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Eosinophilic syndromes

David J Hermel, Ali Nael, Yi-Tsung Lu, Jackson Kim, Russell K Brynes, Maria Vergara-Lluri, Mojtaba Akhtari
Gelatinous bone marrow transformation (GBMT) is a rare pathologic entity of unclear etiology characterized by adipose cell atrophy, focal hematopoietic tissue hypoplasia, and a distinct eosinophilic substance that stains with Alcian blue at pH 2.5. It is traditionally described in the context of malnutrition and cachexia from generalized disease and is important to identify because of its potential reversibility. Several recent case reports have described GBMT in patients with chronic myeloid leukemia (CML) on the first-generation tyrosine-kinase inhibitor (TKI) imatinib...
April 2019: Anti-cancer Drugs
Kalliopi Moustaka, Eirini Maleskou, Andromachi Lambrianidou, Stelios Papadopoulos, Marilena E Lekka, Theoni Trangas, Eirini Kitsiouli
Τhe effect of docosahexaenoic acid (DHA, an omega-3 polyunsaturated fatty acid) upon the proliferation of EoL-1 (Eosinophilic leukemia) cell line was assessed, while additional cellular events during the antiproliferative action were recorded. DHA inhibited EoL-1 cells growth dose-dependently by inducing growth arrest at G0/1 phase of the cell cycle. After DHA addition to the cells, the expression of MYC oncogene was decreased, PTAFR -mRNA overexpression was observed which was used as a marker of differentiation, and PLA2G4A -mRNA increase was recorded...
March 7, 2019: Nutrients
Larisa Pinte, Cristian Baicuș
BACKGROUND: In the last years an uprising interest for a relatively unknown entity, eosinophilic ascites (EA), has been recorded. Our aim is to investigate the potential causes of EA development, as well as clinical, laboratory, endoscopic and radiologic features, management and outcome in these patients. METHODS: The following research was performed on PubMed (MEDLINE) database using the medical subject headings [Mesh] terms "Ascites" AND "Eosinophils"...
December 1, 2018: Romanian Journal of Internal Medicine
Michelle Cancel, Mingchen Song
Objective: Antisynthetase syndrome is a condition that includes interstitial lung disease and inflammatory myositis in its definition. The interstitial lung disease of this syndrome can vary in severity and if not identified soon enough, can lead to severe respiratory failure. Here we present a patient who had a working diagnosis of acute eosinophilic pneumonia. He initially improved after prolonged hospitalization and course of high dose steroids. CT chest revealed interval improvement in his bilateral ground glass and reticular opacities but residual fibrotic interstitial lung disease...
2019: Respiratory Medicine Case Reports
Peng Wu, Yuanguo Chen, Haibo Zhang, Chen Li
RATIONALE: Idiopathic hypereosinophilic syndrome (IHES) is a rare disease in which patients which present with eosinophilia-associated damage. Previous studies focused on organ damage from increased eosinophilic granulocytosis. We report IHES in a patient who presented with multiple organ damage (MOD). PATIENT CONCERNS: A 52-year-old male presented with MOD, including myocardial damage suggestive of myocardial infarction, cardiac tamponade, respiratory failure, skin damage, and gastrointestinal damage...
March 2019: Medicine (Baltimore)
Seong-Ik Kim, Beomseok Jeon, Jeongmo Bae, Jae Kyung Won, Han-Joon Kim, Jeemin Yim, Yun Joong Kim, Sung-Hye Park
Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a progressive degenerative white matter disorder caused by mutations in the tyrosine kinase domain of the CSF1R gene. ALSP is often misdiagnosed as other diseases due to its rarity and various clinical presentations such as Parkinsonism, pyramidal signs, cognitive impairment and/or psychiatric symptoms. We describe an autopsy case of ALSP with a CSF1R mutation. A 61-year-old woman presented insidious-onset gait difficulty for 12 years since her age of 49, and premature ovarian failure since her age of 35...
February 2019: Experimental Neurobiology
Kevin Z Huang, Evan S Dellon
BACKGROUND AND AIMS: Eosinophilic gastrointestinal disorders (EGIDs) have been linked with atopic conditions, connective tissue disorders, and autoimmunity, but the association between EGIDs and autonomic dysfunction has not been extensively described. We aimed to assess this association and determine the prevalence of autonomic dysfunction in our EGID population. METHODS: In this retrospective cohort study, we analyzed adult patients in the University of North Carolina Eosinophilic Esophagitis (EoE)/EGID database who also had a diagnosis of comorbid autonomic dysfunction (postural orthostatic tachycardia syndrome [POTS] or gastroparesis)...
March 2019: Journal of Gastrointestinal and Liver Diseases: JGLD
Haixia Zhang, Bingwu Xiang, Hui Chen, Xiang Chen, Tao Cai
BACKGROUND: The KMT2A gene encoded lysine methyltransferase plays an essential role in regulating gene expression during early development and hematopoiesis. To date, 92 different mutations of KMT2A have been curated in the human gene mutation database (HGMD), resulting in Wiedemann-Steiner syndrome (WDSTS) and intellectual disability (ID)/developmental delay (DD). CASE PRESENTATION: In this report, we present a de novo heterozygous deletion mutation [c.74delG; p...
March 6, 2019: BMC Medical Genetics
Guangcai Ren, Han Wang, Yuanyuan Yan, Fan Liu, Miaorong Huang, Ruiai Chen
Hydropericardium-hepatitis syndrome (HHS) is characterized by pericardial effusion and hepatitis and causes huge economic losses in the poultry industry in China. In this study, a strain of fowl adenoviruses (FAdV-4) (GX-1) was isolated from liver samples of diseased chickens with HHS. Phylogenetic analysis based on complete genome gene revealed that GX-1 clustered with the C-type fowl adenovirus and was serotyped as FAdV-4. Pathogenicity testing showed that the GX-1 strain caused 100% mortality in 10-day-old specific pathogen-free chickens at a dose of 104 tissue culture infective doses (TCID50) within 3 d post-infection...
February 28, 2019: Poultry Science
Raghavendra L. Girijala, Imaad Siddiqi, Young Kwak, David Wright, Dhruti B. Patel, Leonard H. Goldberg
Background: Drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) or drug-induced hypersensitivity (DIHS) is a rare and feared complication of frequently used medications such as anticonvulsants, sulfonamides, and allopurinol. To date, no reports of hydroxychloroquine-induced pustular DRESS syndrome have been associated with Epstein-Barr virus (EBV) reactivation nor imitated other cutaneous adverse drug reactions as in our patient. Observation: A 56-year-old female presented with a diffuse cutaneous eruption involving the face, trunk, extremities, and palms approximately two weeks after the initiation of hydroxychloroquine therapy for a suspected Sjögren's-like process with inflammatory cervical lymphadenopathy...
February 1, 2019: Journal of Drugs in Dermatology: JDD
Mahmood Tavassoli, Hassan Abolhassani, Reza Yazdani, Mohsen Ghadami, Gholamreza Azizi, Sina Abdolrahim Poor Heravi, Tannaz Moeini Shad, Mostafa Kokabee, Masoud Movahedi, Hormoz Abdshahzadeh, Mohammad Gharagozlou, Nima Rezaei, Hossein Esmaeilzadeh, Soheila Aleyasin, Asghar Aghamohammadi
BACKGROUND: Hyper IgE syndromes (HIES) are distinct disease characterized by recurrent cutaneous and lung infections, eczema and elevated serum IgE level. METHODS: In this study, clinical manifestations, immunologic findings and genetic studies of all patients with HIES in the Iranian national registry database were evaluated. RESULTS: 129 HIES patients with median age of 14.0 (9.0-24.0) years were followed for a total of 307.8 patient-years...
February 23, 2019: Pediatric Allergy and Immunology
Nooreen Hussain, Preeti Patel, Jonathan Yin, Rachael Davis, Ossama Ikladios
Tumor necrosis factor antagonists (anti-TNF) are increasingly prescribed as maintenance therapy for a variety of autoimmune conditions. Therefore, frequent monitoring and awareness of side effects are of the utmost importance. Adalimumab is known to cause peripheral eosinophilia, but there are few reports of more severe adverse events. Loeffler's endocarditis is a rare and fatal disease characterized by eosinophilic infiltration of the endomyocardium leading to fibrosis and restrictive cardiomyopathy. Herein we describe a 72 year old female on adalimumab therapy for two years for rheumatoid arthritis presenting with Loeffler's endocarditis...
2019: Journal of Community Hospital Internal Medicine Perspectives
Marta García-Clemente, Ana Isabel Enríquez-Rodríguez, Marta Iscar-Urrutia, Beatriz Escobar-Mallada, Miguel Arias-Guillén, Francisco Julián López-González, Claudia Madrid-Carbajal, Liliana Pérez-Martínez, Teresa Gonzalez-Budiño
OBJECTIVE: The aim of our study was to determine the tomographic findings and prevalence of bronchiectasis in our population of patients with severe asthma, and to identify factors associated with the presence of bronchiectasis in these patients. MATERIALS AND METHODS: We retrospectively collected data from the medical histories of patients referred to the asthma unit of our hospital, with a diagnosis of severe asthma between 2015 and 2017. Patients with ABPA, cystic fibrosis, immunodeficiency or systemic disease were excluded...
February 20, 2019: Journal of Asthma: Official Journal of the Association for the Care of Asthma
Ying Jiang, Bi-Li Zhang, Wen-Hong Wang
OBJECTIVE: To study the clinical features of nephrotic syndrome (NS) accompanied by eosinophilia in children. METHODS: A retrospective analysis was performed for the clinical manifestations, laboratory findings and treatment outcomes of 18 cases of eosinophilia (15 children, 3 of whom also had eosinophilia at the second recurrence) in children with NS. RESULTS: Of the 18 cases, 16 (89%) had mild eosinophilia, 1 (6%) had moderate eosinophilia, and 1 (6%) had severe eosinophilia...
February 2019: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Hakan Öztürk, Neslihan Ekşi Bozbulut, Sinan Sarı, Ödül Eğritaş Gürkan, Hakan Sözen, Ali Sapmaz, Aydın Dalgıç, Buket Dalgıç
OBJECTIVES: Our aim was to determine potentially adverse effects of immunosuppressive protocols after liver transplantation in children. MATERIALS AND METHODS: The medical records of 60 children who underwent liver transplant retrospectively analyzed. Corticosteroid, tacrolimus, and mycophenolate mofetil were the primary immunosuppressive agents used in our center. RESULTS: The mean age of children was 6.1 years, ranging from 3 months to 17 years (34 boys, 26 girls)...
January 2019: Experimental and Clinical Transplantation
G Y Yu, X Hong, W Li, Y Y Zhang, Y Gao, Y Chen, Z Y Zhang, X Y Xie, Z G Li, Y Y Liu, J Z Su, W X Zhu, Z P Sun
Immunoglobulin G4-related sialadenitis (IgG4-RS) is a newly recognized immune-mediated disease and one of immunoglobulin G4-related diseases (IgG4-RD). Our multidisciplinary research group investigated the clinicopathological characteristics and diagnosis of IgG4-RS during the past 10 years. Clinically, it showed multiple bilateral enlargement of major salivary glands (including sublingual and accessory parotid glands) and lacrimal glands. The comorbid diseases of head and neck region including rhinosinusitis, allergic rhinitis, and lymphadenopathy were commonly seen, which could occur more early than enlargement of major salivary glands...
February 18, 2019: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
Iain Mactier, Jonathan R Dalzell, David Carrick
CLINICAL INTRODUCTION: A 50-year-old woman presented with an inferoposterior ST-elevation myocardial infarction (STEMI) and underwent emergency percutaneous coronary intervention (PCI). Angiography revealed acute occlusion of the circumflex and right coronary (RCA) arteries. PCI was uncomplicated. Her medical history included asthma, hypertension and chronic sinusitis.Three months later, she presented with a non-STEMI (NSTEMI), and angiogram showed a new focal stenosis in the left anterior descending artery...
February 14, 2019: Heart: Official Journal of the British Cardiac Society
Haris Sheikh, Mahrukh Siddiqui, Syed Mohammad Mazhar Uddin, Aatera Haq, Uzair Yaqoob
Eosinophilic myocarditis (EM) is a rare form of myocarditis. As there is extreme diversity in its manifestations, the true incidence is difficult to assess and no proper epidemiological criteria are present. It generally presents with a wide array of clinical manifestations. Clinical presentation tends to differ in cases and not all the patients show the same signs and symptoms. The etiology of EM often remains obscure but potential causes have been identified which may include hypersensitivity to drugs, exposure to certain viruses and parasites, and hyper-eosinophilic syndromes...
December 3, 2018: Curēus
A M Sarkar, S Akhter, M R Khan, M Saha, M R Alam, C K Ghosh, D S Ahmed, A R Miah, P K Roy
Several mechanisms have been proposed to explain the symptoms of functional dyspepsia but actual pathogenesis is still poorly understood. Recent studies support duodenal abnormality to be the most important causal link to explain symptoms and to understand abnormal pathophysiology of functional dyspepsia. The aim of this prospective observational study is to compare eosinophil count in duodenal mucosa between patients with functional dyspepsia and control subjects without dyspepsia and was done at the department of Gastroenterology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from December 2015 to December 2016...
January 2019: Mymensingh Medical Journal: MMJ
Joseph T Rabban, Emily Chan, Julie Mak, Charles Zaloudek, Karuna Garg
Pathology-based screening of uterine smooth muscle tumors (uSMT) for morphology suggestive of fumarate hydratase deficiency (FH-d morphology) has been proposed as a method to identify women at increased risk for hereditary leiomyomatosis renal cell carcinoma (HLRCC) syndrome. For 5 years our clinical diagnostic practice has evaluated all women with any type of uSMT for FH-d morphology (defined, at low magnification, as staghorn shaped blood vessels and alveolar pattern edema and, at high magnification, as tumor macronucleoli surrounded by a halo and cytoplasmic eosinophilic globules) and, when present, used the pathology report to advise genetic counseling to further evaluate for HLRCC syndrome...
February 6, 2019: American Journal of Surgical Pathology
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