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Eosinophilic syndromes

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https://www.readbyqxmd.com/read/30317003/c-reactive-protein-as-a-diagnostic-tool-in-differential-diagnosis-of-hypereosinophilic-syndrome-and-anca-negative-eosinophilic-granulomatosis-with-polyangiitis
#1
Amélie Leurs, Cécile Chenivesse, Benjamin Lopez, Jean-Baptiste Gibier, Guillaume Clément, Matthieu Groh, Marie-Christine Copin, Delphine Staumont, Geoffrey Mortuaire, Marie-Hélène Balquet, Frédéric Dezoteux, Nathalie Bautin, Anne-Laure Buchdahl, Noémie Le Gouellec, Nicolas Etienne, Louis Terriou, Sylvain Dubucquoi, Myriam Labalette, Sandrine Morell-Dubois, Hélène Maillard-Lefebvre, Marc Lambert, Eric Hachulla, David Launay, Jean-Emmanuel Kahn, Pierre-Yves Hatron, Guillaume Lefèvre
No abstract text is available yet for this article.
October 11, 2018: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/30315710/eosinophilia-and-reduced-stat3-signalling-affect-neutrophil-cell-death-in-autosomal-dominant-hyper-ige-syndrome
#2
Susan Farmand, Bernhard Kremer, Monika Häffner, Katrin Pütsep, Peter Bergman, Mikael Sundin, Henrike Ritterbusch, Maximilian Seidl, Marie Follo, Philipp Henneke, Birgitta Henriques-Normark
The autosomal-dominant hyper-IgE syndrome (HIES), caused by mutations in signal transducer and activator of transcription 3 (STAT3), is a rare primary immunodeficiency which predisposes to mucocutaneous candidiasis and staphylococcal skin and lung infections. This infection phenotype is suggestive of defects in neutrophils, but data on neutrophil functions in HIES are inconsistent. This study was undertaken to functionally characterize neutrophils in STAT3-deficient HIES patients and to analyse whether the patients` eosinophilia affects the neutrophil phenotype in S...
October 13, 2018: European Journal of Immunology
https://www.readbyqxmd.com/read/30303819/somatic-mutations-of-tsc2-or-mtor-characterize-a-morphologically-distinct-subset-of-sporadic-renal-cell-carcinoma-with-eosinophilic-and-vacuolated-cytoplasm
#3
Ying-Bei Chen, Leili Mirsadraei, Gowtham Jayakumaran, Hikmat A Al-Ahmadie, Samson W Fine, Anuradha Gopalan, S Joseph Sirintrapun, Satish K Tickoo, Victor E Reuter
The differential diagnosis of renal cell neoplasms with solid or nested architecture and eosinophilic cytoplasm has become increasingly complex. Despite recent advances in classifying a number of entities exhibiting this morphology, some tumors remain in the unclassified category. Here we describe a morphologically distinct group of sporadic renal cell carcinoma (RCC) with predominantly nested architecture, eosinophilic, and remarkably vacuolated cytoplasm retrospectively identified from a cohort of previously unclassified tumors...
October 9, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/30301732/dress-syndrome-associated-acute-necrotizing-eosinophilic-myocarditis-with-giant-cells
#4
Robert Kowtoniuk, Mamatha Pinninti, William Tyler, Sanjay Doddamani
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is an uncommon drug hypersensitivity reaction caused by a wide variety of agents. It has a characteristic latent period between 2 and 8 weeks from the onset of drug ingestion followed by a slow resolution with the potential for relapse. Despite being a potentially fatal disease, little is understood about its variable clinical presentation and why it can present long after removal of the offending drug. Visceral organ involvement typically occurs, but rarely results in clinically manifested cardiac injury...
October 8, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/30300850/atypical-anti-mog-syndrome-with-aseptic-meningoencephalitis-and-pseudotumor-cerebri-like-presentations
#5
Ram N Narayan
OBJECTIVE: To describe 2 atypical cases with Anti-MOG antibody related demyelinating syndrome. METHODOLOGY: Case series. RESULTS: We present two cases. Case 1 is an 18-year-old woman who presented with headache, blurred vision, and papilledema and was initially diagnosed with pseudotumor cerebri syndrome. CSF showed mildly elevated opening pressure and lymphocytic pleocytosis and a diagnosis of aseptic meningitis was considered. MRI brain and spinal cord revealed longitudinally extensive bilateral simultaneous optic neuritis and multiple spinal cord lesions...
October 3, 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/30283713/allergic-diseases-and-immune-mediated-food-disorders-in-pediatric-acute-onset-neuropsychiatric-syndrome
#6
Jaime S Rosa, Joseph D Hernandez, Janell A Sherr, Bridget M Smith, Kayla D Brown, Bahare Farhadian, Talia Mahony, Sean A McGhee, David B Lewis, Margo Thienemann, Jennifer D Frankovich
Background: The prevalence and impact of allergic and immune-mediated food disorders in pediatric acute-onset neuropsychiatric syndrome (PANS) are mostly unknown. Objective: We sought to explore the prevalence of atopic dermatitis (AD), asthma, allergic rhinitis (AR), IgE-mediated food allergies (FAs), and other immune-mediated food disorders requiring food avoidance in patients with PANS. In addition, to further understand the extent of food restriction in this population, we investigated the empiric use of dietary measures to improve PANS symptoms...
September 1, 2018: Pediatric Allergy, Immunology, and Pulmonology
https://www.readbyqxmd.com/read/30281364/succinate-dehydrogenase-deficient-renal-cell-carcinoma-a-short-review
#7
Gang Wang, Priya Rao
Succinate dehydrogenase (SDH) is a mitochondrial enzyme complex composed of 4 protein subunits (SDHA, SDHB, SDHC, and SDHD). Germ line mutations of the genes encoding these SDH subunits result in hereditary syndromes harboring pheochromocytomas/paragangliomas, gastrointestinal stromal tumors, renal cell carcinomas, and pituitary adenomas. SDH-deficient renal cell carcinomas are rare, with a mean age of 38 to 40 years. Histologically, these tumors show a characteristic appearance that includes a solid, nested, or tubular architecture with variable cysts...
October 2018: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/30280106/spontaneous-intramural-esophageal-rupture-an-uncommon-presentation-of-eosinophilic-esophagitis-requiring-endoscopic-clipping
#8
Tooba Tariq, Asad Rizvi, Karun Badwal, Ransome Eke
Spontaneous intramural esophageal rupture (SIER) is a form of acute esophageal trauma defined as an injury deeper than a Mallory-Weiss tear but not extending completely through the muscular propria as in Boerhaave syndrome. SIER is a rare complication of eosinophilic esophagitis (EoE); after extensive literature review, we found 7 case reports of SIER complicating EoE. We present a case of SIER complicating EoE in a 46-year-old man with an atypical presentation requiring endoscopic clipping to achieve successful hemostasis...
2018: ACG Case Reports Journal
https://www.readbyqxmd.com/read/30277205/case-report-space-occupying-lesion-in-the-liver-related-to-preprandial-hunger-and-hepatic-visceral-larva-migrans
#9
Kye-Yeung Park, Hoon-Ki Park, Hwan-Sik Hwang, Jae-Sook Ryu, Kyeong-Geun Lee, Ki-Seok Jang
Visceral larva migrans (VLM) is one of the clinical syndromes of human toxocariasis. We report a case of hepatic VLM presenting preprandial malaise and epigastric discomfort in a 58-year-old woman drinking raw roe deer blood. The imaging studies of the abdomen showed a 74-mm hepatic mass featuring hepatic VLM. Anti- Toxocara canis IgG was observed in enzyme-linked immunosorbent assay (ELISA) and western blot. Despite anthelmintic treatment, the patient complained of newly developed cough and skin rash with severe eosinophilia...
October 1, 2018: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/30257810/esophageal-dysmotility-an-intrinsic-feature-of-megacystis-microcolon-hypoperistalsis-syndrome-mmihs
#10
Samuel A Kocoshis, Monique L Goldschmidt, Jaimie D Nathan, Khalil I El-Chammas, Alexander J Bondoc, Gregory M Tiao, Maria H Alonso, Agozie C Ubesie, Conrad R Cole, Ajay Kaul
OBJECTIVES: Megacystis-microcolon-hypoperistalsis syndrome (MMIHS) also called Berdon's Syndrome, is a smooth muscle myopathy that results in an enlarged bladder, microcolon, and small bowel hypoperistalsis. In our series of six patients with this disorder, all had disordered swallowing. Therefore, we prospectively characterized esophageal structure and function in all. METHODS: Diagnoses had been established by contrast radiography, small bowel manometry, and urodynamic studies...
September 7, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/30250322/-histiocitosis-de-c%C3%A3-lulas-de-langerhans-con-compromiso-vertebral
#11
Manuel Giraldo-Grueso, María C Villegas, David L Rodríguez, Edison Rodríguez
Introducción: La histiocitosis de células de Langerhans (HCL) es un trastorno histiocítico raro y su incidencia exacta se mantiene desconocida; se ha diagnosticado en todos los grupos de edad, pero es más común en los primeros 3 años de vida. Se caracteriza por lesiones únicas o múltiples de tipo osteolítico causadas por proliferación clonal de células histológicamente similares a las células de Langerhans; su presentación clínica es heterogénea. Caso clínico: Presentamos el caso de una paciente de sexo femenino de 7 años, con dificultad para la marcha y debilidad progresiva en los miembros inferiores de 5 días de evolución...
2018: Boletín Médico del Hospital Infantil de México
https://www.readbyqxmd.com/read/30249857/a-rare-case-of-hypereosinophilic-syndrome-presenting-with-unilateral-proptosis-and-torticollis
#12
Jayanta K Das, Krati Gupta, Saurabh Deshmukh, Richa Shrivastava
Hypereosinophilic syndrome (HES) is a spectrum of myeloproliferative disorder, which is characterized by persistent and marked blood eosinophilia and damage to multiple organs due to eosinophilic infiltration. Idiopathic HES is identified after ruling out all other causes of eosinophilia. Poor prognosis is usually associated with cardiac involvement and malignant transformation of blood cells. We report a rare case of HES in an 8-year-old boy who presented with unilateral proptosis and torticollis. The patient responded well to corticosteroid therapy with reduction of proptosis and torticollis and normalization of serum eosinophil count...
October 2018: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/30237972/acute-pulmonary-embolism-and-deep-vein-thrombosis-secondary-to-idiopathic-hypereosinophilic-syndrome
#13
Dezhi Li, Li Xu, Dianjie Lin, Shujuan Jiang, Saran Feng, Ling Zhu
Acute pulmonary embolism (PE) is a most dangerous complication that needs prompt treatment to reduce potentially death. There are many well-known prognostic factors indicate the morbidity and mortality in various thromboembolic events. Persistent eosinophilia in peripheral blood can lead to tissue infiltration and even organ damage, but the urgent event of thromboembolism in pulmonary provoked by eosinophil eosinophilia in idiopathic hypereosinophilic syndrome (HES) is relative an unusual presentation. In this paper, we present two cases of patients with multiple PE and deep vein thrombosis secondary to the idiopathic HES...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/30223114/an-in-depth-characterization-of-a-large-cohort-of-adults-patients-with-eosinophilic-esophagitis
#14
Lyvia Y Leigh, Jonathan M Spergel
BACKGROUND: Eosinophilic esophagitis (EoE) is a chronic allergic, immune-mediated disease associated with increased risk of comorbid atopic conditions. OBJECTIVE: We aim to perform an in-depth characterization of a large cohort of manually verified adult EoE patients including evaluation of less studied associations such as pollen food allergy syndrome, anaphylaxis, autoimmunity and psychiatric comorbidities. METHODS: We performed manual retrospective electronic medical record review of 1,218 patients with EoE identified by ICD-9 and 10 codes from the University of Pennsylvania Health Systems (UPHS)...
September 14, 2018: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/30216468/diagnosis-and-management-of-nsaid-exacerbated-respiratory-disease-n-erd-a-eaaci-position-paper
#15
Marek L Kowalski, Ioana Agache, Sevim Bavbek, Arzu Bakirtas, Miguel Blanca, Grażyna Bochenek, Matteo Bonini, Enrico Heffler, Ludger Klimek, Tanya M Laidlaw, Joaquim Mullol, Ewa Niżankowska-Mogilnicka, Hae-Sim Park, Marek Sanak, Mario Sanchez-Borges, Silvia Sanchez-Garcia, Glenis Scadding, Masami Taniguchi, Maria J Torres, Andrew A White, Aleksandra Wardzyńska
NSAID-exacerbated respiratory disease (N-ERD) is a chronic eosinophilic, inflammatory disorder of the respiratory tract occurring in patients with asthma and/or chronic rhinosinusitis with nasal polyps (CRSwNP), symptoms of which are exacerbated by NSAIDs, including aspirin. Despite some progress in understanding of the pathophysiology of the syndrome, which affects 1/10 of patients with asthma and rhinosinusitis, it remains a diagnostic and therapeutic challenge. In order to provide evidence-based recommendations for the diagnosis and management of N-ERD, a panel of international experts was called by the EAACI Asthma Section...
September 14, 2018: Allergy
https://www.readbyqxmd.com/read/30214482/coronary-artery-dissection-in-the-puerperium-a-case-report-and-literature-review
#16
Farshad Tahmasebi, Alice Hurrell, Amie Ford, Manish Gupta, Damien Geindreau, Dominic Pimenta, Constantinos O'Mahony
Spontaneous coronary artery dissection is a rare but important cause of acute coronary syndrome. Its relevance for women during the puerperium mandates awareness and understanding amongst obstetric healthcare professionals. The aetiology of the increased risk in pregnancy has not been fully elucidated, but include medial eosinophilic angitis, pregnancy-induced degeneration of collagen in conjunction with the stresses of parturition, and rupture of the vasa vasorum. The risk of mortality necessitates prompt diagnosis, usually by angiography...
September 2018: Obstetric Medicine
https://www.readbyqxmd.com/read/30210745/the-spectrum-of-hypereosinophilia-and-associated-clonal-disorders-a-real-world-data-based-on-combined-retrospective-and-prospective-analysis-from-a-tropical-setting
#17
Sreejesh Sreedharanunni, Neelam Varma, Man Updesh Singh Sachdeva, Shano Naseem, Pankaj Malhotra, Deepak Bansal, Amita Trehan, Subhash Varma
Objective: To determine the frequency, etiological spectrum and treatment outcome of hypereosinophilia (HE) and hypereosinophilic syndromes (HES) in a tropical setting. Methods: A retrospective analysis of hospital data of five years (January 2009 to December 2013) and a comprehensive prospective evaluation of patients presenting with HE/HES over a period of 33 months (January 2014 to September 2016) was performed. Results: HE/HES was diagnosed in a total of 125 patients during the study period with an estimated prevalence of 0...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/30197069/eosinophilic-granulomatosis-with-polyangiitis-clinical-pathology-conference-and-review
#18
REVIEW
Eveline Y Wu, Michelle L Hernandez, J Charles Jennette, Ronald J Falk
Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is a small vessel vasculitis associated with asthma and eosinophilia. Despite its rarity, continuous gains are being made in understanding the disease with knowledge advancements regarding its epidemiology, heterogeneous clinical manifestations, management, and outcomes. Large knowledge gaps remain, however, particularly surrounding pathophysiologic and diagnostic uncertainties. There is still an incomplete understanding of the interplay between the eosinophilic and vasculitic processes that are features of disease pathogenesis...
September 2018: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/30179138/churg-strauss-syndrome-presenting-as-acute-necrotizing-eosinophilic-myocarditis-concise-review-of-the-literature
#19
Muhammad Ali Chaudhry, Luanda Grazette, Andrew Yoon, Adrian Correa, Michael W Fong
Acute eosinophilic myocarditis (EM) is a rare form of heart failure that is characterized by myocardial eosinophilic infiltration usually in association with peripheral eosinophilia. The underlying cause is variable and can include allergic reactions, parasitic infection, idiopathic hypereosinophilic syndrome, malignancy, Loeffler's syndrome, Churg-Strauss syndrome (CSS), early giant cell myocarditis and malignancy. The course is potentially fatal, and early diagnosis and treatment with steroids is essential...
September 3, 2018: Current Hypertension Reviews
https://www.readbyqxmd.com/read/30176295/drug-reaction-with-eosinophilia-and-systemic-symptoms-dress-syndrome-identified-in-the-electronic-health-record-allergy-module
#20
Anna R Wolfson, Li Zhou, Yu Li, Neelam A Phadke, Ohn A Chow, Kimberly G Blumenthal
BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but severe hypersensitivity reaction that remains poorly characterized in the United States. OBJECTIVE: To identify and describe DRESS syndrome cases in an integrated health care system using electronic health record (EHR) allergy module free-text searches. METHODS: We identified DRESS syndrome cases with rash, absolute eosinophil count of 500/L or more, organ involvement, and a European Registry of Severe Cutaneous Adverse Reactions to Drugs and Collection of Biological Samples score of 2 or more by reviewing those patients from 1980 to 2016 whose EHR allergic reaction matched DRESS-related key words...
August 31, 2018: Journal of Allergy and Clinical Immunology in Practice
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