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Gorham's disease
Gorham's disease (pronounced GOR-amz), also known as Gorham vanishing bone disease and phantom bone disease,〔(【引用サイトリンク】url=http://www.diseasesdatabase.com/ddb31515.htm )〕 is a very rare skeletal condition of unknown cause, characterized by the uncontrolled proliferation of distended, thin-walled vascular or lymphatic channels within bone, which leads to resorption and replacement of bone with angiomas and/or fibrosis.〔Gorham LW, Stout AP. Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone): its relation to hemangiomatosis. J Bone Joint Surg () 1955;37-A:985-1004.〕〔Ross JL., Schinella R., and Shenkman L. Massive osteolysis: An unusual cause of bone destruction. The American Journal of Medicine 1978; 65(2): 367-372.〕 Current treatments are experimental only.
==Signs and symptoms==
The symptoms of Gorham's disease vary depending on the bones involved. It may affect any part of the skeleton, but the most common sites of disease are the shoulder, skull, pelvic girdle, jaw, ribs, and spine.〔MÖLLER, G., Priemel, M., Amling, M. Werner, M., Kuhlmey, A. S., Delling, G. "The Gorham-Stout syndrome (Gorham's massive osteolysis) A REPORT OF SIX CASES WITH HISTOPATHOLOGICAL FINDINGS." Journal of Bone and Joint Surgery (Br) 81B.3 (1999): 501-6. Web. 2 Sep 2011.〕〔Yalniz E., Alicioglu B., Benlier E., Yilmaz1 B., Altaner S. Gorham-Stout Disease of the Humerus. JBR–BTR, 2008, 91: 14-17.〕〔Duffy B, Manon R, Patel R, Welsh JS, et al. A case of Gorham's disease with chylothorax treated curatively with radiation therapy. Clin Med Res. 2005;3:83–6.〕〔Lee WS, Kim SH, Kim I, et al. Chylothorax in Gorham's disease. J Korean Med Sci. 2002;17:826–9.〕
In some cases there are no symptoms until a fracture occurs either spontaneously or following minor trauma, such as a fall. There may be an acute onset of localized pain and swelling. More commonly there is pain of no apparent cause that increases in frequency and intensity over time and may eventually be accompanied by weakness and noticeable deformity of the area. The rate of progression is unpredictable and the prognosis can be difficult. The disease may stabilize after a number of years, go into spontaneous remission, or, in cases involving the chest and upper spine, prove fatal. Recurrence of the disease following remission can also occur. Involvement of the spine and skull base may cause a poor outcome from neurological complications. In many cases, the end result of Gorham's disease is severe deformity and functional disability.〔〔〔Chattopadhyay P, Bandyopadhyay A, Das S, Kundu A J. Gorham's disease with spontaneous recovery. Singapore Med J 2009; 50(7).〕
Symptoms such as difficulty breathing and chest pain may be present if the disease is present in the ribs, scapula, or thoracic vertebrae. These may indicate that the disease has spread from the bone into the chest cavity. The breathing problems may be misdiagnosed as asthma, because the damage done to the lungs can cause the same types of changes to lung function testing that are seen in asthma.〔 Extension of the lesions into the chest may lead to the development of chylous pleural and pericardial effusions. Chyle is rich in protein and white blood cells that are important in fighting infection. The loss of chyle into the chest can have serious consequences, including infection, malnutrition, and respiratory distress and failure. These complications or their symptoms, such as difficulty breathing, chest pain, poor growth or weight loss, and infection have sometimes been the first indications of the condition.〔〔〔
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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