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Chronic granulomatous disease

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https://www.readbyqxmd.com/read/29145578/the-changing-paradigm-of-management-of-liver-abscesses-in-chronic-granulomatous-disease
#1
David M Straughan, Kaitlin C McLoughlin, John E Mullinax, Beatriz E Marciano, Alexandra F Freeman, Victoria L Anderson, Gulbu Uzel, Sa D C Azoury, Rebecca Sorber, Humair S Quadri, Harry L Malech, Suk See DeRavin, Natasha Kamal, Christopher Koh, Christa S Zerbe, Douglas B Kuhns, John I Gallin, Theo Heller, Steven M Holland, Udo Rudloff
Background: Chronic granulomatous disease (CGD) is a rare genetic disorder causing recurrent infections. Over one quarter of patients develop hepatic abscesses and liver dysfunction. Recent reports suggest disease-modifying treatment with corticosteroids is effective for these abscesses. Comparison of corticosteroid therapy to traditional invasive treatments has not been performed. Methods: Records of 268 patients with CGD treated at the National Institutes of Health (NIH) from 1980 to 2014 were reviewed...
November 14, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/29145499/clinical-tool-for-disease-phenotyping-in-granulomatous-lung-disease
#2
Lori J Silveira, Matthew Strand, Michael V Van Dyke, Margaret M Mroz, Anna V Faino, Dana M Dabelea, Lisa A Maier, Tasha E Fingerlin
BACKGROUND: Exposure to beryllium may lead to granuloma formation and fibrosis in those who develop chronic beryllium disease (CBD). Although disease presentation varies from mild to severe, little is known about CBD phenotypes. This study characterized CBD disease phenotypes using longitudinal measures of lung function. METHODS: Using a case-only study of 207 CBD subjects, subject-specific trajectories over time were estimated from longitudinal pulmonary function and exercise-tolerance tests...
2017: PloS One
https://www.readbyqxmd.com/read/29141716/hansen-s-disease-and-rheumatoid-arthritis-crossover-of-clinical-symptoms-a-case-series-of-18-patients-in-the-united-states
#3
Sarah M Labuda, John S Schieffelin, Jeffrey G Shaffer, Barbara M Stryjewska
Hansen's Disease (HD) is a rare, chronic granulomatous infection of the skin and peripheral nerves caused by the noncultivable organism Mycobacterium leprae. Arthritis is the third most common symptom of HD. Subjects with both confirmed HD on skin biopsy and chronic arthritis were identified at the National Hansen's Disease Program (NHDP). We conducted a series of medical chart reviews and extracted and logged personally deidentified data into a database and carried out descriptive analyses. Eighteen of 261 subjects presented to the NDHP with both HD and chronic arthritis between 2001 and 2015...
October 23, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/29135572/long-term-observational-studies-of-chronic-granulomatous-disease
#4
Maria Kanariou, Kleopatra Spanou, Sofia Tantou
PURPOSE OF REVIEW: Chronic granulomatous disease (CGD) is a primary immunodeficiency, with a defect of phagocytes in killing specific pathogens. CGD is characterized by severe recurrent bacterial and fungal infections and dysregulated inflammatory response. Since its first description as fatal disease about 60 years ago, a significant improvement in outcome has been achieved in the last 20 years. The purpose of this review is to framework recent advances in CGD immunopathogenesis, management of disease manifestation and cure of CGD patients...
November 10, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29132925/successful-transcatheter-arterial-antimicrobial-and-steroid-therapy-for-refractory-liver-abscess-in-chronic-granulomatous-disease-a-case-report-and-review-of-literature
#5
Taito Kitano, Yuki Nishikawa, Satoru Sueyoshi, Noriko Horikawa, Hiroyuki Nakagawa, Sayaka Yoshida
Hepatic abscess in chronic granulomatous disease (CGD) is very refractory and frequently requires multiple surgeries with frequent morbidities. Although surgical interventions are often required, patients are often not able to have surgery for various reasons. We present the case of a 21-year-old man with recurrent hepatic abscess in CGD. We could not provide surgical interventions due to the lack of a fluid cavity and the patient's refusal, and therefore we administered transcatheter arterial antimicrobial and steroid therapy...
November 10, 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/29132304/the-three-cyba-variants-rs4673-rs1049254-and-rs1049255-are-benign-new-evidence-from-a-patient-with-cgd
#6
Jinqiao Sun, Min Wen, Ying Wang, Danru Liu, Wenjing Ying, Xiaochuan Wang
BACKGROUND: Chronic granulomatous disease (CGD) is an inherited immunodeficiency disease caused by the defect of NADPH oxidase. Mutations in CYBB or CYBA gene may result in membrane subunits, gp91phox or p22phox, expression failure respectively and NADPH oxidase deficiency. Previous study showed that three variants, c.214 T > C (rs4673), c.521 T > C (rs1049254) and c.(*)24G > A (rs1049255), in CYBA gene form a haplotype, which are associated with decreased reactive oxygen species generation...
November 13, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/29122311/-uveitis-diagnostic-work-up-recommendations-from-an-expert-committee
#7
P Sève, B Bodaghi, S Trad, J Sellam, D Bellocq, P Bielefeld, D Sène, G Kaplanski, D Monnet, A Brézin, M Weber, D Saadoun, P Cacoub, C Chiquet, L Kodjikian
INTRODUCTION: Diagnostic work-up of uveitis involves many uncertainties. Search for an etiology should take into account the epidemiology of uveitis and focus on the most severe diseases or those, which can be treated. This work was undertaken to establish recommendations for the diagnosis work-up of uveitis. METHODS: Recommendations were developed by a multidisciplinary panel of 15 experts, including internists, ophthalmologists and a rheumatologist and are based on a review of the literature with regard to effectiveness of investigations and the results of the ULISSE study, which is the first prospective study assessing the efficiency of a standardized strategy for the etiological diagnosis of uveitis...
November 6, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29114418/stop-being-so-sensitive-an-exceptionally-rare-report-of-ustekinumab-induced-sub-acute-hypersensitivity-pneumonitis
#8
Azka Ali, Jason Chertoff, Christopher Harden, Dara Wakefield, James Wynne
Hypersensitivity pneumonitis (HSP) is a rare syndrome characterised by granulomatous inflammatory lung disease due to repeated sensitisation from a specific antigen. We present the case of a 61-year old male veteran with a history of nodular eczema who presented with 2 weeks of progressive dyspnoea on exertion and pleuritic chest pain. The patient was started on ustekinumab 5 weeks prior to presentation. Initial workup revealed ground-glass opacities on computed tomography (CT) scan of the chest. Cardiac workup was unrevealing with a normal myocardial perfusion stress test...
October 2017: Turkish Journal of Anaesthesiology and Reanimation
https://www.readbyqxmd.com/read/29111085/il-37-and-leprosy-a-novel-cytokine-involved-in-the-host-response-to-mycobacterium-leprae-infection
#9
Jorge Rodrigues de Sousa, Renata Lima Prudente, Leonidas Braga Dias Junior, Francisca Regina Oliveira Carneiro, Mirian Nacagami Sotto, Juarez Antonio Simões Quaresma
Leprosy is a chronic infectious granulomatous disease caused by Mycobacterium leprae, in which the clinical outcome depends on the pattern of the host immune response. Because it is a spectral disease, leprosy is a good model for studying the immunology of the pathogen-host relationship. Although previous studies have characterized the participation of cytokine profiles such as Th1, Th2, Th7, Treg, Th9, and Th22 responses in leprosy, the role of new cytokines such as IL-37 have not yet been described for the spectral model of the disease...
October 27, 2017: Cytokine
https://www.readbyqxmd.com/read/29098128/hot-tub-lung-a-diagnostic-challenge
#10
Hassaan Yasin, William E Mangano, Paras Malhotra, Ali Farooq, Hesham Mohamed
Hot tub lung (HTL) is a granulomatous lung disease thought to occur as a result of a hypersensitivity response to non-tuberculous mycobacteria (NTM). Typical radiographic findings are diffuse micronodular and/or ground glass opacities. We report an interesting case of HTL that presented with unique radiographic features, making its diagnosis a predicament. A 56-year-old immunocompetent female with chronic dyspnea and dry cough was found to have subcentimeter cavitary nodules, predominantly in the upper lung zones...
August 27, 2017: Curēus
https://www.readbyqxmd.com/read/29097349/spectrum-of-imaging-findings-of-chronic-granulomatous-disease-a-single-center-experience
#11
Minah Lee, Mu Sook Lee, Jeong Sub Lee, Su Yeon Ko, Sun Young Jeong
The purpose of this pictorial essay is to present and summarize findings of various images of chronic granulomatous disease (CGD). CGD represents a heterogeneous group of disorders caused by defective generation of respiratory bursts in human phagocytes. This defect results in abnormal phagocytic functions and defective killing of bacteria by phagocytes. CGD may involve many organs and present with recurrent infections and inflammations. Radiologists should consider the possibility of CGD when a patient presents with atypical and recurrent infection...
November 2017: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
https://www.readbyqxmd.com/read/29096002/balamuthia-mandrillaris-granulomatous-amebic-encephalitis-with-renal-dissemination-in-a-previously-healthy-child-case-report-and-review-of-the-pediatric-literature
#12
Kareem W Shehab, Khalid Aboul-Nasr, Sean P Elliott
Balamuthia mandrillaris is a recently described ameba known to cause a subacute to chronic central nervous system infection called granulomatous amebic encephalitis. Evidence suggests that apparently immunocompetent persons are at risk for disease and show a similar nonspecific presentation to that of immunodeficient persons. However, evidence of hematogenous dissemination, which has been found in immunodeficient patients, has been lacking in immunocompetent patients. Here, we describe a previously healthy patient with B mandrillaris-associated granulomatous amebic encephalitis in whom both central nervous system and renal disease were found during autopsy, which suggests hematogenous dissemination...
October 31, 2017: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29094889/chronic-rhinosinusitis
#13
Ahmad R Sedaghat
Chronic rhinosinusitis is an inflammatory disease of the paranasal sinuses that occurs in 1% to 5% of the U.S. POPULATION: It may significantly decrease quality of life. Chronic rhinosinusitis is defined by the presence of at least two out of four cardinal symptoms (i.e., facial pain/pressure, hyposmia/anosmia, nasal drainage, and nasal obstruction) for at least 12 consecutive weeks, in addition to objective evidence. Objective evidence of chronic rhinosinusitis may be obtained on physical examination (anterior rhinoscopy, endoscopy) or radiography, preferably from sinus computed tomography...
October 15, 2017: American Family Physician
https://www.readbyqxmd.com/read/29090204/subcutaneous-sarcoidosis-occurring-in-both-chin-and-toe
#14
Kwang Seog Kim, Dong Seob Lim, Jun Ho Choi, Jae Ha Hwang, Sam Yong Lee
Sarcoidosis is a systemic inflammatory disease characterized by non-caseating granulomas of unknown origin. Of the fewer than 6% of sarcoidosis cases that occur in subcutaneous tissue, most occur on the face or forearm, but rarely in the toe. A 33-year-old man was admitted to our institute with a 2-cm mass on his chin and a 0.5-cm mass on his right fourth toe. Based on preoperative ultrasonography, epidermal cysts were suspected, and histopathological tests were performed after removing the masses. Histopathologically, the chin and toe tissue samples showed chronic granulomatous inflammation, without necrosis, indicative of sarcoidosis...
September 2017: Arch Craniofac Surg
https://www.readbyqxmd.com/read/29081405/the-enigma-of-granulomatous-mastitis-a-series
#15
Muhammad Umair Bashir, Alexius Ramcharan, Sarah Alothman, Sabine Beaugris Pa-C, Sarosh Ahmad Khan, Mohammed A Sbeih, Ryan Engdahl
Granulomatous mastitis is a chronic inflammatory breast disease with an enigmatic clinical presentation that can delay diagnosis and perpetuate ineffective treatments. We report our experience with the diagnosis and management of eighteen cases of granulomatous mastitis in the setting of an urban public hospital. The patients were identified after a retrospective review of pathology and surgery databases. Demographic, clinical, radiographic, histopathological data and treatment responses were reviewed. Out of a total of 18 patients, 8 patients were noted to be from Mexico...
February 20, 2017: Breast Disease
https://www.readbyqxmd.com/read/29080069/5-aminosalicylic-acid-modulates-the-immune-response-in-chronic-beryllium-disease-subjects
#16
Brian J Day, Jie Huang, Briana Q Barkes, May Gillespie, Li Li, Lisa A Maier
INTRODUCTION: Chronic beryllium disease (CBD) is characterized by accumulation of macrophages and beryllium-specific CD4(+) T cells that proliferate and produce Th1 cytokines. 5-Amino salicylic acid (5-ASA) is currently used to treat inflammatory bowel disease and has both antioxidant and anti-inflammatory actions. We hypothesized that 5-ASA may be a beneficial therapeutic in CBD. METHODS: Seventeen CBD patients were randomized 3:1 to receive 5-ASA 500-mg capsules or placebo four times daily for 6 weeks orally...
October 27, 2017: Lung
https://www.readbyqxmd.com/read/29075621/pancreatic-involvement-in-pediatric-inflammatory-bowel-disease
#17
REVIEW
Javier Martín-de-Carpi, Melinda Moriczi, Gemma Pujol-Muncunill, Victor M Navas-López
Inflammatory bowel disease (IBD) is a chronic condition that includes two clinical entities: Crohn's disease and ulcerative colitis. Although both entities mainly affect the gastrointestinal tract are considered multisystemic diseases and may present extraintestinal manifestations involving other organs and systems. Pancreatic involvement in Pediatric IBD includes a heterogeneous group of clinical entities like acute pancreatitis, chronic pancreatitis, autoimmune pancreatitis, asymptomatic exocrine pancreatic insufficiency, increased pancreatic enzyme levels, structural abnormalities, and granulomatous inflammation...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29063637/phenotypic-prenatal-diagnosis-of-chronic-granulomatous-disease-cgd-a-useful-tool-in-the-absence-of-molecular-diagnosis
#18
Manasi Kulkarni, Maya Gupta, Manisha Madkaikar
Chronic Granulomatous Disease (CGD) is an inherited immunodeficiency disorder affecting the microbicidal function of the phagocytes. It is characterized by susceptibility to recurrent infections leading to significant morbidity and mortality. Antibacterial and antifungal prophylaxis though has significantly reduced the rate and severity of the infections; the breakthrough infections still remain a challenge. Currently allogenic hematopoietic stem cell transplantation is the only curative option which is very expensive and unavailable for many due to lack of suitable donor...
October 24, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29062247/frequency-of-mycobacterium-bovis-and-mycobacteria-in-primary-immunodeficiencies
#19
Ezgi Ulusoy, Neslihan Edeer Karaca, Güzide Aksu, Cengiz Çavuşoğlu, Necil Kütükçüler
AIM: Susceptibility to mycobacterial diseases is observed in some primary immunodeficiency diseases. In this study, we aimed to evaluate mycobacterial infections in primary immunodeficiency diseases. MATERIAL AND METHODS: Patients under follow-up by Ege University Pediatric Immunology Department for severe combined and combined immunodeficiencies, interleukin 12/ interferon gamma receptor deficiency, nuclear factor kappa-beta essential modulator deficiency and chronic granulomatosis disease were evaluated retrospectively in terms of the frequency and characteristics of mycobacterial infections using a questionnaire form for demographic properties, clinical features and laboratory tests...
September 2017: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/29050515/cytologic-and-ultrastructural-findings-of-bronchoalveolar-lavage-in-patients-with-chronic-granulomatous-disease
#20
Mikako Warren, Hiroyuki Shimada
Background Chronic granulomatous disease (CGD) is a hereditary immunodeficiency caused by mutations in genes encoding nicotinamide adenine dinucleotide phosphate oxidase enzyme complex, which lead to the inability to kill intracellular pathogens. Patients with CGD are susceptible to recurrent bacterial and fungal infections in their early lives. Although the recent survival rate has been significantly improved, early diagnosis is critical to prevent multiple organ impairment. In 1950s, CGD was first described as a disease with recurrent infections and visceral infiltration of granulomas and pigmented histiocytes...
January 1, 2017: Pediatric and Developmental Pathology
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