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https://www.readbyqxmd.com/read/28302646/eosinophil-derived-il-4-drives-progression-of-myocarditis-to-inflammatory-dilated-cardiomyopathy
#1
Nicola L Diny, G Christian Baldeviano, Monica V Talor, Jobert G Barin, SuFey Ong, Djahida Bedja, Allison G Hays, Nisha A Gilotra, Isabelle Coppens, Noel R Rose, Daniela Čiháková
Inflammatory dilated cardiomyopathy (DCMi) is a major cause of heart failure in children and young adults. DCMi develops in up to 30% of myocarditis patients, but the mechanisms involved in disease progression are poorly understood. Patients with eosinophilia frequently develop cardiomyopathies. In this study, we used the experimental autoimmune myocarditis (EAM) model to determine the role of eosinophils in myocarditis and DCMi. Eosinophils were dispensable for myocarditis induction but were required for progression to DCMi...
March 16, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28290180/non-bacterial-thrombotic-endocarditis-of-aortic-valve-due-to-hypereosinophilic-syndrome
#2
Harveen Lamba, Salil Deo, Salah Altarabsheh, Yakov Elgudin, Allen Markowitz, Soon Park
Hypereosinophilic syndrome (HES) is a rare hematological disorder, which may present with cardiac involvement. The case is presented of a 61-year-old male patient with isolated aortic stenosis secondary to non-bacterial thrombotic endocarditis and HES. The patient underwent successful aortic valve replacement with a bioprosthesis and remained recurrence-free at the 18-month follow up. A review of the current literature is also presented and cardiac manifestations, clinical presentation and surgical issues in the care of patients with this rare condition are discussed...
November 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28290178/multiple-valvular-complications-of-hypereosinophilic-syndrome
#3
Zoltán Pozsonyi, Szabolcs Benedek, Pál Sármán, Lívia Jánoskuti, Tivadar Hüttl, Astrid Apor
Endomyocardial fibrosis (EMF) is the most common cardiac abnormality in hyeperosinophilic syndrome (HES), sometimes complicated with mitral valve disease. Mitral valve disease without ventricular manifestation is very rare, however. Case reports link HES to prosthetic valve thrombosis (PVT), but the optimal type of prosthetic valve in HES is not known. Herein is reported the case of a young female HES patient with secondary mitral valve degeneration and severe regurgitation. A mechanical prosthetic valve was implanted six months after she was diagnosed with HES, but despite anticoagulation and antiplatelet therapy she developed PVT three months later...
November 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28284183/multiparametric-cardiac-magnetic-resonance-imaging-cmr-for-the-diagnosis-of-loeffler-s-endocarditis-a-case-report
#4
Mareike Gastl, Patrick Behm, Christoph Jacoby, Malte Kelm, Florian Bönner
BACKGROUND: Endocarditis parietalis fibroplastica Löfflein (EPF) is a rare form of primary restrictive cardiomyopathy with poor prognosis. It is generally caused by hypereosinophilic syndrome with eosinophilic penetration of the heart. This leads to congestive heart failure in three different stages. As a frequent manifestation of neoplastic diseases, cardiac involvement means poor prognosis. CASE PRESENTATION: The present report deals with a case of EPF caused by non-specified T-cell lymphoma (T-NOS)...
March 11, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28263223/hypereosinophilic-syndrome-after-liver-transplantation-a-case-report-and-a-review-of-the-literature
#5
Wiebke Aulbert, Robin Kobbe, Christian Breuer, Andrea Briem-Richter, Hansjörg Schäfer, Florian Brinkert, Anne Dettmar, Markus J Kemper, Enke Grabhorn
Persistently elevated eosinophil granulocytes in the peripheral blood in children is challenging due to a complex diagnosis especially after solid organ transplantation and can lead to difficulties in finding an underlying causative factor.We report a 12-year-old boy who developed severe hypereosinophilia eleven years after liver transplantation (LT) due to biliary atresia. Accompanying symptoms were recurrent fever, fatigue, elevated liver enzymes, abdominal pain and significant weight loss. After exclusion of secondary causes of eosinophilia, an idiopathic hypereosinophilic syndrome (I-HES) was diagnosed...
March 4, 2017: Transplantation
https://www.readbyqxmd.com/read/28219563/repeated-prosthetic-mitral-valve-thrombosis-in-fluke%C3%A2-infection-induced-hypereosinophilic-syndrome
#6
Yiran Zhang, Liang Ma
A 43-year-old female patient with mitral valve stenosis and hypereosinophilia on admission received mechanical mitral valve replacement. During the 2 years after the first surgery, the patient underwent two mitral valve revision operations for repeated prosthetic mitral valve thrombosis. During the 2-year period, the patient's absolute eosinophil count was found elevated repeatedly. Finally, fluke antibody detection revealed fluke infection and, after praziquantel treatment, a normal level of absolute eosinophil count has been maintained and recurrent thrombosis has not occurred for 18 months...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28178599/the-targeted-eosinophil-lowering-effects-of-dexpramipexole-in-clinical-studies
#7
Steven I Dworetzky, Gregory T Hebrank, Donald G Archibald, Ian J Reynolds, Wildon Farwell, Michael E Bozik
Dexpramipexole, an orally bioavailable small molecule previously under clinical development in amyotrophic lateral sclerosis, was observed during routine safety hematology monitoring to demonstrate pronounced, dose- and time-dependent eosinophil-lowering effects, with minor reductions on other leukocyte counts. Analysis of hematology lab values across two double-blind, randomized placebo-controlled clinical trials at total daily doses ranging from 50mg to 300mg demonstrated that dexpramipexole consistently and markedly lowered peripheral blood eosinophils...
January 16, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28174010/a-case-of-t-cell-lymphoproliferative-disorder-associated-with-hypereosinophilia-with-excellent-response-to-mycophenolate-mofetil
#8
Amr Hanbali, Marwan Shaheen, Feras Alfraih, Wafa Al-Otaibi, Riad El Fakih, Tarek Owaidah, Syed Ahmed
Hypereosinophilic syndrome (HES) is a group of rare blood disorders characterized by a persistent elevation of blood eosinophil count ⩾1.5×10(9)/L and clinical manifestations attributable to eosinophilia or tissue hypereosinophilia. Lymphocytic variant of HES (HES-L) is a known subtype according to World Health Organization classification. It is well documented in the literature that patients with HES-L are predisposed to develop T-cell lymphoma. We report a case of T-cell lymphoproliferation associated with hypereosinophilia, which has been successfully treated with mycophenolate mofetil, with resolution of skin lesions and normalization of eosinophil count and immunoglobulin E level...
February 1, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28168065/rapid-change-in-mental-status-in-a-patient-with-hypereosinophilia
#9
Hanyin Wang, John K Erban
We present the case of a 48-year-old female with acute onset altered mental status, who was found to have eosinophilia, elevated troponin, and embolic strokes. Extensive testing for autoimmune, infectious, and coronary artery etiologies was unremarkable. After a cardiac MRI revealed focal myocardial hyperenhancement, the patient underwent an endomyocardial biopsy with findings consistent with eosinophilic myocarditis. The patient was diagnosed of idiopathic hypereosinophilic syndrome and started on prednisone and apixaban...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28111307/jak1-gain-of-function-causes-an-autosomal-dominant-immune-dysregulatory-and-hypereosinophilic-syndrome
#10
Kate L Del Bel, Robert J Ragotte, Aabida Saferali, Susan Lee, Suzanne M Vercauteren, Sara A Mostafavi, Richard A Schreiber, Julie S Prendiville, Min S Phang, Jessica Halparin, Nicholas Au, John M Dean, John J Priatel, Emily Jewels, Anne K Junker, Paul C Rogers, Michael Seear, Margaret L McKinnon, Stuart E Turvey
No abstract text is available yet for this article.
January 19, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28102002/pseudoherpetic-transient-acantholytic-dermatosis-grover-disease-case-series-and-review-of-the-literature
#11
Kiran Motaparthi
Three cases of pseudoherpetic transient acantholytic dermatosis (Grover disease) are presented, followed by a brief review of prior reports. All three patients were above the age of 60 and presented with a pruritic eruption composed of papules with or without vesicles distributed on the trunk. For all three patients, the clinical differential diagnosis included drug eruption but did not include Grover disease; in one patient, the clinical impression included herpesvirus infection. Similar histologic and immunohistochemical findings were demonstrated in all three cases...
January 18, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28094869/loss-of-fas-fasl-signaling-does-not-reduce-apoptosis-in-sharpin-null-mice
#12
Christopher S Potter, Kathleen A Silva, Victoria E Kennedy, Timothy M Stearns, Harm HogenEsch, John P Sundberg
Mice with mutations in SHANK-associated RH domain interactor (Sharpin) develop a hypereosinophilic auto-inflammatory disease known as chronic proliferative dermatitis. Affected mice have increased apoptosis in the keratinocytes of the skin, esophagus, and forestomach driven by extrinsic TNF receptor mediated apoptotic signaling pathways. FAS receptor signaling is an extrinsic apoptotic signaling mechanism frequently involved in inflammatory skin diseases. Compound mutations in Sharpin and Fas or Fasl were created to determine if these death domain proteins influenced the cutaneous phenotype in Sharpin null mice...
January 17, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28094164/disorders-characterized-by-predominant-or-exclusive-dermal-inflammation
#13
Mark R Wick
Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions. Included among the lymphoid lesions are viral exanthems, pigmented purpuras, gyrate erythemas, polymorphous light eruption, lupus tumidus, and cutaneous lymphoid hyperplasia. Neutrophilic infiltrates are represented by infections, Sweet syndrome, pyoderma gangrenosum, and hidradenitis suppurativa, as well as a group of so-called "autoinflammatory" dermatitides comprising polymorphonuclear leukocytes...
December 14, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28070732/the-clinical-and-laboratory-spectrum-of-dedicator-of-cytokinesis-8-immunodeficiency-syndrome-in-patients-with-a-unique-mutation
#14
Arnon Broides, Amarilla B Mandola, Jacov Levy, Baruch Yerushalmi, Vered Pinsk, Michal Eldan, George Shubinsky, Nurit Hadad, Rachel Levy, Amit Nahum, Miriam Ben-Harosh, Atar Lev, Amos Simon, Raz Somech
Mutations in the dedicator of cytokinesis 8 (DOCK8) gene cause a combined immunodeficiency usually diagnosed as autosomal recessive hyper IgE syndrome. We sought to reveal the varying manifestations in patients with a unique mutation in DOCK8 gene by a retrospective medical record review. Ten patients from five consanguineous families and three tribes were included. Seven patients were homozygous for the c.C5134A, p.S1711X mutation, and the remaining three patients were their siblings manifesting hyper IgE syndrome features without a genetic diagnosis...
January 10, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28065613/anca-negative-churg-strauss-syndrome-presenting-as-acute-multiple-cerebral-infarcts-a-case-report
#15
Klearchos Psychogios, Ilias Evmorfiadis, Spyros Dragomanovits, Athanasios Stavridis, Konstantinos Takis, Loukas Kaklamanis, Stathis Pantelis
Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Elevated cardiac enzymes and cardiac magnetic resonance imaging were consistent with endomyocarditis. The simultaneous presence of history of asthma, sinusitis, hypereosinophilia, and vasculitis led to the diagnosis of EGPA...
January 5, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28052805/hypereosinophilic-syndrome
#16
REVIEW
Heung R Noh, Gil G Magpantay
BACKGROUND: Hypereosinophilic syndrome (HES) is a myeloproliferative disorder characterized by persistent eosinophilia associated with multiple organ damage. OBJECTIVE: To increase awareness of the rare but potentially life-threatening disease and to provide a brief overview of the clinical presentation, diagnosis, and management of HES. METHODS: Pertinent data from the patient's clinical course was incorporated with salient articles on the topic...
January 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28005278/igg4-related-disease-and-lymphocyte-variant-hypereosinophilic-syndrome-a-comparative-case-series
#17
Mollie N Carruthers, Sujin Park, Graham W Slack, Bakul I Dalal, Brian F Skinnider, David F Schaeffer, Jan P Dutz, Joanna K Law, Fergal Donnellan, Vladimir Marquez, Michael Seidman, Patrick C Wong, Andre Mattman, Luke Y C Chen
OBJECTIVE: To compare the clinical and laboratory features of IgG4-related disease (IgG4-RD) and lymphocyte-variant hypereosinophilic syndrome (L-HES), two rare diseases that often present with lymphadenopathy, gastrointestinal symptoms, eosinophilia, and elevated immunoglobulins/IgE. METHOD: Comparative case series of 31 patients with IgG4-RD and 13 patients with L-HES. RESULTS: Peripheral blood eosinophilia was present in eight of 31 patients with IgG4-RD compared to 13 of 13 patients with L-HES (median eosinophils 0...
December 22, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27911676/tolerance-and-efficacy-with-simultaneous-use-of-two-monoclonal-antibodies-for-a-patient-with-hypereosinophilic-syndrome-and-ulcerative-colitis
#18
Bhavisha Patel, Joseph H Butterfield, Catherine R Weiler, Sunanda V Kane
The development and utilization of monoclonal antibodies (mAbs) have been of great interest in all fields of medicine. A substantial increase in the production and development of mAbs has occurred because these biologic agents are proving to be effective and less toxic given their targeted mechanism of action. However, data are limited on coadministration of two or more mAbs. With the increasing availability of mAbs and the comorbidities of some patients, assessment is needed of the ability to safely use multiple mAbs for an individual patient...
December 2016: Monoclonal Antibodies in Immunodiagnosis and Immunotherapy
https://www.readbyqxmd.com/read/27896330/biventricular-thrombus-in-hypereosinophilic-syndrome-presenting-with-shortness-of-breath
#19
Abdul Baqi, Shahan Waheed, Fateh Ali Tipoo, Aamir Hameed Khan
A 48 years old male presented to clinic with 12 months of low grade fever with shortness of breath which has progressively worsened with no associated weight loss, night sweats or loss of appetite. There was no prior history of chronic illness before the current illness. Laboratory workup revealed a high white blood cell count with predominant eosinophils. Chest X-ray was normal. Transthoracic echocardiography and Cardiac Magnetic Resonance showed biventricular thrombi. On further extensive workup the findings were consistent with hypereosinophilic syndrome...
June 2016: Turkish Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27887955/treatment-of-hypereosinophilic-syndrome-with-cutaneous-involvement-with-the-jak-inhibitors-tofacitinib-and-ruxolitinib
#20
Brett King, Alfred Ian Lee, Jaehyuk Choi
No abstract text is available yet for this article.
November 22, 2016: Journal of Investigative Dermatology
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