing incidence of Stenotrophomonas maltophilia ( S. maltophilia ) infections raises concern because of the high fatality/case ratio. This study aimed to evaluate the risk factors for infection and mortality associated with S. maltophilia bloodstream infections (BSIs) in children and compare them with Pseudomonas aeruginosa BSIs., Methods: All BSIs caused by S. maltophilia (n:73) and P. aeruginosa (n:80) were enrolled in this study between January 2014 and December 2021 at the Medical School of Ege University., Results: Previous Pediatric Intensive Care Unit (PICU) admission, prior glycopeptide, and carbapenem use were significantly more common in patients with S. maltophilia BSIs ( P = 0.044, P = 0.009, and P = 0.001, respectively) than with P. aeruginosa BSIs. C-reactive protein (CRP) levels were significantly higher in S. maltophilia BSIs ( P = 0.002). Multivariate analysis showed that prior carbapenem use was associated with S. maltophilia BSIs ( P = 0.014, adjusted odds ratio [AOR]: 2.710; 95% confidence interval [CI]: 1.225-5.992). PICU admission because of BSI, prior carbapenem and glycopeptide use, neutropenia, and thrombocytopenia were significantly more common in patients with mortality because of S. maltophilia BSIs ( P < 0.001, P = 0.010, P = 0.007, P = 0.008, P = 0.004, respectively), while only PICU admission because of BSI, and prior glycopeptide use were significant in multivariate analysis (AOR, 19.155; 95% CI: 2.337-157.018; P = 0.006 and AOR, 9.629; 95% CI: 1.053-88.013; P = 0.045, respectively)., Conclusion: Prior carbapenem use is a significant risk factor for developing S. maltophilia BSIs. PICU admission because of BSI and prior glycopeptide use are risk factors associated with the mortality rate in patients with S. maltophilia BSIs. Therefore, S. maltophilia should be considered in patients with these risk factors, and empirical treatment should include antibiotics for S. maltophilia ., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: The early and late complications after hematopoietic stem cell transplantation (HSCT) determine the patients' prognosis and life quality. We aim to determine the metabolic syndrome development frequency after HSCT in children to find out the risk factors and compare them with healthy adolescents., Methods: Thirty-six children who underwent HSCT at least two years ago were analyzed prospectively and cross-sectionally. Our study included 18 healthy children between the ages of 11 and 17 as a control group. All of the cases were assessed in terms of metabolic syndrome (MS) through the use of Modified WHO Criteria., Results: The patients' median age was 10.6 (5.1-17) years, the median time of follow-up after HCST was 4.1 (2-13.5) years and 70% were male. Two cases were diagnosed with MS (5.6%). When considered in terms of the sub-components of MS, 2 cases (5.6%) were found to have obesity, 17 cases (47%) abnormal glucose tolerance, 11 cases (30.7%) dyslipidemia, and 3 cases (8.6%) hypertension. The MS rate was not different when compared with the 11-17 year-old healthy control group (0 vs. 11%, p=0.48). Myeloablative conditioning regimen (65 vs. 20%) and the increased age at which HSCT was performed were considered to be risk factors in terms of insulin resistance (p=0.025 and 0.002)., Conclusions: Age and conditioning regimens were found to be the risk factors for insulin resistance development. The long-term follow-up of the cases who had undergone HSCT in childhood in terms of MS and its sub-components is important in order to increase life quality., (© 2021 Walter de Gruyter GmbH, Berlin/Boston.)

Chlorogenic (5-CQA), 1,5-, 3,5-, 4,5- and 3,4-dicaffeoylquinic (DCQA) acids were identified and quantified in the methanol extracts of Inula oculus-christi L., I. bifrons L., I. aschersoniana Janka var. aschersoniana, I. ensifolia L., I. conyza (Griess.) DC. and I. germanica L. by HPLC analysis. The amount of 5-CQA varied from 5.48 to 28.44 mg/g DE and the highest content was detected in I. ensifolia. 1,5-DCQA (4.05-55.25 mg/g DE) was the most abundant dicaffeoyl ester of quinic acid followed by 3,5-DCQA, 4,5-DCQA and 3,4-DCQA. The extract of I. ensifolia showed the highest total phenolic content (119.92±0.95 mg GAE/g DE) and exhibited the strongest DPPH radical scavenging activity (69.41±0.55 %). I. bifrons extract was found to be the most active sample against ABTS .+ (TEAC 0.257±0.012 mg/mL) and the best tyrosinase inhibitor. The studied extracts demonstrated a low inhibitory effect towards acetylcholinesterase and possessed low cytotoxicity in concentration range from 10 to 300 μg/mL toward non-cancer (MDCK II) and cancer (A 549) cells., (© 2020 Wiley-VHCA AG, Zurich, Switzerland.)

Hexane extracts of Heracleum verticillatum, H. sibiricum, H. angustisectum, and H. ternatum were studied for their furanocoumarin content antioxidant potential and acetylcholinesterase and α-amylase inhibitory activities. Quantification of the furanocoumarins was performed by 1 H-NMR. Pimpinellin was found to be the main component in the roots of all studied species. Bergapten and imperatorin were the major compounds in the fruits of H. sibiricum and H. verticillatum, respectively, while byakangelicol dominated in H. angustisectum and H. ternatum fruits. The leaf and fruit extracts of H. angustisectum demonstrated the highest DPPH radical scavenging activity and TEAC (IC 50 0.58 mg/mL and 1.83 mm, respectively). The root extracts of H. verticillatum and H. angustisectum were found to be the most effective against acetylcholinesterase (IC 50 0.30 and 0.34 mg/mL, respectively). The studied extracts were not active or demonstrated a weak inhibitory effect (%Inh. up to 29.7) towards α-amylase., (© 2019 Wiley-VHCA AG, Zurich, Switzerland.)

Non-typhoidal Salmonella (NTS) is an important pathogen that causes gastroenteritis, bacteraemia, and focal infections. Herein, we present our experience with bloodstream infections caused by Salmonella in paediatric leukaemia patients, which has been reported for the first time in both Europe and the US. According to our research, NTS might be a cause of serious infections in paediatric haematology-oncology patients. Following a low bacterial diet and increasing the hygiene of both the children and their surroundings would be beneficial in preventing these infections., Competing Interests: The authors declare no conflict of interest.

Objective: To report a case of late-onset self-healing Langerhans cell histiocytosis., Case Description: A 4½-month-old female patient presenting with an eythematopurpuric eruption underwent a skin biopsy for histopathology and was first diagnosed with isolated cutaneous Langerhans cell histiocytosis. Her lesions regressed within a few months and she was retrospectively diagnosed with late-onset self-healing Langerhans cell histiocytosis after being without skin or systemic involvement in a follow-up four years later., Comments: Self-healing Langerhans cell histiocytosis, which is characterized by clonal proliferation of Langerhans cells and presents with cutaneous lesions, is a rare self-limited variant of histiocytosis and can only be diagnosed retrospectively, after the patient remains free from systemic involvement for several years. Although it presents at birth or during the neonatal period, only a few cases of its late-onset type regarding the age of onset have been reported. Purpuric lesions that appear after the neonatal period serve as a clue for late-onset self-healing Langerhans cell histiocytosis and the patients should be monitored regularly for systemic involvement if the diagnosis is confirmed by a cutaneous biopsy.

Introduction: Hemophilic arthropathy is the most important cause of morbidity in patients with hemophilia. The earliest alterations that occur during the development of hemophilic arthropathy can be shown using magnetic resonance imaging (MRI). In addition, various tools have been developed to monitor joint health., Aim: : The purpose of this study was to determine the correlation between these tools when used to assess hemophilia patients., Methods: This cross-sectional study enrolled 38 hemophilia patients between 2 and 18 years of age. Hemophilia Joint Health Score (HJHS) and radiologic scores (Pettersson and Arnold-Hilgartner) were used to evaluate the joints of the patients (n=236). Magnetic resonance imaging (MRI) was performed on 46 joints that were pathologic according to the HJHS. These joints were imaged bilaterally; therefore, 14 normal joints were imaged. In addition, the Functional Independence Score in Hemophilia (FISH) was used to evaluate the joints of 33 patients., Results: The HJHS scores were correlated with the MRI and FISH scores. The annual bleeding rate was not correlated with any scores; however, the number of painful joints was correlated with the MRI scores. The radiologic scores were correlated weakly with progressive score and HJHS., Conclusion: The agreement between the HJHS scores and the MRI scores suggests that the HJHS may be used safely as a first-line tool. We recommend that the FISH should be used in the routine follow-up of hemophilia patients as a functional evaluation tool. Painful joints may be useful in deciding to apply MRI, whereas the bleeding frequency may not be useful.

The essential oils from the flower, leaf, and stem of Echinophora lamondiana B.Yildiz et Z.Bahcecioglu were analyzed by gas chromatography-flame ionization detection and gas chromatography-mass spectrometry. In total, 41, 37, and 44 compounds were identified, which accounted for 98.0, 99.1, and 97.0% of flower, leaf, and stem essential oils, respectively. The monoterpenic hydrocarbons were found to be high in all samples of the essential oils. The major components of essential oils from flower, leaf, and stem of E. lamondiana were δ-3-carene (61.9, 75.0, and 65.9%, respectively), α-phellandrene (20.3, 14.1, and 12.8%, respectively), and terpinolene (2.7, 3.3, and 2.9%, respectively). Flower and leaf essential oils and terpinolene produced biting deterrence similar to 25 nmol/cm(2) N, N-diethyl-meta-toluamide (DEET; 97%) against Aedes aegypti (L.) and Anopheles quadrimaculatus Say. Compounds (+)-δ-3-carene, (R)-(-)-α-phellandrene, and water-distilled essential oils were significantly less repellent than DEET. Among essential oils, leaf oil was the least toxic of the oils, with an LC50 value of 138.3 ppm, whereas flower essential oil killed only 32% larvae, and no mortality of stem oil at highest tested dosages against Ae aegypti was observed. Terpinolene and α-phellandrene showed higher toxicity than δ-3-carene in both the species. In contrast to Ae. aegypti, all the essential oils showed toxicity in An. quadrimaculatus, and toxicity was higher in leaf oil than the other two oils. These results could be useful in finding new, safe, and more effective natural biopesticides and biting deterrent or repellents against Ae. aegypti., (© The Author 2015. Published by Oxford University Press on behalf of the Entomological Society of America.For Permissions, please e-mail: journals.permissions@oup.com.)