7,306 results match your criteria Chronic Granulomatous Disease


A case report of esophageal actinomycosis in an immunocompetent patient and review of the literature.

New Microbiol 2019 Feb 20;42(1). Epub 2019 Feb 20.

Laboratory of Microbiology and Virology, National Institute of Gastroenterology "S. De Bellis", Research Hospital, Castellana Grotte, Bari 70013, Italy.

Actinomycosis is a rare, chronic and slowly progressive granulomatous disease caused by Actinomyces spp., a Gram-positive anaerobic bacterium that rarely affects the esophagus. Although this infection is uncommon, it has been reported in both immunocompromised and immunocompetent individuals. Read More

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February 2019

Papulopustular Dermatitis in X-Linked Chronic Granulomatous Disease.

Front Pediatr 2018 31;6:429. Epub 2019 Jan 31.

Division of Pediatric Allergy and Immunology, Department of Pediatrics, University of Rochester Medical Center, Golisano Children's Hospital, Rochester, NY, United States.

Here we describe two term male infants diagnosed with X-linked CGD who present, in addition to frequent infection, with a unique papulopustular skin rash. CGD is caused by a number of genetic defects that impair phagocyte function. This disease results in recurrent infections and granuloma formation. Read More

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http://dx.doi.org/10.3389/fped.2018.00429DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6365455PMC
January 2019

Genetic and Clinical Profiles of Disseminated Bacillus Calmette-Guérin Disease and Chronic Granulomatous Disease in China.

Front Immunol 2019 29;10:73. Epub 2019 Jan 29.

Department of Infectious Diseases, Huashan Hospital, Fudan University, Shanghai, China.

Disseminated Bacillus Calmette-Guérin disease (D-BCG) in children with chronic granulomatous disease (CGD) can be fatal, while its clinical characteristics remain unclear because both diseases are extremely rare. The patients with CGD receive BCG vaccination, because BCG vaccination is usually performed within 24 h after delivery in China. We prospectively followed-up Chinese patients with CGD who developed D-BCG to characterize their clinical and genetic characteristics. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00073DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6361786PMC
January 2019

PPARγ Deficiency Exacerbates Fibrotic Response to Mycobacteria Peptide in Murine Sarcoidosis Model.

Am J Respir Cell Mol Biol 2019 Feb 11. Epub 2019 Feb 11.

East Carolina University, Dept of Internal Medicine, Greenville, North Carolina, United States ;

We established a murine model of multiwall carbon nanotube (MWCNT)-elicited chronic granulomatous disease which bears similarities to human sarcoidosis pathology including alveolar macrophage deficiency of peroxisome-proliferator-activated receptor gamma (PPARγ). Because lymphocyte reactivity to mycobacterial antigens has been reported in sarcoidosis, we hypothesized that addition of mycobacterial Early Secreted Antigenic Target Protein 6 (ESAT-6) to MWCNT might exacerbate pulmonary granulomatous pathology. MWCNT with or without ESAT-6 peptide-14 were instilled by oropharyngeal route into macrophage-specific PPARγ KO or wild-type mice. Read More

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http://dx.doi.org/10.1165/rcmb.2018-0346OCDOI Listing
February 2019
1 Read

Fifteen-minute consultation: Recognising primary immune deficiencies in children.

Arch Dis Child Educ Pract Ed 2019 Feb 7. Epub 2019 Feb 7.

Department of Paediatrics, Institute of Clinical Sciences, University of Gothenburg, Gothenburg, Sweden.

Children with primary immunodeficiency syndromes present with broad variation of clinical features and the consequences are often severe if not promptly recognised. Here, support is provided for the general paediatrician to recognise primary immunodeficiencies among the many children they meet in their clinical practice. Read More

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http://dx.doi.org/10.1136/archdischild-2018-315484DOI Listing
February 2019
1 Read

Clinical and Genetic characteristics of Chinese Pediatric Patients with Chronic Granulomatous Disease.

Pediatr Allergy Immunol 2019 Feb 4. Epub 2019 Feb 4.

China National Clinical Research Center for Respiratory Diseases, Department of Respiratory Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, China, 100045.

Background: Chronic granulomatous disease (CGD) is a rare disease in China, and very little large-scale studies have been conducted to date. We aimed to investigate the clinical and genetic features of CGD in Chinese pediatric patients.

Methods: Pediatric patients with CGD from Beijing Children's Hospital, Capital Medical University, China were enrolled from January 2006 to December 2016. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/pai.13033
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http://dx.doi.org/10.1111/pai.13033DOI Listing
February 2019
2 Reads
3.397 Impact Factor

Aortitis and pachymeningitis: an unusual combination in granulomatosis with polyangiitis (myeloperoxidase-associated vasculitis).

BMJ Case Rep 2019 Jan 28;12(1). Epub 2019 Jan 28.

Internal Medicine, Maricopa Integrated Health System, Phoenix, Arizona, USA.

Aortitis and pachymeningitis are uncommon manifestations of the antineutrophil cytoplasmic antibody-associated vasculitides, a group of systemic autoimmune diseases mainly affecting small vessels. We present a case of a 71-year-old woman with a remote history of idiopathic chronic granulomatous pachymeningitis complicated with aortic thickening due to autoimmune aortitis. Read More

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http://dx.doi.org/10.1136/bcr-2018-226795DOI Listing
January 2019
1 Read

Hypertrophic cervical spine pachymeningitis due to sarcoidosis: a case report.

Hosp Pract (1995) 2019 Feb 6:1-5. Epub 2019 Feb 6.

d Pathology , Lehigh Valley Health Network , Allentown , PA , USA.

Hypertrophic pachymeningitis (HP) is a chronic, progressive diffuse inflammatory condition that leads to thickening of the dura mater and can be idiopathic or associated with sarcoidosis among other disorders. In this case report, we present a rare case of cervical spine HP in a 29-year-old woman in the post-partum period, who had a history of pituitary adenoma and juvenile rheumatoid arthritis. Magnetic resonance imaging (MRI) of the spine revealed a soft tissue mass and moderate cord compression. Read More

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https://www.tandfonline.com/doi/full/10.1080/21548331.2019.1
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http://dx.doi.org/10.1080/21548331.2019.1575645DOI Listing
February 2019
8 Reads

The macrophage-specific V-ATPase subunit ATP6V0D2 restricts inflammasome activation and bacterial infection by facilitating autophagosome-lysosome fusion.

Autophagy 2019 Jan 25:1-16. Epub 2019 Jan 25.

a Department of Immunology , School of Basic Medicine, Tongji Medical College, Huazhong University of Science and Technology , Wuhan , China.

Macroautophagy/autophagy is a conserved ubiquitous pathway that performs diverse roles in health and disease. Although many key, widely expressed proteins that regulate autophagosome formation followed by lysosomal fusion have been identified, the possibilities of cell-specific elements that contribute to the autophagy fusion machinery have not been explored. Here we show that a macrophage-specific isoform of the vacuolar ATPase protein ATP6V0D2/subunit d2 is dispensable for lysosome acidification, but promotes the completion of autophagy via promotion of autophagosome-lysosome fusion through its interaction with STX17 and VAMP8. Read More

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http://dx.doi.org/10.1080/15548627.2019.1569916DOI Listing
January 2019
3 Reads
11.753 Impact Factor

Analysis of the rs2476601 polymorphism of PTPN22 in Mexican mestizo patients with leprosy.

Biomed Rep 2019 Feb 7;10(2):127-132. Epub 2019 Jan 7.

Laboratory of Immunology, Faculty of Biological and Chemical Sciences, Autonomous University of Sinaloa, Culiacán, Sinaloa 80010, México.

Leprosy, a human chronic granulomatous disease caused by , remains endemic in certain countries despite the use of multidrug therapy. Recently, several host genes modulating the immune responses to infection have been suggested to influence the acquisition and clinical course of leprosy. Lymphoid protein tyrosine phosphatase, encoded by the protein tyrosine phosphatase non-receptor type 22 () gene, serves a negative regulatory role in T cell activation. Read More

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http://dx.doi.org/10.3892/br.2019.1184DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341406PMC
February 2019

Sarcoidosis Presenting as Acute Respiratory Distress Syndrome.

Case Rep Med 2018 23;2018:6465180. Epub 2018 Dec 23.

Department of Pulmonary and Critical Care Medicine-Jefferson Health, Abington, PA, USA.

Sarcoidosis is a multisystem granulomatous disease of unknown origin. It typically involves the lungs and mediastinal lymph nodes in a chronic fashion. However, acute syndrome has been reported possibly in response to systemic release of proinflammatory cytokines. Read More

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http://dx.doi.org/10.1155/2018/6465180DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323477PMC
December 2018
1 Read

Two cases of Osteoarticular Mucor menace: A diagnostic and management conundrum.

Drug Discov Ther 2018 ;12(6):374-378

Department of Medicine, All India Institute of Medical Sciences.

Mucormycosis is an uncommon aggressive fungal infection usually seen in immunocompromised hosts or patients with burns and trauma. The common presentations include rhino-orbital-cerebral and pulmonary involvement. Osteoarticular involvement is a rare presentation of this disease. Read More

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http://dx.doi.org/10.5582/ddt.2018.01064DOI Listing
January 2018
1 Read

Predictive value of the red blood cell distribution width-to-platelet ratio for hepatic fibrosis.

Scand J Gastroenterol 2019 Jan 20:1-6. Epub 2019 Jan 20.

f Department of Laboratory Medicine , The Affiliated Hospital of Shaoxing University , Shaoxing , China.

Aims: The red blood cell distribution width-to-platelet ratio (RPR) has been reported to be an indicator of hepatic fibrosis in patients with chronic hepatitis B (HBV), nonalcoholic fatty liver disease (NAFLD) or chronic hepatitis C (HCV). However, no research has explored the RPR in all patients with hepatic fibrosis. There is a recognized need to establish whether the RPR could assess hepatic fibrosis and reflect the severity of fibrosis, regardless of the patient's etiology. Read More

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http://dx.doi.org/10.1080/00365521.2018.1558786DOI Listing
January 2019
5 Reads

Bleeding Assessment in female patients with the Hermansky-Pudlak syndrome - A Case Series.

Eur J Haematol 2019 Jan 19. Epub 2019 Jan 19.

Department of Surgery, School of Medicine, Medical Sciences Campus, Universityof Puerto Rico.

The Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous group of autosomal recessive disorders characterized by: oculocutaneous albinism (OCA); bleeding diathesis; and other systemic complications including: chronic granulomatous colitis, and pulmonary fibrosis. Despite HPS being a rare genetic disease worldwide, it is the most common single-gene disorder in the island of Puerto Rico (PR), particularly in the northwestern region, where it occurs with a frequency of 1:1,800 and where carrier frequency is estimated to be 1 out of 21 citizens. HPS thus represents a significant public health issue in PR. Read More

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http://dx.doi.org/10.1111/ejh.13210DOI Listing
January 2019
1 Read

(p47)-deficient chronic granulomatous disease: comprehensive genetic and flow cytometric analysis.

Blood Adv 2019 Jan;3(2):136-147

Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD.

Mutations in (p47) cause autosomal recessive chronic granulomatous disease (CGD) with abnormal dihydrorhodamine (DHR) assay and absent p47 protein. Genetic identification of mutations is complicated by adjacent highly conserved (>98%) pseudogenes ( and ). has GTGT at the start of exon 2, whereas the pseudogenes each delete 1 GT (ΔGT). Read More

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http://dx.doi.org/10.1182/bloodadvances.2018023184DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341190PMC
January 2019
4 Reads

Laryngeal Tuberculosis in Pregnant Women: A Case Report and Review of the Literature.

Cureus 2018 Nov 5;10(11):e3545. Epub 2018 Nov 5.

Internal Medicine, Hospital Nacional Arzobispo Loayza, Universidad Nacional Mayor De San Marcos, Lima, PER.

Tuberculosis is the most frequent granulomatous disease but the involvement of the larynx is rare. Immunosuppressed patients have a higher risk of developing this clinical form due to primoinfection or reactivation of latent tuberculosis. It is common to confuse the diagnosis of laryngeal tuberculosis with laryngeal cancer because they have similar macroscopic lesions and both produce dysphonia of chronic evolution. Read More

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http://dx.doi.org/10.7759/cureus.3545DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6324851PMC
November 2018
3 Reads

A rare case of a prostatic abscess, bacteremia and chronic granulomatous disease associated with Klebsiella pneumoniae.

J Infect Chemother 2019 Jan 11. Epub 2019 Jan 11.

Department of Infectious Diseases, The University of Tokyo; Department of Infection Control and Prevention, The University of Tokyo.

Chronic granulomatous disease (CGD) is a primary immunodeficiency disease characterized by severe recurrent infections such as pneumonia, liver and skin infections. However, prostatic abscesses are rare as only two cases have been reported thus far. We present the case of a 41-year-old patient with CGD who was admitted to the hospital with fever and subsequently, Klebsiella pneumoniae was identified on blood culture. Read More

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http://dx.doi.org/10.1016/j.jiac.2018.11.015DOI Listing
January 2019
1 Read
1.384 Impact Factor

Takayasu arteritis: differential diagnosis in a teenager with severe acute kidney injury - a case report.

J Bras Nefrol 2019 Jan 10. Epub 2019 Jan 10.

Hospital Geral de Fortaleza, Departamento de Nefrologia, Fortaleza, CE, Brasil.

Takayasu arteritis (TA) is a chronic granulomatous inflammatory condition of unknown cause that involves large vessels - particularly the aorta and its branches - such as the carotid, coronary, pulmonary, and renal arteries. The left subclavian artery is the most frequently involved vessel. Stenosis of the renal artery has been reported in 23-31% of the cases and may result in malignant hypertension, ischemic renal disease, decompensated heart failure, and premature death. Read More

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http://dx.doi.org/10.1590/2175-8239-JBN-2018-0174DOI Listing
January 2019
2 Reads

Exon skipping in CYBB mRNA and skewed inactivation of X chromosome cause late-onset chronic granulomatous disease.

Pediatr Hematol Oncol 2019 Jan 11:1-9. Epub 2019 Jan 11.

a Department of Pediatrics , Ehime University Graduate School of Medicine , Toon , Ehime , Japan.

Chronic granulomatous disease (CGD) is a hereditary immunodeficiency syndrome caused by a defect in the NADPH oxidase complex, which is essential for bactericidal function of phagocytes. Approximately 70% of patients with CGD have a mutation in the CYBB gene on the X chromosome, resulting in defective expression of gp91, one of the membrane-bound subunits of NADPH oxidase. Although most patients with X-linked CGD are males, owing to transmission of this disease as an X-linked recessive trait, there are female patients with X-linked CGD. Read More

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https://www.tandfonline.com/doi/full/10.1080/08880018.2018.1
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http://dx.doi.org/10.1080/08880018.2018.1522402DOI Listing
January 2019
7 Reads

The roles of NADPH oxidase in modulating neutrophil effector responses.

Mol Oral Microbiol 2019 Jan 11. Epub 2019 Jan 11.

Department of Microbiology and Immunology, University of Louisville, Louisville, Kentucky.

Neutrophils are phagocytic innate immune cells essential for killing bacteria via activation of a wide variety of effector responses and generation of large amounts of reactive oxygen species (ROS). Majority of the ROS in neutrophils is generated by activation of the superoxide-generating enzyme nicotinamide adenine dinucleotide phosphate (NADPH) oxidase. Independent of their anti-microbial function, NADPH oxidase-derived ROS have emerged as key regulators of host immune responses and neutrophilic inflammation. Read More

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http://doi.wiley.com/10.1111/omi.12252
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http://dx.doi.org/10.1111/omi.12252DOI Listing
January 2019
7 Reads

Proteome analysis of human neutrophil granulocytes from patients with monogenic disease using data-independent acquisition.

Mol Cell Proteomics 2019 Jan 10. Epub 2019 Jan 10.

Wellcome Trust Centre for Cell Biology, University of Edinburgh, Germany

Neutrophil granulocytes are critical mediators of innate immunity and tissue regeneration. Rare diseases of neutrophil granulocytes may affect their differentiation and/or functions. However, there are very few validated diagnostic tests assessing the functions of neutrophil granulocytes in these diseases. Read More

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http://www.mcponline.org/lookup/doi/10.1074/mcp.RA118.001141
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http://dx.doi.org/10.1074/mcp.RA118.001141DOI Listing
January 2019
5 Reads

Melkersson-Rosenthal syndrome: a case report of a rare disease with overlapping features.

Allergy Asthma Clin Immunol 2019 5;15. Epub 2019 Jan 5.

2Department of Neuroscience, Division of Dentistry, University of Padua, Via Giustiniani 2, 35128 Padua, Italy.

Background: Melkersson-Rosenthal syndrome (MRS) is a rare, neuro-mucocutaneous disease which presents as orofacial swelling, facial palsy and fissured tongue. These symptoms may occur simultaneously or, more frequently, with a oligosymptomatic or monosymptomatic pattern. Swelling, that is the most common initial finding, may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue. Read More

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https://aacijournal.biomedcentral.com/articles/10.1186/s1322
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http://dx.doi.org/10.1186/s13223-018-0316-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320604PMC
January 2019
5 Reads

Interleukin-4 Receptor Alpha Expressing B Cells Are Essential to Down-Modulate Host Granulomatous Inflammation During Schistosomasis.

Front Immunol 2018 18;9:2928. Epub 2018 Dec 18.

International Center for Genetic Engineering and Biotechnology (ICGEB), Cape Town Component, Cape Town, South Africa.

Schistosomiasis (bilharzia) is a parasitic helminth disease that can cause severe inflammatory pathology leading to organ damage in humans. Failure of the host to regulate egg-driven granulomatous inflammation causes host morbidity during chronic infection with . Although the importance of B cells in regulating pathology during chronic infection has been well defined, the specific contribution of IL-4Rα-expressing B cells is still unknown. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2018.02928
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http://dx.doi.org/10.3389/fimmu.2018.02928DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305417PMC
December 2018
8 Reads

The role of biologics in the treatment of chronic granuloma annulare.

Int J Dermatol 2019 Jan 7. Epub 2019 Jan 7.

David Geffen School of Medicine at University of California, Los Angeles, CA, USA.

Background: Granuloma annulare (GA), a benign inflammatory skin disease, is considered a Th1-type delayed hypersensitivity reaction. Localized GA is likely to resolve spontaneously, whereas disseminated GA (DGA) may persist for decades and can be resistant to treatment. Biologics including TNF-α inhibitors have been proposed and utilized as salvage therapy for GA and other related diseases, interstitial granulomatous dermatitis (IGD), and actinic granuloma (AG). Read More

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http://dx.doi.org/10.1111/ijd.14350DOI Listing
January 2019
5 Reads

Prevalence of Mycobacterium avium subspecies paratuberculosis IS 900 DNA in biopsy tissues from patients with Crohn's disease: histopathological and molecular comparison with Johne's disease in Fars province of Iran.

BMC Infect Dis 2019 Jan 7;19(1):23. Epub 2019 Jan 7.

Department of Pathology, School of Veterinary Medicine, Shiraz University, PO Box 71345-1731, Shiraz, Iran.

Background: Crohn's disease is a chronic enteritis of humans that affects the gastrointestinal tract, especially the terminal ileum, cecum and colon. The etiology of this disease is still unknown but seems to be multifactorial. There are reports about the potential link between Crohn's disease in humans and the causative agent of Johne's disease in ruminants. Read More

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http://dx.doi.org/10.1186/s12879-018-3619-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322312PMC
January 2019
3 Reads

Ethical considerations of using a single minor donor for three bone marrow harvests for three HLA-matched siblings with primary immunodeficiency.

Pediatr Blood Cancer 2019 Jan 4:e27602. Epub 2019 Jan 4.

Duke University Medical Center, Durham, North Carolina.

Allogeneic hematopoietic stem cell transplantation is curative for primary immunodeficiencies. Bone marrow from an unaffected human leukocyte antigen (HLA)-identical sibling donor is the ideal graft source. For minor donors, meaningful consent or assent may not be feasible, and permission from parents or legal guardians is considered acceptable. Read More

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http://dx.doi.org/10.1002/pbc.27602DOI Listing
January 2019

Rubella Virus-Associated Cutaneous Granulomatous Disease: a Unique Complication in Immune-Deficient Patients, Not Limited to DNA Repair Disorders.

J Clin Immunol 2019 Jan 3;39(1):81-89. Epub 2019 Jan 3.

Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Department of Pediatrics, University of Washington, Seattle, WA, USA.

The association of immunodeficiency-related vaccine-derived rubella virus (iVDRV) with cutaneous and visceral granulomatous disease has been reported in patients with primary immunodeficiency disorders (PIDs). The majority of these PID patients with rubella-positive granulomas had DNA repair disorders. To support this line of inquiry, we provide additional descriptive data on seven previously reported patients with Nijmegen breakage syndrome (NBS) (n = 3) and ataxia telangiectasia (AT) (n = 4) as well as eight previously unreported patients with iVDRV-induced cutaneous granulomas and DNA repair disorders including NBS (n = 1), AT (n = 5), DNA ligase 4 deficiency (n = 1), and Artemis deficiency (n = 1). Read More

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http://link.springer.com/10.1007/s10875-018-0581-0
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http://dx.doi.org/10.1007/s10875-018-0581-0DOI Listing
January 2019
7 Reads

Cytomegalovirus in Pediatric Hematopoietic Stem Cell Transplantation: A Case-Based Panel Discussion of Current Challenges.

J Pediatric Infect Dis Soc 2018 Dec;7(suppl_2):S72-S74

Division of Infectious Diseases, Department of Pediatrics, Seattle Children's Hospital, Washington.

Cytomegalovirus (CMV) remains a significant contributor to morbidity and death after pediatric solid and stem cell transplantation. Decisions regarding prevention and treatment often lack pediatric-specific data to drive decision making. We present here a case-based discussion around some of these specific topics and focus on approaches to CMV prevention, post-CMV secondary prophylaxis options, and identification and treatment of resistant CMV infection, including emerging antiviral agents and the use of cytotoxic CMV-specific T-cells, in the setting of pediatric hematopoietic stem cell transplantation. Read More

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http://dx.doi.org/10.1093/jpids/piy104DOI Listing
December 2018

Immunoglobulin (Ig)G-4 related myositis - A new entity?

Neuromuscul Disord 2019 Jan 24;29(1):70-74. Epub 2018 Nov 24.

Department of Neuropathology, Charité - Universitätsmedizin Berlin, Charitéplatz 1 / Virchowweg 15 (int.) Berlin, Germany. Electronic address:

Immunoglobulin (Ig)G4-related disease is an uncommon systemic autoimmune disorder characterized by infiltration of IgG4 plasma cells in different organs and elevated levels of IgG4 in peripheral blood. So far, only one case of myositis with abundant IgG4 plasma cells has been reported and classified as 'polymyositis'. We present an unusual case of chronic inflammatory myopathy in a context of rheumatoid arthritis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09608966183104
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http://dx.doi.org/10.1016/j.nmd.2018.11.009DOI Listing
January 2019
7 Reads

Porcine Circovirus type 2 - Systemic disease on pig farms and associated knowledge of key players in the pig industry in Central Uganda.

Int J Vet Sci Med 2018 Dec 28;6(2):178-185. Epub 2018 Aug 28.

College of Veterinary Medicine, Animal Resources and Biosecurity, Makerere University, P.O. BOX 7062, Kampala, Uganda.

Porcine Circovirus type 2 (PCV2) infections and associated diseases have been rarely studied in Africa. There is no report of PCV2 infection-associated morbidity and the level of awareness of stakeholders has never been investigated in Uganda. This cross sectional survey investigated the occurrence of Porcine Circovirus type 2 - systemic disease (PCV2-SD) among pigs and the associated level of awareness of stakeholders in Central Uganda. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S23144599183006
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http://dx.doi.org/10.1016/j.ijvsm.2018.08.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6286401PMC
December 2018
7 Reads

Pulmonary Sarcoidosis: Prognostic Factors at Diagnosis in Patients from North of Portugal.

Reumatol Clin 2018 Dec 14. Epub 2018 Dec 14.

Faculdade de Medicina-Universidade do Porto, Porto, Portugal; Pneumology Department and Diffuse Lung Disease Study Group/Centro Hospitalar Universitário de São João, Porto, Portugal. Electronic address:

Background: Sarcoidosis is a multisystemic granulomatous disease that affects the lungs in more than 90% of the patients. It is associated with a variable clinical course and considering all the different forms of disease presentation, there are an absence of reliable clinical prognostic markers that can predict the outcome at diagnosis.

Objective: The aim of our study was to investigate prognostic factors at diagnosis in a population of sarcoidosis patients from Northern Portugal. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S1699258X183023
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http://dx.doi.org/10.1016/j.reuma.2018.10.004DOI Listing
December 2018
8 Reads

Chronic dacryocystitis caused by sarcoidosis.

Arch Soc Esp Oftalmol 2018 Dec 14. Epub 2018 Dec 14.

Servicio de Anatomía Patológica, Fundació Althaia, Xarxa Assistencial Universitària de Manresa, Manresa, Barcelona, España.

Sarcoidosis of lacrimal sac is a very uncommon disease. When the patient has symptoms of lacrimal sac diseases, it is necessary to take into account the histological evaluation, to demonstrate the involvement of the lacrimal sac by this pathology. In patients with sarcoidosis history, the treatment is surgical and it is suggested to do an external dacryocystorhinostomy, in order to take a biopsy, to get a diagnosis and to establish the incidence of this pathology. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S03656691183033
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http://dx.doi.org/10.1016/j.oftal.2018.10.010DOI Listing
December 2018
7 Reads

Unusual presentation of Q fever in a kidney-pancreas transplant recipient.

Transpl Infect Dis 2018 Dec 12:e13037. Epub 2018 Dec 12.

Division of Infectious Diseases, Carver College of Medicine University of Iowa, Iowa City, Iowa.

Q fever is uncommon in solid organ transplant (SOT) recipients. We describe a case of granulomatous lung disease as an unusual presentation of chronic Q fever in a kidney-pancreas transplant recipient. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/tid.13037
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http://dx.doi.org/10.1111/tid.13037DOI Listing
December 2018
11 Reads

The long-term prognostic significance of sarcoidosis-associated pulmonary hypertension - A cohort study.

Clin Immunol 2018 Dec 10. Epub 2018 Dec 10.

Department of Medicine 'B', Sheba Medical Center, Tel-Hashomer, Israel; Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel; Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel. Electronic address:

Background: Sarcoidosis is a multisystem, chronic, progressive, granulomatous disease. Sarcoidosis-associated pulmonary hypertension is a well described, but not common, complication of sarcoidosis. In small scale studies, it has been previously described as manifestation of advanced disease and was found to be associated increased morbidity and mortality. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15216616183072
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http://dx.doi.org/10.1016/j.clim.2018.12.012DOI Listing
December 2018
3 Reads

Disease-Specific Enteric Microbiome Dysbiosis in Inflammatory Bowel Disease.

Front Med (Lausanne) 2018 20;5:304. Epub 2018 Nov 20.

Department of Bacteria, Parasites and Fungi, Statens Serum Institut, Copenhagen, Denmark.

Inflammatory Bowel disease (IBD) is traditionally divided into Crohn's disease (CD) and ulcerative colitis (UC). UC is a relapsing non-transmural inflammatory disease that is restricted to the colon and is characterized by flare-ups of bloody diarrhea. CD is a chronic, segmental localized granulomatous disease that can affect any part of the entire gastrointestinal tract. Read More

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http://dx.doi.org/10.3389/fmed.2018.00304DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6256240PMC
November 2018
7 Reads

Narrative review of therapies for chronic enteropathies in dogs and cats.

J Vet Intern Med 2019 Jan 6;33(1):11-22. Epub 2018 Dec 6.

Department of Veterinary Clinical Sciences, College of Veterinary Medicine, Iowa State University, Ames, Iowa.

Background: The optimal medical treatment for chronic enteropathy (CE) in dogs and cats is controversial. Sequential treatment using diet, antimicrobials, and immunosuppressive drugs is the most common strategy used by clinicians.

Objectives: To review the evidence for the effectiveness of dietary, drug, and alternative health interventions for inducing clinical remission in dogs and cats with CE. Read More

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http://dx.doi.org/10.1111/jvim.15345DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335544PMC
January 2019
7 Reads

Immunopathology of Aspergillus Infections in Children with Chronic Granulomatous Disease and Cystic Fibrosis.

Authors:
Adilia Warris

Pediatr Infect Dis J 2018 Dec 10. Epub 2018 Dec 10.

MRC Centre for Medical Mycology, Aberdeen Fungal Group, Institute of Medical Sciences, University of Aberdeen, UK.

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http://dx.doi.org/10.1097/INF.0000000000002265DOI Listing
December 2018
3 Reads

Two atypical presentations of lepra reactions.

Int J Mycobacteriol 2018 Oct-Dec;7(4):390-393

Army College of Medical Sciences, New Delhi, India.

Leprosy or Hansen's disease is a chronic infectious granulomatous disease with varied presentation, especially in the setting of lepra reactions. We report two such atypical presentations each of Type I and Type II Lepra reactions; the first being an elderly male presenting with fever, while the second case being of a young boy being evaluated for cervical lymphadenitis. Read More

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http://dx.doi.org/10.4103/ijmy.ijmy_105_18DOI Listing
December 2018
9 Reads

Atypical presentation of necrobiosis lipoidica in a pediatric patient.

Pediatr Dermatol 2019 Jan 2;36(1):e31-e33. Epub 2018 Dec 2.

Dermatology Department, University of Health Sciences, Şişli Hamidiye Etfal Training and Research Hospital, İstanbul, Turkey.

Necrobiosis lipoidica (NL) is a rare chronic granulomatous inflammatory skin disease characterized by brownish-red papules and yellowish plaques with atrophic centers, which usually affect the legs, bilaterally. The average age of onset is 30-40 years, and there are very few reported cases of necrobiosis lipoidica in children. A nondiabetic girl aged 14 years presented to our clinic with a history of an asymptomatic, 7 × 5-cm single red plaque over her back in the interscapular area, which had started 5 years ago. Read More

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http://doi.wiley.com/10.1111/pde.13716
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http://dx.doi.org/10.1111/pde.13716DOI Listing
January 2019
16 Reads

Chronic granulamatous disease: Two decades of experience from a paediatric immunology unit in a country with high rate of consangineous marriages.

Scand J Immunol 2019 Feb 23;89(2):e12737. Epub 2019 Jan 23.

Department of Pediatric Immunology, Ege University Faculty of Medicine, Izmir, Turkey.

Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by susceptibility to bacterial and fungal infections resulting from the inadequacy of phagocytic leucocytes to produce reactive oxygen radicals. CGD is a genetically heterogeneous disease with an X-linked recessive (XR-CGD) form caused by mutations in the CYBB (OMIM #300481) gene encoding the gp91(phox) protein, and an autosomal recessive (AR-CGD) form caused by mutations in the CYBA (OMIM #608508), NCF1 (OMIM #608512), NCF2 (OMIM #608515) and NCF4 (OMIM #601488) genes encoding p22(phox), p47(phox), p67(phox) and p40(phox), respectively. The genetic mutation of one of the cytosolic p47phox/p67phox proteins and membrane-bound gp91phox/p22phox proteins, which constitutes the NADPH oxidase enzyme complex, causes the disease. Read More

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http://dx.doi.org/10.1111/sji.12737DOI Listing
February 2019
9 Reads

Bartonella infections in cats and dogs including zoonotic aspects.

Parasit Vectors 2018 Dec 4;11(1):624. Epub 2018 Dec 4.

Departament de Medicina i Cirurgia Animals, Facultat de Veterinària, Universitat Autònoma de Barcelona, 08193, Bellaterra, Spain.

Bartonellosis is a vector-borne zoonotic disease with worldwide distribution that can infect humans and a large number of mammals including small companion animals (cats and dogs). In recent years, an increasing number of studies from around the world have reported Bartonella infections, although publications have predominantly focused on the North American perspective. Currently, clinico-pathological data from Europe are more limited, suggesting that bartonellosis may be an infrequent or underdiagnosed infectious disease in cats and dogs. Read More

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http://dx.doi.org/10.1186/s13071-018-3152-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6280416PMC
December 2018
2 Reads

Granulomatous amebic encephalitis caused by Acanthamoeba in an immuncompetent child.

Turk J Pediatr 2018 ;60(3):340-343

Division of Infectious Diseases, İstanbul University İstanbul Faculty of Medicine, İstanbul, Turkey.

Sütçü M, Aktürk H, Gülümser-Şişko S, Acar M, Erol OB, Somer A, Bilgiç B, Salman N. Granulomatous amebic encephalitis caused by Acanthamoeba in an immuncompetent child. Turk J Pediatr 2018; 60: 340-343. Read More

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http://dx.doi.org/10.24953/turkjped.2018.03.019DOI Listing
January 2018
4 Reads

Hepatic leptospiral infections in dogs without obvious renal involvement.

J Vet Intern Med 2019 Jan 29;33(1):141-150. Epub 2018 Nov 29.

Department of Veterinary Medicine, University of Cambridge, Cambridge, United Kingdom.

Background: Reports of chronic hepatitis in dogs caused by Leptospira spp. are confined to small case series. Fluorescence in situ hybridization (FISH) allows the identification of spirochetes in liver samples. Read More

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http://dx.doi.org/10.1111/jvim.15340DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335520PMC
January 2019
12 Reads

Granulomatous Lobular Mastitis: Antituberculous Treatment and Outcome in 22 Patients.

Breast Care (Basel) 2018 Oct 5;13(5):359-363. Epub 2018 Jul 5.

Department of Breast Surgery, The Second Hospital of Shandong University, Jinan, Shandong, China.

Background: Granulomatous lobular mastitis (GLM) is a rare chronic inflammatory condition of the breast. The purpose of this study was to describe antituberculous treatment of GLM and the long-term follow-up outcome.

Methods: This retrospective study included 22 patients who had been histopathologically diagnosed with GLM at the Second Hospital of Shandong University from January 2011 to March 2015. Read More

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http://dx.doi.org/10.1159/000487935DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6257198PMC
October 2018
13 Reads

Human Chronic Necrotizing Granulomatous Meningoencephalitis: A Novel Case Report.

Case Rep Neurol 2018 Sep-Dec;10(3):302-308. Epub 2018 Oct 24.

Geisinger Medical Center, Danville, Pennsylvania, USA.

Necrotizing and granulomatous meningoencephalitis are common central nervous system diseases known to affect canines. To date, necrotizing granulomatous meningoencephalitis has yet to be described in humans. Current studies of presumed pathogenesis and possible treatment options have only been described in canines. Read More

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https://www.karger.com/Article/FullText/494079
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http://dx.doi.org/10.1159/000494079DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6243906PMC
October 2018
14 Reads

Long-term use of Le Fort I osteotomy for the management of nasopharyngeal rhinosporidiosis: A case series.

Ear Nose Throat J 2018 Oct-Nov;97(10-11):E36-E43

Department of Craniofacial Surgery, Nitte Meenakshi Institute of Craniofacial Surgery, Deralakatte, Mangalore, Karnataka, India.

Rhinosporidiosis is a rare, chronic, granulomatous infection of the mucous membranes that mainly involves the nose and nasopharynx; it occasionally involves the pharynx, conjunctiva, larynx, trachea and, rarely, the skin. The characteristic clinical features of this disease include the formation of painless polyps in the nasal mucosa or the nasopharynx that bleed easily on touch. At our center, excision of the lesion with a Le Fort I osteotomy is carried out in patients (1) in whom two or more previous attempts at excision of biopsy-proven rhinosporidiosis arising from the nasal mucosa was carried out or (2) in whom the rhinosporidiosis arises from the nasophayrngeal mucosa and/or extranasal sites. Read More

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November 2018
4 Reads
0.881 Impact Factor

Idiopathic granulomatous mastitis: A retrospective cohort study between 44 patients with different treatment modalities.

Ann Med Surg (Lond) 2018 Dec 9;36:162-167. Epub 2018 Nov 9.

Department of Surgery, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

Background: Idiopathic granulomatous mastitis (IGM) is an uncommon benign chronic inflammatory disease which can clinically and radiographically mimic abscess or breast cancer. Definitive diagnosis was made by histopathology and exclusion of an identifying etiology. Optimal treatment has not been yet established. Read More

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http://dx.doi.org/10.1016/j.amsu.2018.11.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6240599PMC
December 2018
1 Read

Approach to Molecular Diagnosis of Chronic Granulomatous Disease (CGD): an Experience from a Large Cohort of 90 Indian Patients.

J Clin Immunol 2018 Nov 23;38(8):898-916. Epub 2018 Nov 23.

National Institute of Immunohaematology-ICMR, 13th floor, KEM Hospital Campus, Mumbai, Parel, 400012, India.

Background: Chronic granulomatous disease (CGD) is characterized by mutation in any one of the five genes coding NADPH oxidase components that leads to functional abnormality preventing the killing of phagocytosed microbes by affecting the progression of a respiratory burst. CGD patients have an increased susceptibility to infections by opportunistic and pathogenic organisms. Though initial diagnosis of CGD using a nitroblue tetrazolium (NBT) test or dihydrorhodamine (DHR) test is relatively easy, molecular diagnosis is challenging due to involvement of multiple genes, presence of pseudogenes, large deletions, and GC-rich regions, among other factors. Read More

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http://dx.doi.org/10.1007/s10875-018-0567-yDOI Listing
November 2018
10 Reads

[Chronic granulomatous disease as a rare differential diagnosis of inflammatory bowel disease].

Z Gastroenterol 2018 Dec 22;56(12):1507-1512. Epub 2018 Nov 22.

I. Medizinische Klinik und Poliklinik, Gastroenterologie und Hepatologie, Universitätsmedizin Mainz.

Background:  Chronic granulomatous disease is a rare disease with a prevalence of approximately 150 cases in Germany. An intestinal manifestation that mimics chronic inflammatory bowel disease (IBD) has only been described in a few cases. As a result of a deficient superoxide-synthesis, frequent and recurrent infections caused by rare pathogens have been described. Read More

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http://dx.doi.org/10.1055/a-0732-5865DOI Listing
December 2018
13 Reads

[Chronic granulomatous disease: multiple infections as clinical presentation. Pediatric case report].

Arch Argent Pediatr 2018 Dec;116(6):e744-e748

Servicio de Infectología, Hospital de Niños Sor María Ludovica.

Chronic granulomatous disease is an uncommon primary immunodeficiency due to a defect of the killing activity of phagocytes, caused by mutations in any of the genes encoding subunits of the superoxide-generating phagocyte NADPH oxidase system. The incidence is 1 in 250 000 live births. It can occur from infancy to adulthood, usually in children under 2 years. Read More

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http://dx.doi.org/10.5546/aap.2018.e744DOI Listing
December 2018
2 Reads