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Chronic granulomatous disease

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https://www.readbyqxmd.com/read/28333712/disseminated-lichtheimia-ramosa-infection-after-hematopoietic-stem-cell-transplantation-in-a-child-with-chronic-granulomatous-disease
#1
Michael Winstead, John Ozolek, Andrew Nowalk, John Williams, Mark Vander Lugt, Philana Lin
Mucormycosis is uncommon in patients with chronic granulomatous disease (CGD). We report a 7-year-old boy with X-linked CGD and absent oxidative burst who developed fatal Lichtheimia ramosa infection with fungal thrombosis of the kidneys, spleen, and other organs following hematopoietic stem cell transplantation (HSCT). Lichtheimia infection is rarely reported in patients with CGD and could be related to iatrogenic immunosuppression.
March 22, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28332028/infection-profile-in-chronic-granulomatous-disease-a-23-year-experience-from-a-tertiary-care-center-in-north-india
#2
Amit Rawat, Pandiarajan Vignesh, Avinash Sharma, Jitendra K Shandilya, Madhubala Sharma, Deepti Suri, Anju Gupta, Vikas Gautam, Pallab Ray, Shivaprakash M Rudramurthy, Arunaloke Chakrabarti, Kohsuke Imai, Shigeaki Nonoyama, Osamu Ohara, Yu L Lau, Surjit Singh
PURPOSE: Chronic granulomatous disease (CGD) is an inherited phagocytic disorder characterized by recurrent infections with usually catalase-positive organisms. Infections in CGD from developing countries are expected to be different from those in the Western countries. We report the profile of infections in children diagnosed with CGD from a tertiary care center in North India. METHODOLOGY: Case records of children diagnosed with CGD at Pediatric Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India, from August 1993 to April 2016 (23 years) were analyzed...
March 22, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28331742/negative-pressure-wound-therapy-in-chronic-inflammatory-breast-diseases
#3
Ozan Barış Namdaroğlu, Hilmi Yazıcı, Ahmet Mücteba Öztürk, Savaş Yakan, Mehmet Yıldırım, Ahmet Deniz Uçar, Nazif Erkan
Mastitis is inflammation of breast tissue that may or may not originate from an infection. Two different forms of mastitis have been described, lactational and non-lactational. Lactational mastitis is the most common type and generally conservative therapy that includes milk removal and physical therapy provides symptomatic relief, but antibiotic therapy is also needed. Common types of non-lactational mastitis are periductal mastitis and idiopathic granulomatous mastitis. Treatment includes antibiotics, drainage, and surgery, but usually this is a chronic process and a therapeutic management algorithm for chronic breast inflammation is unclear and has no consensus...
April 2016: J Breast Health (2013)
https://www.readbyqxmd.com/read/28331079/characterization-and-vaccine-potential-of-membrane-vesicles-produced-by-francisella-noatunensis-sup-orientalis-in-an-adult-zebrafish-model
#4
Leidy Lagos, Julia I Tandberg, Urska Repnik, Preben Boysen, Erik Ropstad, Deepa Varkey, Ian T Paulsen, Hanne C Winther-Larsen
Vaccine development against extracellular bacteria has been important for the sustainability of the aquaculture industry. In contrast, infections with intracellular pathogens remain largely an unresolved problem. Francisella noatunensis subspecies orientalis (Fno) are Gram-negative, facultative intracellular bacteria that cause the disease francisellosis in fish. Francisellosis is commonly characterized as a chronic granulomatous disease with high morbidity and can result in high mortality depending on the host...
March 22, 2017: Clinical and Vaccine Immunology: CVI
https://www.readbyqxmd.com/read/28329923/-actinomycosis-involving-chronic-pancreatitis-a-case-report-with-literature-review
#5
Seong Jae Yeo, Chang Min Cho, Min Kyu Jung, Ki Ju Kim, Myung Hi Kim, Seung Hyun Cho, Gab Chul Kim, An Na Seo
Actinomycosis is a slowly progressive, chronic infectious disease. It is caused by the genus Actinomyces, which are gram-positive anaerobic bacteria. It presents as a mass-like lesion, composed of bacterial nidus and characteristic granulomatous inflammatory fibrosis. As such, it has frequently been mistaken for a malignancy. Surgical resection is a common procedure in these patients prior to a definite diagnosis. Although actinomycosis can occur in a variety of regions, including oral-cervicofacial, thoracic, and abdominopelvic cavities, the involvement of the pancreas is very rare...
March 25, 2017: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
https://www.readbyqxmd.com/read/28329526/chronic-granulomatous-disease-as-a-risk-factor-for-cutaneous-lupus-in-childhood
#6
Sandrina Carvalho, Susana Machado, Rita Sampaio, Margarida Guedes, Júlia Vasconcelos, Diogo Semedo, Manuela Selores
Chronic granulomatous disease (CGD) is a primaryimmunodeficiency disorder that affects the phagocyticcells of the innate immune system. It is characterizedby recurrent or persistent infections with granulomaformation. Lupus-like lesions have been reported incarriers of CGD and less frequently, in patients withCGD. Immunological study in these patients areusually negative. We describe the case of an 8-yearoldboy with CGD who developed chronic and acutecutaneous lupus erythematous with angular cheilitis,oral ulcers, Raynaud phenomenon, and positiveserologies for antinuclear, anticentromere, and anti-Saccharomyces cerevisiae antibodies...
March 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28328616/borderline-tuberculoid-leprosy-masquerading-as-granuloma-annulare-a-clinical-and-histological-pitfall
#7
Tian Hao Zhu, Faranak Kamangar, Marc Silverstein, Maxwell A Fung
INTRODUCTION: Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae, an intracellular acid-fast bacillus that tends to infect the skin and peripheral nerves. Because of the wide array of cutaneous manifestation, diagnosis is not always straightforward, especially in nonendemic regions of the world such as the United States. CASE REPORT: The authors report an unusual case of borderline tuberculoid leprosy in an 80-year-old white woman from California...
April 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28328160/co-morbidities-in-severe-asthma-clinical-impact-and-management
#8
REVIEW
Celeste Porsbjerg, Andrew Menzies-Gow
Patients with severe asthma represent a minority of the total asthma population, but carry a majority of the morbidity and healthcare costs. Achieving better asthma control in this group of patients is therefore of key importance. Systematic assessment of patients with possible severe asthma to identify treatment barriers and triggers of asthma symptoms, including co-morbidities, improves asthma control and reduces healthcare costs and is recommended by international guidelines on management of severe asthma...
March 22, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28326206/chronic-hepatitis-with-liver-granulomas-in-a-patient-with-granuloma-annulare-a-case-report-and-review-of-the-literature
#9
Majid Alsahafi, Mohammed I AlJasser, Sunil Kalia, H M Yang, Alnoor Ramji
Granuloma annulare (GA) is a benign granulomatous skin disorder of unknown etiology. GA is rarely associated with liver diseases. We report a unique case of chronic hepatitis with liver granulomas in a patient with GA. Despite an extensive workup, no clear etiology for the hepatitis was found. Based on the possible immune pathophysiology of GA and the presence of liver granulomas, the patient was treated with prednisone and azathioprine which resulted in complete normalization of the liver enzymes and concurrent improvement of GA...
2017: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/28321344/pituitary-adenoma-and-hyperprolactinemia-accompanied-by-idiopathic-granulomatous-mastitis
#10
Sebahattin Destek, Vahit Onur Gul, Serkan Ahioglu, Kursat Rahmi Serin
Idiopathic granulomatous mastitis (IGM) is a rare chronic inflammatory disease of the breast, and its etiology remains not fully elucidated. IGM is observed more often in patients with autoimmune disease. Hyperprolactinemia is observed during pregnancy, lactation, and a history of oral contraceptive use. A 39-year-old patient with no history of oral contraceptive use presented with complaints such as redness, pain, and swelling in her left breast. Ultrasound and magnetic resonance imaging (MRI) revealed a suspicious inflamed mass lesion...
2017: Case Reports in Endocrinology
https://www.readbyqxmd.com/read/28320834/early-intracellular-trafficking-of-granulibacter-bethesdensis-in-human-macrophages
#11
Jessica Chu, Margery G Smelkinson, David W Dorward, Kol A Zarember, John I Gallin
Granulibacter bethesdensis is a Gram-negative bacterium that infects patients with Chronic Granulomatous Disease (CGD), a primary immunodeficiency marked by a defect in NOX2, the phagocyte nicotinamide adenine dinucleotide phosphate oxidase. Previous studies have shown that NOX2 is essential for killing of G. bethesdensis by neutrophils and monocytes and that bacteriostatic activity of monocyte derived macrophages (MDM) requires NOX2 and IFNγ-pretreatment. To determine if G. bethesdensis evades phagolysosomal killing, a host defense pathway intact in both normal and CGD MDM, or occupies a distinct intracellular niche in CGD MDM, we assessed the trafficking patterns of this organism...
March 20, 2017: Infection and Immunity
https://www.readbyqxmd.com/read/28318195/-serratia-infections-should-we-think-about-primary-immunodeficiencies
#12
Alicia Montaner Ramón, Laura Murillo Sanjuán, Cristina Martínez Faci, Carmelo Guerrero Laleona, Carmen Rodríguez-Vigil Iturrate
Chronic granulomatous disease (CGD) is a primary immunodeficiency with an incidence of 1/200,000-250,000 live births. CGD affects mainly male patients, most of the mutations being X-linked, and autosomal recessive forms occur more frequently in communities with greater numbers of consanguineous marriages. CGD is characterized by sensitivity to recurrent and severe bacterial and fungal infections, with formation of granulomas due to the inability of phagocytes to generate reactive oxygen compounds, necessary for the intracellular death of phagocytic microorganisms...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28300422/an-oral-lesion-as-the-primary-clinical-manifestation-of-sarcoidosis
#13
I Gill, J Siddiqi
An oral lesion as the first clinical presentation of sarcoidosis is extremely rare. We present the case of a 39-year-old woman who was referred to the oral and maxillofacial surgery department with a persistent asymptomatic nodular lesion in the hard palate. This was located adjacent to a grossly carious upper first molar and a provisional diagnosis of chronic periapical granuloma was made. An incisional biopsy of the lesion was carried out, which unexpectedly revealed the presence of a non-caseating granulomatous inflammatory reaction...
March 16, 2017: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/28294378/cutaneous-crohn-disease-with-superimposed-psoriasis-a-unique-case-with-overlapping-histology
#14
Ben J Friedman, Bahar Dasgeb, Jason B Lee
Crohn disease is an idiopathic, chronic inflammatory disorder of the gastrointestinal tract. We recently encountered a unique case in which a patient with longstanding Crohn disease presented with skin lesions with histopathologic features of both psoriasis and granulomatous inflammation suggestive of cutaneous Crohn disease. To our knowledge, this has not been described concomitantly in the same patient, in the same lesions. Review of the literature suggests that the intersection of these two histopathological reaction patterns may not be pure coincidence...
March 14, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28288455/diagnostic-tools-for-doctors-evaluation-of-tattoo-complications
#15
Jørgen Serup
Diagnosis of tattoo complications is a multi-facetted field since many clinical entities and disease mechanisms are represented. Infections, allergies, and pigment foreign body reactions with granuloma are the major groups. The clinician needs a structured approach to diagnosis and an armamentarium of standard tests. Diagnosis primarily builds on patient history, objective clinical examination, and punch biopsy, supplemented with microbiology testing, ultrasound scanning, and clinical photography. Evaluation of allergic tattoo reactions and allergy to pigments by patch testing is not applicable and has a falsely negative outcome except for the diagnosis of allergy to metals and preservatives...
2017: Current Problems in Dermatology
https://www.readbyqxmd.com/read/28288231/-primary-immunodeficiencies-in-seriously-ill-children-report-of-3-clinical-cases
#16
Leticia Yáñez, Pamela Lama, Carolina Rivacoba, Juanita Zamorano, María Angélica Marinovic
Primary immunodeficiency diseases (PID) are congenital disorders secondary to an impaired immune response. Infections, autoimmune disorders, atopy, and lymphoproliferative syndromes are commonly associated with this disorder. OBJECTIVE: To present and discuss 3 infants diagnosed with PID. CLINICAL CASES: The cases are presented of three patients with PID diagnosed during their first admission to a Paediatric Intensive Critical Care Unit. The first patient, a 4-month-old infant affected by a severe pneumonia, and was diagnosed as a Severe Combined Immunodeficiency Disease...
February 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28287132/crispr-cas9-generated-p47-phox-deficient-cell-line-for-chronic-granulomatous-disease-gene-therapy-vector-development
#17
Dominik Wrona, Ulrich Siler, Janine Reichenbach
Development of gene therapy vectors requires cellular models reflecting the genetic background of a disease thus allowing for robust preclinical vector testing. For human p47(phox)-deficient chronic granulomatous disease (CGD) vector testing we generated a cellular model using clustered regularly interspaced short palindromic repeats (CRISPR)/Cas9 to introduce a GT-dinucleotide deletion (ΔGT) mutation in p47(phox) encoding NCF1 gene in the human acute myeloid leukemia PLB-985 cell line. CGD is a group of hereditary immunodeficiencies characterized by impaired respiratory burst activity in phagocytes due to a defective phagocytic nicotinamide adenine dinucleotide phosphate (NADPH) oxidase...
March 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28282232/an-atypical-case-of-leishmaniasis-associated-with-chronic-duodenitis-in-a-dog
#18
Ignacio Ayala, Luis Jesus Bernal, Juan Diego Garcia-Martinez, Miguel Angel Gomez, Jose Antonio Navarro, Antonio Bernabe
We describe an atypical case of duodenal leishmaniasis in a boxer dog presenting with chronic diarrhea and poor general condition. Antidiarrheic therapy was previously administered without success and inflammatory bowel disease localized to the small intestine was suspected, given the chronic clinical signs and by ruling out other known causes of gastrointestinal inflammation. Endoscopic biopsy of duodenum showed a moderate increase in lamina propria lymphocytes, plasma cells, and macrophages. Basophilic bodies were seen in the cytoplasm of numerous macrophages, suggestive of Leishmania spp, confirmed by immunostaining, and a diagnosis of granulomatous duodenitis associated to Leishmania infection was made...
March 2017: Journal of the American Animal Hospital Association
https://www.readbyqxmd.com/read/28271562/erythema-nodosum-in-association-with-idiopathic-granulomatous-mastitis-a-case-series-and-review-of-the-literature
#19
R Fruchter, C Castilla, E Ng, M K Pomeranz, A N Femia
Idiopathic granulomatous mastitis (IGM) is a chronic inflammatory disease characterized by tender, erythematous, indurated breast plaques with associated edema, drainage, and scar formation.(1) IGM is often mistaken for breast carcinoma or infectious mastitis.(1,2) Histopathology readily distinguishes IGM from breast carcinoma, as the primary finding in IGM is granulomas centered around mammary lobules.(3) Nevertheless, differentiating IGM from bacterial mastitis and other mimickers, such as atypical mycobacterial infections or sarcoidosis, can be more difficult...
March 8, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28270364/new-frontiers-in-the-therapy-of-primary-immunodeficiency-from-gene-addition-to-gene-editing
#20
REVIEW
Donald B Kohn, Caroline Y Kuo
The most severe primary immune deficiency diseases (PIDs) have been successfully treated with allogeneic hematopoietic stem cell transplantation for more than 4 decades. However, such transplantations have the best outcomes when there is a well-matched donor available because immune complications, such as graft-versus-host disease, are greater without a matched sibling donor. Gene therapy has been developed as a method to perform autologous transplantations of a patient's own stem cells that are genetically corrected...
March 2017: Journal of Allergy and Clinical Immunology
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