Makino Yuko, Shimanuki Yuri, Fujiwara Noriko, Morio Yoshiteru, Sato Koichi, Yoshimoto Jiro, Gunji Yoko, Suzuki Tsutomu, Sasaki Shin-ichi, Iwase Akihiko, Kawasaki Seiji, Takahashi Kazuhisa, Seyama Kuniaki
Department of Respiratory Medicine, Juntendo University, School of Medicine, Tokyo, Japan.
Intern Med. 2008;47(4):281-5. doi: 10.2169/internalmedicine.47.0475. Epub 2008 Feb 15.
A 38-year-old woman was admitted due to lymphangioleiomyomatosis (LAM)-associated massive chylous ascites and progressive cachexia. She was incidentally diagnosed to have ascites during her regular physical check-up two years previously and LAM was revealed as its underlying cause. Periodic paracentesis was required to ameliorate ascites-associated symptoms, but resulted in lymphocytopenia, malnutrition, and deterioration of general status. Ascites was refractory to diuretics and fat-restricted diet. Peritoneovenous shunt (Denver shunt) was placed and thereafter ascites has been managed successfully without any complications for one year after the placement. Peritoneovenous shunt should be considered in LAM patients whose chylous ascites can not be managed with conservative treatments.
一名38岁女性因淋巴管平滑肌瘤病（LAM）相关的大量乳糜性腹水和进行性恶病质入院。两年前她在定期体检时偶然被诊断出患有腹水，随后发现其潜在病因是LAM。需要定期进行腹腔穿刺以改善与腹水相关的症状，但这导致了淋巴细胞减少、营养不良和全身状况恶化。腹水对利尿剂和低脂饮食治疗无效。放置了腹腔静脉分流术（丹佛分流术），此后在放置后的一年里腹水得到了成功控制，没有出现任何并发症。对于乳糜性腹水无法通过保守治疗控制的LAM患者，应考虑进行腹腔静脉分流术。
