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    6913 results match your criteria Chronic Granulomatous Disease

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    J Zoo Wildl Med 2017 Sep;48(3):929-932
    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. Read More

    Hypercalcemic Disorders in Children.
    J Bone Miner Res 2017 Sep 15. Epub 2017 Sep 15.
    Academic Endocrine Unit, Radcliffe Department of Medicine, University of Oxford, Oxford, UK.
    Hypercalcemia is defined as a serum calcium concentration that is greater than 2 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. Read More

    The Choroidal Vascularity Index Decreases and Choroidal Thickness Increases in Vogt-Koyanagi-Harada Disease Patients During a Recurrent Anterior Uveitis Attack.
    Ocul Immunol Inflamm 2017 Sep 15:1-7. Epub 2017 Sep 15.
    a The First Affiliated Hospital of Chongqing Medical University , Chongqing Key Laboratory of Ophthalmology and Chongqing Eye Institute , Chongqing , P.R. China.
    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. Read More

    Subcellular localization and function study of a secreted phospholipase C from Nocardia seriolae.
    FEMS Microbiol Lett 2017 Sep;364(17)
    College of Fisheries, Guangdong Ocean University, Zhanjiang 524088, PR China.
    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. Read More

    Gene Therapy Approaches to Immunodeficiency.
    Hematol Oncol Clin North Am 2017 Oct 29;31(5):823-834. Epub 2017 Jun 29.
    Infection, Immunity, Inflammation, Molecular and Cellular Immunology Section, University College London, UCL Great Ormond Street Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. Electronic address:
    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. Read More

    Inflammatory myopathy associated with antimitochondrial antibodies: A distinct phenotype with cardiac involvement.
    Semin Arthritis Rheum 2017 Jun 13. Epub 2017 Jun 13.
    Department of Medicine, Division of Rheumatology, Johns Hopkins University, School of Medicine, Baltimore, MD. Electronic address:
    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. Read More

    Host immune status-specific production of gliotoxin and bis-methyl-gliotoxin during invasive aspergillosis in mice.
    Sci Rep 2017 Sep 8;7(1):10977. Epub 2017 Sep 8.
    Fungal Pathogenesis Unit, LCIM, NIAID, NIH, Bethesda, MD, USA.
    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. Read More

    Mutation in an exonic splicing enhancer site causing chronic granulomatous disease.
    Blood Cells Mol Dis 2017 Jul 18;66:50-57. Epub 2017 Aug 18.
    Sanquin Research, and Landsteiner Laboratory, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands. Electronic address:
    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. Read More

    Hepatitis C virus mediated chronic inflammation and tumorigenesis in the humanised immune system and liver mouse model.
    PLoS One 2017 8;12(9):e0184127. Epub 2017 Sep 8.
    Institute of Molecular and Cell Biology, Singapore, Singapore.
    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. Read More

    Neutrophils as Components of Mucosal Homeostasis.
    Cell Mol Gastroenterol Hepatol 2017 Nov 19;4(3):329-337. Epub 2017 Jul 19.
    Department of Medicine, University of Colorado School of Medicine, Aurora, Colorado.
    Inflammatory responses in the intestinal mucosa inevitably result in the recruitment of neutrophils (polymorphonuclear leukocytes [PMNs]). Epithelial cells that line the mucosa play an integral role in the recruitment, maintenance, and clearance of PMNs at sites of inflammation. The consequences of such PMN-epithelial interactions often determine tissue responses and, ultimately, organ function. Read More

    Pleuroparenchymal Fibroelastosis: A Review of Histopathologic Features and the Relationship Between Histologic Parameters and Survival.
    Am J Surg Pathol 2017 Sep 4. Epub 2017 Sep 4.
    Departments of *Histopathology §Radiology, Royal Brompton and Harefield NHS Foundation Trust †Interstitial Lung Disease Unit, Royal Brompton Hospital, Imperial College ‡National Heart and Lung Institute, Imperial College, London, UK.
    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. Read More

    Xanthoma-like Skin Changes in an Elderly Woman with a Normal Lipid Profile.
    Acta Dermatovenerol Croat 2017 Jul;25(2):167-169
    Professor Joanna Maj, MD, PhD, Department and Clinic of Dermatology, Venereology and Allergology, Wroclaw Medical University, Chałubińskiego 1, PL-50-368 Wrocław, Poland;
    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. Read More

    Th17-Inducing Cytokines IL-6 and IL-23 Are Crucial for Granuloma Formation during Experimental Paracoccidioidomycosis.
    Front Immunol 2017 21;8:949. Epub 2017 Aug 21.
    Department of Biochemistry and Immunology, School of Medicine of Ribeirão Preto, University of São Paulo, Ribeirão Preto, Brazil.
    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. Read More

    Recent advances in understanding and treating chronic granulomatous disease.
    F1000Res 2017 11;6:1427. Epub 2017 Aug 11.
    Paediatric Immunology and Haematopoietic Stem Cell Transplantation, Great North Childrens' Hospital, Newcastle upon Tyne, UK.
    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. Read More

    Splicing dysfunction and disease: The case of granulopoiesis.
    Semin Cell Dev Biol 2017 Sep 1. Epub 2017 Sep 1.
    Australian Regenerative Medicine Institute, Monash University, Clayton, Victoria 3800, Australia. Electronic address:
    Splicing is a ubiquitous process in eukaryotic cells, long recognised as contributing to diversity of the transcriptome. More specifically, splicing fine-tunes the transcriptome output for highly individual outcomes at different stages of cell development, in specific timeframes, which when perturbed result in significant human diseases. Granulopoiesis provides a particularly well studied example of how splicing can be a highly flexible but tightly regulated process. Read More

    Is idiopathic granulomatous mastitis a surgical disease? The jury is still out.
    Ann Transl Med 2017 Aug;5(15):309
    Department of Surgery, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
    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. Read More

    A case of relapsed lung abscess caused by Eubacterium brachy infection following an initial diagnosis of pulmonary actinomycosis.
    Respir Med Case Rep 2017 17;22:171-174. Epub 2017 Aug 17.
    Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
    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. Read More

    Primary Immunodeficiency Diseases: Current and Emerging Therapeutics.
    Front Immunol 2017 9;8:937. Epub 2017 Aug 9.
    Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Bethesda, MD, United States.
    Primary immunodeficiency diseases (PID) result from defects in genes affecting the immune and other systems in many and varied ways (1, 2). Until the last few years, treatments have been largely supportive, with the exception of bone marrow transplantation. However, recent advances in immunobiology, genetics, and the explosion of discovery and commercialization of biologic modifiers have drastically altered the landscape and opportunities in clinical immunology. Read More

    Dermoscopy of borderline tuberculoid leprosy.
    Int J Dermatol 2017 Aug 22. Epub 2017 Aug 22.
    Department of Dermatology, S. Nijalingappa Medical College, Bagalkot, Karnataka, India.
    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. Read More

    NOX5 and p22phox are two novel regulators of human monocytic differentiation into dendritic cells.
    Blood 2017 Aug 22. Epub 2017 Aug 22.
    INSERM-U1149, CNRS-ERL8252, Centre de Recherche sur l'Inflammation (CRI), Paris, France;
    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. Read More

    Oral manifestations as the first presenting sign of Crohn's disease in a pediatric patient.
    J Clin Exp Dent 2017 Jul 1;9(7):e934-e938. Epub 2017 Jul 1.
    BDS, MSc, University of Washington School of Dentistry, Department of Oral & Maxillofacial Surgery, Seattle, WA.
    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. Read More

    Host regulation of liver fibroproliferative pathology during experimental schistosomiasis via interleukin-4 receptor alpha.
    PLoS Negl Trop Dis 2017 Aug 21;11(8):e0005861. Epub 2017 Aug 21.
    Cytokines and Diseases Group, International Centre for Genetic Engineering and Biotechnology, Cape Town Component, Cape Town, South Africa.
    Interleukin-4 receptor (IL-4Rα) is critical for the initiation of type-2 immune responses and implicated in the pathogenesis of experimental schistosomiasis. IL-4Rα mediated type-2 responses are critical for the control of pathology during acute schistosomiasis. However, type-2 responses tightly associate with fibrogranulomatous inflammation that drives host pathology during chronic schistosomiasis. Read More

    Advances in basic and clinical immunology in 2016.
    J Allergy Clin Immunol 2017 Aug 19. Epub 2017 Aug 19.
    Division of Immunology, Boston Children's Hospital, and Department of Pediatrics, Harvard Medical School, Boston, Mass.
    Advances in basic immunology in 2016 included studies that further characterized the role of different proteins in the differentiation of effector T and B cells, including cytokines and proteins involved in the actin cytoskeleton. Regulation of granule formation and secretion in cytotoxic cells was also further described by examining patients with familial hemophagocytic lymphohistiocytosis. The role of prenylation in patients with mevalonate kinase deficiency leading to inflammation has been established. Read More

    A comprehensive review and update on Crohn's disease.
    Dis Mon 2017 Aug 18. Epub 2017 Aug 18.
    Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh, M2, C Wing, 200 Lothrop Street, Pittsburgh, PA 15213, United States.
    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. Read More

    Granulomatous rosacea: a case report.
    J Med Case Rep 2017 Aug 20;11(1):230. Epub 2017 Aug 20.
    Department of Dermatology, University Hospital Hassan II, 202 Hay Mohamadi, Fez, Morocco.
    Background: Granulomatous rosacea is a rare chronic inflammatory skin disease with an unknown origin. The role of Demodex follicularum in its pathogenesis is currently proved.

    Case Presentation: We report a case of a 54-year-old Moroccan man with a 3-month history of erythematous, nonpruritic papules on the lateral side around the eyes. Read More

    Biological and functional characterization of bone marrow-derived mesenchymal stromal cells from patients affected by primary immunodeficiency.
    Sci Rep 2017 Aug 15;7(1):8153. Epub 2017 Aug 15.
    Department of Pediatric Hematology/Oncology, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
    Mesenchymal stromal cells (MSCs) represent a key component of bone marrow (BM) microenvironment and display immune-regulatory properties. We performed a detailed analysis of biological/functional properties of BM-MSCs derived from 33 pediatric patients affected by primary immune-deficiencies (PID-MSCs): 7 Chronic Granulomatous Disease (CGD), 15 Wiskott-Aldrich Syndrome (WAS), 11 Severe Combined Immunodeficiency (SCID). Results were compared with MSCs from 15 age-matched pediatric healthy-donors (HD-MSCs). Read More

    Histoplasmosis by Histoplasma capsulatum var. duboisii Observed at the Laboratory of Pathological Anatomy of Lomé in Togo.
    J Pathog 2017 18;2017:2323412. Epub 2017 Jul 18.
    Department of Pathology, University Teaching Hospital of Lomé, Lomé, Togo.
    Our study aimed to describe the epidemiological, clinical, and diagnostic aspects of African histoplasmosis in Togo through a descriptive and cross-sectional study on histological diagnosed African histoplasmosis in Pathology Department of Lomé from 2002 to 2016 (15 years). A total of 17 cases of African histoplasmosis were diagnosed. The sex ratio (M/F) was 1. Read More

    Mycetoma: An Update.
    Indian J Dermatol 2017 Jul-Aug;62(4):332-340
    Department of Dermatology, Maulana Azad Medical College, New Delhi, India.
    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. Read More

    Two simultaneous mycetomas caused by Fusarium verticillioides and Madurella mycetomatis.
    Rev Inst Med Trop Sao Paulo 2017 3;59:e55. Epub 2017 Aug 3.
    Hospital General de México "Dr. Eduardo Liceaga", Servicio de Dermatología, Ciudad de México, México.
    Mycetoma is a chronic granulomatous disease, classified into eumycetoma caused by fungi and actinomycetoma due to aerobic filamentous actinomycetes. Mycetoma can be found in geographic areas near the Tropic of Cancer. Mexico is one of the countries in which actinomycetoma is endemic. Read More

    A curious case of growth failure and hypercalcemia: Answers.
    Pediatr Nephrol 2017 Aug 7. Epub 2017 Aug 7.
    Division of Nephrology, University of Toronto, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
    Background: Sarcoidosis is a multisystem granulomatous disease of unknown etiology that rarely presents in childhood. Here, we report a case of pediatric sarcoidosis, presenting with renal failure and hypercalcemia.

    Case Diagnosis/treatment: A previously well 14-year-old Caucasian boy was admitted to the Hospital for Sick Children, Canada, for hypertension and renal failure following work-up by his family physician for initial concerns of growth failure. Read More

    A curious case of growth failure and hypercalcemia: Questions.
    Pediatr Nephrol 2017 Aug 7. Epub 2017 Aug 7.
    Division of Nephrology, University of Toronto, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
    Background: Sarcoidosis is a multisystem granulomatous disease of unknown etiology that rarely presents in childhood. Here, we report a case of pediatric sarcoidosis presenting with renal failure and hypercalcemia.

    Case Diagnosis/treatment: A previously well 14-year-old Caucasian boy was admitted to the Hospital for Sick Children, Canada, for hypertension and renal failure following work-up by his family physician for initial concerns of growth failure. Read More

    Common Variable Immunodeficiency and Liver Involvement.
    Clin Rev Allergy Immunol 2017 Aug 7. Epub 2017 Aug 7.
    Division of Rheumatology/Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 Health Sciences Drive, Davis, CA, 95616, USA.
    Common variable immunodeficiency (CVID) is a primary B-cell immunodeficiency disorder, characterized by remarkable hypogammaglobulinemia. The disease can develop at any age without gender predominance. The prevalence of CVID varies widely worldwide. Read More

    Granulomas within renal epithelial neoplasms in patients with sarcoidosis: A causal association?
    Pathol Res Pract 2017 Jul 23. Epub 2017 Jul 23.
    Surgical Pathology Division, Department of Pathology, University of Illinois at Chicago, 840 S. Wood St., Suite 130 CSN, Chicago, IL, 60612, United States; Hematologic Pathology Division, Department of Pathology, University of Illinois at Chicago, 840 S. Wood St., Suite 130 CSN, Chicago, IL, 60612, United States.
    Sarcoidosis is a chronic granulomatous disease that can involve virtually every organ system, but most commonly presents as lung, skin, or lymph node disease. Although kidney involvement is usually clinically silent, granulomatous interstitial nephritis - the hallmark of renal sarcoidosis - can lead to functional impairment and organ failure. Also, recent studies have suggested an association between sarcoidosis and an increased risk of developing kidney tumors. Read More

    Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome.
    Autoimmun Rev 2017 Oct 2;16(10):1036-1043. Epub 2017 Aug 2.
    Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Université Paris Descartes, Paris 5, Paris, France. Electronic address:
    Objective: Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap.

    Methods: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria. Read More

    Development of Hodgkin lymphoma in a patient with sarcoidosis.
    Reumatismo 2017 Aug 3;69(2):84-87. Epub 2017 Aug 3.
    Department of Hematology, Katip Celebi University, Ataturk Education and Research Hospital, Izmir.
    Sarcoidosis is a chronic granulomatous disease of unknown etiology characterized by non-caseified granulomas in many different organs and systems. The disease most frequently manifests with bilateral hilar lymphadenopathy and infiltrations in the lungs and skin, as well as with eye lesions. It may mimic a number of systemic diseases and/or accompany them. Read More

    Granulomas in the gastrointestinal tract: deciphering the Pandora's box.
    Virchows Arch 2017 Aug 4. Epub 2017 Aug 4.
    School of Pathology and Laboratory Medicine, University of Western Australia, Perth, Australia.
    Granulomas are organised collection of activated histiocytes induced by a persistent antigen stimulus. A wide variety of antigens encountered by the gastrointestinal tract are of this nature and hence the resulting granulomatous inflammation represents a tissue reaction pattern. The potential causes can be broadly classified as infections or non-infectious immune reactions. Read More

    Pulmonary and sinus fungal diseases in non-immunocompromised patients.
    Lancet Infect Dis 2017 Jul 31. Epub 2017 Jul 31.
    Department of Medical Microbiology, Postgraduate Institute of Medical Education & Research, Chandigarh, India.
    The human respiratory tract is exposed daily to airborne fungi, fungal enzymes, and secondary metabolites. The endemic fungi Histoplasma capsulatum, Coccidioides spp, Blastomyces dermatitidis, and Paracoccidioides brasiliensis, and occasionally Aspergillus fumigatus, are primary pulmonary pathogens of otherwise healthy people. Such infections resolve in most people, and only a few infections lead to disease. Read More

    Pyoderma gangrenosum in a patient with chronic granulomatous disease: A case report.
    Medicine (Baltimore) 2017 Aug;96(31):e7718
    1st Department of Internal Medicine, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.
    Rationale: The simultaneous occurrence of pyoderma gangrenosum (PG) and chronic granulomatous disease (CGD) is uncommon and few cases have been reported worldwide.

    Patient Concerns: PG is a rare, chronic, ulcerative, neutrophilic skin disease of unknown etiology that requires immunosuppressive treatment. CGD belongs to Primary Immune Deficiencies in which the main defect lies in an inability of the phagocytic cells to generate superoxide making patients susceptible to serious, potentially life-threatening bacterial and fungal infections. Read More

    Idiopathic Granulomatous Mastitis: A Clinical Puzzle in Breast Lump Cases.
    J Clin Diagn Res 2017 Jun 1;11(6):PD14-PD15. Epub 2017 Jun 1.
    Junior Resident, Department of General Surgery, SCB, Medical College, Cuttack, Odisha, India.
    Idiopathic granulomatous mastitis (IGM) is a rare benign disease, characterized by chronic inflammation and granulomatous disease process. A middle aged lady with breast lump for six months with equivocal mammographic and ultrasound results underwent lumpectomy and biopsy. Ruling out all other possible granulomatous diseases and malignancy, a diagnosis of IGM was made. Read More

    Kidney Disease in Human Immunodeficiency Virus-seropositive Patients: Absence of Human Immunodeficiency Virus-associated Nephropathy was a Characteristic Feature.
    Indian J Nephrol 2017 Jul-Aug;27(4):271-276
    Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.
    Human immunodeficiency virus (HIV) infection can cause a broad spectrum of renal diseases. However, there is paucity of Indian data on the patterns of renal lesions in HIV-seropositive patients. The aim of the present study was to delineate the spectrum of renal lesions in HIV/acquired immunodeficiency syndrome patients. Read More

    Short communication: Passive shedding of Mycobacterium avium ssp. paratuberculosis in commercial dairy goats in Brazil.
    J Dairy Sci 2017 Oct 26;100(10):8426-8429. Epub 2017 Jul 26.
    Department of Veterinary, Universidade Federal de Viçosa (UFV), Av. P. H. Rolfs, s/n, CEP 36570-900, Viçosa, Minas Gerais, Brazil. Electronic address:
    Goat farming is a low-cost alternative to dairy production in developing countries. In Brazil, goat production has increased in recent years due in part to the implementation of programs encouraging this activity. Mycobacterium avium ssp. Read More

    Haematopoietic stem cell transplantation for primary immunodeficiency syndromes: A 5-year single-centre experience.
    J Paediatr Child Health 2017 Jul 28. Epub 2017 Jul 28.
    Department of Immunology, Sydney Children's Hospital, Sydney, New South Wales, Australia.
    Aim: Haematopoietic stem cell transplantation (HSCT) is a central therapy in the treatment of primary immunodeficiency diseases (PIDs). Over the past 5 years, outcomes have been greatly improved due to earlier diagnosis, improved donor availability, advancements in graft manipulation and the use of less toxic preparative regimens. We present a 5-year audit of HSCT for PID at a single Australian tertiary hospital. Read More

    Allogeneic Reduced-Intensity Hematopoietic Stem Cell Transplantation for Chronic Granulomatous Disease: a Single-Center Prospective Trial.
    J Clin Immunol 2017 Jul 28. Epub 2017 Jul 28.
    Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, USA.
    Purpose: The purpose of this study was to evaluate engraftment and adverse events with a conditioning and prophylactic regimen intended to achieve high rates of engraftment with minimal graft-versus-host disease (GVHD) in allogeneic transplantation for chronic granulomatous disease in a single center.

    Methods: Forty patients, 37 male, with chronic granulomatous disease were transplanted. Transplant products were matched sibling peripheral blood stem cells (PBSCs) in four and matched unrelated donor (MUD) bone marrow in three, and one patient received mismatched unrelated PBSCs. Read More

    Factors related to recurrence of idiopathic granulomatous mastitis: what do we learn from a multicentre study?
    ANZ J Surg 2017 Jul 27. Epub 2017 Jul 27.
    Laboratory of Nutrigenomics and Epidemiology, Department of Food Engineering, İzmir İnstitute of Technology, Izmir, Turkey.
    Background: Idiopathic granulomatous mastitis (IGM) is a rare chronic inflammatory disease of the breast with unknown aetiology. Its treatment is controversial and the recurrence rate is high. The objectives of this study were to examine the demographic, sociocultural and clinical characteristics observed among a large cohort of IGM patients from Turkey and to identify factors related to the recurrence of IGM. Read More

    Differential Signalling and Kinetics of Neutrophil Extracellular Trap Release Revealed by Quantitative Live Imaging.
    Sci Rep 2017 Jul 26;7(1):6529. Epub 2017 Jul 26.
    Laboratory of Translational Immunology, Department of Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands.
    A wide variety of microbial and inflammatory factors induce DNA release from neutrophils as neutrophil extracellular traps (NETs). Consensus on the kinetics and mechanism of NET release has been hindered by the lack of distinctive methods to specifically quantify NET release in time. Here, we validate and refine a semi-automatic live imaging approach for quantification of NET release. Read More

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