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Chronic granulomatous disorder

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https://www.readbyqxmd.com/read/29204384/approach-to-a-child-with-primary-immunodeficiency-made-simple
#1
REVIEW
Dhrubajyoti Sharma, Ankur K Jindal, Amit Rawat, Surjit Singh
Primary immunodeficiency disorders (PIDs) are a group of disorders affecting the capability to fight against infection. These include defects in T cells and B cells affecting cell-mediated and humoral immunity, respectively, combined humoral and cell-mediated immunodeficiency, defects in phagocytosis, complement defects, and defects in cytokine or cytokine signalling pathways which are detrimental for immune function. Depending upon the type and severity, age at onset of symptoms can vary from neonatal period to late childhood...
November 2017: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/29145578/the-changing-paradigm-of-management-of-liver-abscesses-in-chronic-granulomatous-disease
#2
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/29102585/laryngeal-sarcoidosis-in-a-child-case-report
#3
Gabriel Vega-Cornejo, Priscila Ayala-Buenrostro
Sarcoidosis is a chronic, multisystemic, granulomatous disorder. Our patient was a 2-year-old girl with multiple airway conditions and a partial response to inhaled and systemic steroids. She was positive for acute phase reactants and negative for antibodies. Polymerase chain reaction revealed atypical Mycobacteria and she was negative for Mycobacterium tuberculosis. Laryngeal sarcoidosis was diagnosed by histopathology in a biopsy of larynx that revealed a chronic granulomatous inflammatory process with Langhans giant cells and acute and ulcerated areas with changes compatible with tuberculosis...
November 1, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/29097349/spectrum-of-imaging-findings-of-chronic-granulomatous-disease-a-single-center-experience
#4
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/29063637/phenotypic-prenatal-diagnosis-of-chronic-granulomatous-disease-cgd-a-useful-tool-in-the-absence-of-molecular-diagnosis
#5
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/28970295/infectious-and-non-infectious-complications-in-primary-immunodeficiency-disorders-an-autopsy-study-from-north-india
#6
Kirti Gupta, Amit Rawat, Parimal Agrawal, Ankur Jindal, Ritambhra Nada, Biman Saikia, Koon Wing Chan, Yu Lung Lau, Ranjana Walker Minz, Surjit Singh
BACKGROUND: Primary immunodeficiency disorders (PID) include a wide spectrum of inherited disorders characterised by functional abnormalities of one or more components of the immune system. Recent updates from the genomic data have contributed significantly to its better understanding with identification of new entities. Diagnosis is always challenging due to their variable clinical presentation. With the evolution of molecular diagnosis, many of these children are being diagnosed early and offered appropriate therapy...
September 28, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28955342/mass-cytometry-identifies-distinct-lung-cd4-t-cell-patterns-in-l%C3%A3-fgren-s-syndrome-and-non-l%C3%A3-fgren-s-syndrome-sarcoidosis
#7
Ylva Kaiser, Tadepally Lakshmikanth, Yang Chen, Jaromir Mikes, Anders Eklund, Petter Brodin, Adnane Achour, Johan Grunewald
Sarcoidosis is a granulomatous disorder of unknown etiology, characterized by accumulation of activated CD4(+) T cells in the lungs. Disease phenotypes Löfgren's syndrome (LS) and "non-LS" differ in terms of clinical manifestations, genetic background, HLA association, and prognosis, but the underlying inflammatory mechanisms largely remain unknown. Bronchoalveolar lavage fluid cells from four HLA-DRB1*03(+) LS and four HLA-DRB1*03(-) non-LS patients were analyzed by mass cytometry, using a panel of 33 unique markers...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28941269/tuberculoid-leprosy-masquerading-as-systemic-lupus-erythematosus-an-interesting-observation
#8
Vijay Zawar, Shrikant Kumavat, Manoj Pawar, Dipti Desai
Leprosy is a chronic granulomatous infectious multisystem disease that may present with protean manifestations. It mimics many systemic and dermatological disorders. Here we report a case in which an elderly female presented with malar rash, intermittent fever, and arthralgia. Her diagnosis was significantly delayed due to a close clinical resemblance to systemic lupus erythematosus. It is important to be aware of such manifestations of leprosy and improve awareness of it in clinicians to avoid misdiagnosis and delay in treatment...
September 2017: Acta Dermatovenerologica Alpina, Panonica, et Adriatica
https://www.readbyqxmd.com/read/28914984/hypercalcemic-disorders-in-children
#9
REVIEW
Victoria J Stokes, Morten F Nielsen, Fadil M Hannan, Rajesh V Thakker
Hypercalcemia is defined as a serum calcium concentration that is greater than two standard deviations above the normal mean, which in children may vary with age and sex, reflecting changes in the normal physiology at each developmental stage. Hypercalcemic disorders in children may present with hypotonia, poor feeding, vomiting, constipation, abdominal pain, lethargy, polyuria, dehydration, failure to thrive, and seizures. In severe cases renal failure, pancreatitis and reduced consciousness may also occur and older children and adolescents may present with psychiatric symptoms...
November 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28895850/gene-therapy-approaches-to-immunodeficiency
#10
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/28871936/xanthoma-like-skin-changes-in-an-elderly-woman-with-a-normal-lipid-profile
#11
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/28856149/is-idiopathic-granulomatous-mastitis-a-surgical-disease-the-jury-is-still-out
#12
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/28828164/oral-manifestations-as-the-first-presenting-sign-of-crohn-s-disease-in-a-pediatric-patient
#13
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
https://www.readbyqxmd.com/read/28826742/a-comprehensive-review-and-update-on-crohn-s-disease
#14
Mahesh Gajendran, Priyadarshini Loganathan, Anthony P Catinella, Jana G Hashash
The term inflammatory bowel disease (IBD) refers principally to two major categories of chronic relapsing inflammatory intestinal disorders: Crohn's disease (CD) and ulcerative colitis (UC). In the United States, it is currently estimated that about 1.5 million people suffer from IBD, causing considerable suffering, mortality and economic loss every year. Yet the cause of IBD is unknown, and until we understand more, prevention or cure will not be possible. There is a lot of variation in the incidence and prevalence of CD based on geographic region, environment, immigrant population, and ethnic groups...
August 18, 2017: Disease-a-month: DM
https://www.readbyqxmd.com/read/28794542/mycetoma-an-update
#15
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/28727298/nine-patients-with-chronic-granulomatous-disease-having-selective-neck-dissection-for-severe-cervical-lymphadenitis
#16
L R Wingfield, J Liu, M Hu, D Bianchi, K Hauck, B Driscoll, J I Gallin, H L Malech, S M Holland, C Van Waes
No abstract text is available yet for this article.
July 20, 2017: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28681028/osteomyelitis-and-lung-abscess-due-to-aspergillus-fumigatus-in-a-chronic-granulomatous-disease-patient
#17
S Mamishi, K Zomorodian, F Saadat, S Z Jalali, M Geramishoar
BACKGROUND AND PURPOSE: Chronic granulomatous disease (CGD) is an inherited disorder of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. This disorder results in recurrent life-threatening bacterial and fungal infections. Aspergillus species are the most common fungal infections in these patients. CASE REPORT: Herein, we present a case of fungal infection in a girl with CGD. We confirmed aspergillosis through the positive microscopic and macroscopic examinations, as well as radiology results...
September 2016: Advances in Medical Mycology (Iran)
https://www.readbyqxmd.com/read/28650935/fulminant-sepsis-due-to-granulibacter-bethesdensis-in-a-4-year-old-boy-with-x-linked-chronic-granulomatous-disease
#18
Erick F Mayer, Philip Gialanella, Iona Munjal, Charlotte Cunningham-Rundles, Jasmeen Dara
Granulibacter bethesdensis is a Gram-negative bacillus described as a pathogen exclusively in patients with chronic granulomatous disease, a phagocytic disorder that impairs the ability to clear catalase-producing organisms. Granulibacter usually causes chronic and recurrent lymphadenopathies. We report the fatal case of a 4-year-old boy with chronic granulomatous disease, who presented with sepsis after a few days of abdominal pain and diarrhea.
December 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28638981/use-of-systemic-glucocorticoids-and-the-risk-of-major-osteoporotic-fractures-in-patients-with-sarcoidosis
#19
O A Oshagbemi, J H M Driessen, A Pieffers, E F M Wouters, P Geusens, P Vestergaard, J van den Bergh, F M E Franssen, F de Vries
This study revealed the risk of major osteoporotic fracture in patients with sarcoidosis exposed to glucocorticoids. Current use of glucocorticoids was associated with a risk of fracture, with no difference between patients with and without sarcoidosis. Sarcoidosis per se was not associated with an increased fracture risk. INTRODUCTION: Sarcoidosis is a multi-organ, chronic inflammatory, granulomatous disorder that most frequently affects the lungs, lymph nodes, skin, eyes, and liver, but may occur in any organ, including the bones...
June 21, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/28611513/brain-changes-detected-by-functional-magnetic-resonance-imaging-and-spectroscopy-in-patients-with-crohn-s-disease
#20
REVIEW
Kun Lv, Yi-Hong Fan, Li Xu, Mao-Sheng Xu
Crohn's disease (CD) is a chronic, non-specific granulomatous inflammatory disorder that commonly affects the small intestine and is a phenotype of inflammatory bowel disease (IBD). CD is prone to relapse, and its incidence displays a persistent increase in developing countries. However, the pathogenesis of CD is poorly understood, with some studies emphasizing the link between CD and the intestinal microbiota. Specifically, studies point to the brain-gut-enteric microbiota axis as a key player in the occurrence and development of CD...
May 28, 2017: World Journal of Gastroenterology: WJG
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