Severe chronic non-bacterial osteomyelitis associated with MPO deficiency
نویسندگان
چکیده
Introduction We report a severe case of chronic non-bacterial osteomyelitis (CNO) associated with total myeloperoxidase (MPO) deficiency. Chronic non-bacterial osteomyelitis (CNO) is often considered an autoinflammatory disease. In pediatric literature CNO is often referred to as chronic recurrent multifocal ostemyelitis (CRMO). CNO is occasionally associated with extremely rare monogenic disease but in most cases the etiology is unknown. MPO deficiency is characterized by low levels of leukocyte MPO, and in some cases even total lack of the enzyme. The condition is seldom associated with pathology; severe Candida infection occurs in about 5% of cases and there is a slightly increased frequency of minor infections.
منابع مشابه
MPO deficiency confers impaired processing of neutrophil reactive oxygen species in a patient with severe CRMO
Introduction We report a severe case of chronic recurrent multifocal osteomyelitis (CRMO) associated with total myeloperoxidase (MPO) deficiency. The etiology of CRMO is in most cases unknown, and this is to our knowledge the first case associated with MPO-deficiency. Leukocyte MPO-deficiency renders neutrophils unable to process superoxide to secondary reactive oxygen species (ROS). Partial MP...
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Background: Myeloperoxidase (MPO), an iron-containing protein, is found in the azurophilic granules of neutrophils (PMNs), and catalyzes the conversion of hydrogen peroxide and chloride ions (Cl) into hypochlorous acid, which plays an important role in oxygen-dependent bacterial killing. The enzyme was first isolated in 1941, and deficiency of MPO was first described in 1954. Fewer than 5% of p...
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