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A 79-year-old woman, diagnosed for cancer of the ovary, had a central catheter that was placed with difficulty through the right internal jugular vein intraoperatively. After oophorectomy, it was realized that the catheter was knotted. Thus, the central venous catheter was removed successfully using a traction technique in the operating room. Central venous catheter use may result in various complications, although it has been used as an invasive method for hemodynamic monitoring and fluid and drug infusion. Here, we present catheter knotting in a case with solutions for this complication, under literature review.

This paper presents a numerical investigation of the air wake around a generic surface combatant ship called NATO-Generic Destroyer (NATO-GD). Naval surface combatants with flight decks must be designed taking aerodynamic concerns into account. Most of the previous studies have employed the Simple Frigate Shape (SFS) and its modified version (SFS2) to investigate the airwake. However, these generic geometries do not accurately represent modern warship designs. To address this, a modern geometry called NATO-GD proposed by the NATO Research Task Group, which represents the features of a modern destroyer, was utilized in the present work. The objective is to examine the air wake on the helicopter deck to ensure the safe operation of air vehicles such as helicopters and drones. The three-dimensional, transient airflow around the ship was solved using the unsteady Reynolds-Averaged Navier-Stokes (URANS) and Detached Eddy Simulation (DES) turbulence models. Besides, the effect of inflow was investigated by comparing uniform velocity inlet and atmospheric boundary layer (ABL) for various wind-over-deck (WOD) angles. The numerical approach was verified for the URANS turbulence model using the Grid Convergence Index (GCI) method. The numerical uncertainty was calculated with four different methods and the uncertainty was found between 1.6% and 2.1%. A detailed discussion of the flow field above the flight deck was conducted to compare the URANS and DES models fairly. It was concluded that employing the ABL profile as a boundary condition is more suitable for achieving accurate ship aerodynamics calculations. The ABL velocity profile makes a significant difference in the velocity components. According to the URANS results, these deviations are found as 8.23% in the x-component, 1.25% in the y-component and 4.89% in the z-component. The deviations were calculated using the root mean square error (RMSE) method. Furthermore, although the numerical results of the URANS and DES models were similar at some points, detailed flow field analysis is only possible with the DES results to determine safe approach patterns for air vehicles. Various wind speeds, directions, and the resulting wake structures were tested to analyze the wake behavior over the helicopter deck under different air conditions. When the wind comes from the port side with 15 degrees (R15) it wind changes the intense turbulence region and creates a low turbulence area on the starboard side while R30 wind causes small scale vortices breaking this region.

Purpose – The study aims to determine the impact of sustainable manufacturing on environmental performance through government regulation and eco-innovation in Indonesian small and medium-sized enterprises (SMEs). Findings – The results indicate sustainable manufacturing plays a significant role in SMEs' environmental performance and regulations, and eco-innovation can moderate it. It also reveals that government regulation has a positive and significant effect on environmental performance. Moreover, eco-innovation has a positive and significant effect on environmental performance. Practical implications – The findings of this study indicate that SMEs can embrace sustainable manufacturing practices and achieve their long-term sustainability goals by adhering to regulations, collaborating with stakeholders and implementing eco-friendly innovations. Originality/value – This research uncovers ground-breaking perspectives on the evolution of scientific knowledge about the impact of eco-innovation, regulatory measures and sustainable manufacturing practices on the environmental performance of SMEs.

Objective. Chronic diseases, which caused 36 million deaths in 2008, are the most common cause of death worldwide. Exercise is one of the non-pharmacological treatment methods. Although exercise benefits are well known, more than half of the population does not exercise due to the burden of exercise. The objectives of the current study were to evaluate the Turkish version of the Exercise Therapy Burden Questionnaire (ETBQ-T) and to investigate its reliability and validity. Methods. A total of 100 participants (female: 69, male: 31) who were diagnosed with at least one chronic disease participated in the translation validity and reliability analysis of the study. Cross-cultural adaptation of the ETBQ-T was performed according to Beaton’s guidelines. The ETBQ-T, the European Quality of Life 5 Dimensions (EQ-5D), pain, satisfaction, and self-efficacy were applied for convergent validity. The ETBQ-T was retested to examine its reliability after 7 days. Results. The internal consistency and reliability were excellent (intraclass correlation coefficient=0.959; Cronbach’s α=0.919). The standard error of measurement was reported as 5.35. The minimum detectable difference was also demonstrated at 1.35. The ETBQ-T had a good correlation with pain (r=0.545, p

Abstract Background Broodstock nutritional programming improves the offspring utilization of plant-based diets in gilthead sea bream through changes in hepatic metabolism. Attention was initially focused on fatty acid desaturases, but it can involve a wide range of processes that remain largely unexplored. How all this can be driven by a different genetic background is hardly underlined, and the present study aimed to assess how broodstock nutrition affects differentially the transcriptome and genome-wide DNA methylome of reference and genetically selected fish within the PROGENSA® selection program. Results After the stimulus phase with a low fish oil diet, two offspring subsets of each genetic background received a control or a FUTURE-based diet. This highlighted a different hepatic transcriptome (RNA-seq) and genome-wide DNA methylation (MBD-seq) pattern depending on the genetic background. The number of differentially expressed transcripts following the challenge phase varied from 323 in reference fish to 2,009 in genetically selected fish. The number of discriminant transcripts, and associated enriched functions, were also markedly higher in selected fish. Moreover, correlation analysis depicted a hyper-methylated and down-regulated gene expression state in selected fish with the FUTURE diet, whereas the opposite pattern appeared in reference fish. After filtering for highly represented functions in selected fish, 115 epigenetic markers were retrieved in this group. Among them, lipid metabolism genes (23) were the most reactive following ordering by fold-change in expression, rendering a final list of 10 top markers with a key role on hepatic lipogenesis and fatty acid metabolism (cd36, pitpna, cidea, fasn, g6pd, lipt1, scd1a, acsbg2, acsl14, acsbg2). Conclusions Gene expression profiles and methylation signatures were dependent on genetic background in our experimental model. Such assumption affected the magnitude, but also the type and direction of change. Thus, the resulting epigenetic clock of reference fish might depict an older phenotype with a lower methylation for the epigenetically responsive genes with a negative methylation-expression pattern. Therefore, epigenetic markers will be specific of each genetic lineage, serving the broodstock programming in our selected fish to prevent and mitigate later in life the risk of hepatic steatosis through changes in hepatic lipogenesis and fatty acid metabolism.

Objective: Heterogeneity among Helicobacter pylori (H. pylori) strains in the expression of CagA and outer membrane proteins (OMPs) is an important factor influencing clinical outcomes. We investigated the expression of H. pylori cagA, omp6 (hopA), omp13 (oipA), omp18, and omp20 (alpA) genes and their associations with clinicopathological findings. Materials and Methods: Endoscopic biopsies from the gastric antrum and corpus of 120 patients were initially examined using a rapid urease test (RUT), histopathology, and culture. Biopsy specimens from 101 patients were analyzed for H. pylori cagA, omp6, omp13, omp18, omp20, 16S rRNA, and ureA gene expression by Real-Time Reverse Transcription-Polymerase Chain Reaction (RT-PCR). Total RNA was extracted from antral and corpus biopsies and cDNA was synthesized. Results: Forty-eight patients were positive for H. pylori infection based on both RUT and histopathology or culture positivity. H. pylori was detected in 77 of 101 (76.2%) patients by RT-PCR. Of the 48 patients with H. pylori infection, 45 (93.7%) were RT-PCR positive. The gene expression frequencies of cagA, omp6, omp13, omp18, and omp20 in the antrum and corpus of 45 patients were 30 (66.7%), 35 (77.8%), 37 (82.2%), 45 (100.0%), and 37 (82.2%), respectively. No significant association was found between gene expression frequency and endoscopic findings. However, the expression level of the omp13 gene was significantly lower in patients with gastric ulcers than in patients with non-erosive gastritis. There was no association between gene expression frequency or level and histopathological parameters. The expression levels of the omp6 and omp13 genes were significantly higher in the corpus than in the antrum. Conclusions: Neither cagA nor OMPs gene expression had a statistically significant impact on patient’s clinical outcomes in our hospital. The omp6 and omp13 genes may play an important role in the pathogenesis of H. pylori infection.

Order picking is referred as a critical process of selecting items requested by a customer in a warehouse. Meeting the demand of every customer is the main objective in this area. Large warehouses pose a challenge since the order-picking process is slowed considerably by the lengthy time it takes to transport items across the warehouse. Throughout the study, the system is hoped to develop proper procedures in the order-picking process. In handling this scenario, the decision-makers need to take any possible action to ensure the warehouses can keep operating and meeting the requirements and satisfaction of the customers. Due to this, the study’s main objective is to determine whether the Floyd–Warshall algorithm or the dynamic programming method will give the most accurate shortest path and minimum travel distance for order pickers. Two data sets (nine nodes and nineteen nodes) are used to determine the optimal path and minimum travel distance for the order picker to meet and satisfy customer orders for the warehouse. The two models were modified and applied to address real-world case studies from the automotive manufacturing company in Malaysia. The results show a big difference between the total distance by 113.48% for 19 nodes. Through this finding, the company may choose which method suits their preferences. Concurrently, this study may also contribute to problem-solving issues in any warehouse operation with a similar procedure.

Background: This research endeavors to enhance supplier selection processes by combining the Analytic Network Process (ANP) and Technique for Order of Preference by Similarity to Ideal Solution (TOPSIS) methodologies, with a specific focus on sustainability criteria. Method: Initially comprising 21 sub-criteria derived from prior research, the selection criteria are refined to 17, eliminating redundant elements. The core principle guiding this refinement is the comprehensive coverage of economic, social, and environmental dimensions, essential for sustainable supplier evaluation. Results: The study’s outcomes underscore the paramount importance of economic criteria (0.0652) in supplier selection, followed by environmental (0.0343) and social dimensions (0.0503). Key sub-criteria contributing significantly to this evaluation encompassed consistent product quality, competitive raw material pricing, proficient labor capabilities, recycling potential, punctual delivery performance, and effective waste management practices. Conclusions: These sub-criteria are thoughtfully integrated into the sustainable assessment framework, aligning seamlessly with the economic, environmental, and social criteria.

BackgroundVitamin D insufficiency is named “the pandemic of our era” by some experts. World Health Organization warns against a “deadlier outbreak” than the COVID-19 pandemic. Critical evidence is hereby for future pandemic prevention, with special emphasis on Vitamin D.MethodsA cross-sectional study was conducted with 172 unvaccinated adult participants, who presented to the emergency department. Blood measurements, radiological findings, and demographic features were evaluated in the four categories of “healthy adults, COVID-19 outpatients, hospitalized inpatients on the wards, and in the ICU.”ResultsResults were statistically significant in association with age, gender, weight, Vitamin D, glucose, urea, creatinine, leucocyte, aspartate transaminase, hemoglobin, C-reactive protein, troponin, platelet/thrombocyte, ferritin, D-dimer, triglycerate, glycated haemoglobin, lactate dehydrogenase measurements, and chest computed tomography features (each p

Road accidents occurred everywhere along the expressway, especially at the inter-urban expressway, due to its high accessibility to wide-ranging highway networks. This study uses dataset from Shah Alam Expressway from 2013 to 2017 to determine the pattern of road accidents which includes the identification of high-risk locations at the expressway as well as the attributes to the accidents at those locations. In order to determine the high-risk location, the weightage point method and accident crash frequency method have been adopted. From the two methods applied, three popular identical sites have been identified as high-risk locations at both the westbound and eastbound. For the west bound, segments 19, 46 and 63 are considered as high risk whereas for the east bound, the high-risk locations are at segments 19, 46 and 60. Most of the accidents at the high-risk locations for west and east bounds caused no injury to road users and happened during daytime. Appropriate countermeasures should be done by local authorities in order to reduce accidents at the above-mentioned area. However, special countermeasures should be considered for segments 8, 28, 31 and 32 at the west bound whereby road accidents frequently happened during nighttime at these locations. On the other hand, a special scheme and countermeasure can be implemented for segment 46 at the eastbound where motorcyclists played a major involvement in road accidents at this location. Future research may consider applying some spatial statistics for detailed analysis to the accident dataset.

Cardiac valvular surgery in patients after lung transplantation is a challenging procedure, reports are scarce. We report a 29-year-old patient who underwent concomitant mitral valve reconstruction and implantation of a decellularized aortic homograft two years after bilateral lung transplantation.

The rapid growth of cities is characterized by the "pressure" in the form of increasingly dense urban areas, slums, traffic congestion, unemployment in the cities, and the number of illegal housing in the suburbs. This issue demonstrates the need for a balance between urban and rural areas. The balance is obtained through the interaction, and the interaction there is a process of "transfer" in the form of the human population, natural resources, and other supporting components. This view of the phenomenon makes many researchers conducting various studies in the context of the interaction between rural and urban. Furthermore, the study of the interaction of cities such as Salatiga and Semarang are in fact joined in the same region, KSN Kedungsepur. Semarang and surrounding developments as Semarang Metropolitan Region (SMR) are the main attraction for the people who are around Semarang that caused an increase in the spatial interactions between Semarang and surrounding areas. From some areas belonging to KSN Kedungsepur, there are only two areas with the status of the city of Semarang city as a centre of KSN and Salatiga. This becomes interesting, unique conditions for studying the phenomenon under study is the interaction of the cities. The method used in this research was a quantitative method with descriptive analysis. Data was collected through a questionnaire survey technique primary by taking a random sample of migrants from Salatiga City and studied at the city of Semarang. The results of the study there were four mobility characteristics formed between Salatiga and Semarang, namely, commuting-boarding, boarding-commuting, boarding and boarding-permanent.

This research was done to measure the added value of goat and sheep with cut and carry systemand influencing factors of goat/sheep productivity and to measure the added value of manure to palmproductivity. The research was done in Deli Serdang Regency by determining two districts as researcharea: Tanjung Morawa District and Bangun Purba District. Location was determined by purposivesampling technique based on population of small ruminants and area of oil palm plantation in bothdistricts. Sample consisted of 50 goat farmers and 50 sheep farmers. Results indicated that farmers have18 to 24 heads of goats or sheep. Performance of goat and sheep reproduction tended to similar togenereral performance. Amount of doe, feed from palm oil and intercropped plant, livestock type andlocation influence significantly at livestock productivity (P

Touraine Solente Gole syndrome is a rare hereditary syndrome of primary pachydermoperiostosis, with the characteristic triad of pachydermia (or elephant like skin), periostosis and acropachia. A 27-year-old patient presented with aesthetic deformity of forehead due to deep skin folds and coarsening of facial features due to progressive thickening of skin. Associated palmoplantar hyperkeratosis with broadened of finger and toe tips and digital clubbing were noticed. Dermatologic evaluation revealed cutis verticis gyrata of scalp, seborrhoeic hyperplasia of face and hyperhidrosis. Natural history of the disease and aetiopathogenesis were reviewed. Aesthetic correction of forehead through frontal rhytidectomy was attempted.

We demonstrate a method for unmanned aerial vehicle based structure from motion mapping and show it to be a viable option for large scale, high resolution terrain modeling. Current methods of large scale terrain modeling can be cost and time prohibitive. We present a method for integrating low cost cameras and unmanned aerial vehicles for the purpose of 3D terrain mapping. Using structure from motion, aerial images taken of the landscape can be reconstructed into 3D models of the terrain. This process is well suited for use on unmanned aerial vehicles due to the light weight and low cost of equipment. We discuss issues of flight path planning and propose an algorithm to assist in the generation of these paths. The structure from motion mapping process is experimentally evaluated in three distinct environments: ground based testing on man-made environments, ground based testing on natural environments, and airborne testing on natural environments. Ground based testing on natural environments was shown to be extremely useful for camera calibration, and the resulting models were found to have a maximum error of 4.26 cm and standard deviation of 1.50 cm. During airborne testing, several areas of approximately 30,000 m2 were mapped. These areas were mapped with acceptable accuracy and a resolution of 1.24 cm.

Direct quantification of fossil fuel CO2 (CO2ff) in atmospheric samples can be used to examine several carbon cycle and air quality questions. We collected in situ CO2, CO, and CH4 measurements and flask samples in the boundary layer and free troposphere over Sacramento, California, USA, during two aircraft flights over and downwind of this urban area during spring of 2009. The flask samples were analyzed for Δ14CO2 and CO2 to determine the recently added CO2ff mole fraction. A suite of greenhouse and other trace gases, including hydrocarbons and halocarbons, were measured in the same samples. Strong correlations were observed between CO2ff and numerous trace gases associated with urban emissions. From these correlations we estimate emission ratios between CO2ff and these species, and compare these with bottom-up inventory-derived estimates. Recent county level inventory estimates for carbon monoxide (CO) and benzene from the California Air Resources Board CEPAM database are in good agreement with our measured emission ratios, whereas older emissions inventories appear to overestimate emissions of these gases by a factor of two. For most other trace species, there are substantial differences (200–500%) between our measured emission ratios and those derived from available emission inventories. For the first flight, we combine in situ CO measurements with the measured CO:CO2ff emission ratio of 14 ± 2 ppbCO/ppmCO2 to derive an estimate of CO2ff mole fraction throughout this flight, and also estimate the biospheric CO2 mixing ratio (CO2bio) from the difference of total and fossil CO2. The resulting CO2bio varies dramatically from up to 8 ± 2 ppm in the urban plume to −6 ± 1 ppm in the surrounding boundary layer air. Finally, we use the in situ estimates of CO2ff mole fraction to infer total fossil fuel CO2 emissions from the Sacramento region, using a mass balance approach. The resulting emissions are uncertain to within a factor of two due to uncertainties in wind speed and boundary layer height. Nevertheless, this first attempt to estimate urban-scale CO2ff from atmospheric radiocarbon measurements shows that CO2ff can be used to verify and improve emission inventories for many poorly known anthropogenic species, separate biospheric CO2, and indicates the potential to constrain CO2ff emissions if transport uncertainties are reduced.

This research was carried out in the Meat and Fish Products Co. Sincan Plant (Ankara,Turkey) between March 2002-May 2002. Total 666 broiler carcasses, 461 of which was cut-up with hand using a knife and 205 of which was cut up with cutting saw, formed the research material. Cut-up carcasses were classified in 6 groups according to their weights. The carcasses were weighted as a whole in both methods and after their weights were recorded, they were cut-up into 4 parts as breast, legs, wings and neck. It was determined that process of cutting up with knife lasted for 60 s and for 17 s with cutting saw. As a result of cutting up, net income increase was determined as 13.82 percent in cutting up with knife and as 15.61 in cutting up with cutting saw with summer prices and as 3.73 percent in cutting up with knife and as 5.6 percent in cutting up with cutting saw with winter prices.

Background: A 28-year-old man was referred to the neurology department of our hospital with difficulty of social interaction, impairment in carrying out daily life activities and muscle rigidity. He had a history of head trauma 3 years ago. Neurological examination revealed bradykinesia, hypophonic speech, resting and postural tremor, rigidity, spasticity, hyperreflexia and psychosis.

The aim of this study is to assess the influence of darbepoetin on the development of peritoneal fibrosis in rats induced by Chlorhexidine gluconate (0.1%) and ethanol (15%) and to determine the effect on peritoneal tissue levels of MMP–2 and TIMP–2, possible important factors in progression of peritoneal fibrosis. Twenty-four female Wistar albino rats were divided into three groups. The first group (CH group) received 3 ml/200g daily intraperitoneal injections of Chlorhexidine gluconate (0.1%) and ethanol (15%) dissolved in saline to induce chemical peritonitis; group 2 (ESA group) received 3 ml/200g daily injections of Chlorhexidine gluconate (0.1%) and ethanol (15%) dissolved in saline and also darbepoetin 12.5 microgr/ per kilogram/ day subcutaneously on the first and seventh days; group 3 (Control group) received intraperitoneal 0.9% saline (3 ml/200g/d) through the right lower quadrant by 21 gauge needle. The study duration was fourteen days. On the fifteenth day rats were sacrificed, parietal peritoneum samples were obtained from the left anterior abdominal wall. Pathological samples were examined using Hematoxyline & Eosin (HE) stains. The thickness, vasculpathy, and inflammation were determined by light microscopy. MMP-2 and TIMP-2 were studied immunohistochemically by monoclonal antibody staining. The activity of MMP-2 on peritoneal tissue was studied by gelatin zymography and TIMP–2 protein level was analysed by ELISA, biochemically. The decrease in thickness of parietal peritoneum in group ESA was statistically significant when compared to CH group (p0.05). Immunohistochemically, darbepoetin was shown to decrease MMP-2 expression on parietal peritoneum in CH group (p0.05). Biochemically the ratio of active MMP–2 to proMMP–2 was more significantly increased in the ESA group than in the CH group (p

Background: Investigate alterations in the expression and localization of carbohydrate units in rat retinal cells exposed to cisplatin toxicity. Aims: The aim of the study was to evaluate putative protective effects of selenium on retinal cells subjected to cisplatin. Study Design: Animal experiment. Methods: Eighteen healthy Wistar rats were divided into three equal groups: 1. Control, 2. Cisplatin and 3. Cisplatin+selenium groups. After anesthesia, the right eye of each rat was enucleated. Results: Histochemically, retinal cells of control groups reacted with α-2,3-bound sialic acid-specific Maackia amurensis lectin (MAA) strongly, while cisplatin reduced the staining intensity for MAA. However, selenium administration alleviated the reducing effect of cisplatin on the binding sites for MAA in retinal cells. The staining intensity for N-acetylgalactosamine (GalNAc residues) specific Griffonia simplicifolia-1 (GSL–1) was relatively slight in control animals and cisplatin reduced this slight staining for GSL-1 further. Selenium administration mitigated the reducing effect of cisplatin on the binding sites for GSL-1. A diffuse staining for N-acetylglucosamine (GlcNAc) specific wheat germ agglutinin (WGA) was observed throughout the retina of the control animals. In particular, cells localized in the inner plexiform and photoreceptor layers are reacted strongly with WGA. Compared to the control animals, binding sites for WGA in the retina of rats given cisplatin were remarkably decreased. However, the retinal cells of rats given selenium reacted strongly with WGA. Conclusion: Cisplatin reduces α-2,3-bound sialic acid, GlcNAc and GalNAc residues in certain retinal cells. However, selenium alleviates the reducing effect of cisplatin on carbohydrate residues in retinal cells.

Background: Artcure diffusional patch (ADP) is a novel transdermal therapeutic system that started to be used in the last decade for lumbar disc herniation (LDH). Previous studies have reported early results of the therapy. In this study, we aimed to evaluate the medium- to long-term functional outcomes of this treatment in LDH patients and examine factors predicting the need for surgery after treatment., Methods: Totally, 270 patients with single-level LDH were included. ADP was applied transdermally to the lumbar regions of the patients. Outcomes measures included the Oswestry Disability Index (ODI) and leg and low back visual analog scales (VAS). Herniations were graded using the Michigan State University (MSU) classification. The predictive factors for surgery need were analyzed with logistic regression analysis., Results: The average follow-up duration was 43.8 ± 4.8 months. The average VAS and ODI scores of the patients decreased significantly at the first month and final visits compared to admission values (p < 0.001). Within the follow-up period, 19 (7.0%) patients underwent surgery. The results of the multivariate analysis showed that ≥ 2 MSU grade of the herniation was a significant predictive factor for operation need (HR: 7.32 (1.87-28.57), p = 0.004)., Conclusion: Single-dose ADP therapy is a safe and feasible treatment option in the treatment of single-level LDH and can achieve favorable functional and pain scores at medium- to long-term. Patients with MSU grade two and three herniations should be monitored more closely, as they are more likely to experience a later surgical intervention., Clinical Trial Number: Not applicable., Competing Interests: Declarations. Ethical approval and consent to participate: This research has been approved by the Institutional Ethics Committee (Yalova University Human Research Ethics Comittee, IRB:2022/59). Informed consent was obtained from all patients. The research was conducted in compliance with the Declaration of Helsinki, and all methods were carried out in accordance with relevant guidelines and regulations. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests., (© 2025. The Author(s).)

Objectives: Extrahepatic portal vein obstruction is the most common cause of portal hypertension in children. This study aimed to evaluate the causes, clinical, laboratory and endoscopic findings, treatment approaches, long-term results, and prognosis of extrahepatic portal vein obstruction in children., Materials and Methods: We retrospectively evaluated 82 patients who were followed up with the diagnosis of extrahepatic portal vein obstruction at Gazi University Pediatric Gastroenterology clinic between January 1, 2011, and October 31, 2021., Results: Among our patients, 58.5% were male, median age at presentation was 36 months (range, 1 month to 6 years), and the follow-up period was 2 years (range, 1-14 years). The most common reasons for admission were gastrointestinal bleeding and splenomegaly. All patients diagnosed with incidentally detected obstruction were children under 5 years of age. The most frequently detected risk factors were history of umbilical vein catheterization (50%) and presence of procoagulant status (31%). Esophageal varices (94.8%), variceal bleeding (73.2%), hypersplenism (64.6%), and growth failure (13.4%) were the most common complications. Weight and/or height z-scores were lower in children with esophageal varices, variceal bleeding, and hypersplenism. Endoscopic treatments were more frequently applied to those with splenomegaly, thrombocytopenia, red color sign, and esophageal variceal bleeding. Rates of splenomegaly, hypersplenism, esophageal variceal bleeding, advanced varicose veins, and portal biliopathy were higher in patients who underwent portosystemic shunt surgery than in patients who did not undergo it., Conclusions: Morbidity and mortality rates of variceal bleeding in extrahepatic portal vein obstruction were lower than bleeding seen in cirrhotic prehepatic portal hypertension. In our study, no patients died from extrahepatic portal vein obstruction-related complications. One patient died from tuberculous meningitis.

Objectives: Inborn errors of bile acid synthesis are rare genetic disorders that usually present as neonatal cholestasis and liver disease in older children and adults. The symptomatology of inborn errors of bile acid synthesis can markedly vary among individuals, ranging from mild to severe conditions. Diagnosis is based on genetic tests and/or urine liquid secondary ionization mass spectrometry. Here, we have described characteristics of patients who were diagnosed with inborn errors of bile acid synthesis in our department., Materials and Methods: We retrospectively evaluated data from patients diagnosed with inborn errors of bile acid synthesis by urine bile acid analysis and/or genetic tests between 2013 and 2023., Results: Ten patients (8 boys, 2 girls) born to consanguineous parents were diagnosed with inborn errors of bile acid synthesis during the study period. Six patients were diagnosed with 3β-hydroxy-Δ5-C27-steroid dehydrogenase deficiency, 2 patients with peroxisomal acyl-CoA oxidase 2 deficiency, and 2 patients with peroxisome biogenesis disorder. In patients with 3β-hydroxy-Δ5-C27-steroid dehydrogenase deficiency, 3 patients were monitored with cholic acid treatment, 2 underwent liver transplant due to liver failure, and 1 patient died from liver failure. Ursodeoxycholic acid treatment was given to patients with acyl-CoA oxidase 2 deficiency. Cholic acid was given to patients with peroxisome biogenesis disorder., Conclusions: Inborn errors of bile acid synthesis can cause a variety of liver diseases, from asymptomatic liver enzyme elevation to cirrhosis. Clinical findings may include neurological symptoms and fat and fat-soluble vitamin malabsorption complications. Deficiency of 3β-hydroxy-Δ5-C27-steroid dehydrogenase is the most common bile acid synthetic defect presenting in cholestasis in infancy and childhood. Cholic acid is effective for most patients with inborn errors of bile acid synthesis. If patients do not receive an early diagnosis, progressive liver disease or other serious complications may develop.

Objectives: Giant cell hepatitis is an important diagnostic consideration in early childhood, especially for patients who present with jaundice. Different diseases may play a role in their etiology. In this study, we presented pediatric patients in our center diagnosed with giant cell hepatitis., Materials and Methods: Liver biopsies performed in our hospital between January 2010 and December 2023 were retrospectively evaluated, and biopsies with histological giant cell formation were included in the study. Demographic, clinical, and histopathological characteristics of the patients were obtained from the medical records., Results: Our study included 31 cases of giant cell hepatitis. Twenty-four (90.3%) of the patients were male, and the median age was 4 months (range, 1-148 mo). The most common clinical findings were jaundice (93.5%). Among the 31 patients, idiopathic giant cell hepatitis was observed in 13 cases (41.9%), and etiology was elucidated in 18 patients. The most common diagnoses in these patients were progressive familial intrahepatic cholestasis, biliary atresia, and autoimmune hepatitis (22.6%, 19.4%, and 9.7%, respectively). The most common accompanying histopathologic findings were canalicular cholestasis (87.1%), lobular cholestasis (54.8%), and pericellular fibrosis (35.5%). Liver transplant was required in 10 patients (32.3%). Nine patients (29%) died from various reasons. Although follow-up of 13 patients (41.9%) are ongoing, 9 patients (29%) are no longer being followed., Conclusions: Few studies in the pediatric age group on giant cell hepatitis have been conducted in the literature. Similar to our series, etiology could not be specified in most of the patients in these reports. Additional research is needed to improve our understanding of the disease process, uncover any other potential causes, and create specific treatment options.

Progressive familial intrahepatic cholestasis represents a group of disorders characterized by defective bile excretion, which causes a multitude of clinical symptoms of variable severity and usually begins in childhood. During the past few decades, a number of gene sequence variants have been shown to be associated with progressive familial intrahepatic cholestasis, and new subtypes continue to be discovered. Sequence variants of the ubiquitinspecific peptidase 53 gene have previously been associated with a novel autosomal recessive form of cholestasis with coincident normal or low γ-glutamyl transferase, with mild phenotypes. Here, we present 2 siblings with novel homozygous sequence variants in the ubiquitin-specific peptidase 53 gene with acute-on-chronic liver failure who underwent liver transplant.

Abernethy syndrome is a rare congenital vascular anomaly. In this condition, blood from the portal system is diverted to the caval system through a portocaval shunt, entirely or partially bypassing the liver. Prevalence figures in the literature range from 1 per 30000 population up to 1 per 50000 population. Abernethy syndrome is classified into 2 types based on the absence (type 1) or presence (type 2) of an intrahepatic portal vein. Abernethy syndrome can manifest with hepatic encephalopathy, pulmonary hypertension, severe osteoporosis, gastrointestinal bleeding, cirrhosis, hepatopulmonary syndrome, and abdominal pain. Hepatocellular carcinoma is a less common presentation. Type 1 Abernethy syndrome is usually treated with a liver transplant, whereas type 2 is addressed by closing the shunt either endovascularly or surgically. Here, we present cases to highlight that surgical shunt ligation is an effective treatment for type 2 Abernethy malformation when the shunt cannot be closed endovascularly.