444 research outputs found

    Nasopharyngeal Myoepithelial Carcinoma Mimicking Nasopharyngeal Carcinoma

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    AbstractMyoepithelial carcinoma (malignant myoepithelioma) (MC) is a rare tumor, defined as a malignant salivary neoplasm composed almost exclusively of tumor cells with myoepithelial differentiation. It can arise in unusual location sites, such as the nasopharynx, and may be difficult to approach. Nasopharyngeal MC can sometimes present as a nasopharyngeal mass which may be mistaken for primary nasopharyngeal carcinoma (NPC). The treatment strategy for nasopharyngeal MC is different from NPC, and maximal surgical resection of the main lesion is still considered as the mainstay of therapy. Herein we present a 32-year-old man with a nasopharyngeal mass which was initially mistaken as NPC, and which was later confirmed as MC after a comprehensive review of the pathology

    Multilocus Short Sequence Repeat Analysis of Mycobacterium avium subsp. paratuberculosis Isolates from Dairy Herds in Northeastern United States of a Longitudinal Study Indicates Low Shedders are Truly Infected

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    ABSTRACT The objectives of this study were to evaluate whether low shedders of Mycobacterium avium subsp. paratuberculosis (MAP) were pass-through animals or whether they were truly infected. We also evaluated whether these animals were possibly infected by the supershedders. The MAP isolates were obtained from a longitudinal study that involved three different herds in the northeastern US. The shedding levels of animals at each culturepositive occasion were determined. Selected isolates were collected from all animals that were culture-positive at the same time super-shedders were present in the herds and from super-shedders. Using a multilocus short sequence repeat (MLSSR) approach we found 15 different strains from a total of 142 isolates analyzed. The results indicated herd-specific infections; a clonal infection in herd C with 89% of animals sharing the same strain, different strains in herds A and B. In herd C, 100% and in herd A, 17 to 70% of cows shed the same strain as that of contemporary super-shedders at a given collection date. About 82% of available tissue samples were culture-positive indicating a true infection. Taken together the results of MAP strain-typing and shedding levels, we conclude that at least 50% of low shedders have same strain as that of a contemporary super-shedder. The results of this study indicate that very few cows had characteristics of a possible pass-through animal; many more cows were actively infected. The sharing of same strain of low shedders with the contemporary super-shedders suggests that low shedders may be infected as adults by the super-shedders

    Homozygous familial hypercholesterolaemia: new insights and guidance for clinicians to improve detection and clinical management. A position paper from the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society

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    AIMS: Homozygous familial hypercholesterolaemia (HoFH) is a rare life-threatening condition characterized by markedly elevated circulating levels of low-density lipoprotein cholesterol (LDL-C) and accelerated, premature atherosclerotic cardiovascular disease (ACVD). Given recent insights into the heterogeneity of genetic defects and clinical phenotype of HoFH, and the availability of new therapeutic options, this Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society (EAS) critically reviewed available data with the aim of providing clinical guidance for the recognition and management of HoFH. METHODS AND RESULTS: Early diagnosis of HoFH and prompt initiation of diet and lipid-lowering therapy are critical. Genetic testing may provide a definitive diagnosis, but if unavailable, markedly elevated LDL-C levels together with cutaneous or tendon xanthomas before 10 years, or untreated elevated LDL-C levels consistent with heterozygous FH in both parents, are suggestive of HoFH. We recommend that patients with suspected HoFH are promptly referred to specialist centres for a comprehensive ACVD evaluation and clinical management. Lifestyle intervention and maximal statin therapy are the mainstays of treatment, ideally started in the first year of life or at an initial diagnosis, often with ezetimibe and other lipid-modifying therapy. As patients rarely achieve LDL-C targets, adjunctive lipoprotein apheresis is recommended where available, preferably started by age 5 and no later than 8 years. The number of therapeutic approaches has increased following approval of lomitapide and mipomersen for HoFH. Given the severity of ACVD, we recommend regular follow-up, including Doppler echocardiographic evaluation of the heart and aorta annually, stress testing and, if available, computed tomography coronary angiography every 5 years, or less if deemed necessary. CONCLUSION: This EAS Consensus Panel highlights the need for early identification of HoFH patients, prompt referral to specialized centres, and early initiation of appropriate treatment. These recommendations offer guidance for a wide spectrum of clinicians who are often the first to identify patients with suspected HoFH
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