Recent advances in Rosai-Dorfman-Destombes disease (RDD), notably molecular testing, targeted therapy, and PET-CT imaging, hold promise for better recognition and improved outcomes. This study presents patients diagnosed and treated in a "real world" setting, where navigating limited resources must be considered. This retrospective single-center review includes 15 adult patients diagnosed with RDD at Vancouver General Hospital between November 2015 and October 2023.
View Article and Find Full Text PDFCognitive control is the ability to allocate attention away from stimuli that are irrelevant to achieving a goal, towards stimuli that are. When conflict is anticipated, attention is biased in a global, top-down manner called proactive control and this effortful type of cognitive control is engaged before stimulus onset. The list-wise congruency proportion (LWPC) effect, where the Stroop congruency effect is reduced when there are more incongruent than congruent trials compared to vice versa, has been viewed as one of the prime signatures of this type of cognitive control.
View Article and Find Full Text PDFWe compared liquid chromatography tandem mass spectrometry (LC-MS/MS) against Binding Site immunonephelometry (BSIN) with regards to these methods' abilities to diagnose IgG4-related disease (IgG4-RD). IgG subclasses were gathered from laboratory from December 2011 to December 2020. The IgG4-RD positive and negative patients were diagnosed according to the ACR/EULAR classification criteria by extensive chart review.
View Article and Find Full Text PDFLive birth (viviparity) has arisen repeatedly and independently among animals. We sequenced the genome and transcriptome of the viviparous Pacific beetle-mimic cockroach and performed comparative analyses with two other viviparous insect lineages, tsetse flies and aphids, to unravel the basis underlying the transition to viviparity in insects. We identified pathways undergoing adaptive evolution for insects, involved in urogenital remodeling, tracheal system, heart development, and nutrient metabolism.
View Article and Find Full Text PDFAlemtuzumab has been associated with the emergence of secondary autoimmune diseases. We report a case of a patient with relapsing-remitting multiple sclerosis who developed a refractory immune thrombocytopaenia associated with vasculitis, myelofibrosis and later Guillain-Barré syndrome following alemtuzumab. The medical community should be aware of unusual and unexpected adverse events that may be associated with alemtuzumab, especially when occurring simultaneously in the same patient.
View Article and Find Full Text PDFObjective: To evaluate whether corpus callosum (CC) lesions are inextricably linked to CNS symptoms of Susac Syndrome (SuS) by reviewing published cases to find instances where: 1) CC lesions occur without CNS symptoms, and 2) whether patients with CNS symptoms lack CC lesions.
Methods: 100 reported cases of SuS were identified in PubMed. Clinical symptoms, para-clinical testing and MRI data were collected both at presentation and for any available follow-up and analyzed.
Objectives: We present a full autopsy with a focused radiology and pathologic review of the coronary arteries. We hope that the results described in this article will help create better diagnostic measures and prevent future coronary artery vasculitis misdiagnosis.
Methods: A full autopsy was performed on the body of Dr Myung Choong Yoon, with full consent from the family, within the department of pathology and laboratory medicine at Vancouver General Hospital.
There has been a recent resurgence of research on the characterization of phage for therapeutic use due to the morbidity and mortality associated with treatment failures in cases of multidrug-resistant infections. A bacteriophage isolated from activated sludge that targets ATCC19606 was characterized by electron microscopy, genome sequencing, comparative genomics, and a host range analysis. The morphology of phage DMU1 resembles phages in .
View Article and Find Full Text PDF: Serum IgG4 is typically measured to investigate for Immunoglobulin G4-related Disease (IgG4-RD), a fibroinflammatory condition associated with polyclonal increase in serum IgG4. However, increased IgG4 can also be monoclonal, and little is known about IgG4 myeloma. : We describe two cases of IgG4 myeloma without clinical, radiologic, or laboratory features of IgG4-related disease.
View Article and Find Full Text PDFBackground: Retroperitoneal fibrosis (RPF) is characterized by the proliferation of fibrous tissue in the retroperitoneum. The majority of RPF cases are due to idiopathic or IgG4-related disease. Recent studies on IgG4-related disease have shown rituximab to be an effective treatment.
View Article and Find Full Text PDFBackground: Hypereosinophilia (HE, persistent peripheral blood eosinophilia > 1.5 × 10 /L) and hypereosinophilic syndrome (HES, HE with end-organ damage) are classified as primary (due to a myeloid clone), secondary (due to a wide variety of reactive causes), or idiopathic. Diagnostic evaluation of eosinophilia is challenging, in part because secondary causes of HE/HES such as lymphocyte-variant HES (L-HES) and vasculitis are difficult to diagnose, and emerging causes such as immunoglobulin G4-related disease (IgG4-RD) have rarely been examined.
View Article and Find Full Text PDFUnderstanding the extent to which ecological divergence is repeatable is essential for predicting responses of biodiversity to environmental change. Here we test the predictability of evolution, from genotype to phenotype, by studying parallel evolution in a salmonid fish, Arctic charr (Salvelinus alpinus), across eleven replicate sympatric ecotype pairs (benthivorous-planktivorous and planktivorous-piscivorous) and two evolutionary lineages. We found considerable variability in eco-morphological divergence, with several traits related to foraging (eye diameter, pectoral fin length) being highly parallel even across lineages.
View Article and Find Full Text PDFThis article will review the structure and function of IgG4, methods of measuring serum IgG4 concentrations, clinical conditions associated with increased and decreased serum IgG4, and the test characteristics of serum IgG4 in the diagnosis and management of Immunoglobulin G4-Related Disease (IgG4-RD). The four subclasses of IgG were discovered in 1964 through experiments on monoclonal IgG in patients with myeloma. Since 2001, interest in measuring serum IgG subclasses has increased dramatically due to the emergence of IgG4-RD, a multisystem fibroinflammatory condition wherein polyclonal serum IgG4 concentration is increased in approximately 70% of cases.
View Article and Find Full Text PDFWe present an atypical presentation of Rosai-Dorfman disease (RDD). Due to its overlap with IgG4-related disease (IgG4-RD), this case proved to be a diagnostic dilemma. Our case is an example of the importance of having a broad-based differential and, ultimately, an in-depth histopathological review.
View Article and Find Full Text PDFMicrobiol Resour Announc
October 2019
is an opportunistic human pathogen that is part of the / (ACB) complex. Here, we report the complete genome sequence of strain M2.
View Article and Find Full Text PDFBackground: An increasing body of literature suggests a positive, neuroprotective effect for testosterone on cognition in older men. However, randomized clinical trials (RCTs) examining the effects of testosterone supplementation (TS) on cognitive function have been inconclusive.
Objective: To investigate the potential for TS to prevent cognitive decline in otherwise cognitively healthy older men, by examining the differential effects of TS on cognitively healthy older men in RCTs.
IgG4-related disease is a fibro-inflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. This review focuses on the hematologic manifestations of IgG4-related disease, including lymphadenopathy, eosinophilia, and polyclonal hypergammaglobulinemia.
View Article and Find Full Text PDF