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KMID : 0367419750160120060
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Journal of Korean Pediatric Society
1975 Volume.16 No. 12 p.60 ~ p.64
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A Case of Klippel-Trenaunay-Weber Syndrome
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盧鎭英/Ro, Jin Yung
曺義鉉/申相滿/李尙圭/Jo, Eui Hyun/Shin, Sang Man/Lee, Sang Jhoo
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Abstract
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A case of Klippel-Trenaunay-Weber syndrome with macrodactylia in a 11-year-old boy is presented who was admitted to our Kyung Hee Medical Center with Chief complaints of nevus flammeus, swelling of the left lower leg and foor for 3 years duration.
This parient was born with an wxtensive nevus flammeus of left leg and left arm with hypertrophy of left leg since at age of 8. There is no history of similar illness in the family.
Physical examination revealed nevus flammeus of left leg and foot with macrodactylia of great toes on both feet and right middle finger.
Radiologic studies: chest X-ray showed no abnormalitis, X-ray of the both hands revealed enlargement of all phalangeal bones in right middle finger. Venography of left lower leg revealed unusually dilated lower leg veins with marked engorgement suggestive of veins malformation.
Laboratory data including Hb, Ht, C.B.C., total cholesterol, alkaline phosphatase, serum protein, S.G.O.T., S.G.P.T. calcium phosphorus, urinalysis were within normal limit exept sed. rate(Wintrobe) 50mm/hr, 2(^+) C.R.P. globulin 3.8gm% and albumin 3.7gm%.
Patient was treated with elastic bandaging of the left leg to reduce edema and prevent stasis dermatitis.
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