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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。

相似文献

1
[Biochemistry of benign-symmetrical lipomatosis (adenolipomatosis Launois-Bensaude, Madelung's disease)].[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]
Wien Klin Wochenschr. 1976 Feb 6;88(3):94-101.
2
Multiple symmetric lipomatosis: a defect in adrenergic-stimulated lipolysis.多发性对称性脂肪瘤病:肾上腺素能刺激的脂肪分解缺陷。
J Clin Invest. 1977 Dec;60(6):1221-9. doi: 10.1172/JCI108881.
3
[The benign symmetrical lipomatosis (adenolipomatosis Launois-Bensaude; Madelung's fat neck)].
Schweiz Med Wochenschr. 1976 Apr 24;106(17):576-80.
4
[Abnormalities of the lipid and glucide metabolism in Launois-Bensaude lipomatosis].[劳诺瓦-本索德脂肪瘤病中的脂质和糖代谢异常]
Presse Med (1893). 1969 Dec 13;77(53):2001-2.
5
Mediastino-abdominal lipomatosis: deep accumulation of fat mimicking a respiratory disease and ascites. Clinical aspects and metabolic studies in vitro.
Q J Med. 1984 Autumn;53(212):453-63.
6
[Surgical treatment of Launois-Bensaude disease. The value of liposuction].[劳诺瓦-本索德病的外科治疗。抽脂术的价值]
Ann Chir Plast Esthet. 1990;35(2):128-33.
7
[Madelung's lipomatosis of the neck--expression of an alcohol-induced endocrine disorder?].[颈部马德隆脂肪过多症——酒精性内分泌紊乱的表现?]
HNO. 1986 Nov;34(11):474-6.
8
Multiple symmetric lipomatosis (Launois-Bensaude syndrome) - adipose tissue insensitivity to cyclic AMP.多发性对称性脂肪瘤病(劳诺瓦-本索德综合征)——脂肪组织对环磷酸腺苷不敏感。
J Clin Endocrinol Metab. 1979 Aug;49(2):307-9. doi: 10.1210/jcem-49-2-307.
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Unusual distribution of the lower body fatty tissue: classification, treatment, and differential diagnosis.下半身脂肪组织的异常分布:分类、治疗及鉴别诊断。
Ann Plast Surg. 2008 Jul;61(1):2-8. doi: 10.1097/SAP.0b013e318156debe.
10
Induction of benign symmetric lipomatosis (Launois-Bensaude syndrome) in a female patient after liver transplantation.
Dermatology. 2008;216(4):337-40. doi: 10.1159/000115968. Epub 2008 Feb 12.

引用本文的文献

1
Subcutaneous adipose tissue fatty acid desaturation in adults with and without rare adipose disorders.成年人中罕见脂肪代谢障碍与非罕见脂肪代谢障碍患者的皮下脂肪组织脂肪酸去饱和作用。
Lipids Health Dis. 2012 Feb 3;11:19. doi: 10.1186/1476-511X-11-19.