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[一名杂合子镰状细胞病患者在暴露于高海拔环境时因脾梗死导致的急性腹痛]

[Acute abdominal pain due to splenic infarction in a patient with heterozygous sickle cell disease exposed to high altitude].

作者信息

Ruiz Semba Edgar, Garavito Rentería Jorge, Jiménez Bustamante Jorge, Arteaga Caro Ronal, García Del Aguila José Luis, Chávez Gil Vannya

机构信息

Servicio de Medicina Interna, Hospital Arzobispo Loayza, Líma, Perú.

出版信息

Rev Gastroenterol Peru. 2006 Oct-Dec;26(4):386-9.

PMID:17211489
Abstract

Hemoglobinopathy S, Depranocytosis or Sickle Cell Disease is the most common hemoglobinopathy in the world. In its heterozygous form (Sickle Cell Trait), it affects 8% of the black population in the U.S. and 25% of the black population in Africa, and is found less frequently in the Mediterranean area, India, Middle East and Latin America. The basic alteration is a substitution of glutamic acid by valin in the sixth position of the beta globin chain, which causes polymerization at low oxygen tension thereby distorting the structure of erythrocytes and increasing blood viscosity, which, in turn, generates obstructions of the capillary arterial blood flow to different areas of the body thus causing microinfarctions. Although Splenic Infarction is rare, it is recognized as a serious complication of Heterozygous Sickle Cell Disease (Sickle Cell Trait). We present the case of a 21 year-old mestizo male patient who came in with an acute case of abdominal pain after arriving to work in the Casapalca mining city (located in the Peruvian Andes at 4200 m.a.s.l.) and was referred to our Hospital in Lima for exams. We present the case because it is an unusual cause of acute abdominal pain, and because this condition is rare in Peru and there are few publications about it.

摘要

血红蛋白病S、异常细胞增多症或镰状细胞病是世界上最常见的血红蛋白病。以其杂合子形式(镰状细胞性状)存在时,它影响美国8%的黑人人口以及非洲25%的黑人人口,在地中海地区、印度、中东和拉丁美洲则较少见。基本改变是β珠蛋白链第六位的谷氨酸被缬氨酸取代,这导致在低氧张力下发生聚合,从而扭曲红细胞结构并增加血液粘度,进而造成身体不同部位的毛细血管动脉血流阻塞,引发微梗死。虽然脾梗死很少见,但它被认为是杂合子镰状细胞病(镰状细胞性状)的一种严重并发症。我们报告一例21岁的混血男性患者,他在抵达卡萨帕尔卡矿业城市(位于秘鲁安第斯山脉海拔4200米处)工作后出现急性腹痛,随后被转诊至利马我们的医院进行检查。我们报告该病例是因为它是急性腹痛的一种不寻常病因,且这种病症在秘鲁很罕见,关于它的出版物也很少。

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[Acute abdominal pain due to splenic infarction in a patient with heterozygous sickle cell disease exposed to high altitude].[一名杂合子镰状细胞病患者在暴露于高海拔环境时因脾梗死导致的急性腹痛]
Rev Gastroenterol Peru. 2006 Oct-Dec;26(4):386-9.
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[Splenic infarction at high altitude, Huaraz-Peru (3,100 masl)].[秘鲁瓦拉斯(海拔3100米)的高原脾梗死]
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Splenic infarction associated with sickle cell trait at low altitude.低海拔地区与镰状细胞性状相关的脾梗死
Hematology. 2015 Dec;20(10):607-9. doi: 10.1179/1607845415Y.0000000024. Epub 2015 Jul 2.
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[2 cases of acute sickle cell crisis in subjects with sickle cell trait following high altitude exposure].2例具有镰状细胞性状的受试者在高海拔暴露后发生急性镰状细胞危象
Schweiz Med Wochenschr. 1989 Sep 30;119(39):1358-9.
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Non hypoxia-related splenic infarct in a patient with sickle cell trait and infectious mononucleosis.
Acta Haematol. 2001;105(1):53-6. doi: 10.1159/000046534.
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[Spleen infarction and S hemoglobinopathies S in the high altitude lands].[高海拔地区的脾梗死与血红蛋白病S]
Rev Gastroenterol Peru. 2012 Jan-Mar;32(1):68-78.
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Splenic syndrome in patients at high altitude with unrecognized sickle cell trait: splenectomy is often unnecessary.高海拔地区未被识别出镰状细胞性状患者的脾脏综合征:脾切除术通常没有必要。
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Sickle cell trait as a cause of splenic infarction while climbing Mt. Fuji.镰状细胞性状作为攀登富士山时脾梗死的一个原因。
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Pulmonary embolism and splenic infarction in a patient with sickle cell trait.一名具有镰状细胞性状的患者发生肺栓塞和脾梗死。
Am J Hematol. 1990 Apr;33(4):279-81. doi: 10.1002/ajh.2830330414.
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The splenic syndrome in individuals with sickle cell trait.具有镰状细胞性状个体的脾脏综合征
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