8,085 results match your criteria Chronic Granulomatous Disease

A Case of Adalimumab-Induced Granulomatous Interstitial Nephritis.

Cureus 2021 Jun 28;13(6):e15986. Epub 2021 Jun 28.

Nephrology, University Hospital Galway, Galway, IRL.

Adalimumab is a monoclonal antibody targeting tumour necrosis factor-alpha (TNF-alpha) and is used for the treatment of numerous autoimmune conditions. There is a paucity of evidence linking adalimumab with granulomatous interstitial nephritis (GIN). We describe a renal biopsy-proven case of GIN secondary to adalimumab therapy. Read More

View Article and Full-Text PDF

A Case of Sarcoid-like Granulomatous Lung Disease with Subacute Progression in Silicosis.

Intern Med 2021 Jul 30. Epub 2021 Jul 30.

Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan.

A 67-year-old man was admitted to our hospital with cough and fatigue. He had had long-term exposure to silica due to cement processing. Chest computed tomography showed bilateral centrilobular nodules, and hilar and mediastinal lymphadenopathy with calcification, suggesting chronic silicosis. Read More

View Article and Full-Text PDF

A doggy tale: Risk of zoonotic infection with Bordetella bronchiseptica for cystic fibrosis (CF) patients from live licenced bacterial veterinary vaccines for cats and dogs.

J Clin Pharm Ther 2021 Jul 30. Epub 2021 Jul 30.

Laboratory for Disinfection and Pathogen Elimination Studies, Northern Ireland Public Health Laboratory, Nightingale (Belfast City) Hospital, Belfast, UK.

What Is Known And Objective: Live-attenuated bacterial veterinary vaccines can constitute an infection risk for individuals with any defect in their phagocytic function, including chronic granulomatous disease, leukocyte adhesion deficiency, myeloperoxidase deficiency, as well as Chediak-Higashi syndrome, from accidental acquisition of licenced attenuated live bacterial vaccine, at vaccination or from their vaccinated pet. Ownership of small companion animals, including cats and dogs, is popular within the cystic fibrosis (CF) community. These animals require vaccines as part of their routine care, which may involve live viral and bacterial vaccines, with potential for infection in the CF owner. Read More

View Article and Full-Text PDF

Malignant and Non-Malignant Causes of Hypercalcemia: A Retrospective Study at a Tertiary Care Hospital in Pakistan.

Cureus 2021 Jun 22;13(6):e15845. Epub 2021 Jun 22.

Medicine, Endocrinology, Aga Khan University Hospital, Karachi, PAK.

Background: Hypercalcemia is a common electrolyte abnormality presenting with a variety of symptoms. The common causes are primary hyperparathyroidism and malignancy associated with hypercalcemia. However, iatrogenic hypercalcemia with the overzealous use of vitamin D has now emerged as another important cause of hypercalcemia over the past decade. Read More

View Article and Full-Text PDF

Endothelial Function and Oxidative Stress in X-Linked, gp91 Deficiency, Chronic Granulomatous Disease.

JACC Case Rep 2020 Aug 5;2(10):1480-1483. Epub 2020 Aug 5.

Department of Cardiovascular Regeneration and Medicine, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima, Japan.

Two patients with X-linked chronic granulomatous disease without NAPDH oxidase activity and with high responses of flow-mediated vasodilation are reported. Bone marrow transplantation restored oxidative stress to the levels of those in healthy subjects and decreased flow-mediated vasodilation to the levels of those in healthy subjects in both of the patients. (). Read More

View Article and Full-Text PDF

Retrospective study of elderly onset sarcoidosis in Tunisian patients.

Sarcoidosis Vasc Diffuse Lung Dis 2021 28;38(2):e2021016. Epub 2021 Jun 28.

Department of Internal Medicine, Hedi Chaker University Hospital, Sfax, Tunisia.

Background: Sarcoidosis is a systemic granulomatous disease of unknown etiology. It affects mostly young adults. In the elderly, the presentation of this disease is different, often posing positive diagnosis problems. Read More

View Article and Full-Text PDF

Editorial: Interstitial Lung Disease in Primary Immunodeficiencies.

Front Immunol 2021 8;12:699126. Epub 2021 Jul 8.

UCL Respiratory, University College London, London, United Kingdom.

View Article and Full-Text PDF

NADPH Oxidase Limits Collaborative Pattern-Recognition Receptor Signaling to Regulate Neutrophil Cytokine Production in Response to Fungal Pathogen-Associated Molecular Patterns.

J Immunol 2021 08 23;207(3):923-937. Epub 2021 Jul 23.

Department of Pediatrics, School of Medicine, Washington University in St. Louis, St. Louis, MO;

Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by genetic defects in leukocyte NADPH oxidase, which has both microbicidal and immunomodulatory roles. Hence, CGD is characterized by recurrent bacterial and fungal infections as well as aberrant inflammation. Fungal cell walls induce neutrophilic inflammation in CGD; yet, underlying mechanisms are incompletely understood. Read More

View Article and Full-Text PDF

Primary biliary cirrhosis in early childhood - A rare case report.

Int J Surg Case Rep 2021 Jul 18;85:106215. Epub 2021 Jul 18.

Pir Abdul Qadir Shah Jeelani Institute of Medical Sciences, Gambat, Sindh, Pakistan.

Introduction And Importance: Primary biliary cirrhosis (PBC) is a chronic and progressive autoimmune liver disease with no known etiology. This disease is mainly characterized by granulomatous destruction of intrahepatic biliary ducts, severe peri-portal inflammation, and ultimate progress to liver fibrosis and cirrhosis. Here, we report a five-year-old girl diagnosed with PBC, presented to us with end-stage liver disease for liver transplantation. Read More

View Article and Full-Text PDF

Crohn's disease with pulmonary granuloma in a child: a case report and review of the literature.

Transl Pediatr 2021 Jun;10(6):1728-1736

Department of Pediatrics, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai, China.

Crohn's disease (CD) is a chronic granulomatous disease that affects the gastrointestinal system. Additionally, CD has multiple extraintestinal manifestations, and bronchopulmonary manifestations are extremely rare. Pulmonary lesions can occur before the diagnosis of CD; thus, pulmonary manifestations are often overlooked, which leads to misdiagnoses. Read More

View Article and Full-Text PDF

Application progress of multiple imaging modalities in Takayasu arteritis.

Int J Cardiovasc Imaging 2021 Jul 21. Epub 2021 Jul 21.

Department of Cardiology, West China Hospital of Sichuan University, Guo Xue Xiang No.37, Chengdu, Sichuan, 610041, China.

Takayasu arteritis (TA) is a chronic, idiopathic, granulomatous large vessel vasculitis of unknown etiology. The clinical manifestations of TA are incredibly variable, mainly depending on the location of the lesions. In the light of its insidious progress and the diversity of clinical manifestations, a substantial proportion of patients might experience a considerable delay in diagnosis, which leads to irreversible malignant complications, highlighting the importance of early diagnosis. Read More

View Article and Full-Text PDF

HSCT in two brothers with CGD arising from mutations in CYBC1 corrects the defect in neutrophil function.

Clin Immunol 2021 Aug 16;229:108799. Epub 2021 Jul 16.

Paediatric Haematopoietic Stem Cell Transplant Unit, Great North Children's Hospital (GNCH), Victoria Wing, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, United Kingdom; Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE2 4HH, United Kingdom.

Homozygous mutations in cytochrome b-245 chaperone 1 (CYBC1) have been recently described as causing recurrent infections and inflammatory disease in an Icelandic cohort and a patient from Saudi Arabia, by destabilising the dimerisation of gp91 with p22, manifesting as phenotypic chronic granulomatous disease (CGD). Haematopoietic stem cell transplantation is the treatment of choice in CGD, though experience of transplantation in this subtype of CGD is limited to a brief description in one patient. We provide clinical and transplant data for two Icelandic brothers with CGD due to homozygous p. Read More

View Article and Full-Text PDF

Sarcoidosis Presenting with Bilateral Optic Disc Edema as the Initial Presentation.

J Coll Physicians Surg Pak 2021 Jul;30(7):861-863

Department of Ophthalmology, Haydarpasa Numune Training and Research Hospital, University of Health Sciences, Istanbul, Turkey.

Sarcoidosis is a chronic, progressive, granulomatous inflammatory disease of unknown etiology that affects multiple organ systems. Granulomatous anterior uveitis is the most common eye finding, and optic nerve involvement at presentation is rarely seen. Here, we report a 43-year man referred to our clinic with eye pain, decreased vision, floaters and bilateral papillitis, vitritis and cystoid macular edema. Read More

View Article and Full-Text PDF

Case Report: BCG-Triggered Hemophagocytic Lymphohistiocytosis in an Infant With X-Linked Recessive Mendelian Susceptibility to Mycobacterial Disease Due to a Variant of Chronic Granulomatous Disease.

Front Pediatr 2021 29;9:687538. Epub 2021 Jun 29.

Department of Pediatrics, College of Medicine and Health Sciences, United Arab Emirates University, Al Ain, Abu Dhabi, United Arab Emirates.

In the United Arab Emirates, BCG (Bacillus Calmette-Guérin) is administered to all newborns. We present here a young infant with an inborn error of immunity (IEI) who developed fatal adverse events to this live-attenuated vaccine. This male infant received BCG (Serum Institute of India Pvt. Read More

View Article and Full-Text PDF

A Rare Case of Renal Sarcoidosis.

Cureus 2021 Jun 7;13(6):e15494. Epub 2021 Jun 7.

Northwell Hofstra School of Medicine at Staten Island University Hospital, Northwell Health, Staten Island, USA.

Sarcoidosis is a multisystem granulomatous disorder characterized by non-caseating granulomas in multiple organs. It most commonly involves lungs and it is very rare to find isolated cases affecting other organ systems with no associated pulmonary findings. We hereby present a case of a young 30-year-old male who was referred to the hospital by his primary medical doctor due to right eye pain secondary to iritis and acute kidney injury (AKI). Read More

View Article and Full-Text PDF

Clinical Features of Mycetoma and the Appropriate Treatment Options.

Res Rep Trop Med 2021 8;12:173-179. Epub 2021 Jul 8.

Department of Dermatology, Smt. SCL Hospital, Smt. N.H.L. Municipal Medical College, Ahmedabad, Gujarat, India.

Mycetoma is a chronic, suppurative and debilitating granulomatous infection seen mainly in tropical and subtropical areas and is now declared as a neglected tropical disease by the World Health Organization. The clinical diagnosis is usually characterized by a classical triad of localized swelling, underlying sinus tracts, and production of grains or granules, but unusual presentations are also seen. It is classified into eumycetoma caused by the fungus, and actinomycetoma caused by the bacteria. Read More

View Article and Full-Text PDF

Impaired respiratory burst contributes to infections in PKCδ-deficient patients.

J Exp Med 2021 Sep 15;218(9). Epub 2021 Jul 15.

Department of Pediatrics, Division of Allergy and Clinical Immunology, Tehran University of Medical Sciences, Tehran, Iran.

Patients with autosomal recessive protein kinase C δ (PKCδ) deficiency suffer from childhood-onset autoimmunity, including systemic lupus erythematosus. They also suffer from recurrent infections that overlap with those seen in patients with chronic granulomatous disease (CGD), a disease caused by defects of the phagocyte NADPH oxidase and a lack of reactive oxygen species (ROS) production. We studied an international cohort of 17 PKCδ-deficient patients and found that their EBV-B cells and monocyte-derived phagocytes produced only small amounts of ROS and did not phosphorylate p40phox normally after PMA or opsonized Staphylococcus aureus stimulation. Read More

View Article and Full-Text PDF
September 2021

Achromobacter xylosoxidans Pneumonia in a Young Child with Chronic Granulomatous Disease-a Case-Based Review.

J Clin Immunol 2021 Jul 14. Epub 2021 Jul 14.

Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.

View Article and Full-Text PDF

Treatment Results of Intralesional Steroid Injection and Topical Steroid Administration in Pregnant Women with Idiopathic Granulomatous Mastitis.

Eur J Breast Health 2021 Jul 24;17(3):283-287. Epub 2021 Jun 24.

Department of Radiology, Van Yüzüncü Yıl University Faculty of Medicine, Van, Turkey.

Objective: Idiopathic granulomatous mastitis (IGM) is an inflammatory and chronic benign breast disease that has proven difficult to diagnose and treat. Since most treatment modalities cannot be used in pregnant patients, the choice of treatment is more difficult and the need for surgery is more pressing. In this first and innovative study, we assess the results of local corticosteroid therapy of IGM in pregnant women. Read More

View Article and Full-Text PDF

False-positive HIV serology, Candida lusitaniae pneumonia, and a novel mutation in the CYBB gene.

Immunobiology 2021 Jul 2;226(4):152110. Epub 2021 Jul 2.

Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. Electronic address:

Background: Chronic granulomatous disease (CGD) presents with a myriad of clinical manifestations pertaining to both immunodeficiency and hyperinflammation. Although Candida infection is a signature organism for patients with CGD, C. lusitaniae pneumonia in CGD has rarely been reported. Read More

View Article and Full-Text PDF

Ganglionar tuberculosis infection evolving to hemophagocytic lymphohistiocytosis after anti-programmed cell death 1 treatment for high-risk melanoma: a case report.

J Med Case Rep 2021 Jul 8;15(1):350. Epub 2021 Jul 8.

Oncology Center, Hospital Sírio Libanês (HSL), Rua Dona Adma Jafet, 91, 2nd floor, Building A, São Paulo, SP, 01308-050, Brazil.

Background: Hemophagocytic lymphohistiocytosis is a rare, potentially fatal syndrome of immune hyperactivation. Here we describe a ganglionar tuberculosis evolving to hemophagocytic lymphohistiocytosis following adjuvant immunotherapy in a melanoma patient.

Case Presentation: A 76-year-old Caucasian male with melanoma started with fever, diffuse petechiae, splenomegaly, anemia, thrombocytopenia, hypofibrinogenemia, and hyperferritinemia 2 months following completion of adjuvant treatment with nivolumab. Read More

View Article and Full-Text PDF

Comparison of Mycobacterium avium subsp. paratuberculosis infection in cattle, sheep and goats in the Khuzestan Province of Iran: Results of a preliminary survey.

Vet Med Sci 2021 Jul 6. Epub 2021 Jul 6.

Department of Clinical Sciences, Faculty of Veterinary Medicine, Shahid Chamran University of Ahvaz, Ahvaz, Iran.

Background: Paratuberculosis or Johne's disease, the chronic infectious granulomatous enteritis of ruminants, is a worldwide infection, which is caused by Mycobacterium avium subsp. paratuberculosis (MAP). The most common symptoms of this disease in cattle are loss of milk production, weight loss and diarrhoea, whereas in sheep and goats, the symptoms are emaciation, anorexia and severe disability. Read More

View Article and Full-Text PDF

Microbiological profile in chronic granulomatous disease patients in a single Brazilian primary immunodeficiencies center.

Allergol Immunopathol (Madr) 2021 1;49(4):141-148. Epub 2021 Jul 1.

Department of Pediatrics, Universidade de São Paulo, São Paulo, Brazil.

Background: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency. Infections of the lungs, skin, lymph nodes, and liver are the hallmark of CGD with frequent initial manifestations of the disease. The aim of the present study was to describe the sites of infections and their causative agents in 38 CGD pediatric patients. Read More

View Article and Full-Text PDF

Prevalence and characteristics of cutaneous sarcoidosis in Argentina.

Int J Womens Dermatol 2021 Jun 19;7(3):280-284. Epub 2021 Jan 19.

Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

Background: Sarcoidosis is a multisystem granulomatous disease of unknown etiology. The incidence is higher in women than in men, according to some studies. Studies regarding prevalence and characteristics of cutaneous sarcoidosis in our region are scarce. Read More

View Article and Full-Text PDF

Postauricular swelling secondary to salmonella extradural abscess in a toddler: a near miss condition.

BMJ Case Rep 2021 Jul 1;14(7). Epub 2021 Jul 1.

Otorhinolaryngology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

Postauricular swelling is usually encountered in an emergency setting in otorhinolaryngology, resulting from complication of acute or chronic suppurative otitis media. Besides that, postauricular swelling may occur secondary to various other conditions including infectious disease, tumour, vascular malformation, granulomatous condition and even trauma. Children less than 2 years old are prone to fall and up to 10% sustain traumatic brain injury without any obvious signs of neurological deficit. Read More

View Article and Full-Text PDF

Anakinra Activates Superoxide Dismutase 2 to Mitigate Inflammasome Activity.

Int J Mol Sci 2021 Jun 18;22(12). Epub 2021 Jun 18.

Department of Medicine and Surgery, University of Perugia, 06132 Perugia, Italy.

Inflammasomes are powerful cytosolic sensors of environmental stressors and are critical for triggering interleukin-1 (IL-1)-mediated inflammatory responses. However, dysregulation of inflammasome activation may lead to pathological conditions, and the identification of negative regulators for therapeutic purposes is increasingly being recognized. Anakinra, the recombinant form of the IL-1 receptor antagonist, proved effective by preventing the binding of IL-1 to its receptor, IL-1R1, thus restoring autophagy and dampening NLR family pyrin domain containing 3 (NLRP3) activity. Read More

View Article and Full-Text PDF

Immunological Aspects of X-Linked Chronic Granulomatous Disease Female Carriers.

Antioxidants (Basel) 2021 Jun 1;10(6). Epub 2021 Jun 1.

Department of Systems Medicine, University of Rome Tor Vergata, via Montpellier 1, 00133 Rome, Italy.

X-linked Granulomatous Disease (XL-CGD) carriers were previously thought to be clinically healthy because random X-chromosome inactivation (XCI) allows approximately half of their phagocytes/monocytes to express functional gp91phox protein. This supports the NADPH oxidase activity necessary for the killing of engulfed pathogens. Some XL-CGD carriers suffer from inflammatory and autoimmune manifestations as well as infections, although the skewed-XCI of a mutated allele is reported to be exclusively determinant for infection susceptibility. Read More

View Article and Full-Text PDF

Bronchoalveolar lavage fluid cell subsets associate with the disease course in Löfgren's and non-Löfgren's sarcoidosis patients.

Respir Med 2021 Jun 24;186:106521. Epub 2021 Jun 24.

Respiratory Medicine Division, Department of Medicine, Solna, Karolinska Institutet, SE-171 76, Stockholm, Sweden; Respiratory Medicine Division, Department of Medicine Solna, And Center for Molecular Medicine (CMM), Karolinska Institutet; and Respiratory Medicine, Theme Inflammation and Infection, Karolinska University Hospital, Stockholm, Sweden.

Background: Sarcoidosis is a multisystem granulomatous inflammatory disorder, that predominantly involves the lungs. Patients with Löfgren's syndrome (LS) are characterized by acute onset and usually have the HLA-DRB1*03 (DR3) allele and a good prognosis. Non-LS patients are usually DR3 and are more likely to develop chronic disease. Read More

View Article and Full-Text PDF

Apoptosis levels in bovine Johne's disease ileal lesions and association with bacterial numbers.

Vet Pathol 2021 Jun 30:3009858211025790. Epub 2021 Jun 30.

8797University College Dublin, Dublin, Ireland.

Johne's disease (JD) is a chronic granulomatous enteritis caused by subsp. (MAP). While it is generally accepted that MAP employs immune subversion mechanisms, aspects of the host-pathogen relationship are not fully understood. Read More

View Article and Full-Text PDF

Histopathological progression of hidradenitis suppurativa/acne inversa : A morphological study with a closer look on the early changes of the folliculosebaceous apocrine apparatus.

Wien Med Wochenschr 2021 Jun 29. Epub 2021 Jun 29.

Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital, Dresden, Germany.

Background: It is generally acknowledged that the first morphological change of hidradenitis suppurativa/acne inversa (HS/AI) consists of infundibular plugging of the folliculosebaceous apocrine apparatus, which is followed by acute and chronic inflammation, cysts with sinus formation, and fibrosis. Alternatively, it has been hypothesized that HS/AI is primarily a neutrophilic autoinflammatory disease and that the follicular plugging typical of this disease is secondary to inflammation.

Objective: To review the sequence of the changes that mark the disease development, we have performed a histopathologic study on the surgical material from a series of axillary and inguinal/perineal cases. Read More

View Article and Full-Text PDF