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自1972年作者报道了第1例脂膜炎伴发α_1—抗胰蛋白酶(α_1—AT)缺乏以来,至今共有21例报道。本文对已报告的21例进行分析。21例患者中女性10例,男性11例。女性年龄19～65岁,男性7～51岁,儿童1例。在各种促发原因中,皮肤创伤后发病者8例。另外,与遗传及家庭环境有关,其中表型MZ者3例,表型ZZ者16例。血清α_1—AT值为12～180mg/dl,有12例低于40mg/dl,α_1—AT值与疾病病情间无直接联系。21例临床表现除有结节性脂膜炎的一般症状外,有以下特点:(1)高熟;(2)有17例为散发结节,斑块,有时呈弥漫性分布。结节、斑块、弥漫性水肿部位有油状液体外流,13例为黄色,1例为棕色,3例为血清—
Since 1972, the authors reported the first cases of lipid peritonitis with α 1-antitrypsin (α 1 -AT) deficiency, so far a total of 21 cases reported. This article has been reported in 21 cases were analyzed. Among the 21 patients, 10 were women and 11 were males. Female age 19 to 65 years old, male 7 to 51 years old, 1 child. In a variety of causes of development, the incidence of skin trauma in 8 cases. In addition, with the genetic and family environment, including 3 cases of phenotype MZ, 16 cases of phenotype ZZ. Serum α_1-AT value of 12 ~ 180mg / dl, 12 cases less than 40mg / dl, α_1-AT values and disease conditions are not directly linked. 21 cases of clinical manifestations in addition to the general symptoms of nodular panniculitis, the following characteristics: (1) high ripening; (2) 17 cases of disseminated nodules, plaques, and sometimes diffuse distribution. Nodular, plaque, diffuse edema oily liquid outflow site, 13 cases were yellow, 1 case was brown, 3 cases of serum -