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大红细胞症和假性白化病:硒缺乏的表现

Macrocytosis and pseudoalbinism: manifestations of selenium deficiency.

作者信息

Vinton N E, Dahlstrom K A, Strobel C T, Ament M E

机构信息

Department of Pediatrics, University of California, Los Angeles.

出版信息

J Pediatr. 1987 Nov;111(5):711-7. doi: 10.1016/s0022-3476(87)80247-0.

DOI:10.1016/s0022-3476(87)80247-0
PMID:3117996
Abstract

Selenium levels were low in four children receiving long-term total parenteral nutrition (TPN) who developed erythrocyte macrocytosis (3/4), loss of pigmentation of hair and skin (2/4), elevated transaminase and creatine kinase activities (2/4), and profound muscle weakness (1/4). Initial mean selenium levels in serum and hair were 38 +/- 11 (SEM) ng/mL and 0.34 +/- 0.13 micrograms/g, respectively. Mean serum vitamin B12, folate, and vitamin E levels were normal. Intravenous supplementation with selenium was begun daily at 2 micrograms/kg/day. After 3 to 6 months, serum selenium levels rose almost threefold to 81 +/- 22 ng/mL. During this same time, erythrocyte mean corpuscular volume fell from 115 +/- 8 fL to 88 +/- 7 fL in the three children with macrocytosis. After 6 to 12 months of supplementation, hair selenium content had increased threefold to 1.02 +/- 0.19 micrograms/g. The two children with decreased pigmentation became darker skinned and their hair color changed from blonde to dark brown; a third child's hair, which had been blonde, also became darker. Transaminase and creatine kinase activities returned to near normal in those affected and, in the one child with severe myopathy, muscle weakness improved. Erythrocyte macrocytosis and loss of skin and hair pigmentation are previously undescribed manifestations of selenium deficiency. We recommend routine supplementation of TPN solution with selenium to avoid the clinical and biochemical syndrome of selenium deficiency in patients receiving long-term TPN.

摘要

在4名接受长期全胃肠外营养(TPN)的儿童中,硒水平较低,这些儿童出现了红细胞大细胞性贫血(3/4)、头发和皮肤色素沉着减退(2/4)、转氨酶和肌酸激酶活性升高(2/4)以及严重肌无力(1/4)。血清和头发中最初的平均硒水平分别为38±11(SEM)ng/mL和0.34±0.13μg/g。血清维生素B12、叶酸和维生素E水平正常。开始每天以2μg/kg/天的剂量静脉补充硒。3至6个月后,血清硒水平几乎增加了两倍,达到81±22 ng/mL。在此期间,3名患有大细胞性贫血的儿童红细胞平均体积从115±8 fL降至88±7 fL。补充6至12个月后,头发中的硒含量增加了两倍,达到1.02±0.19μg/g。两名色素沉着减退的儿童皮肤颜色变深,头发颜色从金色变为深棕色;第三名原本是金色头发的儿童头发也变深了。受影响儿童的转氨酶和肌酸激酶活性恢复到接近正常水平,在患有严重肌病的一名儿童中,肌无力有所改善。红细胞大细胞性贫血以及皮肤和头发色素沉着减退是先前未描述过的硒缺乏表现。我们建议在TPN溶液中常规补充硒,以避免接受长期TPN治疗的患者出现硒缺乏的临床和生化综合征。

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1
Macrocytosis and pseudoalbinism: manifestations of selenium deficiency.大红细胞症和假性白化病:硒缺乏的表现
J Pediatr. 1987 Nov;111(5):711-7. doi: 10.1016/s0022-3476(87)80247-0.
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