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Beta-ketothiolase (mitochondrial acetoacetyl-CoA thiolase, T2) deficiency is an autosomal recessive disorder characterized by impaired metabolism of ketones and isoleucine. In this study, we report on the first two siblings with T2 deficiency from Libya. Both siblings presented with ketoacidosis, but the severity and outcomes were quite distinctive. T2 deficiency in patient 1, the younger sister, manifested as recurrent severe episodes of ketoacidosis during the first year of life. She unfortunately experienced neurodevelopmental complications, and died at 14 months old, after her 5th episode. In contrast, patient 2, the elder brother, experienced only one ketoacidotic episode at the age of 4 years. He recovered uneventfully and has continued to achieve age-appropriate development to date. Upon analysis, the siblings’ blood acylcarnitine profiles had shown increased levels of C5:1 and C5-OH carnitine. ACAT1 mutational analysis revealed patient 2 is homozygotic for a novel mutation–c.674C > A (p.Ala225Glu); this mutation was then confirmed by familial analysis. Transient expression analysis of c.674C > A mutant T2 cDNA revealed neither potassium ion-activated acetoacetyl-CoA thiolase activity, which represents T2 activity, nor mutant T2 protein. Therefore, this mutation is truly pathogenic. Interestingly, the incidence of T2 deficiency may be high among the Arab population. This disease should be considered in the differential diagnosis for unexplained ketoacidosis in children. Patients with T2 deficiency could have a favorable outcome if diagnosed and treated early.

Abstract In Egypt, while many studies have focused on the radiometry and mineralogy of black sands, research on their effects on nearby aquatic organisms is rare. This study aimed to assess the combined effects of heat stress (HS) and black sand nanoparticles (BS-NPs) on renal function, antioxidant responses (TAC, SOD, CAT), neuro-stress indicators (AchE, cortisol), and to conduct histopathological investigations in the kidney and spleen tissues of African catfish Clarias gariepinus over a 15-day period to exposure to control, HS (32 °C), BS (6.4 g/kg diet) and HS + BS groups. The outcomes revealed that thermal stress alone showed no significant difference from the control. However, creatinine and uric acid levels were significantly higher in the BS-NPs and HS + BS-NPs groups (p

Beta-ketothiolase deficiency is an inherited disorder of ketone body metabolism and isoleucine catabolism. It typically manifests as recurrent ketoacidotic episodes with characteristic abnormalities in the urinary organic acid profile. However, several challenges in the diagnosis of beta-ketothiolase deficiency have been encountered: atypical presentations have been reported and some other disorders, such as succinyl-CoA:3-oxoacid CoA transferase and 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiencies, can mimic the clinical and/or biochemical signs of beta-ketothiolase deficiency. A final diagnosis of beta-ketothiolase deficiency requires an enzymatic assay and/or a molecular analysis, but some caveats must be considered. Despite the reported missed cases, screening programs have successfully identified an increasing number of patients with beta-ketothiolase deficiency. Early diagnosis and management of beta-ketothiolase deficiency will enable prevention of its serious acute and chronic complications and ultimately improve the prognosis.

This work deals with the plankton stratigraphy of the subsurface Upper Cretaceous-Palaeogene succession of theEast Bahariya Concession based on planktonic foraminifera and calcareous nannofossils. The examination of the cuttings from five wells: AQSA-1X, KARMA-E-1X, KARMA-3X, KARMA-NW-1X and KARMA-NW-5X is biostratigraphically evaluated. It is possible to identify the planktonic foraminifera as well as the calcareous nannofossil biozones. The analyses of calcareous nannofossils revealed the presence of several hiatuses. Information obtained from well data such as seismic facies analysis for the studied area has enabled classification of the Upper Cretaceous Palaeogene succession into five major 2nd order depositional sequences, separated by four major depositional sequence boundaries (SB1, SB2, SB3 and SB4). The Upper Cretaceous-Palaeogene succession in the East Bahariya is dividedinto 17 systems tracts. These systems tracts are: 7 System tracts of probable Cenomanian age, (the sequence stratigraphic framework as well as the cycles and system tracts of the Cenomanian Bahariya Formation match well with thoseof CATUNEANUet al., 2006); 4 System tracts of Turonianage, 2 System tracts of Campanian-Maastrichtian ageand 4 System tracts of Eocene age.

Background: Traditional microvascular anastomosis can be timeconsuming, which raises the risk of thrombosis. Damage to the endothelium during the process triggers a repair mechanism that involves platelet aggregation, smooth muscle cell proliferation, and thickening of the endothelium. Microvascular anastomosis plays a vital role in free flap transfers and replantation surgeries. Although standard suturing remains the preferred technique, it presents challenges due to its technical difficulty, extended duration, and potential for vessel wall trauma. Fibrin glue, derived from a combination of autologous cryoprecipitate and thrombin, was originally introduced for peripheral nerve repair in humans. The success of these early uses led to its expanded application in areas such as wound closure, skin grafting, and osteotomy healing. This study aims to evaluate the effectiveness of fibrin adhesive in addressing these challenges within the context of microvascular anastomosis. Methods: This experimental study included 22 male Sprague-Dawley albino rats, the rats were divided into two groups Group (1): Experimental group, we used 4 core sutures with 9/0 polypropylene placed 90 degrees from each other and 0.1 ml of fibrin glue at site of anastomosis. Group (2): As a control group we used the standard repair technique of the transected artery by 5-8 primary sutures with 9/0 polypropylene. Results: The use of fibrin adhesive significantly decreased both the number of sutures required and the time needed to complete the anastomosis. The immediate and long-term patency rates were not adversely affected by the application of fibrin glue. Histological analysis of the anastomosed vessels revealed no significant differences between the two techniques. Conclusions: In conclusion, the application of fibrin adhesive did not produce any adverse effects in microvascular anastomosis. The authors advocate for using fibrin adhesive in clinical settings, particularly in more complex cases where multiple microvascular anastomoses are necessary. [ABSTRACT FROM AUTHOR]

Background: Cow's milk allergy is a predictable immune response to certain proteins found in cow's milk. This reaction consistently causes a range of symptoms, such as gastrointestinal, respiratory, and skin issues, after consuming the problematic food. This condition affects approximately 2-5% of infants. Objectives: The aim of the current study was to evaluate the validity of CoMiSS in children suspected to have Cow's Milk Protein Allergy (CMPA). Methods: A prospective observational study was conducted among 100 targeted patients presenting with one or more of the following CMPA symptoms: cutaneous (atopic dermatitis and urticaria), respiratory (cough and dyspnea, rhinitis), and gastrointestinal (digestive regurgitation, vomiting, rectal bleeding, constipation and diarrhea) at Pediatrics department, Sohag university hospitals, Egypt from 1st of December 2022 to 31st August, 2023, CoMiSS, 2016 and elimination diet and food challenge test were applied to all cases. Results: comparison between Confirmed CMA (positive challenge test) and non-CMA (negative challenge test) regarding CoMiSS at first visit. There was statistically significant difference between confirmed CMA and non-CMA according to cut off point of CoMiSS either < 12 or = 12 (P-value < 0.001). 9 cases had CoMiSS < 12 and confirmed CMA by food challenge elimination test. On the other hand, 2 of cases had CoMiSS = 12 and confirmed No CMA by food challenge test. 64 cases had CoMiSS = 12 and confirmed CMA by diet elimination and food challenge test and assessment of ROC curve showed that the accuracy of CoMiSS in diagnosis of CMA, it was 89 %. The percentage of Sensitivity was (87.7 %), Specificity (92.6 %), Positive predictive value % (96.9 %) when the score is = 12, Negative predictive value % (73.5 %). Conclusion: The accuracy of CoMiSS in diagnosis of CMA was 89 % with sensitivity (87.7 %), specificity (92.6 %), positive predictive value % (96.9 %) when the score is = 12 & negative predictive value % (73.5 %) so, CoMiSS is an easy-to-use and practical awareness tool for evaluating CM-related symptoms but cannot be considered a stand alone diagnostic tool for CMA. [ABSTRACT FROM AUTHOR]

The alkaline rocks are known for enriching rare lithophilic elements, including lithium, uranium, and tin, which negatively impact aquatic life. This study offers an intensive investigation of the influence of alkaline rocks on Nile Tilapia (Oreochromis niloticus). The variation in blood profile, the induction of antioxidant enzymes, morphological erythrocyte, and histological structure have been conducted for the fish after 15 days of exposure to alkaline rocks powder with a dose of 100 μg/L. As a result, there was a pronounced decrease in blood profiles, such as platelets and white blood cell counts. There was a failure in the liver and kidney functions. Moreover, it shows an increase in superoxide dismutase (SOD) and catalase (CAT) activities as antioxidant biomarkers. Also, exposure to alkaline rocks induced DNA mutation and erythrocyte distortion. We concluded that the bulk alkaline rocks induced changes in the hemato-biochemical and antioxidant parameters of Nile tilapia. Additionally, exposure to bulk alkaline rock compounds also caused poikilocytosis and nuclear abnormalities of RBCs. This draws our attention to the seriousness of climatic changes, the erosion of rocks, and their access to water.

Commercially pure copper samples were successfully deformed by parallel tubular channel angular pressing (PTCAP) up to different passes at room temperature. The effects of the PTCAP number of passes on the microstructure, mechanical properties, microhardness and wear resistance of the processed samples were fully investigate. The microstructure of processed samples were observed by SEM and showed notable decrease in the grain size with increase number of passes. The mechanical properties of the pure copper in each pass were studied by tensile testing and microhardness method at room temperature. In this respect, UTS, σ 0.2% and microhardness have been markedly improved from 199.03, 102.17 MPa and 67.83 HV as the annealed condition to 331.40, 293.51 MPa and 144.03 HV after the forth pass, respectively. Besides, the elongation percent were decreased while, the wear resistance improved with increased number of PTCAP passes. Ductile fracture with extensive necking zone and many big dimples occurs in annealed samples, while fine dimples were decreased with the deformation final passes of PTCAP processed samples.

Background: Heart rate is an integral part of the clinical assessment of the children with acute illness and is commonly used in pediatric early warning scores. Objective: The aim of this study was to assess our current practice for evaluation and management of bradycardia in children. Patients and methods: The study was conducted in Pediatric Arrhythmia Clinic, Neonatal Intensive Care Unit and Pediatric Intensive Care Unit from March 2020 to August 2021. 61 Neonates and children aged from 1 day to 18 years presenting with bradycardia were included in this study. Predeath bradycardia was excluded. Full medical history, physical examination, basic investigations, 12 leads ECG and echocardiography were performed to all patients. Thyroid function tests, 24 hours Holter monitoring, and stress ECG were done if indicated. Results: 61 patients were included in the study. Heart rate ranged from 44 to 94 beat/minute with mean 69.44 beat/minute, the most common form of presentation was abnormal echocardiography. Hypocalcemia was present in 85.2% of patients. 70.5% of patients had sinus bradycardia, 13% had complete heart block (CHB),10% of patients had long QTc Syndrome with sinus bradycardia, 3.3% had 1st and 2nd degree heart block, 1.6% had ECG changes of ALCAPA with sinus bradycardia and 1.6% had ventricular ectopic. Conclusion: It could be concluded that bradycardia in children needs accurate assessment to rule out the need of acute intervention and to decrease the risk of life-threatening conditions. [ABSTRACT FROM AUTHOR]

A pattern recognition application, using artificial neural networks (ANN), may be categorized as the largest category that covers the use of a neural networks for classifications. It is proved that ANNs are excellent for the applications of complex pattern recognition such as visual images of objects, speech recognition, printed or handwritten characters, and many other types of pattern recognition. Such wide applications are developed, based on the major advantage of the neural networks of being inherently error tolerant, i.e., it can solve problems adequately with information (data) that is uncertain, incomplete, or noisy. The reliable results of the power system security and economical analysis depend on the supply of real-time data base, results of power system state estimation, programs whose major problem is the determination of the network topology configuration. The determination of the topology suffers from the uncertainty of the collected data related to the circuit breakers status, due to the possible communication errors and/or failures. This problem solution matches the potential of the neural networks that can generalize, and after training, they can handle imperfect or incomplete data, providing a degree of fault tolerance. This paper describes an efficient ANN power system topology classifier. The function of such a classifier is to determine the system topology configuration under the presence of incorrect and/or incomplete data concerning the lines and the breakers status. A double layered ANN model is developed and explained as well as the specially efficient training algorithm. Comparative training and test results are reported, and they prove the robustness of this ANN. [ABSTRACT FROM AUTHOR]

Abstract In this work, the production of dimethyl ether (DME) from methanol over natural kaolin clay modified through impregnation with various percentages of H2SO4, WO3, or ZrO2 catalysts was investigated. The prepared catalysts were characterized via X-ray fluorescence, X-ray diffraction, Fourier transform infrared spectroscopy, scanning electron microscopy, and N2-sorption analysis. The acidity of these catalysts was determined through the dehydration of isopropyl alcohol and the chemisorption of pyridine. The catalytic activity performance revealed that the addition of modifiers into kaolin enhanced the latter’s activity toward DME production. In addition, the kaolin clay modified with 10 wt% ZrO2 exhibited excellent activity of 98% conversion with 100% selectivity at 275 °C. Moreover, this catalyst could proceed the reaction for a long time (6 days) without any noticeable deactivation. The remarkable improvement in the catalytic performance achievement was well correlated with the acidity and the structure of the catalysts.

Effective biosafe management strategies are used to decrease world crop damage produced by plant-parasitic nematodes. This study evaluated the efficiency of hydroxyapatite nanoparticles (n-HAP) and mycorrhizal fungi to control the Meloidogyne incognita infecting tomato plants. Application of n-HAP significantly increased the juveniles’ mortality (195.67%) and egg hatching inhibition percentage (80.71%) compared to the untreated control, in vitro. Mycorrhizal and/or n-HAP treatments increased the plant growth parameters (root and shoot length, dry weight, and leaf area) and reduced the negative consequence of nematode infection. This may be due to indirect mechanisms through increasing plant nutrient uptake efficiency and increasing the internal plant resistance against nematode infection. In dual-treated plants, phosphorus, nitrogen, and calcium content recorded the highest value in the nematode-infected plants. Whereas the dual inoculation significantly increased mineral contents in tomato plants compared with control, this may induce the strength of the cell wall of the epidermal layer and cortex and consequently increase the plant resistance against nematode infection. Our results revealed that the application of the plant resistance-stimulants enhanced the plant growth parameters and internal nutrient content and reduced the nematode’s criteria. Consequently, the internal plant resistance against nematode infection was induced.

Objective: This study compares the efficacy of sublingual misoprostol versus intravenous oxytocin in reducing bleeding during and after cesarean delivery. Materials and methods: A randomized clinical trial conducted on 120 pregnant women at term (37–40 weeks) gestation scheduled for elective cesarean delivery, who were assigned to either sublingual misoprostol 400 μg or intravenous infusion of 20 units of oxytocin after delivery of the neonate. The main outcome measures were blood loss at and 2 hours after cesarean delivery, change in hematocrit value, need for any additional oxytocic drugs, and drug-related side effects. Results: The overall mean blood loss was significantly lower in the misoprostol group compared to the oxytocin group (490.75 ± 159.90 mL vs. 601.08 ± 299.49 mL; p = 0.025). However, changes in hematocrit level (pre- and postpartum) was comparable between both groups. There was a need for additional oxytocic therapy in 16.7% and 23.3% after use of misoprostol and oxytocin, respectively (p = 0.361). Incidence of side effects such as shivering and metallic taste were significantly higher in the misoprostol group compared to the oxytocin group (p