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Chronic granulomatous disease

Deniz Koksal, Aydin Seref Koksal, Ahmet Gurakar
Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease caused by diffuse inflammation, destruction and fibrosis of the intrahepatic bile ducts, ultimately leading to cirrhosis, portal hypertension and liver failure. The pathogenesis of PBC is incompletely understood, but current data suggest roles for genetic susceptibility and environmental factors. PBC is often thought of as an organ-specific autoimmune disease, which mainly targets the liver; however, lung tissue is also a site for autoimmune involvement of PBC...
September 28, 2016: Journal of Clinical and Translational Hepatology
Hai-Jing Yu, Hua Deng, Jian Ma, Shu-Jun Huang, Jian-Min Yang, Yan-Fen Huang, Xiao-Ping Mu, Liang Zhang, Qi Wang
BACKGROUND: Granulomatous mastitis (GM) is a chronic inflammatory breast lesion and its etiology remains incompletely defined. Although mounting evidence suggests the involvement of Corynebacterium in GM, there has been no systematic study of GM bacteriology using -omics technology. METHODS: The bacterial diversity and relative abundances in breast abscesses from 19 women with GM were investigated using 16S rDNA metagenomic sequencing and Sanger sequencing. A quantitative PCR (qPCR) assay was also developed to identify C...
October 21, 2016: International Journal of Infectious Diseases: IJID
Aravind T Reddy, Sowmya P Lakshmi, Raju C Reddy
Peroxisome proliferator-activated receptor γ (PPARγ) is now recognized as an important modulator of leukocyte inflammatory responses and function. Its immunoregulatory function has been studied in a variety of contexts, including bacterial infections of the lungs and central nervous system, sepsis, and conditions such as chronic granulomatous disease. Although it is generally believed that PPARγ activation is beneficial for the host during bacterial infections via its anti-inflammatory and antibacterial properties, PPARγ agonists have also been shown to dampen the host immune response and in some cases exacerbate infection by promoting leukocyte apoptosis and interfering with leukocyte migration and infiltration...
2016: PPAR Research
Olga C Rojas, Rafael B R León-Cachón, Maria Moreno-Treviño, Gloria M González
Mycetoma is a chronic granulomatous, subcutaneous disease endemic in tropical and subtropical countries. It is currently a health problem in rural areas of Africa, Asia and South America. Nine cases of mycetoma were analysed in a retrospective study. All isolates were identified by morphological features. The level of species identification was reached by molecular tools. Definitive identification of fungi was performed using sequence analysis of the ITS of the ribosomal DNA region and the ribosomal large-subunit D1/D2...
October 21, 2016: Mycoses
Maria Pia Bondioni, Vassilios Lougaris, Giuseppe Di Gaetano, Tiziana Lorenzini, Annarosa Soresina, Francesco Laffranchi, Diego Gatta, Alessandro Plebani
PURPOSE: The purpose of this study is to evaluate the possibility of early detection of pulmonary fungal infections by lung CT scan in chronic granulomatous disease (CGD). METHODS: A retrospective study on 14 patients affected with CGD for a total of 18 infectious episodes was performed. Revision of clinical data and CT scan analysis before and after treatment was performed. RESULTS: The presence of lung nodules <30 mm was evaluated in 18 infectious episodes in 14 patients...
October 21, 2016: Journal of Clinical Immunology
Huan-Ting Lin, Takashi Okumura, Yukinori Yatsuda, Satoru Ito, Hiromitsu Nakauchi, Makoto Otsu
Stable gene transfer into target cell populations via integrating viral vectors is widely used in stem cell gene therapy (SCGT). Accurate vector copy number (VCN) estimation has become increasingly important. However, existing methods of estimation such as real-time quantitative PCR are more restricted in practicality, especially during clinical trials, given the limited availability of sample materials from patients. This study demonstrates the application of an emerging technology called droplet digital PCR (ddPCR) in estimating VCN states in the context of SCGT...
October 2016: Human Gene Therapy Methods
Michela Malvisi, Fiorentina Palazzo, Nicola Morandi, Barbara Lazzari, John L Williams, Giulio Pagnacco, Giulietta Minozzi
Paratuberculosis in cattle is a chronic granulomatous gastroenteritis caused by Mycobacterium avium subsp. paratubercolosis (MAP) which is endemic worldwide. In dairy herds, it is responsible for huge economic losses. However, current diagnostic methods do not detect subclinical infection making control of the disease difficult. The identification of MAP infected animals during the sub-clinical phase of infection would play a key role in preventing the dissemination of the pathogen and in reducing transmission...
2016: PloS One
Y Hayashi, Yoshiki Ishii, J Nagasawa, S Arai, H Okada, F Ohmi, T Umetsu, Y Machida, K Kurasawa, A Takemasa, S Suzuki, T Senoh, T Sada, K Hirata
Sarcoidosis is a chronic granulomatous disease that can affect multiple organs. The lungs, eyes, and skin are known to be highly affected organs in sarcoidosis. There have been reports based on random muscle biopsy that 32-80% of systemic sarcoidosis comprises noncaseating granulomas; however, muscle involvement in sarcoidosis is generally asymptomatic and has an unknown frequency. We describe a case of acute to subacute sarcoid myositis of the skeletal and extraocular muscles. Typical ophthalmic involvement (manifested by infiltration of the ocular adnexa, intraocular inflammation, or infiltration of the retrobulbar visual pathways) and extraocular sarcoid myositis (as with the present case) is infrequently reported...
October 7, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
Thiago Almeida Pereira, Wing-Kin Syn, Frederico Figueiredo Amâncio, Pedro Henrique Diniz Cunha, Julia Fonseca Morais Caporali, Guilherme Vaz de Melo Trindade, Elisângela Trindade Santos, Márcia Maria Souza, Zilton Araújo Andrade, Rafal P Witek, William Evan Secor, Fausto Edmundo Lima Pereira, José Roberto Lambertucci, Anna Mae Diehl
BACKGROUND: Symptomatic acute schistosomiasis mansoni is a systemic hypersensitivity reaction against the migrating schistosomula and mature eggs after a primary infection. The mechanisms involved in the pathogenesis of acute schistosomiasis are not fully elucidated. Osteopontin has been implicated in granulomatous reactions and in acute hepatic injury. Our aims were to evaluate if osteopontin plays a role in acute Schistosoma mansoni infection in both human and experimentally infected mice and if circulating OPN levels could be a novel biomarker of this infection...
October 2016: PLoS Neglected Tropical Diseases
Owen M Slater, Jeanine Peters-Kennedy, Manigandan Lejeune, David Gummer, Bryan Macbeth, Amy Warren, Tomy Joseph, Hong Li, Cristina W Cunha, Pádraig J Duignan
Malignant catarrhal fever-like clinical disease was diagnosed in a free-ranging bighorn sheep ( Ovis canadensis ) from Alberta, Canada, in June 2015. Antemortem and gross pathology findings included muscle atrophy, marked weight loss, and bilaterally symmetric alopecia with hyperpigmentation and crusting over the face, medial surfaces of the pinnae, dorsal trunk, distal limbs, perineal area, and tail. Histologically, the skin lesions were characterized by granulomatous mural folliculitis with numerous multinucleated giant cells and fewer lymphocytes and eosinophils consistent with previous reports of chronic ovine herpesvirus-2 (OvHV-2) infection...
October 17, 2016: Journal of Wildlife Diseases
Laila Alawdah, Ahmad Nahari, Dayel Alshahrani, Musa Fagih, Shahid Ghazi, Abdulrahman Al-Hussaini
Gastrointestinal sarcoidosis is a rare disease with very limited data in children. Here we report the first pediatric case of successful treatment with infliximab. The first case was an 8-year-old Saudi girl who presented with fever, weight loss, and abdominal pain that was followed in a few months with hematemesis and development of hepatosplenomegaly. The second case was a 9-year-old Sudanese boy who manifested with vomiting, epigastric pain, and weight loss. On upper endoscopy, both cases demonstrated severe erosive nodular gastric mucosa...
September 2016: Saudi Journal of Gastroenterology: Official Journal of the Saudi Gastroenterology Association
Selahattin Çaliskan, Emrah Özsoy, Selçuk Kaba, Orhan Koca, Metin Isak Öztürk
INTRODUCTION: Xanthogranulomatous pyelonephritis is a chronic destructive granulomatous inflammation of the kidney. This pathology was firstly described in 1916 and a small number of patient series were reported in the literature. In this study, we aimed to report the patients with xanthogranulomatous pyelonephritis in our nephrectomy cases. METHODS: The patients who underwent nephrectomy and were diagnosed with xanthogranulomatous pyelonephritis in our hospital database were reviewed retrospectively...
October 2016: Archives of Iranian Medicine
S F Lax
Endometritis is nowadays rare in developed countries and typically shows a subclinical or mild course; therefore, there are probably more cases of endometritis than diagnosed but they lack clinical relevance. In the fertile period of life it can be the reason for vaginal bleeding and infertility. The most common causes for non-specific endometritis are residual placental tissue after abortion or childbirth, intrauterine interventions, lesions within the uterine cavity, such as endometrial polyps, endometrial hyperplasia and neoplasms, intrauterine devices (IUD) and cervical stenosis...
October 13, 2016: Der Pathologe
Marta Maciejewska, Delphine Adam, Loïc Martinet, Aymeric Naômé, Magdalena Całusińska, Philippe Delfosse, Monique Carnol, Hazel A Barton, Marie-Pierre Hayette, Nicolas Smargiasso, Edwin De Pauw, Marc Hanikenne, Denis Baurain, Sébastien Rigali
Moonmilk speleothems of limestone caves host a rich microbiome, among which Actinobacteria represent one of the most abundant phyla. Ancient medical texts reported that moonmilk had therapeutical properties, thereby suggesting that its filamentous endemic actinobacterial population might be a source of natural products useful in human treatment. In this work, a screening approach was undertaken in order to isolate cultivable Actinobacteria from moonmilk of the Grotte des Collemboles in Belgium, to evaluate their taxonomic profile, and to assess their potential in biosynthesis of antimicrobials...
2016: Frontiers in Microbiology
Mallary C Greenlee-Wacker, William M Nauseef
Infections, especially with Staphylococcus aureus (SA), commonly cause morbidity and mortality in patients with chronic granulomatous disease (CGD), a condition characterized by a defective phagocyte oxidase. IFN-γ reduces the frequency and consequences of infection in CGD by mechanisms that remain unknown. As IFN-γ promotes bacterial killing, efferocytosis of effete polymorphonuclear neutrophils (PMN), and cytokine production in macrophages-the same macrophage effector functions that are impaired in response to SA-we hypothesized that IFN-γ may reverse these defects and thereby, augment macrophage control of SA during infection...
October 5, 2016: Journal of Leukocyte Biology
Aurélie Gabrion, Isabelle Hmitou, Despina Moshous, Bénédicte Neven, Alain Lefèvre-Utile, Jean-Sébastien Diana, Félipe Suarez, Capucine Picard, Stéphane Blanche, Alain Fischer, Marina Cavazzana, Fabien Touzot
BACKGROUND: Chronic granulomatous disease (CGD) is primary immunodeficiency caused by defective production of reactive oxygen species in phagocytic cells that results in life-threatening infections and severe inflammatory manifestations. The treatment of inflammatory manifestations remains challenging, as it can be associated with an increased risk of infections. Previous studies have shown that CGD phagocytes display a defect in autophagy and a reactive oxygen species-independent activation of the inflammasome...
October 1, 2016: Journal of Allergy and Clinical Immunology
Baruch Wolach, Ronit Gavrieli, Martin de Boer, Karin van Leeuwen, Sivan Berger-Achituv, Tal Stauber, Josef Ben Ari, Menachem Rottem, Yechiel Schlesinger, Galia Grisaru-Soen, Omar Abuzaitoun, Nufar Marcus, Ben Zion Garty, Arnon Broides, Jakov Levy, Polina Stepansky, Amos Etzioni, Raz Somech, Dirk Roos
Chronic granulomatous disease (CGD) is an innate immunodeficiency with a genetic defect of the nicotinamide adenosine dinucleotide phosphate, reduced, oxidase components. This leads to decreased reactive oxygen species (ROS) production, which renders patients susceptible to life-threatening infections. Over the course of 30 years, we diagnosed CGD in 84 patients from 61 families using functional, molecular and genetic studies. The incidence of CGD in Israel is 1.05 per 100,000 live-births in the Jewish population and 1...
October 4, 2016: American Journal of Hematology
Manasi Kulkarni, Mukesh Desai, Maya Gupta, Aparna Dalvi, Prasad Taur, Antony Terrance, Sunil Bhat, Mamta Manglani, Revathi Raj, Ira Shah, Manisha Madkaikar
Chronic granulomatous disease (CGD) is a group of inherited disorder of phagocytes, resulting from mutations in the components of the NADPH oxidase complex. Reduced or absent oxygen radical synthesis seen in these patients leads to impaired killing of intracellular bacteria and fungi. CGD clinically presents with recurrent and life-threatening infections as well as granulomatous inflammatory responses. p47(phox) encoded by the NCF1 gene is the most common autosomal recessive form of CGD which is often clinically milder...
October 3, 2016: Journal of Clinical Immunology
Neslihan Edeer Karaca, Guzide Aksu, Ezgi Ulusoy, Serap Aksoylar, Salih Gozmen, Ferah Genel, Sanem Akarcan, Nesrin Gulez, Tatjana Hirschmugl, Savas Kansoy, Kaan Boztug, Necil Kutukculer
Alterations of immune homeostasis in the gut may result in development of inflammatory bowel disease. A five-month-old girl was referred for recurrent respiratory and genitourinary tract infections, sepsis in neonatal period, chronic diarrhea, perianal abscess, rectovaginal fistula, and hyperemic skin lesions. She was born to second-degree consanguineous, healthy parents. Her elder siblings were lost at 4 months of age due to sepsis and 1 year of age due to inflammatory bowel disease, respectively. Absolute neutrophil and lymphocyte counts, immunoglobulin levels, and lymphocyte subsets were normal ruling out severe congenital neutropenia and classic severe combined immunodeficiencies...
2016: Case Reports in Immunology
Silvia Cellone Trevelin, Célio Xavier Dos Santos, Raphael Gomes Ferreira, Larissa de Sá Lima, Rangel Leal Silva, Cristoforo Scavone, Rui Curi, José Carlos Alves-Filho, Thiago Mattar Cunha, Pérsio Roxo-Júnior, Maria-Célia Cervi, Francisco Rafael Martins Laurindo, John Stephen Hothersall, Andrew M Cobb, Min Zhang, Aleksandar Ivetic, Ajay M Shah, Lucia Rossetti Lopes, Fernando Queiroz Cunha
The reactive-oxygen-species-(ROS)-generating-enzyme Nox2 is essential for leukocyte anti-microbial activity. However its role in cellular redox homeostasis and, consequently, in modulating intracellular signaling pathways remains unclear. Herein, we show Nox2 activation favors thioredoxin-1 (TRX-1)/p40phox interaction, which leads to exclusion of TRX-1 from the nucleus. In contrast, the genetic deficiency of Nox2 or its pharmacological inhibition with apocynin (APO) results in reductive stress after lipopolysaccharide-(LPS)-cell stimulation, which causes nuclear accumulation of TRX-1 and enhanced transcription of inflammatory mediators through nuclear-factor-(NF)-κB...
October 4, 2016: Scientific Reports
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