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1.
The heterogeneous group of 3-methylglutaconic aciduria (3-MGA-uria) syndromes includes several inborn errors of metabolism biochemically characterized by increased urinary excretion of 3-methylglutaconic acid. Five distinct types have been recognized: 3-methylglutaconic aciduria type I is an inborn error of leucine catabolism; the additional four types all affect mitochondrial function through different pathomechanisms. We provide an overview of the expanding clinical spectrum of the 3-MGA-uria types and provide the newest insights into the underlying pathomechanisms. A diagnostic approach to the patient with 3-MGA-uria is presented, and we search for the connection between urinary 3-MGA excretion and mitochondrial dysfunction.  相似文献   

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The medical treatment of acute pancreatitis continues to focus on supportive care, including fluid therapy, nutrition, and antibiotics, all of which will be critically reviewed. Pharmacologic agents that were previously studied were found to be ineffective likely due to a combination of their targets and flaws in trial design. Potential future pharmacologic agents, particularly those that target intracellular calcium signaling, as well as considerations for trial design will be discussed. As the incidence of acute pancreatitis continues to increase, greater efforts will be needed to prevent hospitalization, readmission and excessive imaging in order to reduce overall healthcare costs. Primary prevention continues to focus on post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis and secondary prevention on cholecystectomy for biliary pancreatitis as well as alcohol and smoking abstinence.  相似文献   

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What’s new in pediatric hypertension?   总被引:2,自引:0,他引:2  
Research activities in the field of pediatric hypertension have been increasing in recent years, leading to important new findings in the epidemiology, diagnosis, and treatment of hypertension in children. This review summarizes recent work in these areas, focusing on the epidemic of obesity-related hypertension in children, advances in the drug therapy of childhood hypertension, and the increasing use of ambulatory blood pressure monitoring in evaluation of hypertensive children. Each of these three areas will no doubt develop further over the years ahead, resulting in further advances in the field of childhood hypertension.  相似文献   

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Trichomonas vaginalis is the most common nonviral sexually transmitted disease in the world. Recent research has elucidated several virulence factors that allow T. vaginalis to adhere to extracellular host proteins and evade the host immune system. Further investigation is needed to determine the mechanisms responsible for the perinatal morbidity associated with trichomoniasis and to evaluate the interactions between trichomoniasis and HIV infection. An effort to decrease T. vaginalis associated with perinatal morbidity by metronidazole treatment in pregnancy was unsuccessful. However, more sensitive tools for diagnosis such as polymerase chain reaction may further facilitate future understanding of the epidemiology and risk factors of disease and improve approaches to control this widespread infection. Treatment has remained highly efficacious with single-dose metronidazole, although concern regarding drug resistance has led to more systematic evaluations of the prevalence of metronidazole resistance and alternatives to metronidazole treatment.  相似文献   

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OBJECTIVES: It has been suggested that the variation in the prevalence of irritable bowel syndrome (IBS) may be due to the application of different diagnostic criteria. New criteria for IBS have been proposed (Rome II). It is unknown whether persons meeting different criteria for IBS have similar psychological and symptom features. The aim of this study was to measure the prevalence of IBS according to Manning and Rome definitions of IBS and to evaluate the clinical and psychological differences between diagnostic categories. METHODS: A total of 4500 randomly selected subjects, with equal numbers of male and female subjects aged > or = 18 yr and representative of the Australian population, took part in this study. Subjects were mailed a questionnaire (response rate, 72%). Characteristics measured were gastrointestinal symptoms over the past 12 months, neuroticism and extroversion (Eysenck Personality Questionnaire), anxiety and depression (Delusions-Symptoms-States Inventory), mental and physical functioning (SF-12), and somatic distress (Sphere). RESULTS: The prevalence for IBS according to Manning, Rome I, and Rome II was 13.6% (95% confidence interval [CI] = 3.5-5.1%), 4.4% (CI 6.0-7.8%), and 6.9% (CI 12.3-14.8%), respectively [corrected]. Only 12 persons with Rome I did not also meet Rome II criteria; 196 persons with Manning criteria did not meet Rome II cut-offs. Having IBS regardless of which criteria were used was significantly associated with psychological morbidity, but psychological factors were not important in discriminating between diagnostic categories. However, pain and bowel habit severity independently discriminated between diagnostic groups. CONCLUSIONS: IBS is a relatively common disorder in the community. The new Rome II criteria may be unnecessarily restrictive in practice.  相似文献   

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Glucose allostasis: Emperor’s new clothes?   总被引:1,自引:1,他引:0  
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Heart Failure is a global epidemic, affecting approximately 5 million adults in the U.S.A. The cornerstone of contemporary pharmacological therapy targets the over activated renin-angiotensin-aldosterone and sympathetic autonomic systems. The 2016 focused pharmacologic update on the current Heart Failure Guidelines introduces the use of two newly approved regimens valsartan/sacubitril and ivabradine. Over the last two decades, guideline directed medical therapy has accomplished significant improvement in survival rates among heart failure patients; however these novel compounds were reported to exert additional mortality and morbidity benefits, in heart failure subpopulations with reduced ejection fraction.  相似文献   

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The incidence of fungal infections is steadily rising. Until recently, clinicians have been limited in the number of effective antifungal agents at their disposal. Our traditional antifungal agents have either been hampered by troublesome side effects or a limited spectrum of activity. Due to the rising demand for better antifungal agents there are more agents in development than ever. Voriconazole and caspofungin are the most recent agents approved for use, offer a broad spectrum of activity, and are generally well tolerated. Several other novel agents are moving into clinical trials. A better understanding of these novel agents is critical for clinicians to effectively treat emerging fungal pathogens.  相似文献   

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Perianal lesions are common in patients with Crohn’s disease, and display aggressive behavior in some cases. An accurate diagnosis is necessary for the optimal management of perianal lesions. Treatment of perianal Crohn’s disease includes medical and/or surgical options. Recent discoveries in the pathogenesis of this disease have led to advances in medical and surgical therapy with good results. Perianal lesions in Crohn’s disease remain a challenging aspect for both gastroenterologists and surgeons and lea...  相似文献   

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Recently, two important issues concerning atherogensis have been raised: first - the role of hemostasis in the progression of atherosclerosis, and second - how the results of experimental animal studies can be translated into humans. There is no direct clinical evidence for the role of the coagulation system in the progression of atherosclerosis, but ample experimental data indicate that platelets and coagulation factors have an important role in the progression of both atherosclerosis and thromboembolism. A new scientific approach is thus needed to assess the actual effect of the hemostatic system on molecular and cellular responses in the vasculature. Although experimental studies helped to unravel numerous factors underlying the pathophysiology of atherosclerosis, there is still a significant gap in the translation of the experimental results to the clinic, and this gap needs to bridged to achieve reliable data from scientific research. Direct translation of the results from mouse studies to human is problematic. Clinical trials should be used more often as an early scientific probe, not just as a pathway to the commercialization of pharmaceuticals or for evaluating comparative efficacy of the agents in clinical use.  相似文献   

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Current recommendations on the management of acute myocardial infarction and the use of thrombolysis are reviewed.  相似文献   

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From the characterization of new animal models for the study of disease pathogenesis, to the demonstration of new therapeutic modalities, many developments have revolutionized the field of autoimmune bullous diseases in the past several years. This review highlights many of the significant advances that have taken place in the diagnosis, pathogenesis, and treatment options for pemphigus, pemphigoid, epidermolysis bullosa acquisita, and immunoglobulin (Ig) A-mediated bullous disorders.  相似文献   

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β-lactamases continue to be the leading cause of resistance to β-lactam antibiotics, among gram-negative bacteria. In recent years, both the incidence and the prevalence of extended-spectrum β-lactamases, inhibitor-resistant β-lactamases, AmpC-type enzymes, and both metallo-carbapenemases and nonmetallo-carbapenemases have increased. These β-lactamases provide resistance to oximino-cephalosporins, β-lactam/β-lactamase inhibitor combinations, cephamycins, and carbapenems, respectively. Strains expressing these β-lactamases will generate a host of therapeutic challenges as we begin the 21st century.  相似文献   

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