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[蚕豆作为急性溶血性贫血的病因]

[Broad beans as a cause of acute hemolytic anemia].

作者信息

Riepl R L, Schreiner J, Müller B, Hildemann S, Loeschke K

机构信息

Medizinische Klinik, Universität München.

出版信息

Dtsch Med Wochenschr. 1993 Jun 25;118(25):932-5. doi: 10.1055/s-2008-1059408.

DOI:10.1055/s-2008-1059408
PMID:8519217
Abstract

A previously healthy 17-year-old Greek boy suddenly developed jaundice of sclerae and skin. In addition, physical examination revealed a pale appearance. He also reported feeling tired and weak. The haemoglobin level was 9.6 g/dl, lactate dehydrogenase activity 335 U/l, bilirubin concentration 3.2 mg/dl (direct bilirubin 0.7 mg/dl, indirect bilirubin 2.5 mg/dl), haptoglobin concentration 48.8 mg/dl. As haemolytic anaemia was assumed, direct questioning elicited the fact that the patient had, for the first time in his life, eaten 300 g of broad beans (Vicia faba) on each of two days, namely 3 and 2 days before the appearance of jaundice. Absence of glucose-6-phosphate dehydrogenase activity in the red blood corpuscles confirmed the diagnosis of favism. On symptomatic treatment both the enzyme activities and the bilirubin level fell to normal within one week, and the haemoglobin level was 15.7 g/dl after 4 weeks.

摘要

一名既往健康的17岁希腊男孩突然出现巩膜和皮肤黄疸。此外,体格检查发现其面色苍白。他还自述感到疲倦和虚弱。血红蛋白水平为9.6 g/dl,乳酸脱氢酶活性为335 U/l,胆红素浓度为3.2 mg/dl(直接胆红素0.7 mg/dl,间接胆红素2.5 mg/dl),触珠蛋白浓度为48.8 mg/dl。由于怀疑为溶血性贫血,经直接询问得知,该患者在黄疸出现前3天和2天,生平第一次连续两天每天食用了300克蚕豆(蚕豆属)。红细胞中葡萄糖-6-磷酸脱氢酶活性缺乏确诊为蚕豆病。经对症治疗,酶活性和胆红素水平在一周内降至正常,4周后血红蛋白水平为15.7 g/dl。

相似文献

1
[Broad beans as a cause of acute hemolytic anemia].[蚕豆作为急性溶血性贫血的病因]
Dtsch Med Wochenschr. 1993 Jun 25;118(25):932-5. doi: 10.1055/s-2008-1059408.
2
Jaundice and bilirubin levels in Greek children with favism.患有蚕豆病的希腊儿童的黄疸和胆红素水平。
Arch Dis Child. 1976 Mar;51(3):233-5. doi: 10.1136/adc.51.3.233.
3
Glucose-6-phosphate dehydrogenase deficiency and Gilbert syndrome: a gene interaction underlies severe jaundice without severe hemolysis.葡萄糖-6-磷酸脱氢酶缺乏症与吉尔伯特综合征:基因相互作用是无严重溶血的严重黄疸的基础。
Pediatr Hematol Oncol. 2005 Oct-Nov;22(7):561-6. doi: 10.1080/08880010500198533.
4
Investigations on favism-inducing toxins in broad beans (Vicia faba L.).蚕豆(野豌豆属蚕豆)中引起蚕豆病毒素的研究。
Proc Nutr Soc. 1973 Dec;32(3):100A-101A.
5
Favism and hemolytic anemia in glucose-6-phosphate dehydrogenase-deficient subjects in North Sardinia.撒丁岛北部葡萄糖-6-磷酸脱氢酶缺乏症患者中的蚕豆病和溶血性贫血
Acta Haematol. 1983;70(2):83-90. doi: 10.1159/000206700.
6
[Hemolytic anemia as cause of a marked bilirubin increase in primary biliary cirrhosis].[溶血性贫血作为原发性胆汁性肝硬化患者胆红素显著升高的原因]
Dtsch Med Wochenschr. 1996 Oct 4;121(40):1226-8. doi: 10.1055/s-2008-1043131.
7
[Glucose-6-phosphate dehydrogenase deficiency in children: a case report].[儿童葡萄糖-6-磷酸脱氢酶缺乏症:一例报告]
Rev Chil Pediatr. 2014 Feb;85(1):74-9. doi: 10.4067/S0370-41062014000100010.
8
Favism by proxy in nursing glucose-6-phosphate dehydrogenase-deficient neonates.葡萄糖-6-磷酸脱氢酶缺乏的新生儿护理中通过代理导致的蚕豆病
J Perinatol. 1998 Nov-Dec;18(6 Pt 1):477-9.
9
Glucose-6-phosphate dehydrogenase deficiency. WHO Working Group.葡萄糖-6-磷酸脱氢酶缺乏症。世界卫生组织工作组。
Bull World Health Organ. 1989;67(6):601-11.
10
[A boy with jaundice].[一个患有黄疸的男孩]
Ned Tijdschr Geneeskd. 2011;155:A2020.

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Increased induction of apoptosis in mononuclear cells of a glucose-6-phosphate dehydrogenase deficient patient.葡萄糖-6-磷酸脱氢酶缺乏症患者单核细胞中凋亡诱导增加。
J Mol Med (Berl). 1995 Jan;73(1):47-9. doi: 10.1007/BF00203619.