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Eosinophilic syndromes

Philipp Gauckler, Jae Il Shin, Gert Mayer, Andreas Kronbichler
Peripheral blood eosinophilia (PBE), defined as 500 eosinophils or above per microliter (µL) blood, is a condition that is not uncommon but often neglected in the management of patients with chronic kidney disease (CKD), acute kidney injury (AKI), or patients on renal replacement therapy (RRT). The nature of PBE in the context of kidney diseases is predominantly secondary or reactive and has to be distinguished from primary eosinophilic disorders. Nonetheless, the finding of persistent PBE can be a useful clue for the differential diagnosis of underdiagnosed entities and overlapping syndromes, such as eosinophilic granulomatosis with polyangiitis (EGPA), IgG4-related disease (IgG4-RD), acute interstitial nephritis (AIN), or the hypereosinophilic syndrome (HES)...
December 8, 2018: Journal of Clinical Medicine
Hirotsugu Hashimoto, Genki Usui, Eiji Sakai, Ken Ohata, Teppei Morikawa
In this article, we report the case of a 40-year-old woman with a sessile polyp of the rectosigmoid junction that underwent endoscopic resection. The resected specimen showed spindle cell proliferation with eosinophilic cytoplasm between mucosal crypts, mimicking mucosal prolapse syndrome. However, these were immunohistochemically positive for S-100, indicating neural cell origin. As neural polypoid lesion of the rectum, neurofibroma, perineurioma, schwannoma, and mucosal Schwann cell hamartoma were in the differential diagnosis...
December 12, 2018: International Journal of Surgical Pathology
Ji Young Hong, Joo-Hee Kim, Sunghoon Park, Yong Il Hwang, Ki-Suck Jung, Seung Hun Jang
Background/Aims: Inhaled corticosteroids (ICS) are a treatment of choice for eosinophilic airway diseases, but their efficacy for other causes of chronic cough is controversial. Methods: We conducted a prospective observational study to determine the ICS efficacy and clinical predictors of response to ICS in patients with upper airway cough syndrome (UACS) or unexplained chronic cough (UCC). Sixty-eight patients with UACS and 33 patients with UCC (duration of cough ≥ 8 weeks) were treated with ICS: 250 µg of fluticasone propionate or 400 µg of budesonide twice a day at physician's discretion...
December 13, 2018: Korean Journal of Internal Medicine
Farid Arman, Marina Barsoum, Umut Selamet, Hania Shakeri, Olivia Wassef, Mira Mikhail, Anjay Rastogi, Ramy M Hanna
Circulating antineutrophil cytoplasmic antibodies (ANCAs) are the central pathogenic mechanism for a group of systemic and renal syndromes called the ANCA-associated vasculitis (AAV). The nomenclature has changed from eponymous labeling to granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis. These syndromes predominantly affect the pulmonary and renal systems. We also review the molecular pathology behind ANCAs and associated antigens and infections...
2018: International Journal of Nephrology and Renovascular Disease
Anca Daniela Brăila, Andrei Călin Zavate, Alex Emilian Stepan, Corneliu Sabetay, Jamal Kamel, Mihai Georgescu-Brăila, Alexandru Marian Gogănău, Mircea Ovidiu Ciobanu
The acute abdomen with its many clinical aspects is not a condition that eludes pregnancy; acute appendicitis being the most frequently incriminated, but when it comes to its complication - peritonitis and the pregnant is a teenager, the cases cited in the literature are quite rare. We present the case of a 15-year-old teenager pregnant within 24 weeks admitted from the emergency department with the diagnosis of acute abdomen and operated for generalized peritonitis due to a perforated appendicitis. The microscopic analysis of the specimen indicated the presence of ulceration and extensive and deep and necrosis of the appendicle wall, the residual structures being dissected by a predominantly polymorphonuclear leukocytes inflammatory infiltrate associated with eosinophilic fibrinous deposits...
2018: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
Jan-Philipp Scheide-Noeth, Maximilian Rosen, David Baumstark, Harald Dietz, Thomas D Mueller
Interleukin-5 (IL-5) is a TH 2 cytokine involved in many aspects of eosinophil life. Eosinophilic granulocytes play a pathogenic role in the progression of atopic diseases, such as allergy, asthma and atopic dermatitis and hypereosinophilic syndromes. Here, eosinophils upon activation degranulate leading to the release of proinflammatory proteins and mediators stored in intracellular vesicles termed granula thereby causing local inflammation, which when persisting leads to tissue damage and organ failure. As a key regulator of eosinophil function, IL-5 therefore presents a major pharmaceutical target and approaches to interfere with IL-5 receptor activation are of great interest...
December 7, 2018: Journal of Molecular Biology
Stamatis Karakonstantis, Niki Gryllou, George Papazoglou, Charalampos Lydakis
INTRODUCTION: Eosinopenia has been previously investigated as a marker to differentiate infectious from non-infectious diagnoses and as a prognostic marker. Most previous studies were conducted in intensive care unit patients. Our study focuses on the value of eosinopenia in patients admitted to the Internal Medicine department. METHODS: We retrospectively analyzed 271 consecutive patients with infection and 31 patients presenting with fever or inflammatory syndrome and a non-infectious diagnosis...
November 1, 2018: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
Raffaele Longo, Oana Balasanu, Mathilde Chastenet de Castaing, Eric Chatelain, Mohammed Yacoubi, Marco Campitiello, Nathalie Marcon, Francesca Plastino
BACKGROUND Merkel cell carcinoma (MCC) is a rare, aggressive primary cutaneous neuroendocrine tumor frequently associated with Merkel cell polyomavirus infection. Despite its aggressiveness, a few reports of spontaneous MCC regression have been described in the literature, most of them following incisional biopsy supporting a hypothetical role of surgery-induced inflammation in the process of regression. CASE REPORT We report a case of 69-year-old Caucasian male who was followed for an idiopathic hyper-eosinophilic syndrome...
December 4, 2018: American Journal of Case Reports
Aline Lima-Matos, Eduardo Vieira Ponte, Juliana Pires Viana de Jesus, Paula Cristina Andrade Almeida, Valmar Bião Lima, Namhee Kwon, John Riley, Luane Marques de Mello, Álvaro A Cruz
BACKGROUND: Asthma is a syndrome with multiple phenotypes. Peripheral blood eosinophil counts might be the ideal biomarker to identify subjects with eosinophilic asthma. It is available, inexpensive, and it is associated with eosinophilia in sputum. OBJECTIVE: The aim of this study was to evaluate whether blood eosinophilia is associated with asthma severity and to evaluate whether blood eosinophilia is associated with lack of control of asthma symptoms and airway obstruction...
December 2018: Respiratory Medicine
Jinfeng Lin, Xiaoying Huang, Weihua Zhou, Suyan Zhang, Weiwei Sun, Yadong Wang, Ke Ren, Lijun Tian, Junxian Xu, Zhilong Cao, Zunguo Pu, Xudong Han
RATIONALE: Extensive thrombosis in the portal venous system caused by hypereosinophilic syndrome (HES) is rare, and there is no consensus on anticoagulant and thrombolytic treatments for arteriovenous thrombosis caused by HES. PATIENT CONCERNS: The clinical data of a patient with extensive thrombosis in his portal venous system (superior mesenteric, splenic, hepatic, and portal veins), renal artery thrombosis, and mesenteric thrombosis caused by HES with secondary gastrointestinal bleeding and intestinal necrosis were retrospectively analyzed...
November 2018: Medicine (Baltimore)
Amy Klion
Hypereosinophilic syndromes (HESs) are a heterogeneous group of rare disorders characterized by peripheral eosinophilia and eosinophilic end organ complications. Conventional therapies, including glucocorticoids and cytotoxic and immunomodulatory agents, have variable efficacy and significant toxicity. Although the recent development of agents that target eosinophils, including tyrosine kinase inhibitors and monoclonal antibodies, provides the possibility of more effective, less toxic approaches to treatment of HES, there are little available data to guide their use in these conditions...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
Michele V Quan, Stephen K Frankel, Mehrnaz Maleki-Fischbach, Laren D Tan
BACKGROUND: Granulomatosis with polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophilic granulomatosis with polyangiitis (EGPA) is also a systemic ANCA-associated vasculitis, but EGPA is characterized by eosinophilic as well as granulomatous inflammation and is more commonly associated with asthma and eosinophilia. Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis classification and/or overlaps with more than one category...
November 29, 2018: BMC Pulmonary Medicine
Marek L Kowalski
PURPOSE OF REVIEW: NSAID-Exacerbated Disease (N-ERD) is a chronic eosinophilic inflammatory disorder of the respiratory tract occurring in patients with asthma and/or rhinosinusitis with nasal polyps, whose symptoms are exacerbated by NSAIDs. The purpose of this review is to provide an update on clinical characteristics, pathophysiology, and management of N-ERD, and to emphasize heterogeneity of this syndrome. RECENT FINDINGS: Growing evidence indicates that N-ERD, which has been considered a separate asthma phenotype, is heterogenous, and can be divided in several subphenotypes varying in clinical characteristics...
January 2019: Current Opinion in Pulmonary Medicine
Alvise Berti, Roberto Caporali, Carlomaurizio Montecucco, Giuseppe Paolazzi, Sara Monti
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) usually affect adults and older populations. The well recognized clinical (i.e., granulomatosis with polyangiitis [GPA], microscopic polyangiitis, and eosinophilic GPA) and serological phenotypes (i.e., anti-MPO-ANCA, anti-PR3-ANCA and ANCA negative) within AAV differ substantially for clinical, demographic, and epidemiological features, including age at presentation. Whether and how aging could contribute to the clinical expression of these disease phenotypes is intriguing and still overlooked...
November 28, 2018: Drugs & Aging
Ho Yeon Jung, Sunmin Park, Beomsu Shin, Ji Ho Lee, Seok Jeong Lee, Myoung Kyu Lee, Won Yeon Lee, Suk Joong Yong, Sang Ha Kim
PURPOSE: Although there have been reported cases of drug reactions with eosinophilia and systemic symptoms (DRESS) syndrome caused by antituberculosis drugs, there has been no research to examine its prevalence. This study assessed the prevalence and clinical characteristics of DRESS syndrome caused by antituberculosis drugs. METHODS: The electronic medical records of a cohort consisting of adult patients diagnosed with tuberculosis between July 2006 and June 2010 were reviewed and retrospectively inspected...
January 2019: Allergy, Asthma & Immunology Research
Eugenio De Corso, Roberta Anzivino, Jacopo Galli, Silvia Baroni, Walter Di Nardo, Carla De Vita, Antonio Salvati, Chiara Autilio, Stefano Settimi, Dario Mele, Gaetano Paludetti, Joaquim Mullol
OBJECTIVE: The aim of our study was to analyze the montelukast effectiveness in improving oculonasal symptoms, patient-reported outcomes (PROs), and eosinophilic biomarkers in patients with nonallergic rhinitis eosinophilic syndrome (NARES). METHODS: We enrolled prospectively 80 symptomatic patients treated with 10 mg once a day of montelukast in monotherapy for 2 months. All patients were investigated before and after treatment. Nasal symptoms (nasal obstruction, rhinorrhoea, sneezing, nasal itching), ocular symptoms (redness/puffiness, watery eyes), and other PROs (olfactory dysfunction, difficulty going to sleep, nighttime awakenings, and nasal congestion on awakening) were scored by visual analogic scale...
November 26, 2018: Laryngoscope
Magnus Halland, Nicholas J Talley, Mike Jones, Joseph A Murray, Raquel Cameron, Marjorie M Walker
BACKGROUND: Rumination syndrome is a functional gastrointestinal disorder characterized by effortless, postprandial regurgitation. Duodenal eosinophilia has been described in patients with functional dyspepsia. Because of the significant symptomatic overlap between functional dyspepsia and rumination syndrome, we hypothesized that histological changes might exist among patients with rumination syndrome. METHODS: We included patients with rumination syndrome in whom we had obtained duodenal biopsies and compared these with controls...
November 26, 2018: Digestive Diseases and Sciences
Ernesto Aitella, Fabio De Bartolomeis, Alfonso Savoia, Massimo Fabiani, Marco Romano, Corrado Astarita
BACKGROUND: One-quarter of systemic symptoms associated with chronic spontaneous urticaria (CSU) are related to gastrointestinal complaints (GICs). OBJECTIVES: To investigate the prevalence and features of urticaria-overlapping GICs. METHODS: In this retrospective cross-sectional survey, 1426 consecutive outpatients were observed at our University Department. Only patients suffering from urticaria or GICs with a complete diagnostic work-up including serum total IgE level (Tot-IgE), differential blood count and urticaria activity score (UAS), were evaluated...
2018: PloS One
Rajesh Kumar, Chandni Garg, Uma Nahar Saikia, K L N Rao
Congenital nevi and fibrolipomatous hamartoma are benign tumors of childhood, the latter being very uncommon. Fibrous hamartoma of infancy typically occurs in <2 years of life. The concurrence of these two lesions is extremely rare. We report a case of congenital fibrolipomatous hamartoma and congenital nevus of infancy in a 6-month-old male infant. Clinically, a suspicion of benign versus malignant lesion beneath the giant congenital nevus prompted its surgical removal. The histopathology confirmed it to be a compound lesion with benign melanocytic nevi and fascicles of spindle cells with eosinophilic cytoplasm representing hamartoma...
October 2018: Journal of Indian Association of Pediatric Surgeons
Jiayuan Jiao, Jiming Wu, Jiali Wang, Yaping Guo, Le Gao, Honggang Liang, Jian Huang, Jinhui Wang
CONTEXT: Ma Huang Tang (MHT) has been used to treat influenza, fever, bronchial asthma, etc. as a traditional Chinese medication. However, the anti-inflammation mechanism of MHT remains unclear. OBJECTIVE: The study identifies the possible mechanisms of MHT on ovalbumin (OVA)-induced acute bronchial asthma in mice. MATERIALS AND METHODS: First, an asthma-related protein-protein interaction (PPI) network was constructed. And then, the acute bronchial asthma mice models were established by exposing to aerosolized 1% ovalbumin for 30 min/day for 1 week, and the mice were administered 2...
December 2018: Pharmaceutical Biology
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