In this study, non-interlaced/non-Z single layer and multilayered uniaxial, biaxial and multiaxis preform structures were developed to produce E-glass/polyester composite structures. The data generated from the tensile tests of the composite structures were analyzed by using a regression model. We found that the tensile strength of the E-glass/polyester structures depended on yarn orientation and the number of layers. It was also indicated that the tensile properties of the composite structures were proportional to their total fiber volume fractions. If all the E-glass/polyester structures are made with the same preform packing density, the structures made from coarse fiber show high tensile strength, compared to structures made from fine fiber, due to high fiber volume fraction. All structures showed mode-I type delamination failure which occurred in the out-of-plane direction as a form of interlayer splitting parallel to the applied tensile load direction. Local interlayer failures were observed between warp/warp in uniaxial structures, warp/filling in biaxial structures, and bias/bias and bias/filling in multiaxis structures due to the non-Z yarn reinforcement. The developed regression model was considered as a suitable and viable tool to predict the tensile properties of the composite structures.
Bibliometric studies on inherited metabolic diseases(IMDs) didn't exist in the literature.Our research aims to conduct a bibliometric study to determine the current status,trending topics,and missing points of publications on IMDs.Between 1968-2023, we conducted a literature search with keyword "inherited metabolic disease" in the SCOPUS-database.We included research articles in medicine written in English,published in the final section.We created our data pool using VoSviewer, SciMAT, and Rstudio software programs for bibliometric parameters of the articles that met the inclusion criteria.We performed bibliometric analysis of the data with R-package "bibliometrix" and BibExcel programs.We included 2702 research articles published on IMDs.Top three countries that have written the most articles in this field are; USA(n= 501), United Kingdom(n=182), and China(n= 172).The most preferred keywords by the authors were;newborn screening(n=54), mutation(n=43), phenylketonuria(n=42), children(n=35), genetics(n= 34) and maple syrup urine disease(n=32).Trending topics were osteoporosis, computed tomography, bone marrow transplantation in the early years of the study, chronic kidney disease, urea cycle disorders, next-generation sequencing, newborn screening, and familial hypercholesterolemia in the final years of the study. This study provides clinicians with a new perspective showing that molecular and genetic studies in inherited metabolic diseases will play an essential role in diagnosis and treatment in the future.
Phenylketonuria is treated either with tetrahydrobiopterin (BH4) or with a phenylalanine-restricted diet. Patients in the diet group may tend to consume carbohydrate-rich foods which have a risk for obesity. In this study, the prevalence of obesity+overweight among phenylketonuria patients either treated with phenylalanine-restricted diet or with BH4 were compared.Patients with phenylketonuria were divided into two groups on dietary treatment and BH4 treatment. Body mass index (BMI), BMI-percentile, and z-score values of patients were calculated and classified as underweight, normal-weight, overweight, and obese according to their nutritional status. The annual mean phenylalanine level of each patient is also evaluated. The study was done retrospectively.A total of 130 patients was included. 77 were receiving diet (female (n,%):37, 48.1%; male (n,%):40, 51.9%) and 53 were receiving BH4 (female (n,%):33, 62.3%; male (n,%):20, 37.7%) respectively. According to BMI-z-score, the sum of the ratio of obesity+overweight was found to be 35.1% in the diet group, 16.9% in the BH4 group. Ratio was significantly higher in diet group (p=0.02). When obesity+overweight ratios were examined in terms of female/male distribution, no significant difference was found. Considering the correlation of obesity+overweight ratios with age in two groups, the median age of the patients with normal weight+underweight in the BH4 group were found as 46-months, and the median age of obese+overweight patients was 137-months (p=0.001). For the same situation, there was no significant difference in the dietary treatment group (p=0.92). Mean annual phenylalanine levels were significantly higher in obese+overweight patients (p=0.047) in the BH4 treatment group but this difference was not significant in the diet group (p=0.051).Patients on the phenylalanine-restricted diet have a risk of obesity or overweight. Therefore, attention should be paid not only to the phenylalanine levels of these patients but also to their weight control and dietary content
Ammonia; It is a toxic molecule for the central nervous system resulting from the catabolism of proteins. Its excretion is provided with the urea cycle. Argininemia is a rare subtype of urea cycle disorders. Arginase enzyme catalyzes the last stage of the urea cycle, arginine; urea and ornithine are broken down. The decrease in arginase 1 (ARG1) enzyme activity is responsible for argininemia. The most common presenting symptoms of patients diagnosed with argininemia are progressive spastic diplegia, regression in developmental stages, choreoathetosis, hepatomegaly and seizures. The diagnosis of the disease can be made by detecting the elevation of arginine in body fluids together with the increase in serum ammonia. Neurological findings of these patients can be confused with cerebral palsy. In this case report, we wanted to present a patient with argininemia who was followed up with a diagnosis of cerebral palsy for a long time. Early diagnosis, restricted protein and arginine diet are life-saving in this disease. Argininemia should be kept in mind in patients with unexplained neuromotor retardation.
To describe clinical features, demographic data, and complications of the patients with SRUS, which is a rare cause of rectal bleeding in children. Eleven patients diagnosed with Solitary Rectal Ulcer Syndrome (SRUS) were evaluated. The patients assessed by colonoscopy and the biopsies were investigated. The data evaluated in SPSS Program. The exact Method of the Chi-square test was used to compare groups according to qualitative variables. P <0.05 value was considered statistically significant. The most common symptom of the patients was rectal bleeding followed by abdominal pain and constipation. Lesions were mostly ulcerative in the endoscopic examination. There was a statistically significant relationship between the admission symptom and the response to treatment. Patients with abdominal pain and rectal bleeding had poor responses to treatment. In conclusion, SRUS is not uncommon than is thought in pediatric patients with the symptoms of rectal bleeding and constipation. SRUS should be considered in patients with or without rectal prolapse, with any complaints of any lesions in the rectum, hematochezia, and tenesmus.
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