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    6969 results match your criteria Chronic Granulomatous Disease

    1 OF 140

    Spontaneous Oral Mucosal Bleeding Unmasking Undiagnosed Sarcoidosis: A Case Report.
    Dent Update 2016 May;43(4):353-5
    Sarcoidosis is a multisystem, non-caseating, chronic, granulomatous disease affecting any organ. In the head and neck region, salivary glands and lymph nodes are most commonly involved. Oral manifestations are rare but these lesions, occasionally haemorrhagic, may be the first sign of systemic disease. Read More

    The Changing Paradigm of Management of Liver Abscesses in Chronic Granulomatous Disease.
    Clin Infect Dis 2017 Nov 14. Epub 2017 Nov 14.
    Thoracic and GI Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD.
    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. Read More

    Clinical tool for disease phenotyping in granulomatous lung disease.
    PLoS One 2017 16;12(11):e0188119. Epub 2017 Nov 16.
    Colorado School of Public Health, University of Colorado Anschutz Medical Campus, Aurora, Colorado, United States of America.
    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. Read More

    Hansen's Disease and Rheumatoid Arthritis Crossover of Clinical Symptoms: A Case Series of 18 Patients in the United States.
    Am J Trop Med Hyg 2017 Oct 23. Epub 2017 Oct 23.
    National Hansen's Disease Program, Baton Rouge, Louisiana.
    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). Read More

    Broadleaf Mahonia attenuates granulomatous lobular mastitis‑associated inflammation by inhibiting CCL‑5 expression in macrophages.
    Int J Mol Med 2017 Nov 9. Epub 2017 Nov 9.
    Department of Mammary Disease, Guangdong Provincial Hospital of Chinese Medicine, Guangzhou, Guangdong 510006, P.R. China.
    Granulomatous lobular mastitis (GLM) is a type of chronic mammary inflammation with unclear etiology. Currently systematic corticosteroids and methitrexate are considered as the main drugs for GLM treatment, but a high toxicity and risk of recurrence greatly limit their application. It is therefore an urgent requirement that safe and efficient natural drugs are found to improve the GLM prognosis. Read More

    Long-term observational studies of chronic granulomatous disease.
    Curr Opin Hematol 2017 Nov 10. Epub 2017 Nov 10.
    Department of Immunology-Histocompatibility, Specialized Center & Referral Center for Primary Immunodeficiencies, Paediatric Immunology, 'Aghia Sophia' Children's Hospital, Athens, Greece.
    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. Read More

    Successful transcatheter arterial antimicrobial and steroid therapy for refractory liver abscess in chronic granulomatous disease: A case report and review of literature.
    J Infect Chemother 2017 Nov 10. Epub 2017 Nov 10.
    Nara Prefecture General Medical Center, Department of Pediatrics, Nara, Japan.
    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. Read More

    The three CYBA variants (rs4673, rs1049254 and rs1049255) are benign: new evidence from a patient with CGD.
    BMC Med Genet 2017 Nov 13;18(1):127. Epub 2017 Nov 13.
    Department of Clinical Immunology, Children's Hospital of Fudan University, 399 Wanyuan Road, Shanghai, 201102, China.
    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. Read More

    Janus-Faced Neutrophil Extracellular Traps in Periodontitis.
    Front Immunol 2017 26;8:1404. Epub 2017 Oct 26.
    Clinic of Operative Dentistry, Periodontology and Preventive Dentistry, Saarland University, Homburg, Germany.
    Periodontitis is characterized by PMN infiltration and formation of neutrophil extracellular traps (NETs). However, their functional role for periodontal health remains complex and partially understood. The main function of NETs appears to be evacuation of dental plaque pathogen-associated molecular patterns. Read More

    Comment on: Phenotypic prenatal diagnosis of Chronic Granulomatous Disease (CGD): a useful tool in the absence of molecular diagnosis.
    Scand J Immunol 2017 Nov 9. Epub 2017 Nov 9.
    Departments of Pediatrics and Molecular, Cell, and Cancer Biology, University of Massachusetts Medical School, 55 Lake Avenue North, Worcester, MA, 01655, USA.
    I read with interest the report by Kulkarni, Gupta, and Madkaikar,[1] cited above. It was good to see that modern technology of flow cytometry could be applied to the phenotypic prenatal diagnosis of CGD. However, I disagree with their assessment that flow cytometry for dihydrorhodamine fluorescence is preferable to histochemical detection of nitroblue tetrazolium (NBT) reduction. Read More

    Stop Being So Sensitive: An Exceptionally Rare Report of Ustekinumab-Induced Sub-acute Hypersensitivity Pneumonitis.
    Turk J Anaesthesiol Reanim 2017 Oct 15;45(5):313-317. Epub 2017 Sep 15.
    Department of Internal Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, University of Florida College of Medicine, Florida, USA.
    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. Read More

    IL-37 and leprosy: A novel cytokine involved in the host response to Mycobacterium leprae infection.
    Cytokine 2017 Oct 27. Epub 2017 Oct 27.
    Tropical Medicine Center, Federal University of Pará, Belém, Pará, Brazil; Center of Health and Biological Sciences, State University of Pará, Belém, Pará, Brazil. Electronic address:
    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. Read More

    Evidence for M2 macrophages in granulomas from pulmonary sarcoidosis: a new aspect of macrophage heterogeneity.
    Hum Immunol 2017 Oct 26. Epub 2017 Oct 26.
    Airways Disease Section, National Heart & Lung Institute, Imperial College London, London, UK; Priority Research Centre for Healthy Lungs, Hunter Medical Research Institute, The University of Newcastle, Newcastle, New South Wales, Australia. Electronic address:
    Background: Sarcoidosis is a granulomatous disease of unknown etiology. Macrophages play a key role in granuloma formation with the T cells, having a significant impact on macrophage polarization (M1 and M2) and the cellular composition of the granuloma. This study evaluates macrophage polarization in granulomas in pulmonary sarcoidosis. Read More

    Hot Tub Lung: A Diagnostic Challenge.
    Cureus 2017 Aug 27;9(8):e1617. Epub 2017 Aug 27.
    Internal Medicine, West Virginia University - Charleston Division.
    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. Read More

    Spectrum of imaging findings of chronic granulomatous disease: a single center experience.
    Diagn Interv Radiol 2017 Nov-Dec;23(6):472-477
    Department of Radiology, Jeju National University Hospital, Jeju National University School of Medicine, Jeju, Republic of Korea.
    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. Read More

    Balamuthia mandrillaris Granulomatous Amebic Encephalitis With Renal Dissemination in a Previously Healthy Child: Case Report and Review of the Pediatric Literature.
    J Pediatric Infect Dis Soc 2017 Oct 31. Epub 2017 Oct 31.
    Departments of Pediatrics.
    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. Read More

    Subcutaneous Sarcoidosis Occurring in Both Chin and Toe.
    Arch Craniofac Surg 2017 Sep 26;18(3):207-210. Epub 2017 Sep 26.
    Department of Plastic and Reconstructive Surgery, Chonnam National University Medical School, Gwangju, Korea.
    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. Read More

    [Pulmonary infection by Arthrographis kalrae in patient with chronic granulomatous disease].
    Arch Argent Pediatr 2017 Dec;115(6):e458-e461
    Hospital de Niños Ricardo Gutiérrez, Programa de Infectología Pediátrica, Universidad de Buenos Aires, Facultad de Medicina, Ciudad de Buenos Aires.
    Background: Arthrographis kalrae is a hyaline fungus that grows forming arthroconidia. It is an opportunistic pathogen that causes infections in immunocompromised as in immunocompetent people and has been rarely isolated from human clinical samples.

    Case Report: We describe the case of a male child with primary immunodeficiency who initially presented unilateral pneumonia and progressed to bilateral involvement despite antibiotic, antifungal treatment. Read More

    Sterile nodular panniculitis with lung and lymph node involvement in a Siberian tiger (Panthera tigris altica).
    J Vet Med Sci 2017 Oct 30. Epub 2017 Oct 30.
    State Key Laboratory of Veterinary Biotechnology, Harbin Veterinary Research Institute, Chinese Academy of Agricultural Sciences.
    A 2- to 4-year-old uncastrated male Siberian tiger (Panthera tigris altica) bred in a local wild animal park presented with generalized clinical signs including abdominal pain, fever, lethargy, and anorexia, along with subcutaneous nodules along the trunk. The patient subsequently died of chronic, progressive dyspnea despite 45 days of antibiotic treatment. At necropsy, mesenteric fat inflammation and multiple subcutaneous, peritoneal, and intraabdominal nodules were observed. Read More

    The enigma of granulomatous mastitis: A series.
    Breast Dis 2017 Feb 20. Epub 2017 Feb 20.
    Department of Plastic Surgery, Harlem Hospital Center.
    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. Read More

    5-Aminosalicylic Acid Modulates the Immune Response in Chronic Beryllium Disease Subjects.
    Lung 2017 Oct 27. Epub 2017 Oct 27.
    Department of Medicine, National Jewish Health, 1400 Jackson Street, Denver, CO, USA.
    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. Read More

    Pancreatic Involvement in Pediatric Inflammatory Bowel Disease.
    Front Pediatr 2017 11;5:218. Epub 2017 Oct 11.
    Pediatric Gastroenterology and Nutrition Unit, Hospital Materno Infantil, Málaga, Spain.
    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. Read More

    Mimicking hypersensitivity pneumonitis as an uncommon initial presentation of chronic granulomatous disease in children.
    Orphanet J Rare Dis 2017 Oct 26;12(1):169. Epub 2017 Oct 26.
    Department of Respiratory Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Nanlishi Road 56, Xicheng District, Beijing, China.
    Dry cough, dyspenea and diffuse centrilobular nodules in both lungs of radiologic findings similar to hypersensitivity pneumonitis (HP) are rare initial presentation in chronic granulomatous disease (CGD). CGD is remarkable for increased susceptibility to bacterial and fungal infections as well as high sensitivity to inciting antigens such as Aspergillus species due to dysregulated inflammation. We identified three children who had an initial presentation mimicking HP and were subsequently diagnosed as CGD. Read More

    Phenotypic prenatal diagnosis of Chronic Granulomatous Disease (CGD): a useful tool in the absence of molecular diagnosis.
    Scand J Immunol 2017 Oct 24. Epub 2017 Oct 24.
    National Institute of Immunohaematology-ICMR.
    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. Read More

    Frequency of Mycobacterium bovis and mycobacteria in primary immunodeficiencies.
    Turk Pediatri Ars 2017 Sep 1;52(3):138-144. Epub 2017 Sep 1.
    Department of Pediatrics, Pediatric Allergy and Immunology, Ege University Faculty of Medicine, Izmir, Turkey.
    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. Read More

    Cytologic and Ultrastructural Findings of Bronchoalveolar Lavage in Patients With Chronic Granulomatous Disease.
    Pediatr Dev Pathol 2017 Jan 1:1093526617736188. Epub 2017 Jan 1.
    1 Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, California.
    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. Read More

    Insights from the Genome Sequence of Mycobacterium lepraemurium: Massive Gene Decay and Reductive Evolution.
    MBio 2017 Oct 17;8(5). Epub 2017 Oct 17.
    Global Health Institute, École Polytechnique Fédérale de Lausanne, Lausanne, Switzerland
    Mycobacterium lepraemurium is the causative agent of murine leprosy, a chronic, granulomatous disease similar to human leprosy. Due to the similar clinical manifestations of human and murine leprosy and the difficulty of growing both bacilli axenically, Mycobacterium leprae and M. lepraemurium were once thought to be closely related, although it was later suggested that M. Read More

    Uveitis: Diagnostic work-up. A literature review and recommendations from an expert committee.
    Autoimmun Rev 2017 Oct 14. Epub 2017 Oct 14.
    Service de Rhumatologie, Hôpital Saint-Antoine, AP-HP, UPMC Univ Paris 06, INSERM UMRS 938, DHU i2B, 75012 Paris, France.
    Purpose: Diagnosis of uveitis is difficult. Etiologic investigations should take into account the epidemiology of uveitis and should focus on the most severe forms of the disease and those which can be treated. This study was undertaken to establish recommendations for the diagnosis of uveitis. Read More

    Celiac Disease Associated with a Benign Granulomatous Mass Demonstrating Self-Regression after Initiation of a Gluten-Free Diet.
    Case Rep Gastroenterol 2017 May-Aug;11(2):473-479. Epub 2017 Aug 17.
    Department of Gastroenterology, University of Toledo Medical Center, Toledo, Ohio, USA.
    Celiac disease is a chronic immune-mediated enteropathy in which dietary gluten induces an inflammatory reaction predominantly in the duodenum. Celiac disease is known to be associated with benign small bowel thickening and reactive lymphadenopathy that often regresses after the institution of a gluten-free diet. A 66-year-old male patient with celiac disease presented with abdominal pain and diarrheal illness. Read More

    First report of fatal disseminated microsporidiosis in two inland bearded dragons Pogona vitticeps in Japan.
    JMM Case Rep 2017 Apr 12;4(4):e005089. Epub 2017 Apr 12.
    Laboratory of Veterinary Parasitology, Nippon Veterinary and Life Science University, 1-7-1 Kyonancho, Musashino, Tokyo, Japan.
    Introduction.Encephalitozoon pogonae is a newly described pathogen belonging to the phylum Microsporidia. In Austria and the USA, this species has been isolated from fatal and disseminated cases of captive-bred inland bearded dragons. Read More

    Very Early-Onset Inflammatory Manifestations of X-Linked Chronic Granulomatous Disease.
    Front Immunol 2017 26;8:1167. Epub 2017 Sep 26.
    Department of Pediatrics, CHU Sainte-Justine, University of Montreal, Montreal, QC, Canada.
    Chronic granulomatous disease (CGD) is a rare primary immune deficiency caused by mutations in genes coding for components of the nicotinamide adenine dinucleotide phosphate oxidase, characterized by severe and recurrent bacterial and fungal infections, together with inflammatory complications. Dysregulation of inflammatory responses are often present in this disease and may lead to granulomatous lesions, most often affecting the gastrointestinal (GI) and urinary tracts. Treatment of inflammatory complications usually includes corticosteroids, whereas antimicrobial prophylaxis is used for infection prevention. Read More

    A rare complication of chronic granulomatous disease in a child: constrictive aspergillus pericarditis.
    Turk Kardiyol Dern Ars 2017 Oct;45(7):660-663
    Department of Cardiovascular Surgery, Ege University Faculty of Medicine, İzmir, Turkey.
    A 3-year-old girl with the diagnosis of chronic granulomatous disease (CGD) was hospitalized for bronchopneumonia and congestive heart failure. Her medical history included methylprednisolone medication for autoimmune gastric outlet obstruction. Computed tomography revealed pneumonic infiltrations and pericardial thickening. Read More

    Response to the Letter to the Editor Regarding "Kinetics of Radiological Response of Thoracic Invasive Fungal Disease in Chronic Granulomatous Disease".
    J Clin Immunol 2017 Nov 8;37(8):744-745. Epub 2017 Oct 8.
    Pediatrics Clinic and Institute for Molecular Medicine "A. Nocivelli", Department of Clinical and Experimental Sciences, University of Brescia, Piazzale Spedali Civili 1, 25123, Brescia, Italy.

    Johne's disease in cattle: an in vitro model to study early response to infection of Mycobacterium avium subsp. paratuberculosis using RNA-seq.
    Mol Immunol 2017 Nov 5;91:259-271. Epub 2017 Oct 5.
    Davies Research Centre, School of Animal and Veterinary Sciences, University of Adelaide, Roseworthy, SA 5371, Australia. Electronic address:
    Johne's disease is a chronic granulomatous enteritis caused by Mycobacterium avium subsp. paratubercolosis (MAP) which affects ruminants worldwide and has a significant economic impact. MAP has also been associated with human Crohn's disease, although this connection is not well established. Read More

    An Update on the Treatment and Follow-up of Patients with Primary Biliary Cholangitis.
    Clin Liver Dis 2017 Nov 26;21(4):709-723. Epub 2017 Jul 26.
    Department of Organ Transplant & Liver Center, Liver Care Network and Organ Care Research, Swedish Medical Center, Seattle, 1124 Columbia Street, WA 98101, USA. Electronic address:
    Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by chronic granulomatous lymphocytic cholangitis of the small bile ducts. PBC was a leading indication for liver transplant in the United States; with early diagnosis and treatment, the majority of patients with PBC have a normal life expectancy. Pathogenesis involves inflammatory damage of bile duct epithelium secondary to innate and adaptive immune responses, and toxicity from accumulated bile acids. Read More

    IFN-γ alters the expression of diverse immunity related genes in a cell culture model designed to represent maturing neutrophils.
    PLoS One 2017 5;12(10):e0185956. Epub 2017 Oct 5.
    Department of Pediatrics, University of Colorado Denver, The Anschutz Medical Campus, Aurora, Colorado, United States of America.
    The cytokine interferon-γ (IFN-γ) is approved as a drug to treat chronic granulomatous disease (CGD) and osteopetrosis and is also used in hyperimmunoglobulin E syndromes. Patients with CGD have defects in proteins of the NOX2 NADPH oxidase system. This leads to reduced production of microbicidal ROS by PMNs and recurrent life threatening infections. Read More

    Inflammatory Bowel Disease in Chronic Granulomatous Disease: an emerging problem over a twenty years' experience.
    Pediatr Allergy Immunol 2017 Oct 5. Epub 2017 Oct 5.
    University Department of Pediatrics, Unit of Immune and Infectious Diseases, Bambino Gesù Children's Hospital IRCCS and University of Rome Tor Vergata, Rome, Italy.
    Background: Chronic Granulomatous Disease (CGD) is a primary immunodeficiency of phagocytes, characterized by life-threatening infections and hyperinflammation. Due to survival improvement, Inflammatory Bowel Disease (IBD) is becoming increasingly relevant. Here we report our 20-year experience. Read More

    Infectious and non-infectious complications in primary immunodeficiency disorders: an autopsy study from North India.
    J Clin Pathol 2017 Sep 28. Epub 2017 Sep 28.
    Department of Paediatrics (Allergy and Immunology Unit), Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India.
    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. Read More

    Sinonasal and laryngeal sarcoidosis.
    Proc (Bayl Univ Med Cent) 2017 Oct;30(4):452-454
    Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas.
    Sarcoidosis is a chronic granulomatous inflammation of uncertain etiology that can involve any organ system in the body. Sinonasal and laryngeal involvement is rare, poorly understood, and difficult to diagnose. Additionally, the extent of the disease is variable, and the response to systemic corticosteroids is often poor. Read More

    Aspergillus myofasciitis in a chronic granulomatous disease patient: first case report.
    Infez Med 2017 Sep;25(3):270-273
    Maternal-Infant Department, USL Umbria 2, San Giovanni Battista Hospital, Foligno, Italy.
    Aspergillus myofasciitis is a rare infection of the muscles and their fascial sheaths that has been reported in patients with immune deficiencies of various kinds but, until now, not with chronic granulomatous disease (CGD). Patients affected by CGD are at high risk of invasive aspergillus infections. The case described involves a 14-year-old boy with a severe autosomal recessive CGD who was admitted to hospital with an Aspergillus myofasciitis of the left forearm. Read More

    Granulomatous colitis: more than a canine disease? A case of Escherichia coli-associated granulomatous colitis in an adult cat.
    JFMS Open Rep 2017 Jul-Dec;3(2):2055116917731168. Epub 2017 Sep 19.
    Fregis Veterinary Health Centre, Arcueil, France.
    Case Summary: This report describes a 4-year-old cat with chronic intermittent haematochezia and faecal incontinence of 7 months' duration. Investigation revealed severe colonic multifocal mucosal ulcerations and infiltration of the mucosal lamina propria by large numbers of periodic acid-Schiff-positive macrophages. Fluorescence in situ hybridisation analysis of colonic biopsies revealed multifocal clusters of intracellular Escherichia coli. Read More

    Mass Cytometry Identifies Distinct Lung CD4(+) T Cell Patterns in Löfgren's Syndrome and Non-Löfgren's Syndrome Sarcoidosis.
    Front Immunol 2017 12;8:1130. Epub 2017 Sep 12.
    Respiratory Medicine Unit, Department of Medicine, Solna and Center for Molecular Medicine, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden.
    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. Read More

    Transition From Pediatric to Adult Care by Young Adults With Chronic Granulomatous Disease: The Patient's Viewpoint.
    J Adolesc Health 2017 Sep 22. Epub 2017 Sep 22.
    Intramural Clinical Management and Operations Branch, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland. Electronic address:
    Purpose: Children with chronic illnesses are living longer, prompting health care provider attention to the transition from pediatric to adult care. Transition of care is successful when youth are independent in managing their health. The aims of this study were to identify the strengths and barriers to transition from pediatric to adult care and to determine strategies that could enhance the transition process. Read More

    Sarcoidosis vs tuberculosis: Diagnostic mystery still unresolved.
    Indian J Tuberc 2017 Oct;64(4):243-245
    All India Institute of Medical Sciences, India.
    Sarcoidosis and tuberculosis are chronic, multisystemic, granulomatous disease of alike clinical, radiological and histopathological manifestations. Idiopathic nature of the disease and a strong clinical similarity with tuberculosis make the effectiveness of various clinical examinations for the diagnosis of sarcoidosis difficult in a tuberculosis endemic area. Presently confirmation of a diagnosis of sarcoidosis in most cases requires a biopsy which is often not confirmatory. Read More

    Tuberculoid leprosy masquerading as systemic lupus erythematosus: an interesting observation.
    Acta Dermatovenerol Alp Pannonica Adriat 2017 09;26(3):81-83
    Hindusabha Hospital, Mumbai, Maharashtra, India.
    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. Read More

    Three novel mutations in CYBA among 22 Iranians with Chronic granulomatous disease.
    Int J Immunogenet 2017 Dec 20;44(6):314-321. Epub 2017 Sep 20.
    Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran.
    Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by defect in one of the components of nicotinamide adenine dinucleotide phosphate (NADPH)-oxidase enzyme. The enzyme is at least composed of membrane-bound subunits gp91-phox and p22-phox (also named cytochrome b558 ), and cytosolic ones p40-phox, p47-phox and p67-phox. A defect in the enzyme activity leads to impaired intracellular killing of phagocytic cells. Read More

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