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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]
These granulomas are the main reason for the name granulomatosis with polyangiitis, although it is not an essential feature. Nevertheless, necrotizing granulomas are a hallmark of this disease. Many biopsies can be nonspecific, though, and 50% provide too little information for the diagnosis of GPA. [12]
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 ...
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 diagnosis should be made only after excluding other possible causes of similar symptoms such as tuberculosis. [6] Sarcoidosis may resolve without any treatment within a few years. [2] [5] However, some people may have long-term or severe disease. [5] Some symptoms may be improved with the use of anti-inflammatory drugs such as ibuprofen. [8]
Granulomatous–lymphocytic interstitial lung disease (GLILD) is a lung complication of common variable immunodeficiency disorders (CVID). It is seen in approximately 15% of patients with CVID. [ 1 ] It has been defined histologically as the presence of (non-caseating) granuloma and lymphoproliferation in the lung. [ 1 ]
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 ...
Granulomatous amoebic encephalitis (GAE) [2] is a rare, often fatal, subacute-to-chronic central nervous system disease caused by certain species of free-living amoebae [3] of the genera Acanthamoeba, Balamuthia and Sappinia. [4] [5] The term is most commonly used with Acanthamoeba.