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Mucopolysaccharidosis type IIIA (MPS IIIA) is characterised by a progressive neurological decline leading to early death. It is caused by bi-allelic loss-of-function mutations in SGSH encoding sulphamidase, a lysosomal enzyme required for heparan sulphate glycosaminoglycan (HS GAG) degradation, that results in the progressive build-up of HS GAGs in multiple tissues most notably the central nervous system (CNS). Skin fibroblasts from two MPS IIIA patients who presented with an intermediate and a severe clinical phenotype, respectively, were reprogrammed into induced pluripotent stem cells (iPSCs). The intermediate MPS IIIA iPSCs were then differentiated into neural progenitor cells (NPCs) and subsequently neurons. The patient derived fibroblasts, iPSCs, NPCs and neurons all displayed hallmark biochemical characteristics of MPS IIIA including reduced sulphamidase activity and increased accumulation of an MPS IIIA HS GAG biomarker. Proliferation of MPS IIIA iPSC-derived NPCs was reduced compared to control, but chanism driving reduced neurogenesis remains to be determined but appears downstream of MPS IIIA HS GAG accumulation.Two patients from Huanggang, China, were diagnosed with spotted fever group (SFG) rickettsiosis-caused by spotted fever group rickettsiae (SFGR)-in 2021. This study aimed to investigate the clinical symptoms, laboratory examinations, epidemiological factors, and therapeutic responses in patients with SFG rickettsiosis-an emerging disease in this region. The patients showed a variety of clinical signs and symptoms, such as acute febrile illness with severe headache, myalgia, asthenia, anorexia, eschar, lymphadenopathy, and rash on the trunk and extremities. They exhibited increased neutrophil ratio, mild thrombocytopenia, liver dysfunction, and increased C-reactive protein and procalcitonin levels. Following treatment with doxycycline, the patients recovered completely. This is the first report of Rickettsia japonica infection in Huanggang City, Hubei Province, China. SFGR infection is a tick-borne disease, which can be effectively treated with doxycycline; however, it has a mortality rate of approximately 10% with delays in treatment. The Huanggang area is also a high-risk area for tick-borne severe fever with thrombocytopenia syndrome (SFTS). Therefore, SFTS and SFG rickettsiosis should be carefully diagnosed in this area and clinicians should be alert with respect to the possibility of infections with both SFTS and SFG rickettsiosis.With 14 million new infections each year, the human papillomavirus (HPV) is the most common sexually transmitted infection (STI) among both men and women in the United States (US). Infections with the human papillomavirus (HPV) are responsible for a considerable portion of the global cancer burden. HPV-related oral malignancies are on the rise around the world, according to epidemiological studies. To provide accurate advice to their patients, dental practitioners require thorough, up-to-date HPV-related knowledge. Methods. In this cross-sectional study, data were collected by the purposely constructed questionnaire. A questionnaire composed of the demographic items and items related to the awareness and knowledge about Human papillomavirus. The questionnaire was constructed after a series of discussions between the panel of experts. This panel was composed of a subject specialist, researcher, and language expert. The Cronbach alpha of the questionnaire was calculated. The study will be conducted in the Al Qunfudhah. Results. The Cronbach alpha of the questionnaire was 0.72. ALC-0159 clinical trial Out of a total of 550 respondents, with a mean (SD) age of 47.5 (11.5), the female respondents were 167 (30.4%) while male were 383 (69.6%). 20.5% of the respondents (out of 550) were having awareness of HPV. Implications. Knowledge of HPV-related oral cancer is critical, and it is advised to be taught as part of dental students' basic curriculum and clinical training. This problem can be solved by better educational training programs. Knowledge of HPV-related oral cancer is critical, and it is advised to be taught as part of dental students' basic curriculum and clinical training.The pathological changes of myocarditis include degeneration and necrosis of myocardial cells and infiltration of inflammatory cells in the myocardial interstitium, accompanied by obvious myocardial fibrosis. Myocardial fibrosis is a determinant of ventricular remodeling and an important indicator of the classification of clinical risk factors and has an important value in evaluating the prognosis of heart disease. Cardiac magnetic resonance (CMR) is the "gold standard" for evaluating the shape and function of the heart, and it can show the characteristic pathological changes of myocardial tissue. The traditional gadolinium imaging agent delays the enhanced sequence images to visually show the extent of the affected myocardial fibrosis, but it cannot effectively identify small focal fibrosis or widespread diffuse fibrosis. The CMR longitudinal relaxation time quantitative technique can directly measure the relaxation time (T1) determined by the myocardial tissue and does not depend on the signal strength of the reference tissue and can quantitatively analyze the affected myocardium. In this study, the initial and enhanced quantitative imaging techniques of CMR were used to measure the magnetic value of the myocardium in patients with myocarditis, to explore the diagnostic value of myocardial fibrosis, and to analyze the correlation between cardiac fibrosis and cardiac function.Dieulafoy lesion is a rarely seen, superficial vascular lesion of the gastric mucosa that can lead to severe and recurrent bleeding which can be life-threatening. It is characterized by massive hemorrhages that can occur as a result of protrusion of a large artery from a submucosal defect. Endoscopic injection and mechanical and thermal methods are frequently used in traditional treatment. Herein, we presented a 61-year-old women who was admitted with upper gastrointestinal hemorrhage owing to Dieulafoy lesion who was successfully treated with over-the-scope clip as the first choice of treatment.Hepatic involvement has been reported in 20-30% of patients with systemic sarcoidosis. However, isolated liver involvement is rare. We report a case of cirrhosis with portal hypertension, diagnosed as hepatic sarcoidosis based on high angiotensin-converting enzyme level and presence of multiple non-caseating granulomas in liver histopathology. She improved clinically after treatment with steroid and ursodeoxycholic acid.Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder comprised of venous malformation mostly involving the skin and gastrointestinal (GI) tract but can also involve other visceral organs. The most predominant site of GI tract involvement is the small bowel. In patients with GI lesions, treatment depends on the severity of bleeding, and extent of involvement. Conservative therapy with iron supplementation and blood transfusion is appropriate in cases with mild bleeding but in severe cases endoscopic and surgical interventions would be beneficial. Also, medical therapy with sirolimus significantly reduces bleeding. A 20-year-old woman was referred to our hospital after transfusion of six units of packed cell because of several episodes of lower GI bleeding within the past three months in the form of melena and a single episode of hematochezia. Her last hemoglobin level before admission was 10mg/dl. She underwent various unsuccessful investigations since she was eight years old to find the origin of refractory iron deficiency anemia. In upper endoscopy, five bleeding polypoid lesions were discovered in the jejunum. Lesions were excised by snare polypectomy. Over a six-month follow-up period, no signs of lower GI bleeding were noted and the patient had a normal hemoglobin level.BACKGROUND With an increase in the prevalence and incidence of inflammatory bowel diseases (IBDs), they have become a global challenge. The IBD registry provides complete and timely data, thereby greatly contributing to the estimation of the burden of these diseases and development of control and prevention programs. We aimed to develop an IBD registry software. METHODS The present applied-developmental study had two main stages determining user requirements, and developing the IBD registry software. The software was created using a Web-based software development technology called ASP.NET Core 2. The programming language in this framework was C#, and the SQL Server 2017 was employed to create a strong and integrated software databank in the relational form. RESULTS When determining user requirements, the data elements were classified into two main categories of patient information and visits and tests. Moreover, in this stage, registry functions, including case ascertainment, abstracting, follow-up, quality control, and reporting were identified. In the registry software development stage, the object-oriented conceptual model was designed with five use case diagrams and 59 classes. The user interface comprised the following main sections add patient, find patient, complete source report, report, staff, and drugs. Precise user authentication and authorization were also employed to enhance the security of the developed software. CONCLUSION Development of an IBD registry which can precisely record patients and estimate the incidence, prevalence, and socioeconomic burden of these diseases can assist in planning for the control and prevention of IBD in healthcare systems.BACKGROUND Due to stressful occupational conditions, irregular dietary and sleep schedules, medical students are at increased risk of developing gastrointestinal disorders, gastroesophageal reflux (GERD) in particular, as well as sleep disturbances. Therefore, for the first time, we aimed to assess the correlation between GERD and sleep disturbances among medical students. METHODS The current cross-sectional study was done on 290 medical students at different study periods in Iran during 2018-2019. Age, sex, stage of studying, residence, and body mass index were gathered. The frequency scale for the symptoms of gastroesophageal reflux (FSSG) was utilized to assess gastrointestinal symptoms among them and the Pittsburgh Sleep Quality Index (PSQI) to assess sleep quality. Eventually, the association of sleep disturbances with demographic factors and gastrointestinal symptoms was evaluated. RESULTS Living in the dormitory (p = 0.048; OR 1.73; 95%CI 1.01-2.99) and being overweight (p less then 0.001; OR 3.09; 95%CI 1.58-6.06) were independently correlated with impaired sleep quality. GERD presented either by heartburn (p less then 0.001) or regurgitation (p less then 0.001) was associated with a lower quality of life. CONCLUSION GERD was correlated with poor sleep quality among medical students. In addition, residence in dormitory and being overweight were correlated with poor sleep quality.BACKGROUND Antibiotic resistance is a major cause of Helicobacter pylori (H. pylori ) treatment failures. The increased resistance to clarithromycin and metronidazole has reduced the ability of this therapeutic regimen and prompted researchers to look for other drugs. One of the antibiotics of interest in this regard is furazolidone because of its low drug resistance. The aim of this study is compare two-drug regimens including low-dose and high-dose furazolidone in the treatment of H. pylori. METHODS This study is a clinical trial in which the studied subjects were categorized into two groups. The first group underwent treatment with amoxicillin 1000 mg-BD, furazolidone 100 mg-BD, omeprazole 20 mg-BD, and bismuth subcitrate 240 mg-BD for two weeks (low-dose OFAB). The second group received furazolidone 200 mg-BD (high-dose OFAB). Then eight weeks after completion of the treatment, they were examined in terms of eradication via the UBT test. RESULTS 85 participants completed the study in each group. The response to treatment was 76% and 83% in the low and high-dose groups, respectively, based on intention to treat analysis.
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