. "5906" . "Variable"@en . "5906"^^ . "16333"^^ . . "Chronic multifocal Langerhans cell histiocytosis"@en . 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""@en . . . . . "\u062F\u0627\u0621 \u0647\u0627\u0646\u062F-\u0634\u0648\u0644\u0631-\u0643\u0631\u064A\u0633\u062A\u064A\u0627\u0646"@ar . . . . "D006646"@en . "C96.5" . . . . . "D006646" . "\u0411\u043E\u043B\u0435\u0437\u043D\u044C \u0425\u0435\u043D\u0434\u0430 \u2014 \u0428\u044E\u043B\u043B\u0435\u0440\u0430 \u2014 \u041A\u0440\u0438\u0441\u0447\u0435\u043D\u0430"@ru . "96.5"^^ . . "277.89"^^ . . "*Triad of bulging eyes, breakdown of bone, diabetes insipidus\n* Other symptoms eg. bone pain, facial asymmetry, ear infections, teeth/gum problems, liver and lung disease signs."@en . "277.89" . . . "Hand\u2013Sch\u00FCller\u2013Christian disease"@en . "Surgery, chemotherapy, radiation therapy"@en . . . . . . . . . . . "Chronic multifocal Langerhans cell histiocytosis, previously known as Hand\u2013Sch\u00FCller\u2013Christian disease, is a type of Langerhans cell histiocytosis (LCH), which can affect multiple organs. The condition is traditionally associated with a combination of three features; bulging eyes, breakdown of bone (lytic bone lesions often in the skull), and diabetes insipidus (excessive thirst and passing urine), although around 75% of cases do not have all three features. Other features may include a fever and weight loss, and depending on the organs involved there maybe rashes, asymmetry of the face, ear infections, signs in the mouth and the appearance of advanced gum disease. Features relating to lung and liver disease may occur."@en . . . . 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"Chronic multifocal Langerhans cell histiocytosis, previously known as Hand\u2013Sch\u00FCller\u2013Christian disease, is a type of Langerhans cell histiocytosis (LCH), which can affect multiple organs. The condition is traditionally associated with a combination of three features; bulging eyes, breakdown of bone (lytic bone lesions often in the skull), and diabetes insipidus (excessive thirst and passing urine), although around 75% of cases do not have all three features. Other features may include a fever and weight loss, and depending on the organs involved there maybe rashes, asymmetry of the face, ear infections, signs in the mouth and the appearance of advanced gum disease. Features relating to lung and liver disease may occur. It is due to a genetic mutation in the MAPKinase pathway that occurs during early development. The diagnosis may be suspected based on symptoms and MRI and confirmed by tissue biopsy. Blood tests may show anaemia, and less commonly a low white blood cell count and low platelet count. Treatment may involve surgery, chemotherapy, radiation therapy, and certain medicines. Hand\u2013Sch\u00FCller\u2013Christian disease was named for the American pediatrician , the Austrian neuroradiologist Arthur Sch\u00FCller, and the American internist Henry Asbury Christian, who described it in 1893, 1915 and 1919, respectively. Before the Histiocyte Society classified histiocytoses in the 1980s, the condition was also known as \"Histiocytosis X\", where \"X\" denoted the then unknown cause. It is now known as chronic multifocal Langerhans cell histiocytosis, a subtype of LCH. The disease is rare. Most present between the ages of two and six. The outlook depends on how many and how much organs are affected. In some people the condition is life-threatening."@en . "Rare"@en . . . . "Genetic mutation in the MAPKinase pathway"@en . . . . . . "Chronic multifocal Langerhans cell histiocytosis"@en . . "Dermatology"@en . . . . . . . . . . . . "A child with Hand-Sch\u00FCller-Christian Disease"@en . . . "Age 2-6"@en . . . . . . . . . . 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