与慢性低血糖相关的血小板功能缺陷。
Defect of platelet function associated with chronic hypoglycaemia.
作者信息
Hutton R A, Macnab A J, Rivers R P
出版信息
Arch Dis Child. 1976 Jan;51(1):49-55. doi: 10.1136/adc.51.1.49.
Abstract
Two patients are described with chronic hypoglycaemia; the first having glucose-6-phosphatase deficiency (type I glycogen storage disease), and the second fructose 1:6-diphosphatase deficiency. Both cases were associated with a bleeding diathesis, a defect of platelet aggregation, and a deficiency of platelet adenine nucleotides. The effect on the platelet abnormalities of a period of normoglycaemia was studied in both patients. Correction of the platelet abnormalities occurred rapidly after stabilization of the blood glucose within the normal range. Normal function persisted for the duration of the normoglycaemia, facilitating diagnostic liver biopsy and surgical procedures. A biochemical explanation for the nucleotide deficiency is suggested.
摘要
本文描述了两名患有慢性低血糖症的患者；第一名患者患有葡萄糖-6-磷酸酶缺乏症（I型糖原贮积病），第二名患者患有1:6-二磷酸果糖酶缺乏症。这两个病例均伴有出血倾向、血小板聚集缺陷和血小板腺嘌呤核苷酸缺乏。对两名患者正常血糖期对血小板异常的影响进行了研究。血糖稳定在正常范围内后，血小板异常迅速得到纠正。在正常血糖期内，正常功能持续存在，有利于进行诊断性肝活检和外科手术。本文还提出了核苷酸缺乏的生化解释。
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本文引用的文献
1
Aggregation of blood platelets by adenosine diphosphate and its reversal.二磷酸腺苷引起的血小板聚集及其逆转
Nature. 1962 Jun 9;194:927-9. doi: 10.1038/194927b0.
2
Microdetermination of adenosine diphosphate and adenosine triphosphate in plasma with firefly luciferase system.利用萤火虫荧光素酶系统微量测定血浆中的二磷酸腺苷和三磷酸腺苷。
Anal Biochem. 1966 Dec;17(3):456-73. doi: 10.1016/0003-2697(66)90181-3.
3
Sugar penetration into human blood platelets.糖渗透进入人体血小板。
J Cell Physiol. 1968 Aug;72(1):77-9. doi: 10.1002/jcp.1040720113.
4
Studies on dietary correction of metabolic abnormalities in hepatorenal glycogenosis.肝肾糖原贮积症代谢异常的饮食纠正研究。
Pediatr Res. 1969 Mar;3(2):160-70. doi: 10.1203/00006450-196903000-00008.
5
Effect of metabolic acidosis and alkalosis on human platelet aggregation induced by epinephrine and ADP.代谢性酸中毒和碱中毒对肾上腺素和二磷酸腺苷诱导的人血小板聚集的影响。
Proc Soc Exp Biol Med. 1974 Feb;145(2):743-6. doi: 10.3181/00379727-145-37887.
6
Haemostatic functions of platelets and their disorders.
Sci Basis Med Annu Rev. 1972:222-37.
7
Platelet dysfunction in glycogen storage disease type I.Ⅰ型糖原贮积病中的血小板功能障碍
Blood. 1973 Feb;41(2):235-47.
8
Fasting hypoglycaemia and metabolic acidosis associated with deficiency of hepatic fructose-1,6-diphosphatase activity.与肝果糖-1,6-二磷酸酶活性缺乏相关的空腹低血糖和代谢性酸中毒。
Lancet. 1970 Jul 4;2(7662):13-6. doi: 10.1016/s0140-6736(70)92474-8.
9
Bleeding tendency associated with "new" abnormality of platelet behaviour.
Lancet. 1967 May 6;1(7497):983-5. doi: 10.1016/s0140-6736(67)92363-x.
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