Αρχειοθήκη ιστολογίου

Παρασκευή 22 Ιουνίου 2018

Personal observations of Achenbach syndrome

Clinical and Experimental Dermatology, EarlyView.


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Evolution of Kaposi sarcoma in the past 30 years in a tertiary hospital of the European Mediterranean basin

Clinical and Experimental Dermatology, EarlyView.


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Eosinophilic Gastrointestinal Diseases (EGIDs) beyond Eosinophilic Esophagitis (EoE)

Publication date: Available online 22 June 2018
Source:Annals of Allergy, Asthma & Immunology
Author(s): Maureen Egan, Glenn T. Furuta
ObjectiveEosinophilic Gastrointestinal Diseases (EGIDs) are a rare group of disorders that can have varied clinical presentations dependent on the involved segment within the gastrointestinal tract. Eosinophilic gastritis presents with abdominal pain or vomiting; Eosinophilic gastroenteritis presents with diarrhea and anemia/hypoalbuminemia and Eosinophilic colitis can present with diarrhea or bloody stools. These nonspecific symptoms remain poorly understood as to their relationship to the underlying pathogenesis but a significant percentage of patients are atopic. Here we discuss the current literature on EGIDs to promote a practical approach to the diagnosis and care.Data SourcesReview of published literatureStudy SelectionsClinically relevant literature from 1900-2018 was obtained from a PubMed search. Those which addressed prevalence, diagnosis, clinical manifestations, natural history and treatment of EGID's were reviewed and are summarized here.ResultsOf the total 485 articles found in the search, majority of which were case reports, 71 were selected for review. The natural history of EGIDs is based primarily on case series and small numbers of patients making therapeutic decisions difficult. Treatment remains limited to dietary restriction that is not effective in many patients and topical or systemic corticosteroids whose long-term use is to be avoided if possible.ConclusionsWith the seeming increase in all types of EGIDs, phenotypic descriptions are emerging that will likely promote better understanding of the pathogenesis and identification of novel therapeutic targets.



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Immunoglobulin and T cell receptor genes in Chinese crocodile lizard Shinisaurus crocodilurus

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Publication date: September 2018
Source:Molecular Immunology, Volume 101
Author(s): Francisco Gambón-Deza, David N. Olivieri
Squamata are reptiles that diverged from mammals 300 million years ago. During this period, the immunoglobulin (IG) and T lymphocyte receptor (TCR) genes evolved parallel to mammals. However, unlike mammals whose IG/TCR locus has retained a constant structure throughout evolution, Squamata have witnessed duplications, losses, and/or gains in the domains of their immunoglobulin genes. The recent genome sequencing of Shinisaurus crocodilurus, a representative species of the oldest reptiles, provides an opportunity to contrast the structure of IG and TCR genes from previously studied Squamata. This study revealed ten immunoglobulin genes: five genes for immunoglobulin M (IgM), two for immunoglobulin D (IgD), one for immunoglobulin D2 (IgD2), and two for immunoglobulin Y (IgY). As in other Squamata, there are genes for the λ light chain (IGLV) but not for the κ chain (IGKV). Here, the data shows that in some IgM genes, the cysteine needed to bind the λ chains does not exist, but we present evidence for possible non-covalent binding to the light chain. With respect to TCR, one gene is detected for the α constant chain (TRAC) and two genes for the β constant chain (TRBC); one of which is located in the locus of the variable regions of the heavy chain. As in the rest of the Squamata, genes for the γ/δ T cell receptor were not found. The V gene repertoire is found to be consistent with all other Squamata with few V genes for beta chain of TCR.



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Acquired and genetic host susceptibility factors and microbial pathogenic factors that predispose to nontuberculous mycobacterial infections

Jennifer R Honda | Scott Alper | Xiyuan Bai | Edward D Chan

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Cytokine modulation of atopic itch

Anna M Trier | Brian S Kim

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Treatment Paradigm for Nasal Airway Obstruction

Nasal airway obstruction (NAO) is a common otolaryngic complaint with many potential causes, frequently structural or inflammatory in nature. Patients typically have multiple coexisting factors leading to symptoms. Good patient outcomes require careful preoperative evaluation, including nasal endoscopy, to accurately identify sources of obstruction and tailor intervention appropriately. Common structural causes of NAO include inferior turbinate hypertrophy, nasal septal deviation, and narrowing or collapse of the internal or external nasal valves. The internal nasal valve has the narrowest cross-sectional area within the nasal airway and is thus most sensitive to changes in dimension due to anatomic variation or surgical intervention.

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Anatomy and Physiology of Nasal Obstruction

Nasal obstruction is a common presenting symptom to clinicians and affects up to one-third of the population. There are several factors that cause nasal obstruction, including anatomic, physiologic, and pathophysiologic factors. The anatomy and physiology of nasal obstruction is complicated and is influenced by patency of nasal passages, mucociliary function, airflow receptors, autonomic function, and degree of mucosal inflammation. Common anatomic causes include internal nasal valve stenosis/collapse, septal deviation, and turbinate hypertrophy. Common physiologic causes include sinonasal inflammatory disorders and iatrogenic causes.

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Utility of narrow band imaging in the diagnosis of middle turbinate head edema

Middle turbinate head edema has recently been found to have high specificity for diagnosis of inhalant allergy. However, subtle mucosal edema can be difficult to appreciate under white light endoscopy. Narrow band imaging (NBI) has the potential to demonstrate edema by identifying reduced mucosal vascularity and improve sensitivity for the detection of mucosal edema. Narrow band imaging was assessed to determine its utility in objectifying mucosal edema.

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Effects of combination of balloon Eustachian tuboplasty with methylprednisolone irrigation on treatment of chronic otitis media with effusion in adults

Adult chronic otitis media with effusion (COME) is characterized by Eustachian tube dysfunction and mucosal inflammation, which management has long been a challenge because of high recurrence. This study was to investigate the pathological changes of Eustachian tube mucosa and optimized treatment.

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Stereotactic radiotherapy in three weekly fractions for the management of vestibular schwannomas

This study evaluates the rates of tumor control, hearing preservation and cranial nerve toxicity with the use of CyberKnife stereotactic radiotherapy consisting of 2100 cGy to the 80% isodose line delivered in three weekly fractions to treat vestibular schwannomas.

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Understanding Caregiver Goals, Benefits, and Acceptable Risks of Peanut Allergy Therapies

Peanut allergy affects 1.4-4.5% of children and may be a lifelong allergy in 75%.1 Nearly 50% of peanut allergic individuals have had a past severe reaction.2 Several promising peanut allergy treatments are under development.3 Peanut allergy is associated with anxiety and poor quality of life (QoL) in the child and caregiver, driven by fear the child will react from an accidental exposure—something treatment could prevent.4–10 For these reasons, two therapies, peanut oral immunotherapy (OIT) and epicutaneous immunotherapy (EPIT), have been granted US Food and Drug Administration (FDA) fast-track status.

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Eosinophilic Gastrointestinal Diseases (EGIDs) beyond Eosinophilic Esophagitis (EoE)

Eosinophilic Gastrointestinal Diseases (EGIDs) are a rare group of disorders that can have varied clinical presentations dependent on the involved segment within the gastrointestinal tract. Eosinophilic gastritis presents with abdominal pain or vomiting; Eosinophilic gastroenteritis presents with diarrhea and anemia/hypoalbuminemia and Eosinophilic colitis can present with diarrhea or bloody stools. These nonspecific symptoms remain poorly understood as to their relationship to the underlying pathogenesis but a significant percentage of patients are atopic.

https://ift.tt/2lqEpC1

A difficult situation – balancing critical anticoagulation versus the risk of permanent neurologic deficit: a case report

Anticoagulation is the mainstay of treatment for pulmonary embolism. However, if bleeding unfortunately occurs, the risks and benefits of anticoagulation present a challenge. Management of one hemorrhagic comp...

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Anterior hypopituitarism in a patient with amyloidosis secondary to Crohn’s disease: a case report

Amyloid infiltration of endocrine glands has been reported, mostly in the thyroid, pancreas, adrenals, and testes, but affected patients do not frequently exhibit overt endocrine insufficiency. Here we report ...

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Aggravation of mild axillary hidradenitis suppurativa by microwave ablation: results of a randomized intra-patient controlled trial



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Tips for dyeing hair when you have psoriasis

People with psoriasis can dye their hair safely. However, there are factors to consider before, during, and after applying the dye. In this article, learn about the risks, and seven tips developed to help people with psoriasis to dye their hair safely. We also describe how to prevent flare-ups on the scalp.

https://ift.tt/2MfVASl

Assisting Your Child with Gender Confusion

The preteen and teenage years are a time of self-discovery for many kids. Along this journey, the topic of gender identification may arise. For many, this is an unfamiliar topic that leads to many questions. Here is everything you need to know to help your child as they discover who they really are.

The post Assisting Your Child with Gender Confusion appeared first on ChildrensMD.



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Anesthetic management without perioperative platelet transfusion for cervical laminectomy and laminoplasty in a case of May–Hegglin anomaly

Abstract

May–Hegglin anomaly (MHA) is an inherited autosomal dominant disorder characterized by giant platelets and inclusion bodies in granulocytes, and thrombocytopenia. There is no consensus on the perioperative management of this disorder. We report a case involving a patient with MHA who was perioperatively managed without platelet transfusion for cervical laminectomy and laminoplasty. In our case, the platelet count was measured to be 0.6 × 104/µL using an automatic blood cell counter. Peripheral blood smear and genetic test analyses were performed, leading to a definitive diagnosis of MHA. However, clot retraction, serotonin release, and platelet aggregation were normal. Total intravenous anesthesia with propofol and remifentanil, in combination with intermittent injection of fentanyl, was administered. The total blood loss volume was 300 mL, and perioperative course was uneventful. Visual platelet count and platelet function were preserved in this case, although platelet or red blood cell transfusion was not performed. No bleeding tendency was observed during perioperative management.



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Which treatments work for stretch marks?

Stretch marks are common during periods of sudden growth. While they often fade over time, they can be permanent. Some people try to reduce the appearance of these marks at home using creams or lotions, herbal remedies, self-tanner, or hyaluronic acid. Here, we explore the evidence behind popular methods.

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Synergistic Cytokine Effects as Apremilast Response Predictors in Patients With Psoriasis

Publication date: Available online 21 June 2018
Source:Journal of Allergy and Clinical Immunology
Author(s): Sandra Garcet, Kristine Nograles, Joel Correa da Rosa, Peter H. Schafer, James G. Krueger
•Based on nonlinear multivariate algorithms, IL-17F is the most important predictor of PASI improvement with apremilast, and nonlinear synergistic cytokine effects are more predictive of PASI response to apremilast than linear univariate models.



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Antibody-mediated inhibition of FXIIa blocks downstream bradykinin generation

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Publication date: Available online 21 June 2018
Source:Journal of Allergy and Clinical Immunology
Author(s): Helen Cao, Mark Biondo, Hadi Lioe, Samantha Busfield, Veronika Rayzman, Bernhard Nieswandt, Konrad Bork, Leonard C. Harrison, Priscilla Auyeung, Henriette Farkas, Dorottya Csuka, Matthias Pelzing, Steve Dower, Michael J. Wilson, Andrew Nash, Marc W. Nolte, Con Panousis

Teaser

HAE results in swelling due to KKS dysregulation and bradykinin production. FXII initiates the KKS and we have developed a human antibody that potently inhibits FXII protease activity as a novel prophylactic therapy for HAE.


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Disrupted N-linked glycosylation as a disease mechanism in deficiency of ADA2

Publication date: Available online 21 June 2018
Source:Journal of Allergy and Clinical Immunology
Author(s): Pui Y. Lee, Yuelong Huang, Qing Zhou, Oskar Schnappauf, Michael S. Hershfield, Ying Li, Nancy J. Ganson, Natalia Sampaio Moura, Ottavia M. Delmonte, Scellig S. Stone, Michael J. Rivkin, Sung-Yun Pai, Todd Lyons, Robert P. Sundel, Victor W. Hsu, Luigi D. Notarangelo, Ivona Aksentijevich, Peter A. Nigrovic
Deficiency of adenosine deaminase 2 is characterized by vasculitis, early-onset strokes, immunodeficiency, and bone marrow failure. We describe a novel pathogenic mutation affecting a consensus N-linked glycosylation sequence and illustrate the essential role of glycosylation in the biology of ADA2.



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Vitamin D Supplementation Attenuates Asthma Development Following Traffic-Related Particulate Matter Exposure

Publication date: Available online 21 June 2018
Source:Journal of Allergy and Clinical Immunology
Author(s): Paige Bolcas, Eric Brandt, Zhonghua Zhang, Jocelyn Biagini Myers, Brandy Ruff, Gurjit K. Khurana Hershey
BackgroundRecent literature suggests that children who are vitamin D deficient are uniquely susceptible to the effects of traffic-related air pollution (TRAP) exposure. This is highly significant because large segments of the population reside in zones of high TRAP exposure.ObjectiveTo determine whether vitamin D supplementation mitigates the impact of TRAP exposure on asthma development, asthma exacerbation, and/or airway inflammation, and to determine the timing of vitamin D supplementation that confers maximal health benefit.MethodsUsing established mouse models of asthma, we examined the impact of pre-and post-natal vitamin D supplementation on asthma development as well as the utility of vitamin D as a treatment for established asthma in the context of diesel-exhaust particle (DEP) exposure.ResultsDEP and allergen co-exposure resulted in increased airway hyperresponsiveness (AHR) and accumulation of pathogenic Th2/Th17 cells in the lungs of vitamin D deficient mice compared to control mice. Prenatal and postnatal vitamin D supplementation significantly attenuated the development of AHR, and decreased pulmonary accumulation of Th2/Th17 cells following co-exposure to TRAP and allergen, but not allergen alone. Restoration of normal vitamin D status had no impact on AHR once asthma was already established.ConclusionsOur data establish that vitamin D confers protection against asthma development specifically in the context of TRAP exposure. While vitamin D replacement did not reverse established asthma, restoration of normal vitamin D status in early life significantly attenuated the development of AHR in DEP-exacerbated allergic asthma and reduced lung Th2/Th17 cells, which portend the development of severe asthma.Clinical ImplicationsEarly vitamin D supplementation may be an effective preventive strategy for the development of TRAP-induced asthma.

Graphical abstract

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Teaser

The authors show that restoration of normal vitamin D status in early life attenuates the development of allergic asthma in mice exposed to traffic-related diesel exhaust particles (DEPs).


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Questioning the accuracy of currently available pneumococcal antibody testing

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Publication date: Available online 21 June 2018
Source:Journal of Allergy and Clinical Immunology
Author(s): Joud Hajjar, Aba Al-Kaabi, Carleigh Kutac, James Dunn, William T. Shearer, Jordan S. Orange

Teaser

Clinically significant inter-laboratory differences in Streptococcus pneumoniae antibodies were identified between commercial laboratories. These differences could affect diagnoses and management when assessing humoral deficiencies; thus, commercial laboratory methods should be standardized to obtain similar results.


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Eosinophil degranulation products drive a pro-inflammatory fibroblast phenotype

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Publication date: Available online 21 June 2018
Source:Journal of Allergy and Clinical Immunology
Author(s): Ksenija Bernau, Jonathan P. Leet, Stephane Esnault, Andrea L. Noll, Michael D. Evans, Nizar N. Jarjour, Nathan Sandbo

Teaser

In this study, we found that eosinophil-derived degranulation products stimulate bronchial fibroblasts to release chemotactic factors that promote neutrophil migration. This finding suggests that eosinophil-fibroblast interactions may promote airway neutrophilia in severe asthma.


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Retraction Note to: Monitoring of vecuronium-induced neuromuscular block at the sternocleidomastoid muscle in anesthetized patients

The Editor-in-Chief has retracted this article [1] because the three studies included in the meta-analysis [2,3 and 4] (cited as references 16, 17 and 18) have been retracted due to concerns regarding the data, which has rendered the results of this meta-analysis invalid.



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‘Best Ideas’ to Come to Baltimore for Fifth Annual Metastatic Breast Cancer Conference

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The fifth annual Metastatic Breast Cancer Conference invites participants from around the world to discuss ongoing research, showcase new ideas from young scientists, and bring together advocates who are working with metastatic breast cancer patients. This year, the conference will be hosted by the Johns Hopkins Kimmel Cancer Center and the Johns Hopkins School of Medicine in Baltimore on Nov. 15 and 16.

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Immediate versus delayed reconstruction of head and neck cutaneous melanoma

The Laryngoscope, EarlyView.


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Diagnosing nontuberculous mycobacterial cervicofacial lymphadenitis in children: A systematic review

Publication date: September 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 112
Author(s): S.H. Willemse, M.A.E.M. Oomens, J. De Lange, L.H.E. Karssemakers
ObjectivesWidespread controversy exists regarding correct diagnosing nontuberculous mycobacterial cervicofacial (NTM) lymphadenitis. This study intends to gather the available evidence with respect to diagnosing NTM cervicofacial lymphadenitis.MethodsA review protocol was developed based on the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA)-statement (https://ift.tt/16KM5lR). A comprehensive search was performed in the bibliographic databases PubMed, Embase.com and Wiley/Cochrane Library. 10 Articles fulfilled the inclusion criteria and were included in the review. Assessing risk of bias of the articles was done using the revised Quality Assessment of Diagnostic Accuracy (QUADAS-2) tool.ResultsThis systematic review shows that diagnostic studies of high methodological quality are scarce. Diagnostic accuracy of polymerase chain reaction (PCR), culture, skin testing, auramine staining, Ziehl-Neelsen staining, and immunodiagnostic assays was studied. Culture sensitivity proved to be 41,8%, while polymerase chain reaction has a sensitivity of 71,6%. Both methods showed a specificity of 100%. Sensitivity of Immunodiagnostic assays ranged between 87,5% and 100% and specificity between 81% and 100%. Overall sensitivity of skin tests containing purified protein derivative (PPD-S) was 70% (95% CI [62%–78%]) with an overall specificity of 94% (95% CI [88%–100%]).ConclusionsIn patients with a high clinical suspicion for NTM cervicofacial lymphadenitis, a positive PPD-S skin is indicative for the diagnosis of NTM cervicofacial lymphadenitis. Either PCR or culture is necessary to confirm the diagnosis. Interferon-γ release assays with purified protein derivative stimulation appear to provide good sensitivity and specificity as a non-invasive pre-operative test, but the evidence is weak. More studies of high methodological quality are needed to validate the results of this systematic review.



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Spontaneous hair regrowth in eight patients with severe alopecia areata

Australasian Journal of Dermatology, EarlyView.


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Transoral Robotic Surgery: Image Guidance and Augmented Reality

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Transoral robotic surgery (TORS) is being used increasingly as an alternative to conventional open procedures, as its minimally invasive approach results in significant benefits for the patient. This includes faster recovery times and better functional outcomes, less postoperative pain and morbidity associated with open access, shorter operative times, and a reduction in the risk of infection. However, the confined working spaces and sensory deficit, due to a lack of haptic sensation, and proximity to critical vasculature and other vitally important anatomical structures bestow significant challenges on the surgeon. Recent advances in the field of surgical image guidance show promise in helping address these issues. This overview considers a number of important recent developments, all of which exploit imaging technologies that, to a lesser or greater extent, are integrated with the surgical robot platform. In its most advanced form, this combination of technologies results in a fusion of visual information such that the operative field is combined with navigational cues and representations of key anatomical structures through augmented reality registration and overlay. In terms of registration accuracy, image guidance as applied to TORS procedures is an exciting proposition, since the regions of interest (e.g., the tongue base, oropharynx) are typically adjacent to and enclosed by rigid anatomy. Clinical indications and overall effectiveness are discussed together with the limitations of the current technologies. Where appropriate, directions for future work are identified.
ORL

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Transoral Robotic Surgery for Obstructive Sleep Apnoea-Hypopnoea Syndrome

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Obstructive sleep apnoea-hypopnoea (OSAH) syndrome constitutes a major health care problem. Surgical modalities for the treatment of OSAH are regaining momentum in view of the increasing prevalence of OSAH and the low compliance rates associated with continuous positive airway pressure. There are several investigations to complement clinical examination in accurately determining the level of airway collapse to ensure correct patient selection and a targeted surgical approach. The most commonly employed include drug-induced sleep endoscopy and imaging with the tongue base and epiglottis often revealed as the major sites of airway narrowing during sleep. In the continuing search for the optimal approach to address these areas, transoral robotic surgery (TORS) has been successfully used for tongue base reduction and epiglottoplasty. With sufficient experience, this technique is safe and well tolerated. Meticulous work-up and careful patient selection are crucial. Multiple studies have demonstrated very good short-term results of TORS for OSAH, with significant reduction in both the Apnoea-Hypopnea Index (AHI) and Epworth Sleepiness Score (ESS). With the appropriate infrastructure, proctoring, and access to robotic surgical technology, it is possible for these results to be reproduced more widely. Further prospective long-term clinical evaluation will ultimately determine the exact role of TORS in the treatment of OSAH.
ORL

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High‐risk human papillomavirus in a child with digital pigmented Bowen's disease: Case report and dermoscopic findings

Pediatric Dermatology, EarlyView.


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Proportionate growth of congenital nevi: Blow a balloon to explain

Pediatric Dermatology, EarlyView.


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Involvement of CD8+ T cell subsets in early response to vascular injury in patients with peripheral arterial disease in vivo

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Publication date: Available online 21 June 2018
Source:Clinical Immunology
Author(s): Pawel Maga, Tomasz P. Mikolajczyk, Lukasz Partyka, Mateusz Siedlinski, Mikolaj Maga, Marek Krzanowski, Krzysztof Malinowski, Kevin Luc, Rafal Nizankowski, Deepak L. Bhatt, Tomasz J. Guzik
AimsAdaptive immunity is critical in vascular remodelling following arterial injury. We hypothesized that acute changes in T cells at a percutaneous transluminal angioplasty (PTA) site could serve as an index of their potential interaction with the injured vascular wall.Methods and ResultsT cell subsets were characterised in 45 patients with Rutherford 3–4 peripheral arterial disease (PAD) undergoing PTA. Direct angioplasty catheter blood sampling was performed before and immediately after the procedure. PTA was associated with an acute reduction of α/β-TcR CD8+ T cells. Further characterisation revealed significant reduction in pro-atherosclerotic CD28nullCD57+ cells, effector and effector memory cells, in addition to cells bearing activation and tissue homing/adhesion markers.ConclusionsThe acute reduction observed here is likely due to the adhesion of cells to the injured vascular wall, suggesting that immunosenescent, activated effector CD8+ cells have a role in the early vascular injury immune response following PTA in PAD patients.



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