Abstract A 72-year-old man developed clinical features of giant cell arteritis (GCA) and ipsilateral ophthalmic-distribution zoster, followed within 2 weeks by VZV encephalitis and 2 months later by ...ischemic optic neuropathy. Temporal artery biopsy was histopathologically negative for GCA, but contained VZV antigen and VZV DNA in multiple non-contiguous (skip) areas. The collective clinical and laboratory findings revealed a remarkably close temporal association of zoster, multifocal VZV vasculopathy with temporal artery infection, biopsy-negative VZV-positive GCA and VZV encephalitis.
Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is an emerging systemic fibrosing disorder that develops in the setting of renal insufficiency. Since its ...recognition in 1997, several case reports of NSF have been published in the rheumatology literature, reflecting the increasing incidence and recognition of this disorder in the United States and abroad. As rheumatology professionals are commonly the first to encounter newly developing cases, it is imperative that they recognize the symptoms and comorbidities of NSF and initiate work-up and treatment. To date, the precise causes of NSF have not been established; however, several promising lines of inquiry have led to a new model of the disorder. This model speculates that circulating fibrocytes of bone marrow origin are aberrantly recruited to the skin and other systemic sites by a process likely triggered or exacerbated by endothelial damage.
Context: Recognition of the importance of informatics to the practice of pathology has surged. Training residents in pathology informatics have been a daunting task for most residency programs in the ...United States because faculty often lacks experience and training resources. Nevertheless, developing resident competence in informatics is essential for the future of pathology as a specialty. Objective: The objective of the study is to develop and deliver a pathology informatics curriculum and instructional framework that guides pathology residency programs in training residents in critical pathology informatics knowledge and skills and meets Accreditation Council for Graduate Medical Education Informatics Milestones. Design: The College of American Pathologists, Association of Pathology Chairs, and Association for Pathology Informatics formed a partnership and expert work group to identify critical pathology informatics training outcomes and to create a highly adaptable curriculum and instructional approach, supported by a multiyear change management strategy. Results: Pathology Informatics Essentials for Residents (PIER) is a rigorous approach for educating all pathology residents in important pathology informatics knowledge and skills. PIER includes an instructional resource guide and toolkit for incorporating informatics training into residency programs that vary in needs, size, settings, and resources. PIER is available at http://www.apcprods.org/PIER (accessed April 6, 2016). Conclusions: PIER is an important contribution to informatics training in pathology residency programs. PIER introduces pathology trainees to broadly useful informatics concepts and tools that are relevant to practice. PIER provides residency program directors with a means to implement a standardized informatics training curriculum, to adapt the approach to local program needs, and to evaluate resident performance and progress over time.
Dural lesions mimicking meningiomas Johnson, Mahlon D.; Powell, Suzanne Z.; Boyer, Philip J. ...
Human pathology,
12/2002, Letnik:
33, Številka:
12
Journal Article
Recenzirano
Odprti dostop
Recently, a number of neoplastic and nonneoplastic entities have been reported that radiographically and clinically mimic meningiomas. Because these lesions occur infrequently and may resemble a ...meningioma during intraoperative analysis, they may not be considered in the differential diagnosis. This review (and case illustrations) considers some of the newly recognized and notable lesions that can mimic meningiomas, including solitary fibrous tumors, gliosarcomas, leiomyosarcomas, hemangiopericytomas, melanocytomas, Hodgkin's disease, plasmacytomas, inflammatory pseudotumors, neurosarcoidosis, plasma cell granulomas, Rosai-Dorfman disease, Castleman's disease, xanthomas, rheumatoid nodules, and tuberculomas. Awareness that these lesions involve the dura may facilitate intraoperative recognition and, in some cases, preclude unnecessary additional surgery. HUM PATHOL 33:1211-1226. Copyright 2002, Elsevier Science (USA). All rights reserved.
Restless legs syndrome (RLS) is a neurological disorder characterized by a strong urge to move the legs. Sufferers of RLS often experience chronic sleep deprivation, due to the characteristic ...worsening of symptoms both when at rest and during the night. MRI data, autopsy studies, and a consistent decrease in CSF ferritin all suggest that early-onset RLS is associated with insufficient iron in the brain. In this study, we examined the relationship between the iron regulatory hormone hepcidin and RLS. Hepcidin serves as a hormone that signals iron release from cells by interacting with ferroportin. We measured the expression and concentration of pro-hepcidin in the brain and cerebrospinal fluid of both RLS patients and control individuals. In CSF, we found that pro-hepcidin levels were significantly decreased in early-onset RLS patient samples, but not in late-onset RLS patients, when compared to controls. Conversely, in neuromelanin cells, substantia nigra, and putamen, the concentration of pro-hepcidin in RLS samples is significantly increased compared to controls. Functionally, hepcidin binds to ferroportin to limit iron movement from cells. Therefore, we provide immunocytochemical evidence that ferroportin is expressed by the epithelial cells of the choroid plexus and the ependymal cells lining the ventricles. These data suggest that sites of action for hepcidin include signaling the ventricular system for movement between brain and CSF. At this time, it cannot be determined if the lower levels of pro-hepcidin in the CSF represent a compensatory response to the decreased levels of iron in the brain or a defective signaling mechanism in RLS. Nonetheless, these data support the mounting evidence that there is a biological basis for RLS and the underlying mechanism involves iron management.
The ability of the brain to store a readily bioavailable source of iron is essential for normal neurologic function because both iron deficiency and iron excess in the brain have serious neurologic ...consequences. The blood-brain barrier presents unique challenges to timely and adequate delivery of iron to the brain. The regional compartmentalization of neurologic function and a myriad of cell types provide additional challenges. Furthermore, iron-dependent events within the central nervous system (CNS) are age dependent (e.g., myelination) or region specific (e.g., dopamine synthesis). Thus the mechanisms for maintaining the delicate balance of CNS iron concentration must be considered on a region-specific and age-specific basis. Confounding factors that influence brain iron acquisition in addition to age-specific and region-specific requirements are dietary factors and disease. This article raises and addresses the novel concept of regional regulation of brain iron uptake by reviewing the developmental patterns of iron accumulation and expression of proteins responsible for maintaining iron homeostasis in a region-specific and cell-specific manner. Understanding these mechanisms is essential for generating insights into diseases such as Hallervorden-Spatz syndrome, in which excess iron accumulation in the brain plays a significant role in the disease process, and should also unveil windows of opportunity for replenishing the brain in a state of iron deficiency.
Neurofibromatosis type 1 (NF1) is a common inherited cancer predisposition syndrome. The NF1 gene product, neurofibromin, is hypothesized to function as a tumor suppressor and nearly all NF1 patients ...develop benign peripheral nerve tumors. These neurofibromas presumably arise from NF1 inactivation in S100(+)Schwann cells, but there is no formal proof for this mechanism. We demonstrate that fibro-blasts isolated from neurofibromas carried at least one normal NF1 allele and expressed both NF1 mRNA and protein, whereas the S100(+)cells typically lacked the NF1 transcript. Our findings further indicate that additional molecular events aside from NF1 inactivation in Schwann cells and/or other neural crest derivatives contribute to neurofibroma formation.
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