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Chronic granulomatous disease

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https://www.readbyqxmd.com/read/28811575/biological-and-functional-characterization-of-bone-marrow-derived-mesenchymal-stromal-cells-from-patients-affected-by-primary-immunodeficiency
#1
Nadia Starc, Daniela Ingo, Antonella Conforti, Valeria Rossella, Luigi Tomao, Angela Pitisci, Fabiola De Mattia, Immacolata Brigida, Mattia Algeri, Mauro Montanari, Giuseppe Palumbo, Pietro Merli, Paolo Rossi, Alessandro Aiuti, Franco Locatelli, Maria Ester Bernardo
Mesenchymal stromal cells (MSCs) represent a key component of bone marrow (BM) microenvironment and display immune-regulatory properties. We performed a detailed analysis of biological/functional properties of BM-MSCs derived from 33 pediatric patients affected by primary immune-deficiencies (PID-MSCs): 7 Chronic Granulomatous Disease (CGD), 15 Wiskott-Aldrich Syndrome (WAS), 11 Severe Combined Immunodeficiency (SCID). Results were compared with MSCs from 15 age-matched pediatric healthy-donors (HD-MSCs). Clonogenic and proliferative capacity, differentiation ability, immunophenotype, immunomodulatory properties were analyzed...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28804654/histoplasmosis-by-histoplasma-capsulatum-var-duboisii-observed-at-the-laboratory-of-pathological-anatomy-of-lom%C3%A3-in-togo
#2
Tchin Darré, Bayaki Saka, Abas Mouhari-Touré, Améyo Monique Dorkenoo, Koffi Amégbor, Vincent Palokinam Pitche, Gado Napo-Koura
Our study aimed to describe the epidemiological, clinical, and diagnostic aspects of African histoplasmosis in Togo through a descriptive and cross-sectional study on histological diagnosed African histoplasmosis in Pathology Department of Lomé from 2002 to 2016 (15 years). A total of 17 cases of African histoplasmosis were diagnosed. The sex ratio (M/F) was 1.8. The annual incidence was 1.1 cases. The mean age of the patients was 27.2 ± 0.4 years. All our patients were of social categories with a low socioeconomic level...
2017: Journal of Pathogens
https://www.readbyqxmd.com/read/28794542/mycetoma-an-update
#3
REVIEW
Vineet Relhan, Khushbu Mahajan, Pooja Agarwal, Vijay Kumar Garg
Mycetoma is a localized chronic, suppurative, and deforming granulomatous infection seen in tropical and subtropical areas. It is a disorder of subcutaneous tissue, skin and bones, mainly of feet, characterized by a triad of localized swelling, underlying sinus tracts, and production of grains or granules. Etiological classification divides it into eumycetoma caused by fungus, and actinomycetoma caused by bacteria. Since the treatment of these two etiologies is entirely different, a definite diagnosis after histopathological and microbiological examination is mandatory, though difficult...
July 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28793023/two-simultaneous-mycetomas-caused-by-fusarium-verticillioides-and-madurella-mycetomatis
#4
Alexandro Bonifaz, Mariana Saldaña, Javier Araiza, Patricia Mercadillo, Andrés Tirado-Sánchez
Mycetoma is a chronic granulomatous disease, classified into eumycetoma caused by fungi and actinomycetoma due to aerobic filamentous actinomycetes. Mycetoma can be found in geographic areas near the Tropic of Cancer. Mexico is one of the countries in which actinomycetoma is endemic. We report an extraordinary case of an adult male with double eumycetoma caused by Madurella mycetomatis and Fusarium verticillioides on both feet.
2017: Revista do Instituto de Medicina Tropical de São Paulo
https://www.readbyqxmd.com/read/28780082/granulomas-within-renal-epithelial-neoplasms-in-patients-with-sarcoidosis-a-causal-association
#5
Nasma K Majeed, Andres M Acosta, Andre Kajdacsy-Balla, Suman Setty, Nathan A Aardsma
Sarcoidosis is a chronic granulomatous disease that can involve virtually every organ system, but most commonly presents as lung, skin, or lymph node disease. Although kidney involvement is usually clinically silent, granulomatous interstitial nephritis - the hallmark of renal sarcoidosis - can lead to functional impairment and organ failure. Also, recent studies have suggested an association between sarcoidosis and an increased risk of developing kidney tumors. While a sarcoid-like granulomatous reaction (SLGR) to renal epithelial neoplasms in patients without sarcoidosis has been well documented, direct involvement of the tumor parenchyma by sarcoidosis has been reported only rarely...
July 23, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28780079/antineutrophil-cytoplasmic-antibody-associated-vasculitides-and-igg4-related-disease-a-new-overlap-syndrome
#6
REVIEW
François-Xavier Danlos, Giovanni Maria Rossi, Daniel Blockmans, Giacomo Emmi, Andreas Kronbichler, Stéphane Durupt, Claire Maynard, Luminita Luca, Cyril Garrouste, Bertrand Lioger, Rachel Mourot-Cottet, Robin Dhote, Jean-Benoit Arlet, Thomas Hanslik, Philippe Rouvier, Mikael Ebbo, Xavier Puéchal, Dominique Nochy, Agnès Carlotti, Luc Mouthon, Loïc Guillevin, Augusto Vaglio, Benjamin Terrier
OBJECTIVE: Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. METHODS: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria...
August 2, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28776363/development-of-hodgkin-lymphoma-in-a-patient-with-sarcoidosis
#7
F Gediz, A F Yilmaz, B Payzin, T Yuksel, A O Calli, S Kobak
Sarcoidosis is a chronic granulomatous disease of unknown etiology characterized by non-caseified granulomas in many different organs and systems. The disease most frequently manifests with bilateral hilar lymphadenopathy and infiltrations in the lungs and skin, as well as with eye lesions. It may mimic a number of systemic diseases and/or accompany them. The development of lymphoma in patients with sarcoidosis or the co-occurrence of both diseases is rarely reported in the literature. In this paper we report a female patient followed up with sarcoidosis for three years who developed Hodgkin lymphoma, according to the results of the investigations and biopsy results...
August 3, 2017: Reumatismo
https://www.readbyqxmd.com/read/28774699/pulmonary-and-sinus-fungal-diseases-in-non-immunocompromised-patients
#8
REVIEW
David W Denning, Arunaloke Chakrabarti
The human respiratory tract is exposed daily to airborne fungi, fungal enzymes, and secondary metabolites. The endemic fungi Histoplasma capsulatum, Coccidioides spp, Blastomyces dermatitidis, and Paracoccidioides brasiliensis, and occasionally Aspergillus fumigatus, are primary pulmonary pathogens of otherwise healthy people. Such infections resolve in most people, and only a few infections lead to disease. However, many fungi are directly allergenic by colonising the respiratory tract or indirectly through contact with cell wall constituents and proteases, causing or exacerbating allergic disease...
July 31, 2017: Lancet Infectious Diseases
https://www.readbyqxmd.com/read/28767612/pyoderma-gangrenosum-in-a-patient-with-chronic-granulomatous-disease-a-case-report
#9
Sideris Nanoudis, Afroditi Tsona, Olga Tsachouridou, Petros Morfesis, Georgia Loli, Adamantini Georgiou, Pantelis Zebekakis, Symeon Metallidis
RATIONALE: The simultaneous occurrence of pyoderma gangrenosum (PG) and chronic granulomatous disease (CGD) is uncommon and few cases have been reported worldwide. PATIENT CONCERNS: PG is a rare, chronic, ulcerative, neutrophilic skin disease of unknown etiology that requires immunosuppressive treatment. CGD belongs to Primary Immune Deficiencies in which the main defect lies in an inability of the phagocytic cells to generate superoxide making patients susceptible to serious, potentially life-threatening bacterial and fungal infections...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28764247/idiopathic-granulomatous-mastitis-a-clinical-puzzle-in-breast-lump-cases
#10
Vivek G Nath, Rakesh Sahoo, Avinash Sahoo, Jithendra Kumar Barad, K A Arun
Idiopathic granulomatous mastitis (IGM) is a rare benign disease, characterized by chronic inflammation and granulomatous disease process. A middle aged lady with breast lump for six months with equivocal mammographic and ultrasound results underwent lumpectomy and biopsy. Ruling out all other possible granulomatous diseases and malignancy, a diagnosis of IGM was made. IGM becomes clinically significant as it closely mimics carcinoma breast and some inflammatory and infectious pathology.
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28761228/kidney-disease-in-human-immunodeficiency-virus-seropositive-patients-absence-of-human-immunodeficiency-virus-associated-nephropathy-was-a-characteristic-feature
#11
J Prakash, V Ganiger, S Prakash, M Sivasankar, S Sunder, U Singh
Human immunodeficiency virus (HIV) infection can cause a broad spectrum of renal diseases. However, there is paucity of Indian data on the patterns of renal lesions in HIV-seropositive patients. The aim of the present study was to delineate the spectrum of renal lesions in HIV/acquired immunodeficiency syndrome patients. In this prospective study, all HIV-positive patients of both genders aged >18 years were screened for renal disease. Patients with proteinuria of more than 1 g/24 h were subjected to renal biopsy...
July 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28755949/short-communication-passive-shedding-of-mycobacterium-avium-ssp-paratuberculosis-in-commercial-dairy-goats-in-brazil
#12
D G G Schwarz, M C Lima, M Barros, F L Valente, T M Scatamburlo, N Rosado, C T S A M Oliveira, L L Oliveira, M A S Moreira
Goat farming is a low-cost alternative to dairy production in developing countries. In Brazil, goat production has increased in recent years due in part to the implementation of programs encouraging this activity. Mycobacterium avium ssp. paratuberculosis (MAP) is the causative agent of paratuberculosis, a disease that causes chronic granulomatous enteritis in ruminants, but MAP transmission dynamics are still poorly understood in goats. In a previously published study of our research group, 10 dairy goat farms (467 animals) from Minas Gerais state were analyzed for MAP detection; 2 fecal cultures and 11 milk samples tested positive for MAP by conventional PCR and were confirmed by sequencing...
July 26, 2017: Journal of Dairy Science
https://www.readbyqxmd.com/read/28752571/haematopoietic-stem-cell-transplantation-for-primary-immunodeficiency-syndromes-a-5-year-single-centre-experience
#13
Melissa Norman, Clementine David, Brynn Wainstein, John B Ziegler, Richard Cohn, Richard Mitchell, Tracey O'Brien, Susan Russell, Toby Trahair, Annette Trickett, Katie Frith, Paul Gray
AIM: Haematopoietic stem cell transplantation (HSCT) is a central therapy in the treatment of primary immunodeficiency diseases (PIDs). Over the past 5 years, outcomes have been greatly improved due to earlier diagnosis, improved donor availability, advancements in graft manipulation and the use of less toxic preparative regimens. We present a 5-year audit of HSCT for PID at a single Australian tertiary hospital. METHODS: Retrospective case note review identified diagnosis, pre-transplant medical morbidity, transplant protocol, engraftment, adverse events, post-transplant immune reconstitution and general health...
July 28, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/28752258/allogeneic-reduced-intensity-hematopoietic-stem-cell-transplantation-for-chronic-granulomatous-disease-a-single-center-prospective-trial
#14
Mark Parta, Corin Kelly, Nana Kwatemaa, Narda Theobald, Diane Hilligoss, Jing Qin, Douglas B Kuhns, Christa Zerbe, Steven M Holland, Harry Malech, Elizabeth M Kang
PURPOSE: The purpose of this study was to evaluate engraftment and adverse events with a conditioning and prophylactic regimen intended to achieve high rates of engraftment with minimal graft-versus-host disease (GVHD) in allogeneic transplantation for chronic granulomatous disease in a single center. METHODS: Forty patients, 37 male, with chronic granulomatous disease were transplanted. Transplant products were matched sibling peripheral blood stem cells (PBSCs) in four and matched unrelated donor (MUD) bone marrow in three, and one patient received mismatched unrelated PBSCs...
July 28, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28749045/factors-related-to-recurrence-of-idiopathic-granulomatous-mastitis-what-do-we-learn-from-a-multicentre-study
#15
Erdal Uysal, Atilla Soran, Efe Sezgin
BACKGROUND: Idiopathic granulomatous mastitis (IGM) is a rare chronic inflammatory disease of the breast with unknown aetiology. Its treatment is controversial and the recurrence rate is high. The objectives of this study were to examine the demographic, sociocultural and clinical characteristics observed among a large cohort of IGM patients from Turkey and to identify factors related to the recurrence of IGM. METHODS: The study was designed as a multicentre retrospective study including 22 breast centres in Turkey...
July 27, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28747804/differential-signalling-and-kinetics-of-neutrophil-extracellular-trap-release-revealed-by-quantitative-live-imaging
#16
Maarten van der Linden, Geertje H A Westerlaken, Michiel van der Vlist, Joris van Montfrans, Linde Meyaard
A wide variety of microbial and inflammatory factors induce DNA release from neutrophils as neutrophil extracellular traps (NETs). Consensus on the kinetics and mechanism of NET release has been hindered by the lack of distinctive methods to specifically quantify NET release in time. Here, we validate and refine a semi-automatic live imaging approach for quantification of NET release. Importantly, our approach is able to correct for neutrophil input and distinguishes NET release from neutrophil death by other means, aspects that are lacking in many NET quantification methods...
July 26, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28731661/-melkersson-rosenthal-syndrome-report-of-two-pediatric-cases
#17
Lucas Bordino, Mariana L Juchli, Marcelo Fernández, María de Los Ángeles Fitz Maurice, Andrea Martins, Zaida E Ramírez, Roxana Spini
Melkersson Rosenthal syndrome is a very infrequent disease of unknown etiology, chronic and progressive course, with neurocutaneous disease that affects the orofacial innervation and mucocutaneous tissues with non-caseating granulomatous infiltration. Clinically, it is characterized by the diagnostic triad: recurrent edema of lips and/or face; recurrent and alternating acute peripheral facial paralysis and scrotal or geographic tongue. The condition generally presents as oligosymptomatic or monosymptomatic form...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28727298/nine-patients-with-chronic-granulomatous-disease-having-selective-neck-dissection-for-severe-cervical-lymphadenitis
#18
Laura R Wingfield, Jack Liu, Michael Hu, David Bianchi, Kenneth Hauck, Brian Driscoll, John I Gallin, Harry L Malech, Steven M Holland, Carter Van Waes
Chronic Granulomatous Disease (CGD) is a rare, inherited disorder due to an X-linked or autosomal genetic defect, in which patients experience a high rate of lymphadenitis. To date, no studies have examined the best treatment for severe lymphadenitis in this patient population. We present a nine CGD patient retrospective case series, examining surgical treatment of cervical lymphadenitis. Our evolving surgical strategy shows that an initially more aggressive surgical approach (selective neck dissection) can help prevent recurrent infection...
July 20, 2017: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28717040/flucloxacillin-results-in-suboptimal-voriconazole-plasma-concentrations
#19
Eline W Muilwijk, Bart G J Dekkers, Stefanie Henriet, Paul E Verweij, Bregje Witjes, Astrid M L Oude Lashof, Geert H Groeneveld, Johannes van der Hoeven, Jan Willem C Alffenaar, Frans G M Russel, Frank van de Veerdonk, Roger J M Brüggemann
Combining voriconazole and flucloxacillin is indicated in patient cohorts experiencing both invasive aspergillosis and gram-positive infections (e.g. patients with chronic granulomatous disease or post-influenza pulmonary aspergillosis). We report a highly relevant interaction between voriconazole and flucloxacillin resulting in subtherapeutic voriconazole plasma concentrations in more than 50% of patients, posing a severe threat if not managed properly.
July 17, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28702318/aspergillus-nodules-in-chronic-granulomatous-disease-attributable-to-aspergillus-ochraceus
#20
Samihah Moazam, David W Denning
Aspergillus ochraceus is a rare pulmonary pathogen. A 39 year old male with COPD and chronic granulomatous disease presented with severe breathlessness and recurrent infections. CT scan demonstrated multiple pulmonary nodules diagnosed as chronic pulmonary aspergillosis. The patient's sputum grew Aspergillus ochraceus thrice over 6 months, alongside positive Aspergillus IgG and serum galactomannan. Despite treatment with itraconazole, the patient continued to be symptomatic. We present the first case associating A...
September 2017: Medical Mycology Case Reports
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