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[良性对称性脂肪瘤病(Launois-Bensaude腺脂肪瘤病、马德隆病)的生物化学]

[Biochemistry of benign-symmetrical lipomatosis (adenolipomatosis Launois-Bensaude, Madelung's disease)].

作者信息

MULLER M M, Fuchs H, Schwarzmeier J D, Obiditsch-Mayer I, Freilinger G, Frank O

出版信息

Wien Klin Wochenschr. 1976 Feb 6;88(3):94-101.

PMID:183373
Abstract
  1. Total lipids, total cholesterol, cholesterol esters, phospholipids, triglycerides and free fatty acids as well as the fatty acids profiles of the different lipid classes were determined in serum, lipomatous and normal adipose tissue. Triglycerides were elevated in patient L's serum. The distribution of serum lipoproteins in this patient's serum showed a type IV according to Fredrickson. All other lipid parameters were within the normal range. Palmitoleic acid was increased nearly in all lipid fractions of the patients' sera as well as in the lipids of lipomatous subcutaneous adipose tissue. 2. The lipomatous adipose tissues of the patients showing no histological abnormalities revealed higher levels of cyclic AMP than normal subcutaneous adipose tissue. 3. Serum uric acid was normal (patient E.), between the normal and pathological range (patient L.) and elevated (patient W.). Urinary uric acid excretion was increased in all three patients. 4. 14C-glycine was overincorporated into urinary uric acid in all three patients. 5. Adenine phosphoribosyltransferase activities in the hemolysates were within the normal range. A decrease of hypoxanthine-guanine phosphoribosyltransferase activities could be demonstrated in two patients' (e., w.) erythrocytes. Erythrocyte phosphoribosylpyrophate synthetase activity was slightly increased in patient L.'s and twice the normal value in patient W.'s erythrocytes.
摘要
  1. 在血清、脂肪瘤组织和正常脂肪组织中测定了总脂质、总胆固醇、胆固醇酯、磷脂、甘油三酯和游离脂肪酸以及不同脂质类别的脂肪酸谱。患者L的血清中甘油三酯升高。根据弗雷德里克森分类法,该患者血清中脂蛋白的分布显示为IV型。所有其他脂质参数均在正常范围内。患者血清的几乎所有脂质组分以及脂肪瘤皮下脂肪组织的脂质中,棕榈油酸均增加。2. 组织学无异常的患者的脂肪瘤组织中,环磷酸腺苷水平高于正常皮下脂肪组织。3. 血清尿酸正常(患者E)、在正常和病理范围之间(患者L)以及升高(患者W)。所有三名患者的尿尿酸排泄均增加。4. 所有三名患者中,14C-甘氨酸过度掺入尿尿酸中。5. 溶血产物中的腺嘌呤磷酸核糖基转移酶活性在正常范围内。在两名患者(例如患者E、W)的红细胞中可证明次黄嘌呤-鸟嘌呤磷酸核糖基转移酶活性降低。患者L的红细胞中磷酸核糖焦磷酸合成酶活性略有增加,患者W的红细胞中该酶活性为正常值的两倍。

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