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Aim: The aim of this study was to examine the clinical, laboratory and demographic characteristics of patients diagnosed with cerebrotendinous xanthomatosis (CTX)., Materials and Methods: This study included 11 patients followed up in the Pediatric Metabolism Polyclinic for a diagnosis of CTX. The diagnosis of CTX was made from high blood cholestanol level and CYP27A1 gene analysis. All the cases diagnosed with CTX for whom clinical and laboratory findings were evaluated were included in the study., Results: Evaluation was made of 11 patients from five different families. The diagnosis was established 25 years after symptoms first appeared. The diagnosis was made because of bilateral cataracts in two patients, tendon xanthomas in two, and as a result of family screening in seven. Tendon xanthomas were present in 36.3% of the patients, and there was a history of cataract in 54.5%. In the current study, mental retardation was determined in 72% of the patients, psychiatric findings in 36%, epilepsy in 36%, pyramidal-extrapyramidal findings in 45%, and postural tremor in 54%. In addition, neuropsychiatric symptoms were seen at different rates in patients with different gene alleles. No tendon xanthomas were determined in the cases with c.1263 + 4A>T and c.808C>T mutations. Cataract was determined in all the cases with homozygote c.1263 + 4A>T mutation., Conclusion: In this study, it was determined that the cases were diagnosed late despite the onset of symptoms providing clues for diagnosis at an early age. It was determined that the delay in diagnosis was 25 years., Competing Interests: Declaration of competing interest There are no conflicts of interest., (Copyright © 2024. Published by Elsevier Inc.)

Objectives: COVID-19 escalated inappropriate antibiotic use. We determined the distribution of pathogens causing community-acquired co-infections, the rate, and factors associated with early empiric antibiotic (EEAB) treatment among hospitalized COVID-19 patients., Methods: The Society of Critical Care Medicine Discovery Viral Infection and Respiratory Illness Universal Study (VIRUS) COVID-19 Registry including 68,428 patients from 28 countries enrolled between January 2020 and October 2021 were screened. After exclusions, 7830 patients were included in the analysis. Azithromycin and/or other antibiotic treatment given within the first 3 days of hospitalization was investigated. Univariate and multivariate analyses were performed to determine factors associated with EEAB use., Results: The majority (6214, 79.4%) of patients received EEAB, with azithromycin combination being the most frequent (3146, 40.2%). As the pandemic advanced, the proportion of patients receiving EEAB regressed from 84.4% (786/931) in January-March 2020 to 65.2% (30/46) in April-June 2021 (P < 0.001). Beta-lactams, especially ceftriaxone was the most commonly used antibiotic. Staphylococcus aureus was the most commonly isolated pathogen. Multivariate analysis showed geographical location and pandemic timeline as the strongest independent predictors of EEAB use., Conclusions: EEAB administration decreased as pandemic advanced, which may be the result of intensified antimicrobial stewardship efforts. Our study provides worldwide goals for antimicrobial stewardship programs in the post-COVID-19 era., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Purpose: Our aim is the early detection of mucopolysaccharidosis (MPS) by examining the radiographs taken for reasons other than a metabolic disease, such as infection, trauma, and short stature., Methods: The radiographs of children who applied to outpatient and emergency clinics in our hospital between 01/01/2022 and 31/12/2022 were examined by a pediatric radiologist retrospectively without knowledge of patient information. The MPS enzyme panel and urine glycosaminoglycan analysis were performed in patients having dysostosis multiplex on radiographs. In cases with MPS detected by enzyme and urine analysis, the definitive diagnosis was confirmed by genetic analysis., Results: Skeletal radiographs of 15.104 cases admitted to our hospital were examined (11,270 chest x-ray, 314 lumbosacral spine x-ray, 2970 hand x-ray, 253 pelvis x-ray, 162 skull x-ray, and 135 complete skeletal surveys). In 67 children, dysostosis multiplex was observed in the skeletal X-ray. Among them, seven newly diagnosed MPS cases were detected. Three cases were diagnosed with MPS type 4A, two with MPS type 6, one with MPS type 2 and one with MPS type 3B. Age at diagnosis was 46.2 ± 30.6 months (range; 20-111 months). There was a history of consanguinity in 6 (85.7%) cases., Conclusion: Radiographs can provide clues for diagnosing MPS before the clinical findings become prominent in children admitted to the hospital for other complaints. Therefore, X-ray screening can be performed on children in endemic regions of MPS to search for dysostosis multiplex., Competing Interests: Declaration of competing interest There are no conflicts of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpuse: The carbapenem-resistant Bacteroides fragilis group (CR-BFG) bacteria have been reported in several countries recently with increasing global attention. The high incidence of CR-BFG isolated from our hospitalized patients has become an important problem. Therefore, we aimed to determine the frequency and associated factors for intestinal colonization by carbapenem-non-susceptible BFG (CNS-BFG) among adult patients hospitalized at intensive care units, neurosurgery and internal medicine wards in our hospital., Methods: Rectal swabs (n = 1200), collected from 766 patients between February 2014 and March 2015, were inoculated onto kanamycin-vancomycin-leaked blood agar containing 0.125 mg/L meropenem. The isolates were identified by MALDI-TOF MS. Susceptibility testing was performed by agar dilution method. The carbapenemase gene (cfiA) was detected by PCR. Logistic regression analysis was used to evaluate the associated factors for intestinal colonization by CNS-BFG., Results: A total 180 non-duplicate BFG isolates were obtained from 164 patients. Ten different species, including Parabacteroides distasonis (n = 46, 25.6%), and Bacteroides fragilis (n = 30; 16.6%), were identified. Twenty-five percent of the isolates were non-susceptible to meropenem (MIC >2 mg/L). The highest prevalence of meropenem resistant strains (MIC >8 mg/L) was detected among B. fragilis (n = 12), followed by Parabacteroides spp. (n = 4). All but one B. fragilis strains were cfiA gene positive. Hospital admission, increasing Charlson score, use of antibiotics; including carbapenems in past three months, colonization with other accompanying carbapenem-resistant Gram negative bacteria (Enterobacteriaceae, Acinetobacter baumannii and Pseudomonas aeruginosa), and having undergone surgical operations were significantly associated with RCS- BFG colonization., Conclusions: The high carriage rate of CNS-BFG in hospitalized patients may lead to worse clinical outcomes, such as serious infections and mortality, and deserves attention., Competing Interests: Declaration of competing interest No conflict of interest was declared by the authors., (Copyright © 2021 Indian Association of Medical Microbiologists. Published by Elsevier B.V. All rights reserved.)

Aim: Studies analyzing viral load in COVID-19 patients and any data that compare viral load with chest computerized tomography (CT) severity are limited. This study aimed to evaluate the severity of chest CT in reverse transcriptase polymerase chain reaction (RT-PCR)-positive patients and factors associated with it., Methodology: SARS-CoV-2 RNA was extracted from nasopharyngeal swab samples by using Bio-speedy viral nucleic acid buffer. The RT-PCR tests were performed with primers and probes targeting the RdRp gene (Bioexen LTD, Turkey) and results were quantified as cycle threshold (Ct) values. Chest CT of SARS-CoV-2 RNA-positive patients (n = 730) in a period from 22 March to 20 May 2020 were evaluated. The total severity score (TSS) of chest CT ranged 0-20 and was calculated by summing up the degree of acute lung inflammation lesion involvement of each of the five lung lobes., Results: Of the 284 patients who were hospitalized, 27 (9.5%) of them died. Of 236 (32.3%) patients, there were no findings on CT and 216 (91.5%) of them were outpatients (median age 35 years). TSS was significantly higher in hospitalized patients; 5.3% had severe changes. Ct values were lower among outpatients, indicating higher viral load. An inverse relation between viral load and TSS was detected in both groups. CT severity was related to age, and older patients had higher TSS (p < 0.01)., Conclusion: Viral load was not a critical factor for hospitalization and mortality. Outpatients had considerable amounts of virus in their nasopharynx, which made them contagious to their contacts. Viral load is important in detecting early stages of COVID-19, to minimize potential spread, whereas chest CT can help identify cases requiring extensive medical care., (Copyright © 2020. Published by Elsevier Ltd.)

Objectives: Antimicrobial stewardship programs (ASPs) have an important role in the appropriate utilization of antibiotics. Some of the core strategies recommended for ASPs are pre-authorization and prospective audit and feedback. In Turkey, a unique nationwide antibiotic restriction program (NARP) has been in place since 2003. The aim of this study was to measure the effect of a prospective audit and feedback strategy system along with the NARP., Methods: A prospective quasi-experimental study was designed and implemented between March and June 2017. A computerized pre-authorization system was used as an ASP strategy to approve the antibiotics. During the baseline period, patients with intravenous (IV) antibiotic use ≥72 h were monitored without intervention. In the second period, feedback and treatment recommendations were given to attending physicians in the case of IV antibiotic use ≥72 h. The modified criteria of Kunin et al. and Gyssens et al. were followed for appropriateness of prescribing. Days of therapy (DOT) and length of stay (LOS) were calculated and compared between the two study periods., Results: A total of 866 antibiotic episodes among 519 patients were observed. A significant reduction in systemic antibiotic consumption was observed in the intervention period (575 vs. 349 DOT per 1000 patient-days; p <  0.001). On multivariate analysis, prospective audit and feedback (odds ratio 1.5, 95% confidence interval 1.09-2.04; p = 0.011) and pre-authorization of restricted antibiotics (odds ratio 1.7; 95% confidence interval 1.2-2.31; p =  0.002) were the predictors of appropriate antimicrobial use. Mean LOS was decreased by 2.9 days (p =  0.095)., Conclusions: This study showed that the antimicrobial restriction program alone was effective, but the system should be supported by a tailored ASP, such as prospective audit and feedback., (Copyright © 2019 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Invasive fungal infections (IFI) are on the rise due to increasing numbers of immunosuppressed and critically ill patients. A malignant-looking pulmonary nodule in an immunosuppressed patient may indeed be caused by a fungal organism. We report a patient, who was eventually diagnosed with an IFI caused by an agent of hyalohyphomycosis, Talaromyces sp. determined via molecular methods and succesfully treated with voriconazole.

Aim: The aim of this study was to investigate the relationships between 25-hydroxy-vitamin D (25(OH)D) and insulin resistance in obese adolescents with non-alcoholic fatty liver disease (NAFLD)., Patients and Methods: Eighty-seven obese adolescents (45 girls and 42 boys, mean age: 12.7 ± 1.3 years, mean body mass index standard deviation score (BMI-SDS): 2.1 ± 0.3) and 30 lean subjects (15 girls and 15 boys, mean age: 12.3 ± 1.45 years, mean BMI-SDS: 0.5 ± 0.7) were enrolled for the study. The obese subjects were divided into two subgroups based on the presence or absence of fatty liver with high transaminases (NAFLD group and non-NAFLD group). Fasting blood samples were assayed for 25(OH)D, transaminases, glucose, and insulin levels. Insulin resistance was calculated by the homeostasis model assessment (HOMA-IR)., Results: 25(OH)D measurements were decreased in both obese groups (NAFLD and non-NAFLD) in comparison with the lean group (29.5 ± 18.4 vs. 41.0 ± 17.9 vs. 48.1 ± 22.2 ng/mL). However; the NAFLD group had significantly lower measurements of 25(OH)D than the non-NAFLD group (p < 0.001) and lean group (p < 0.001). 25(OH)D was negatively correlated with HOMA-IR (r = 0.158, p = 0.01) and with alanine aminotransferase (r = 0.794, p = 0.03) in the NAFLD obese group. There was no significant associations between fasting insulin, BMI-SDS and 25(OH)D in obese groups., Conclusion: We suggest that low 25(OH)D occurs commonly in obese adolescents with NAFLD and we demonstrated an association between insufficient vitamin D status and low insulin sensitivity in obese adolescents with NAFLD., (© Crown Copyright 2013 Published by Elsevier Ltd on behalf of Asia Oceania Assoc. for the Study of Obesity. All rights reserved.)

Study Objective: To determine the influence of timing of systemic ketamine administration on postoperative morphine consumption., Design: Prospective randomized study., Setting: Operating rooms, postanesthesia care unit, and gynecology service of a university hospital., Patients: Forty-five patients undergoing laparotomy for benign gynecologic pathologies were randomized into 3 groups., Interventions: In Group 1, before surgical incision, patients received 0.5 mg/kg ketamine IV, followed by normal saline infusion and normal saline IV at wound closure in group 1 (n = 15). In group 2 (n = 15), patients received 0.5 mg/kg ketamine IV before surgery, followed by ketamine infusion 600 mug . kg(-1) . h(-1), until wound closure and normal saline IV at that time. In the other group (group 3, n = 15), patients received normal saline IV before surgery, followed by saline infusion and then 0.5 mg/kg ketamine IV at wound closure. In the postoperative period, patient-controlled analgesia IV morphine was used for postoperative pain relief. First requested analgesic medication time was recorded. Postoperative pain was assessed by measuring morphine consumption at 0 to 2, 0 to 4, and 0 to 24 hours and visual analog scale (VAS) pain scores in response to cough at 2nd, 4th, and 24th hours and during rest at 0 to 2, 0 to 4, and 0 to 24 hours after surgery., Measurement and Main Results: First requested analgesia was shorter in group 1 than the others (P < .01). Mean VAS pain scores in response to cough at 24th hour in groups 2 and 3 were significantly lower than in group 1 (P < .001 and P < .01, respectively). Mean VAS pain scores during rest at 0 to 24 hours in groups 2 and 3 were significantly lower than in group 1 (P < .01 and P < .05, respectively). Morphine consumption was lower in groups 2 and 3 at 0 to 2 hours (P < .001 and P < .01). Moreover, morphine consumption at 0 to 4 hours in group 2 was significantly lower (P < .01)., Conclusions: Lower pain scores and morphine consumption in groups 2 and 3 may be related to higher plasma ketamine concentrations caused by the higher doses and later administration. Our findings suggest that a single preoperative dose of ketamine provided less analgesia compared with other dosing regimens that included intraoperative infusions or postoperative administration.