7,127 results match your criteria Chronic Granulomatous Disease


The role of neutrophils in the pathogenesis of Crohn's disease.

Authors:
Anthony W Segal

Eur J Clin Invest 2018 Jun 21:e12983. Epub 2018 Jun 21.

Division of Medicine, University College London, WC1E 6BT, London.

Crohn's disease (CD) is caused by a trigger, almost certainly enteric infection by one of a multitude of organisms that allows faeces access to the tissues, at which stage the response of individuals predisposed to CD is abnormal. In CD the failure of acute inflammation results in the failure to recruit neutrophils to the inflammatory site, as a consequence of which the clearance of bacteria from the tissues is defective. The retained faecal products result in the characteristic chronic granulomatous inflammation and adaptive immune response. Read More

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[Infections during Immunosuppression].

Praxis (Bern 1994) 2018 Jun;107(13):689-698

3 Klinik für Immunologie, Universitätsspital Zürich.

Infections during Immunosuppression Abstract. Immunomodulating and immunosuppressive therapies are being used more and more frequently. Depending on the mechanism of action and the underlying disease, there is an increased risk of infection with these therapies. Read More

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Chronic Granulomatous Disease First Diagnosed in Adulthood Presenting With Spinal Cord Infection.

Front Immunol 2018 4;9:1258. Epub 2018 Jun 4.

Department of Neurology, Hannover Medical School, Hannover, Germany.

Chronic granulomatous disease (CGD) is a rare genetic immunodeficiency, which is characterized by recurrent severe bacterial and fungal infections caused by a defect in phagocytic cells due to loss of superoxide production. The disease usually manifests within the first years of life. Early diagnosis allows therapeutic intervention to improve the limited life expectancy. Read More

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[Cerebral tuberculosis without meningitis in a immunocompetent child].

Rev Chilena Infectol 2018 Apr;35(2):207-212

Clínica Las Américas, Medellín, Colombia.

Cerebral tuberculosis TB (tuberculomas) without meningitis is an uncommon disease with a high morbidity and mortality. We report on a case that illustrates the complexity of this clinical presentation. An 11 month old, previously healthy male infant was brought to the clinic due to fever present during the last 1. Read More

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April 2018
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Case Report: Pulmonary and Liver Sarcoidosis Suspected of Metastasis.

F1000Res 2018 7;7:288. Epub 2018 Mar 7.

Department of Internal Medicine, Yasuj University of Medical Sciences, Yasuj, Iran.

: Sarcoidosis is a granulomatous disease with unknown cause that can vary from an asymptomatic condition. Almost half of the patients with sarcoidosis have no symptoms. In this article, we describe a sarcoidosis patient with lung and liver engagement; it may be confused with metastasis. Read More

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March 2018
2 Reads

Chronological observation of surgically-treated granuloma faciale implies the necessity of circumspect management for perinasal nodular subset.

J Dermatol 2018 Jun 15. Epub 2018 Jun 15.

Department of Dermatology, Kyorin University School of Medicine, Tokyo, Japan.

Granuloma faciale (GF) is a rare chronic dermatosis with still unknown etiopathology, which usually presents a solitary, asymptomatic, smooth reddish-brown to violaceous plaques or nodules on the face. Various therapeutic approaches, including topical application of corticosteroid or tacrolimus and removal with laser, cryotherapy and surgery have been attempted; however, the outcome has been inconsistent. Herein, we report a case of perinasal nodular GF who repeatedly underwent surgical excisions after the failure of laser treatment. Read More

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June 2018
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Hepatic actinomycosis with immunoglobulin G4-related liver disease: A case report.

Medicine (Baltimore) 2018 Jun;97(24):e11146

Pancreatobiliary Cancer Clinic, Department of Surgery.

Rationale: Immunoglobulin (Ig)G4-related pseudotumors of the liver are very rare diseases that are difficult to distinguish from malignant tumors. They can be usually improved by steroid therapy. Actinomycosis is a chronic, suppurative, granulomatous infection, for which immune suppression is a predisposing factor. Read More

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June 2018
5 Reads

Repurposing of gamma interferon via inhalation delivery.

Adv Drug Deliv Rev 2018 Jun 7. Epub 2018 Jun 7.

Pulmonary, Critical Care & Sleep Medicine, Department of Medicine, State University of New York at Stony Brook, 101 Nicolls Rd, HSC T-17-040, Stony Brook, NY 11794-8172, United States. Electronic address:

Pulmonary diseases frequently involve imbalances in immunity. The inability to control bacteria in tuberculosis is a failed response to a pathogen. Idiopathic pulmonary fibrosis (IPF), a progressive fibrotic lung disease, can lead to respiratory failure and death within 3 years of diagnosis. Read More

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June 2018
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Tuberculosis of the Oral Cavity Misdiagnosed as Precancerous Lesion.

Acta Stomatol Croat 2017 Dec;51(4):326-331

Department of Oral Medicine, School of Dental Medicine, University of Zagreb, Zagreb, Croatia.

Introduction: The aim of this case report was to discuss an extremely rare oral lesion as a result of primary pulmonary tuberculosis.

Case Report: In this case report, the patient with refractory painless ulceration at ventral surface of the tongue was described. Detailed medical history was taken followed by clinical examination of the oral mucosa and palpation of regional lymph nodes. Read More

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December 2017

Common signalling pathways in macrophage and osteoclast multinucleation.

J Cell Sci 2018 Jun 5;131(11). Epub 2018 Jun 5.

Centre for Inflammatory Disease, Imperial College London, London W12 0NN, UK

Macrophage cell fusion and multinucleation are fundamental processes in the formation of multinucleated giant cells (MGCs) in chronic inflammatory disease and osteoclasts in the regulation of bone mass. However, this basic cell phenomenon is poorly understood despite its pathophysiological relevance. Granulomas containing multinucleated giant cells are seen in a wide variety of complex inflammatory disorders, as well as in infectious diseases. Read More

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June 2018
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[Viewing sepsis and autoimmune disease in relation with infection and NETs-formation].

Nihon Saikingaku Zasshi 2018 ;73(2):171-191

Research & Development Center, Fuso Pharmaceutical Industries, Ltd.

Neutrophil has been widely recognized as body's first line of defence against pathogens. NETosis was first reported in 2004 as a programmed cell death of neutrophil and distinguished from apoptosis and necrosis. This phenomenon has been already observed in both basic and clinical research. Read More

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June 2018
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[Cutaneous manifestations in primary immunodeficiency diseases].

Orv Hetil 2018 Jun;159(23):937-947

Bőr-, Nemikórtani és Bőronkológiai Klinika, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.

Primary immunodeficiency diseases (PIDs) are inherited, genetic disorders. The majority of PIDs are diagnosed in infancy or early childhood, but manifestation in adulthood may also occur. Frequent, recurrent and prolonged infections, which respond poorly to treatment may be heralding signs. Read More

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[Rheumatological manifestations in primary immunodeficiency diseases].

Orv Hetil 2018 Jun;159(23):919-928

Klinikai Immunológiai, Felnőtt- és Gyermekreumatológiai Osztály, Országos Reumatológiai és Fizioterápiás Intézet Budapest, Frankel Leó út 38-40., 1023.

Primary immune deficiencies (PIDs) are characterized by quantitative and/or functional abnormalities of the immune system elements. Bone and joint abnormalities are not rare in patients with immunodeficiencies. Joint manifestations, of which arthritis is the most common, occur mainly in humoral PIDs (X-linked agammaglobulinemia, common variable immunodeficiency, and IgA deficiency) and occasionally in defects of the phagocyte system (chronic granulomatous disease, glicogen storage diseases). Read More

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June 2018
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Rhinoscleroma: An Unusual Presentation.

Indian Dermatol Online J 2018 May-Jun;9(3):191-193

Consultant Dermatologist and Dermatopathologist, Pune, Maharashtra, India.

Rhinoscleroma is a chronic granulomatous disease caused by Klebsiella rhinoscleromatis. It commonly affects the nasal cavity and nasopharynx, but it can also involve the larynx, trachea, bronchi, middle ear, and orbit. We are reporting a rare presentation of rhinoscleroma in a middle-aged female patient involving the lips and gums. Read More

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Induces Macropinocytosis to Enter Macrophages.

Biomed Res Int 2018 22;2018:4271560. Epub 2018 Apr 22.

Laboratorio de Infectología, Microbiología e Inmunología Clínicas, Unidad de Investigación en Medicina Experimental, Facultad de Medicina, Universidad Nacional Autónoma de México, Mexico City, Mexico.

is an opportunistic pathogen that infects individuals with cystic fibrosis, chronic granulomatous disease, and other immunocompromised states. survives in macrophages in membrane-bound vacuoles; however, the mechanism by which gains entry into macrophages remains unknown. After macrophage internalization, survival of within a bacteria-containing membrane vacuole (BcCV) is associated with its ability to arrest the maturation of the BcCV. Read More

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April 2018
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Issues in the management of invasive pulmonary aspergillosis in non-neutropenic patients in the intensive care unit: A role for isavuconazole.

IDCases 2018 1;12:7-9. Epub 2018 Mar 1.

Infectious Diseases Division, Department of Medicine, University of Udine and Santa Maria Misericordia University Hospital, Udine, Italy.

Background: Almost half of all cases of invasive aspergillosis (IA) occur in the intensive care unit (ICU), with mortality rates of 70-80% for probable or proven cases. IA has become a major concern among non-neutropenic patients in the ICU with chronic obstructive pulmonary disease (COPD) but although prompt, appropriate antifungal therapy is crucial, diagnosis in this situation is challenging. Criteria for a probable diagnosis in critically ill patients have been proposed to help to expedite therapy. Read More

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March 2018
2 Reads

Sarcoid-resembling granulomatous lung disease secondary to occupational magnetite iron dust exposure.

Respirol Case Rep 2018 Aug 17;6(6):e00331. Epub 2018 May 17.

Respiratory Medicine Royal Darwin Hospital Darwin Australia.

Non-caseating granulomatous pulmonary conditions resembling sarcoidosis secondary to industrial/occupation exposure to magnetite iron ore dusts have been rarely documented in the literature. This is a case report of a 58-year-old blast crew member involved in iron ore/magnetite mining who presented with a 12-month history of chronic dry cough. High-resolution computed tomography revealed bilateral interstitial opacities. Read More

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Disseminated Mycobacterium bovis infection post-kidney transplant following remote intravesical BCG therapy for bladder cancer.

Transpl Infect Dis 2018 May 29:e12931. Epub 2018 May 29.

Department of Internal Medicine, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, MB, Canada.

Intravesical Bacillus Camlette-Guérin (BCG) is the treatment of choice for non-muscle invasive bladder cancer, and has been used successfully for over 40 years. A rare and potentially fatal complication of intravesical BCG therapy is BCG-induced sepsis. We report a rare case in which a patient with end-stage renal disease secondary to chronic granulomatous interstitial nephritis underwent remote, pre-transplant intravesical BCG treatment for high-grade non-invasive papillary bladder carcinoma. Read More

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May 2018
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Leprosy manifesting with type 2 leprae reaction in a patient presenting with chronic fever: A case report.

J Pak Med Assoc 2018 Apr;68(4):653-656

The Indus Hospital, Karachi.

Leprosy is a chronic granulomatous disease involving the skin and nerves, leading to a debilitating condition. Leprosy has been controlled in most parts of the world; therefore physicians are not very well versed in the recognition, management and assessment of this disease. The protean manifestations of leprosy often lead to delays in diagnosis and increase the morbidity. Read More

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April 2018
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Actinomycotic Osteomyelitis of the Mandible Diagnosed Using Matrix Assisted Laser Desorption Ionization-Time of Flight Mass Spectrometry: A Case Report.

J Oral Maxillofac Surg 2018 Apr 26. Epub 2018 Apr 26.

Department Head, Department of Oral and Maxillofacial Surgery, Kobe City Medical Center General Hospital, Kobe, Japan.

Actinomycosis is a rare, chronic, slowly progressive granulomatous disease caused by filamentous gram-positive anaerobic bacteria from the Actinomycetaceae family (genus Actinomyces). It has become a rare condition because of the widespread use of antibiotics. When clinical symptoms are not typical, diagnosis of this condition becomes difficult. Read More

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April 2018
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Retrotransposable genetic elements causing neutrophil defects.

Eur J Clin Invest 2018 May 17:e12953. Epub 2018 May 17.

Sanquin Research, and Landsteiner Laboratory, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.

Background: Retrotransposable elements are stretches of DNA that encode proteins with the inherent ability to insert their own RNA or another RNA by reverse transcriptase as DNA into a new genomic location. In humans, the only autonomous retrotransposable elements are members of the Long INterspersed Element-1 (LINE-1) family. LINE-1s may cause gene inactivation and human disease. Read More

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Primary gingival tuberculosis in pregnancy: A rare combination.

J Indian Soc Periodontol 2018 Mar-Apr;22(2):178-181

Department of Periodontics, Dr. Ziauddin Ahmad Dental College, Faculty of Medicine, Aligarh Muslim University, Aligarh, Uttar Pradesh, India.

Tuberculosis (TB), a common chronic-specific granulomatous disease, has become rare in the developed countries. However, it is still a common cause of morbidity and mortality in India. Although it commonly involves the lungs, its presentation in the oral cavity is quite uncommon. Read More

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May 2018
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A systematic review of the literature of the three related disease entities cheilitis granulomatosa, orofacial granulomatosis and Melkersson - Rosenthal syndrome.

Curr Pediatr Rev 2018 May 14. Epub 2018 May 14.

Department of Pediatrics, Hospital Ostallgäu-Kaufbeuren, Kaufbeuren. Germany.

Background And Objective: Melkersson Rosenthal syndrome (MRS) is a rare disorder of unknown etiology and comprises the triad: orofacial edema, recurrent facial paralysis and lingua plicata. In the current literature confusing heterogeneity exists, mixing together the historically grown terms cheilitis granulomatosa or granulomatous cheilitis, Melkersson Rosenthal syndrome and the umbrella term orofacial granulomatosis (OFG).

Methods: We provide a systematic review comprising all three disease entities of orofacial granulomatosis using the computerized database "Pubmed Medline" entering the key words "orofacial granulomatosis" (141 references), "Melkersson-Rosenthal syndrome" (207 references), "granulomatous cheilitis" or "cheilitis granulomatosa" (102 references) back to 1956. Read More

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New onset colitis in an adult patient with chronic granulomatous disease treated with hematopoietic stem cell transplantation: a diagnostic dilemma.

Allergy Asthma Clin Immunol 2018 8;14:17. Epub 2018 May 8.

1Department of Medicine, Dalhousie University, QEII-Bethune Building Room 442, 1276 South Park Street, Halifax, NS B3H 2Y9 Canada.

Background: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency characterized by recurrent life-threatening bacterial and fungal infections, granuloma formation and intestinal disease. This disease is caused by defects in NADPH oxidase, which result in the inability of phagocytes (neutrophils, monocytes and macrophages) to destroy certain microbes. The only established curative therapy for CGD is hematopoietic stem cell transplantation. Read More

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Allergic and Immunologic Perspectives of Inflammatory Bowel Disease.

Clin Rev Allergy Immunol 2018 May 12. Epub 2018 May 12.

Division of Gastroenterology and Hepatology, Penn State Milton S Hershey Medical Center, 500 University Dr, Hershey, PA, 17033, USA.

Inflammatory bowel disease (IBD) is a chronic immune-mediated inflammatory condition primarily involving the gastrointestinal tract. It includes Crohn's disease (CD), ulcerative colitis (UC), and a less common phenotype-indeterminate colitis. It is thought to result from a complex interplay of environmental, microbial, and host factors including genetic factors, although the exact mechanism is not known. Read More

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Obstructive Lymphangitis Precedes Colitis in Murine Norovirus-Infected Stat1-Deficient Mice.

Am J Pathol 2018 May 17. Epub 2018 May 17.

Department of Comparative Medicine, University of Washington, Seattle, Washington.

Murine norovirus (MNV) is an RNA virus that can prove lethal in mice with impaired innate immunity. We found that MNV-4 infection of Stat1 mice was not lethal, but produced a 100% penetrant, previously undescribed lymphatic phenotype characterized by chronic-active lymphangitis with hepatitis, splenitis, and chronic cecal and colonic inflammation. Lesion pathogenesis progressed from early ileal enteritis and regional dilated lymphatics to lymphangitis, granulomatous changes in the liver and spleen, and, ultimately, typhlocolitis. Read More

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May 2018
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Prevention of Infectious Complications in Patients With Chronic Granulomatous Disease.

J Pediatric Infect Dis Soc 2018 May;7(suppl_1):S25-S30

Division of Infectious Diseases, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee.

Chronic granulomatous disease (CGD) is a primary immunodeficiency that confers a markedly increased risk of bacterial and fungal infections caused by certain opportunistic pathogens. Current evidence supports the use of prophylactic antibacterial, antifungal, and immunomodulatory therapies designed to prevent serious or life-threatening infections in patients with CGD. In this review, we discuss current strategies for the prevention of infections in children and adults with CGD and the evidence that supports those strategies. Read More

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Allogeneic Hematopoietic Cell Transplantation for Chronic Granulomatous Disease: Controversies and State of the Art.

J Pediatric Infect Dis Soc 2018 May;7(suppl_1):S31-S39

Division of Hematology/Oncology/Blood and Marrow Transplant, Department of Pediatrics, Medical College of Wisconsin and Children's Hospital of Wisconsin, Milwaukee.

Chronic granulomatous disease (CGD) is a congenital disorder characterized by recurrent life-threatening bacterial and fungal infections and development of severe inflammation secondary to a congenital defect in 1 of the 5 phagocyte oxidase (phox) subunits of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Hematopoietic cell transplant (HCT) is a curative treatment for patients with CGD that provides donor neutrophils with functional NADPH and superoxide anion production. Many characteristics of CGD, including preexisting infection and inflammation and the potential for cure with mixed-donor chimerism, influence the transplant approach and patient outcome. Read More

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May 2018
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Noninfectious Manifestations and Complications of Chronic Granulomatous Disease.

J Pediatric Infect Dis Soc 2018 May;7(suppl_1):S18-S24

Division of Pediatric Infectious Diseases, Vanderbilt University School of Medicine, Nashville, Tennessee.

Chronic granulomatous disease (CGD), a primary immunodeficiency characterized by a deficient neutrophil oxidative burst and the inadequate killing of microbes, is well known to cause a significantly increased risk of invasive infection. However, infectious complications are not the sole manifestations of CGD; substantial additional morbidity is driven by noninfectious complications also. These complications can include, for example, a wide range of inflammatory diseases that affect the gastrointestinal tract, lung, skin, and genitourinary tract and overt autoimmune disease. Read More

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Infectious Complications in Patients With Chronic Granulomatous Disease.

J Pediatric Infect Dis Soc 2018 May;7(suppl_1):S12-S17

The National Institutes of Health, National Institute of Allergy and Infectious Diseases, Bethesda, Maryland.

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Future of Care for Patients With Chronic Granulomatous Disease: Gene Therapy and Targeted Molecular Medicine.

J Pediatric Infect Dis Soc 2018 May;7(suppl_1):S40-S44

Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Disease, Bethesda, Maryland.

Chronic granulomatous disease is a rare and potentially fatal disorder of neutrophil function. Beyond current medical management and hematopoietic stem cell transplantation, new methods of gene therapy that use lentiviral vectors or gene editing might extend curative therapies to patients who lack a suitable transplantation donor while eliminating the risk of graft-versus-host disease. Furthermore, new therapies focused on altering the biology of phagolysosomes might offer novel targeted treatments for inflammatory complications in patients with chronic granulomatous disease. Read More

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Chronic Granulomatous Disease: Epidemiology, Pathophysiology, and Genetic Basis of Disease.

J Pediatric Infect Dis Soc 2018 May;7(suppl_1):S2-S5

Vanderbilt Vaccine Research Program and Division of Pediatric Infectious Diseases, Department of Pediatrics, Vanderbilt University School of Medicine and Medical Center, Nashville, Tennessee.

Chronic Granulomatous Disease is one of the classic primary immunodeficiencies of childhood. While the incidence and severity of bacterial and fungal infections have been greatly reduced in this patient population, much remains to be learned about the pathophysiology of the disease, particularly for autoinflammatory manifestations. In this review, we examine the epidemiology, pathophysiology, and genetic basis for CGD. Read More

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Considerations in the Diagnosis of Chronic Granulomatous Disease.

J Pediatric Infect Dis Soc 2018 May;7(suppl_1):S6-S11

Division of Allergy and Immunology, Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus.

Chronic granulomatous disease (CGD) is a rare primary immunodeficiency that is caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. The disease presents in most patients initially with infection, especially of the lymph nodes, lung, liver, bone, and skin. Patients with CGD are susceptible to a narrow spectrum of pathogens, and Staphylococcus aureus, Burkholderia cepacia complex, Serratia marcescens, Nocardia species, and Aspergillus species are the most common organisms implicated in North America. Read More

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May 2018
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A holistic approach to the mycetoma management.

PLoS Negl Trop Dis 2018 05 10;12(5):e0006391. Epub 2018 May 10.

Department of Surgery, Faculty of Medicine, Sennar University, Sennar, Sudan.

Mycetoma, one of the badly neglected tropical diseases, it is a localised chronic granulomatous inflammatory disease characterised by painless subcutaneous mass and formation of multiple sinuses that produce purulent discharge and grains. If untreated early and appropriately, it usually spread to affect the deep structures and bone resulting in massive damage, deformities and disabilities. It can also spread via the lymphatics and blood leading to distant secondary satellites associated with high morbidity and mortality. Read More

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Correction to: Raised Serum IL-8 Levels Are Associated with Excessive Fatigue in Female Carriers of X-Linked Chronic Granulomatous Disease in the UK.

J Clin Immunol 2018 Apr 23. Epub 2018 Apr 23.

Institute of Cellular Medicine, Newcastle University, Newcastle Upon Tyne, UK.

The original version of the article, "Raised Serum IL-8 Levels Are Associated with Excessive Fatigue in Female Carriers of X-Linked Chronic Granulomatous Disease in the UK" incorrectly listed the name of the fourth author as Fai W. Ng. The correct spelling of the author's name is WF Ng. Read More

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Host Defense Versus Immunosuppression: Unisexual Infection With Male or Female Differentially Impacts the Immune Response Against Invading Cercariae.

Front Immunol 2018 24;9:861. Epub 2018 Apr 24.

Division of Tropical Medicine and Infectious Diseases, Center of Internal Medicine II, University Medical Center Rostock, Rostock, Germany.

Infection with the intravascular diecious trematode . remains a serious tropical disease and public health problem in the developing world, affecting over 258 million people worldwide. During chronic infection, complex immune responses to tissue-entrapped parasite eggs provoke granulomatous inflammation which leads to serious damage of the liver and intestine. Read More

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April 2018
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Severe Multisystem Involvement of Chronic Granulomatous Disease in a Pediatric Patient.

J Trop Pediatr 2018 May 7. Epub 2018 May 7.

Pediatric Infectious Diseases Department, Istanbul Medical Faculty, Istanbul University, Capa/Fatih, Istanbul 34098, Turkey.

Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disorder identified by recurrent pyogenic and fungal infections infections secondary to defective nicotinamide adenine dinucleotide phosphate oxidase enzyme. In the present study, we demonstrated a case with a history of multiple segmental lung resections because of invasive bronchopulmonary aspergillosis, multifocal hepatic and splenic granulomas, bilateral adnexal calcific foci presumed to be related with old granulomatous infection and finally gastric outlet obstruction secondary to the involvement of the stomach wall thickening with granulomatous tissue. This is an extremely severe case of CGD with multiorgan involvement within a 10-year period after the diagnosis. Read More

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May 2018
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Sarcoid-Like Paracoccidioidomycosis in a Female Urban Dweller: Reviewing a Rare Clinical Condition in Brazil.

Mycopathologia 2018 May 8. Epub 2018 May 8.

Hospital of Clinics of Ribeirão Preto, Division of Dermatology of Internal Medicine Department, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.

We discuss the sarcoid-like clinical presentation, a rare type of infiltrative paracoccidioidomycosis (PCM) that is almost exclusively cutaneous, involves the face and histologically has a tuberculoid granulomatous pattern with few fungi. It is often misdiagnosed. In endemic regions of Brazil, PCM is more common among men from rural areas, while women of a reproductive age appear to be protected. Read More

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Gamma delta T cell expansion in Whipple's disease with muscular granulomatous vasculitis.

Infection 2018 May 3. Epub 2018 May 3.

Department of Internal Medicine and Infectious Diseases, CHU Bordeaux, Hôpital Haut Lévèque, 33600, Pessac, France.

Whipple's disease usually presents as chronic joint pain followed by digestive manifestations. However, many different presentations have been described in the literature. We report here the first proven case of muscular vasculitis related to Whipple's disease, associated with an expansion of circulating activated γδ T lymphocytes. Read More

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[A rare case of ocular scarrings in a patient with Vogt-Koyanagi-Harada disease].

Pan Afr Med J 2017 22;28:313. Epub 2017 Dec 22.

Université Mohamed V Souissi, Service d'Ophtalmologie A, Hôpital des Spécialités, CHU IBN Sina, Rabat, Maroc.

We here report the case of a 27-year old patient, followed-up in our Department for treatment of chronic Vogt-Koyanagi-Harada disease ( VKH disease). Fundus examination showed depigmentation of the retinal pigment epithelium and of the choroid, appearing as a pseudotumoral peripapillary lesion. Vogt-Koyanagi-Harada disease is a multisystem disorder, characterized by bilateral granulomatous panuveitis with serous exudative multifocal retinal detachment. Read More

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When Antimicrobial Therapy Is Not Enough: Respiratory Failure in a Patient with Chronic Granulomatous Disease.

Ann Am Thorac Soc 2018 May;15(5):630-632

Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania.

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Symptomatic hypercalcemia and scarring alopecia as presenting features of sarcoidosis.

Proc (Bayl Univ Med Cent) 2018 Apr 15;31(2):224-226. Epub 2018 Mar 15.

Division of Dermatology, Baylor University Medical Center, Dallas, Texas.

Sarcoidosis is a multisystem granulomatous disease most frequently affecting the lungs, lymph nodes, and eyes. Skin involvement occurs in approximately 25% to 35% of cases, with the scalp uncommonly affected. Abnormal calcium metabolism is associated with sarcoidosis and other granulomatous disorders and most commonly presents as hypercalciuria (40%-60%) and, less frequently, hypercalcemia (10%-20%). Read More

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Immunodetection of hepatic stellate cells in dogs with visceral leishmaniasis.

Parasitol Res 2018 Jun 27;117(6):1829-1837. Epub 2018 Apr 27.

Departamento de Patologia Veterinária, Faculdade de Ciências Agrárias e Veterinárias, Universidade Estadual Paulista (UNESP) , Via de Acesso Prof. Paulo Donato Castellane, s / n , Jaboticabal , SP , 14884-900 , Brasil.

Hepatic stellate cells (HSC), or Ito cells, store vitamin A when at rest but undergo phenotypic changes in situations of liver injury, which may induce fibrosis, and they may participate in the immune response in the liver. The objective of the present study was to investigate the role of HSC in the livers of dogs with visceral leishmaniasis (VL). Twenty-eight livers from dogs infected with VL that were living in an area endemic for the disease were evaluated, among which 13 were asymptomatic (A) and 15 were symptomatic (S). Read More

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June 2018
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Characterization of 4 New Mutations in the CYBB Gene in 10 Iranian Families With X-linked Chronic Granulomatous Disease.

J Pediatr Hematol Oncol 2018 Apr 26. Epub 2018 Apr 26.

Department of Pathology, Tehran University of Medical Sciences (TUMS).

Chronic granulomatous disease (CGD) is an inherited disease of the innate immune system that results from defects in 1 of the 5 subunits of nicotinamide adenine dinucleotide phosphate oxidase complex and leads to life-threatening infections with granuloma formation. During 3 years of study, we recognized 10 male patients with X-linked CGD from a tertiary referral center for immune deficiencies in Iran. The CGD patients were diagnosed according to clinical features and biochemical tests, including nitroblue tetrazolium and dihydrorhodamine-1, 2, 3 tests, performed on patients and their mothers. Read More

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Efficacy of long-term intralesional triamcinolone in Morbihan's disease and its possible association with mast cell infiltration.

Dermatol Ther 2018 Apr 23:e12609. Epub 2018 Apr 23.

Dermatology department, University Hospital of Paracelsus, Medical University of Salzburg, Salzburg, Austria.

Morbihan's disease is characterized by chronic persistent facial edema of the upper half of the face, absence of typical diagnostic findings, and refractoriness to treatment. A 44-year-old man was diagnosed with Morbihan's disease based on clinical signs and histopathology, which showed dermal edema in upper dermis, discrete lymphocytic infiltrate without granulomatous reaction, and mast cell infiltration. After long-term therapy with intralesional triamcinolone a remarkable objective and subjective clinical response was observed. Read More

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A Pilot Study Assessing the Ability of 5-Aminosalicylic Acid to Modulate the Immune Response in Chronic Beryllium Disease.

Ann Am Thorac Soc 2018 Apr;15(Supplement_2):S133

1 Division of Environmental and Occupational Health Sciences, Hollis Laboratory, Department of Medicine, National Jewish Health, Denver, Colorado; and.

Chronic beryllium disease is a granulomatous lung disease, characterized by the accumulation of macrophages and beryllium-specific CD4 T-cells that proliferate and produce T-helper cell type 1 cytokines. Previous studies indicate that beryllium-mediated oxidative stress enhances cytokine response in chronic beryllium disease CD4 T-cells. 5-Aminosalicylic acid (5-ASA) is currently used to treat inflammatory bowel disease and has both antioxidant and antiinflammatory actions. Read More

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April 2018
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Non-Clinical Efficacy and Safety Studies on G1XCGD, a Lentiviral Vector for Ex Vivo Gene Therapy of X-Linked Chronic Granulomatous Disease.

Hum Gene Ther Clin Dev 2018 Jun 17;29(2):69-79. Epub 2018 Apr 17.

1 Institute for Tumor Biology and Experimental Therapy , Georg-Speyer-Haus, Frankfurt, Germany .

Chronic granulomatous disease (CGD) is a debilitating primary immunodeficiency affecting phagocyte function due to the absence of nicotinamide dinucleotide phosphate (NADPH) oxidase activity. The vast majority of CGD patients in the Western world have mutations within the X-linked CYBB gene encoding for gp91 (NOX2), the redox center of the NADPH oxidase complex (XCGD). Current treatments of XCGD are not entirely satisfactory, and prior attempts at autologous gene therapy using gammaretrovirus vectors did not provide long-term curative effects. Read More

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June 2018
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Francisella philomiragia: Think of Chronic Granulomatous Disease.

J Clin Immunol 2018 Apr 16;38(3):257-259. Epub 2018 Apr 16.

Internal Medicine Department, La Paz Universitary Hospital, Madrid, Spain.

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April 2018
2 Reads

A clinical threat in patients with granulomatosis polyangiitis in remission: Subglottic stenosis.

Eur J Rheumatol 2018 Mar 11;5(1):69-71. Epub 2017 Apr 11.

Division of Rheumatology, Department of Internal Medicine, Süleyman Demirel University School of Medicine, Isparta, Turkey.

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatous disease that involves small- and medium-sized arteries and affects the main respiratory tracts and kidneys. Upper respiratory tract involvement usually occurs in 90% of patients, who most frequently present with symptoms of chronic sinusitis. Subglottic stenosis (SS) is a rare and severe complication that is usually observed in approximately 15% of patients. Read More

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March 2018
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