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Chronic granulomatous disease (CGD), also known as Bridges–Good syndrome, chronic granulomatous disorder, and Quie syndrome, [1] is a diverse group of hereditary diseases in which certain cells of the immune system have difficulty forming the reactive oxygen compounds (most importantly the superoxide radical due to defective phagocyte NADPH oxidase) used to kill certain ingested pathogens. [2]
The disease is still sometimes confused with lethal midline granuloma and lymphomatoid granulomatosis, both malignant lymphomas. [ 34 ] The full clinical picture was first presented by Friedrich Wegener (1907–1990), a German pathologist , in two reports in 1936 and 1939, leading to the eponymous name Wegener's granulomatosis or Wegener ...
However, chronic granulomatous disease leads to inadequate H 2 O 2 production, while myeloperoxidase deficiency is characterized by a lack of myeloperoxidase to interact with present H 2 O 2. [ 1 ] [ 2 ] [ 3 ] [ 7 ] Testing with NADPH oxidase-specific assays can lead to positive results for chronic granulomatous disease and negative results for ...
Rhinoscleroma, is a chronic granulomatous bacterial disease of the nose that can sometimes infect the upper respiratory tract. [1] It most commonly affects the nasal cavity—the nose is involved in 95–100 per cent of cases—however, it can also affect the nasopharynx, larynx, trachea, and bronchi. Slightly more females than males are ...
Leishmaniases are a group of human diseases caused by Leishmania genus and transmitted by a sandfly bite can lead to granulomatous inflammation [14] in skin (cutaneous form of the disease) and liver (visceral form), with research suggesting effective granuloma formation to be desirable in the resolution of the disease. [15]
Sarcoidosis (/ ˌ s ɑːr k ɔɪ ˈ d oʊ s ɪ s /; also known as Besnier–Boeck–Schaumann disease) is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. [2] The disease usually begins in the lungs, skin, or lymph nodes. [2]
Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; [7] whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. [8]
The discovery that the gene defect in Blau syndrome involves the CARD15/NOD2 gene has sparked investigation into its function as part of the innate immune system. The innate immune system recognizes pathogen-associated molecular patterns, including bacterial polysaccharides such as muramyl dipeptide, via its pattern recognition receptors, such as NOD2, to induce signaling pathways that ...