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In medical terminology lymphangiomatosis means: lymphatic system (lymph) vessel (angi) tumor or cyst (oma) condition (tosis). Lymphatic vessels reach every part of the body except the central nervous system (the brain and spinal cord), which has its own specialized system. The lymphatic system has three main functions: * to maintain fluid balance, * to defend the body against disease by producing lymphocytes, and * to absorb fats and fat soluble vitamins (A, D, E, and K) from the small intestine and transport them to the blood, bypassing the liver. Lymphangiomatosis, like other lymphatic malformations, is thought to be the result of congenital errors of lymphatic development occurring prior to the 20th week of gestation.〔Faul, J.L., Berry, G.J., Colby, T.V., Ruoss, S.J., Walter, M.B, Rosen, G.D., and Raffin, T.A. Thoracic Lymphangiomas, Lymphangiectasis, Lymphangiomatosis, and Lymphatic Dysplasia Syndrome. Am. J. Respir. Crit. Care Med., Volume 161, Number 3, March 2000, 1037-1046.〕 Lymphangiomatosis is a condition marked by the presence of cysts that result from an increase both in the size and number of thin-walled lymphatic channels that are abnormally interconnected and dilated.〔〔Pernick, Nat. "Soft Tissue Tumors Part 3 Muscle, Vascular, Nerve, Other Lymphangiomatosis." PathologyOutlines.com. PathologyOutlines.com, Inc., 10/17/2009. Web. 6 Sep 2011. ==Signs and symptoms== Lymphangiomatosis is a multi-system disorder. Symptoms depend on the organ system involved and, to varying degrees, the extent of the disease. Early in the course of the disease patients are usually asymptomatic, but over time the abnormally proliferating lymphatic channels that constitute lymphangiomatosis are capable of massive expansion and infiltration into surrounding tissues, bone, and organs.〔〔 Because of its slow course and often vague symptoms, the condition is frequently under-recognized or misdiagnosed.〔Venkatramani R, Ma NS, Pitukcheewanont P, Malogolowkin MH, Mascarenhas L. Gorham’s disease and diffuse lymphangiomatosis in children and adolescents. Pediatr Blood Cancer. 2011 Apr;56(4):667-70〕 Early signs of disease in the chest include wheezing, cough, and feeling short of breath, which is often misdiagnosed as asthma.〔 The pain that accompanies bone involvement may be attributed to "growing pains" in younger children. With bone involvement the first indication for disease may be a pathological fracture. Symptoms may not raise concern, or even be noted, until the disease process has advanced to a point where it causes restrictive compression of vital structures.〔 Further, the occurrence of chylous effusions seems to be unrelated to the pathologic "burden" of the disease, the extent of involvement in any particular tissue or organ, or the age of the patient.〔Zisis C, Spiliotopoulos K, Patronis M, Filippakis G, Stratakos G, Tzelepis G, Bellenis I. Diffuse lymphangiomatosis: are there any clinical or therapeutic standards? J Thorac Cardiovasc Surg. 2007 Jun;133(6):1664-5.〕 This offers one explanation as to why, unfortunately, the appearance of chylous effusions in the chest or abdomen may be the first evidence of the disease. Following are some of the commonly reported symptoms of lymphangiomatosis, divided into the regions/systems in which the disease occurs: • 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Lymphangiomatosis」の詳細全文を読む スポンサード リンク
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