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Chronic granulomatous disease

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https://www.readbyqxmd.com/read/28920786/cryptococcus-neoformans-var-grubii-associated-renal-amyloidosis-causing-protein-losing-nephropathy-in-a-red-kangaroo-macropus-rufus
#1
Mary Irene Thurber, Jenessa Gjeltema, Matthew Sheley, Ray F Wack
A 10-year-old male castrated red kangaroo (Macropus rufus) presented with mandibular swelling. Examination findings included pitting edema with no dental disease evident on examination or radiographs. The results of blood work were moderate azotemia, hypoalbuminemia, and severely elevated urine protein:creatinine ratio (9.9). Radiographs showed an interstitial pattern of the caudal right lung, and an abdominal ultrasound demonstrated scant effusion. Symptomatic and empirical therapy with antibiotics, anti-inflammatory drugs, and an angiotensin-converting enzyme (ACE) inhibitor did not resolve clinical signs...
September 2017: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
https://www.readbyqxmd.com/read/28919373/treatment-of-chronic-granulomatous-disease-associated-fistulising-colitis-with-vedolizumab
#2
Nicholas Campbell, Hugo Chapdelaine
No abstract text is available yet for this article.
September 14, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28917708/type-2-diabetes-mellitus-balb-c-mice-are-more-susceptible-to-granulomatous-amoebic-encephalitis-immunohistochemical-study
#3
Maritza Omaña-Molina, Raquel Sanchez-Rocha, Dolores Hernandez-Martinez, Miriam Romero Grijalva, Citlaltepetl Salinas-Lara, Miriam Rodriguez-Sosa, Imelda Juarez-Avelar, Lizbeth Salazar-Villatoro, Arturo Gonzalez-Robles, Adolfo Rene Mendez-Cruz, Patricia Aley-Medina, Jesus Espinosa-Villanueva, Ismael Castelan-Ramirez, Jacob Lorenzo-Morales
Granulomatous amoebic encephalitis (GAE) is a chronic, difficult to resolve infection caused by amphizoic amoebae of the genus Acanthamoeba, which in most cases occurs in immunosuppressed persons or with chronic diseases such as diabetes. In this study, we describe the early events of A. culbertsoni infection of GAE in diabetic mice model. Diabetes was induced in male BALB/c mice, with a dose of streptozotocin (130 mg/kg). Healthy and diabetic mice were inoculated via intranasal with 1 × 10(6) trophozoites of A...
September 13, 2017: Experimental Parasitology
https://www.readbyqxmd.com/read/28914578/the-choroidal-vascularity-index-decreases-and-choroidal-thickness-increases-in-vogt-koyanagi-harada-disease-patients-during-a-recurrent-anterior-uveitis-attack
#4
Shulin Liu, Liping Du, Qingyun Zhou, Qi Zhang, Ke Hu, Jian Qi, Liang Liang, Chunjiang Zhou, Aize Kijlstra, Peizeng Yang
PURPOSE: To measure changes in the choroidal vascularity index (CVI) in chronic Vogt-Koyanagi-Harada (VKH) disease during a recurrent anterior uveitis attack. METHODS: Forty VKH patients and 40 normal controls were included in this study. Choroidal images were recorded before and during a recurrent anterior uveitis attack, as well as after appropriate treatment. CVI was measured by the binarization technique using ImageJ software (Bethesda, MD). RESULTS: The CVI was 0...
September 15, 2017: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/28911184/subcellular-localization-and-function-study-of-a-secreted-phospholipase-c-from-nocardia-seriolae
#5
Liqun Xia, Haiying Liang, Liang Xu, Jianlin Chen, Michaël Bekaert, Honglian Zhang, Yishan Lu
Fish nocardiosis is a chronic systemic granulomatous disease, and Nocardia seriolae is the main pathogen that causes it. The pathogenesis and virulence factors of N. seriolae are not fully understood. A phospholipase C (PLC), which is likely to be a secreted protein targeting host cell mitochondria, was found by a bioinformatics analysis of the whole genome sequence of N. seriolae. In order to determine the subcellular localization and study the preliminary function of PLC from N. seriolae (NsPLC), in this study gene cloning, secreted protein identification, subcellular localization in host cells and apoptosis detection of NsPLC were carried out...
September 15, 2017: FEMS Microbiology Letters
https://www.readbyqxmd.com/read/28895850/gene-therapy-approaches-to-immunodeficiency
#6
REVIEW
Sujal Ghosh, H Bobby Gaspar
Transfer of gene-corrected autologous hematopoietic stem cells in patients with primary immunodeficiencies has emerged as a new therapeutic approach. Patients with various conditions lacking a suitable donor have been treated with retroviral vectors and a gene-addition strategy. Initial promising results were shadowed by the occurrence of malignancies in some of these patients. Current trials, developed in the last decade, use safer viral vectors to overcome the risk of genotoxicity and have led to improved clinical outcomes...
October 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/28893408/inflammatory-myopathy-associated-with-antimitochondrial-antibodies-a-distinct-phenotype-with-cardiac-involvement
#7
Jemima Albayda, Aamna Khan, Livia Casciola-Rosen, Andrea M Corse, Julie J Paik, Lisa Christopher-Stine
OBJECTIVE: In the context of clinical evaluations performed on our prospective myositis cohort, we noted a striking association of severe cardiac disease in myositis patients with antimitochondrial antibodies. We sought to review all cases of antimitochondrial antibody (AMA) associated myositis in our cohort to describe the clinical features of this disease subset. METHODS: We identified 7 patients with confirmed antimitochondrial antibodies who presented as an inflammatory myopathy...
June 13, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28887465/host-immune-status-specific-production-of-gliotoxin-and-bis-methyl-gliotoxin-during-invasive-aspergillosis-in-mice
#8
Janyce A Sugui, Stacey R Rose, Glenn Nardone, Muthulekha Swamydas, Chyi-Chia R Lee, Kyung J Kwon-Chung, Michail S Lionakis
Delayed diagnosis in invasive aspergillosis (IA) contributes to its high mortality. Gliotoxin (GT) and bis-methyl-gliotoxin (bmGT) are secondary metabolites produced by Aspergillus during invasive, hyphal growth and may prove diagnostically useful. Because IA pathophysiology and GT's role in virulence vary depending on the underlying host immune status, we hypothesized that GT and bmGT production in vivo may differ in three mouse models of IA that mimic human disease. We defined temporal kinetics of GT and bmGT in serum, bronchoalveolar lavage fluid (BALF) and lungs of A...
September 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28886419/mutation-in-an-exonic-splicing-enhancer-site-causing-chronic-granulomatous-disease
#9
Martin de Boer, Karin van Leeuwen, Judy Geissler, Bernd H Belohradsky, Taco W Kuijpers, Dirk Roos
In a male patient suffering from X-linked chronic granulomatous disease (CGD) we found a c.389G>T mutation in exon 5 of the CYBB gene. We have analyzed why 95% of the transcripts of this gene lacked exon 5, leading to a frameshift and premature termination codon. The mutation was located in a region comprising three putative exonic splicing enhancer binding sites, for SRSF1, SRFS2 and SRFS6, according to the ESEfinder Tool (http://rulai.cshl.edu/cgi-bin/tools/ESE3/esefinder.cgi). With the Analyser Splice Tool we calculated the probability of skipping of exon 5 in CYBB mRNA, and by means of Sroogle the number of putative binding motifs for splicing enhancer and splicing silencer proteins (http://astlab...
August 18, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28886065/hepatitis-c-virus-mediated-chronic-inflammation-and-tumorigenesis-in-the-humanised-immune-system-and-liver-mouse-model
#10
Zhiqiang Zheng, Ching Wooen Sze, Choong Tat Keng, Muthafar Al-Haddawi, Min Liu, Sue Yee Tan, Hwee Ling Kwek, Zhisheng Her, Xue Ying Chan, Bhaskar Barnwal, Eva Loh, Kenneth Tou En Chang, Thiam Chye Tan, Yee-Joo Tan, Qingfeng Chen
Hepatitis C is a liver disease caused by infection of the Hepatitis C virus (HCV). Many individuals infected by the virus are unable to resolve the viral infection and develop chronic hepatitis, which can lead to formation of liver cirrhosis and cancer. To understand better how initial HCV infections progress to chronic liver diseases, we characterised the long term pathogenic effects of HCV infections with the use of a humanised mouse model (HIL mice) we have previously established. Although HCV RNA could be detected in infected mice up to 9 weeks post infection, HCV infected mice developed increased incidences of liver fibrosis, granulomatous inflammation and tumour formation in the form of hepatocellular adenomas or hepatocellular carcinomas by 28 weeks post infection compared to uninfected mice...
2017: PloS One
https://www.readbyqxmd.com/read/28877057/pleuroparenchymal-fibroelastosis-a-review-of-histopathologic-features-and-the-relationship-between-histologic-parameters-and-survival
#11
Reena Khiroya, Claudio Macaluso, Maria A Montero, Athol U Wells, Felix Chua, Maria Kokosi, Toby M Maher, Anand Devaraj, Alexandra Rice, Elisabetta A Renzoni, Andrew G Nicholson
Pleuroparenchymal fibroelastosis (PPFE) is now a defined clinicopathologic entity in the updated 2013 ATS/ERS classification of idiopathic interstitial pneumonias (IIPs), which has led to a significant increase in cases being diagnosed at our institution. We have therefore reviewed 43 PPFE cases (58 biopsies in total) to assess whether any clinical or histopathologic features provide prognostic information. A semiquantatitive grading system was used to assess extent of fibroblastic foci, intra-alveolar fibroelastosis, visceral pleural fibrosis, chronic inflammation in areas of fibrosis, vascular fibrointimal thickening, and presence of granulomas...
September 4, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28871936/xanthoma-like-skin-changes-in-an-elderly-woman-with-a-normal-lipid-profile
#12
Piotr Nockowski, Zdzisław Woźniak, Adam Reich, Joanna Maj
Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Imaging assessment and ultrasound examination did not show any specific involvement of internal organs...
July 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/28871251/th17-inducing-cytokines-il-6-and-il-23-are-crucial-for-granuloma-formation-during-experimental-paracoccidioidomycosis
#13
Fabrine Sales Massafera Tristão, Fernanda Agostini Rocha, Daniela Carlos, Natália Ketelut-Carneiro, Camila Oliveira Silva Souza, Cristiane Maria Milanezi, João Santana Silva
Paracoccidioidomycosis (PCM), a chronic granulomatous disease caused by the thermally dimorphic fungus Paracoccidioides brasiliensis and Paracoccidioides lutzii, has the highest mortality rate among systemic mycosis. The T helper 1-mediated immunity is primarily responsible for acquired resistance during P. brasiliensis infection, while susceptibility is associated with Th2 occurrence. Th17 is a population of T CD4(+) cells that, among several chemokines and cytokines, produces IL-17A and requires the presence of IL-1, IL-6, and TGF-β for differentiation in mice and IL-23 for its maintenance...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28868142/recent-advances-in-understanding-and-treating-chronic-granulomatous-disease
#14
REVIEW
Andrew Gennery
A number of recent advances have been made in the epidemiology and treatment of chronic granulomatous disease. Several reports from developing regions describe the presentations and progress of local populations, highlighting complications due to Bacillus Calmette-Guérin vaccination. A number of new reports describe complications of chronic granulomatous disease in adult patients, as more survivors reach adulthood. The complications experienced by X-linked carriers are particularly highlighted in three new reports, confirming that infection and inflammatory or autoimmune conditions are more common and severe than previously recognised...
2017: F1000Research
https://www.readbyqxmd.com/read/28859990/immune-discrepancies-during-in%C3%A2-vitro-granuloma-formation-in-response-to-cutibacterium-formerly-propionibacterium-acnes-infection
#15
Guillaume Ghislain Aubin, Grâce Ada Da Silva, Yoshinobu Eishi, Cédric Jacqueline, Frédéric Altare, Stéphane Corvec, Karim Asehnoune
Cutibacterium (formerly Propionibacterium) acnes is involved in chronic/low-grade pathologies such as sarcoidosis or prosthetic joint infection (PJI). In these diseases, granulomatous structures are frequently observed. In this study, we induced a physiological granulomatous reaction in response to different well-characterized clinical C. acnes isolates in order to investigate the cellular process during granuloma formation. Three C. acnes isolates selected according to their origin (PJI, sarcoidosis and acne) were typed by MLST...
August 30, 2017: Anaerobe
https://www.readbyqxmd.com/read/28856149/is-idiopathic-granulomatous-mastitis-a-surgical-disease-the-jury-is-still-out
#16
Demetrios Moris, Christos Damaskos, Spyridon Davakis, Michail Vailas, Nikolaos Garmpis, Eleftherios Spartalis, Michael Kontos, Konstantinos Kontzoglou
Idiopathic granulomatous mastitis (IGM), is a rare entity of chronic inflammatory disorder of the breast of unknown etiology. Very few cases have been described so far, almost exclusively in women. Here we describe a case of IGM in a 53-year-old man presented with a right breast mass, progressively enlarging during the last 6 months. Due to the findings of clinical examination and CT-scan, the suspicion for a potentially malignant lesion was given and the decision for surgical resection was made. Microscopic analysis of the specimen showed non-caseating granulomas around mammary lobules, findings compatible with IGM...
August 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28856090/a-case-of-relapsed-lung-abscess-caused-by-eubacterium-brachy-infection-following-an-initial-diagnosis-of-pulmonary-actinomycosis
#17
Hideaki Yamakawa, Eri Hagiwara, Masahiro Hayashi, Takuma Katano, Kosuke Isomoto, Ryota Otoshi, Ryota Shintani, Satoshi Ikeda, Kaori Tanaka, Takashi Ogura
We report a rare case of lung abscess due to Eubacterium brachy. In this case, an analysis of the aspirate from frank pus revealed Gram-positive coccobacilli. We initially strongly suspected lung abscess associated with actinomycosis because of the chronic/recurrent clinical course and radio-pathological findings such as a granuloma lesion. Although a biochemical analysis revealed Actinomyces sp., 16S rRNA gene sequencing and a phylogenetic tree analysis of the isolated strain confirmed the presence of E. brachy...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28833001/dermoscopy-of-borderline-tuberculoid-leprosy
#18
Balachandra S Ankad, Punit S Sakhare
INTRODUCTION: Leprosy is a chronic granulomatous condition caused by Mycobacterium leprae, and borderline tuberculoid (BT) leprosy is the most common type of leprosy and presents with large, well- to ill-defined hypopigmented patches. It is known for atypical presentations. Dermoscopy of granulomatous conditions is well documented. However, there is no description of dermoscopy of BT leprosy in the literature. Authors evaluated the dermoscopic patterns of BT leprosy. CASE REPORT: Patients with BT leprosy were studied for dermoscopic patterns...
August 22, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28830888/nox5-and-p22phox-are-two-novel-regulators-of-human-monocytic-differentiation-into-dendritic-cells
#19
Viviana Marzaioli, Margarita Hurtado-Nedelec, Coralie Pintard, Asma Tlili, Jean-Claude Marie, Renato Monteiro, Marie-Anne Gougerot-Pocidalo, Pham My-Chan Dang, Jamel El-Benna
Dendritic cells (DCs) are a heterogeneous population of professional antigen-presenting cells and are key cells of the immune system, acquiring different phenotypes in accordance with their localization during the immune response. A subset of inflammatory DCs is derived from circulating monocytes (Mo), and has a key role in inflammation and infection. The pathways controlling Mo-DC differentiation are not fully understood. Our objective was to investigate the possible role of NADPH oxidases (NOXs) in Mo-DC differentiation...
August 22, 2017: Blood
https://www.readbyqxmd.com/read/28828164/oral-manifestations-as-the-first-presenting-sign-of-crohn-s-disease-in-a-pediatric-patient
#20
Ashley Eckel, Dale Lee, Gail Deutsch, Anthony Maxin, Dolphine Oda
Crohn's disease (CD) is a chronic inflammatory disorder affecting the gastrointestinal (GI) tract. Although the GI tract is the primary site of involvement, many patients, particularly in pediatric cases, first present with non-intestinal manifestations, including oral lesions. Oral manifestations of CD in children occur in around 50-80% of cases, and about 30% of CD cases in children occur first in the mouth. Recognizing such oral lesions in the pediatric population, and requesting a biopsy, may expedite the diagnosis of CD...
July 2017: Journal of Clinical and Experimental Dentistry
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