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[甘露糖治疗先天性红细胞磷酸葡萄糖异构酶缺乏所致溶血性贫血的临床试验]

[Clinical trial of mannose treatment of hemolytic anemia caused by congenital deficiency of erythrocyte glucosephosphate isomerase].

作者信息

Jabłońska-Skwiecińska E, Giro E, Rokicka-Milewska R

机构信息

Zakładu Diagnostyki Laboratoryjnej CMKP, Warszawie.

出版信息

Acta Haematol Pol. 1992;23(2):123-8.

PMID:1488862
Abstract

A therapeutic trial with mannose given intravenously as a 5% solution during 7 consecutive days (daily doses 12.5 g, 25 g and 50 g) was performed in a GPI deficient girl with nonspherocytic hemolytic anemia. The aim of the trial was to substitute glucose--the main red cells metabolic substrate--with mannose, since the glucose metabolism, due to the GPI deficiency, was significantly decreased. An initial good effect of treatment was disturbed with viral infection. No complications due to treatment with mannose were observed.

摘要

对一名患有非球形细胞溶血性贫血的糖基磷脂酰肌醇(GPI)缺乏症女孩进行了一项治疗试验,连续7天静脉注射5%的甘露糖溶液(每日剂量分别为12.5克、25克和50克)。该试验的目的是用甘露糖替代葡萄糖(红细胞的主要代谢底物),因为由于GPI缺乏,葡萄糖代谢显著降低。治疗初期的良好效果因病毒感染而受到干扰。未观察到因甘露糖治疗引起的并发症。

相似文献

1
[Clinical trial of mannose treatment of hemolytic anemia caused by congenital deficiency of erythrocyte glucosephosphate isomerase].[甘露糖治疗先天性红细胞磷酸葡萄糖异构酶缺乏所致溶血性贫血的临床试验]
Acta Haematol Pol. 1992;23(2):123-8.
2
[Hereditary non-spherocytic hemolytic anemia caused by glucosephosphate isomerase deficiency: 1. case observed in Switzerland].
Schweiz Med Wochenschr. 1974 Sep 28;104(39):1379-81.
3
Combined glucose phosphate isomerase and glucose-6-phosphate dehydrogenase deficiency of erythrocytes.红细胞磷酸葡萄糖异构酶和葡萄糖-6-磷酸脱氢酶联合缺乏症
Isr J Med Sci. 1978 Nov;14(11):1186-90.
4
A case of congenital nonspherocytic hemolytic anemia associated with glucosephosphate isomerase (GPI) deficiency-GPI 'Kinki'.一例与葡萄糖磷酸异构酶(GPI)缺乏症-GPI‘近畿型’相关的先天性非球形细胞溶血性贫血。
Nihon Ketsueki Gakkai Zasshi. 1983 Feb;46(1):11-7.
5
Glucosephosphate isomerase (GPI) deficiency hereditary nonspherocytic hemolytic anemia. Report of the first case found in Japanese.葡萄糖磷酸异构酶(GPI)缺乏遗传性非球形细胞溶血性贫血。首例日本病例报告。
Nihon Ketsueki Gakkai Zasshi. 1973 Feb;36(1):65-9.
6
Glucosephosphate isomerase (GPI) deficiency hereditary nonspherocytic hemolytic anemia. Report of the second case found in Japanese.葡萄糖磷酸异构酶(GPI)缺乏遗传性非球形红细胞溶血性贫血。在日本人中发现的第二例病例报告。
Nihon Ketsueki Gakkai Zasshi. 1973 Feb;36(1):70-3.
7
[Phospho-hexo-isomerase deficiency].
Arch Fr Pediatr. 1976 Mar;33(3):217-32.
8
Fine structure of the spleen and liver in glucosephosphate isomerase (GPI) deficiency hereditary nonspherocytic hemolytic anemia. Selective reticulocyte destruction as a mechanism of hemolysis.葡萄糖磷酸异构酶(GPI)缺乏遗传性非球形红细胞溶血性贫血时脾脏和肝脏的精细结构。选择性网织红细胞破坏作为溶血机制。
Nihon Ketsueki Gakkai Zasshi. 1973 Feb;36(1):46-54.
9
Glucosephosphate-isomerase (GPI) deficiency: GPI elyria.葡萄糖磷酸异构酶(GPI)缺乏症:GPI缺陷症。 (你提供的原文“elyria”可能有误,推测这里想表达的是“deficiency”之类更符合语境的词,以上译文是基于合理推测进行的翻译。)
Ann Intern Med. 1974 Jun;80(6):730-2. doi: 10.7326/0003-4819-80-6-730.
10
[Red cell enzyme deficiencies - clinical manifestation and pathophysiology (author's transl)].红细胞酶缺乏症——临床表现与病理生理学(作者译)
Monatsschr Kinderheilkd. 1981 Aug;129(8):444-53.