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血红蛋白汉默史密斯型及特纳综合征患儿大剂量静脉输注去铁胺后的矿物质平衡(铁、铝、铜、锌)

Mineral balance (iron, aluminum, copper, zinc) after high-dose intravenous Desferal in a child with hemoglobin Hammersmith and Turner's syndrome.

作者信息

Hyman C B, Gonick H C, Neufeld N, Agness C L

机构信息

Department of Pediatrics, Cedars-Sinai Medical Center, Los Angeles, California 90048.

出版信息

Am J Pediatr Hematol Oncol. 1989 Winter;11(4):450-5.

PMID:2618982
Abstract

A 12-day balance study with measurements of urine and stool excretion was undertaken to determine the effects of intravenous (i.v.) Desferal (293 mg/kg/24 h x 2) on iron, aluminum, copper, and zinc in a child with Hemoglobin Hammersmith and Turner's syndrome treated as a thalassemia major patient because of symptoms of anemia and ineffective erythropoiesis. Iron balance was positive, 34 mg/3 days baseline. The Desferal infusion induced iron excretion of 117 mg over 48 h, almost equally in stool and urine. This child receives approximately 20 transfusion/i.v. Desferal treatments yearly. If iron excretion is roughly the same with each treatment, it would equal 2,340 mg or 47% of her annual iron intake from transfusion. The i.v. infusions are an important part of this patient's therapy and may also be useful for other chronic transfusion patients for whom subcutaneous Desferal is inadequate for preventing continued iron accumulation. Some patients have successfully received their i.v. Desferal therapy at home, thereby decreasing hospitalization time and cost. Desferal induced moderate aluminum excretion in urine but had no effect on copper or zinc excretion.

摘要

针对一名患有血红蛋白汉默史密斯病和特纳综合征的儿童,因其贫血症状和无效红细胞生成而被当作重型地中海贫血患者进行治疗,开展了一项为期12天的平衡研究,测量尿液和粪便排泄情况,以确定静脉注射(i.v.)去铁胺(293毫克/千克/24小时×2)对铁、铝、铜和锌的影响。铁平衡为正值,基线为34毫克/3天。去铁胺输注在48小时内促使铁排泄117毫克,粪便和尿液中的排泄量几乎相等。该儿童每年接受约20次输血/静脉注射去铁胺治疗。如果每次治疗的铁排泄量大致相同,那么每年将达到2340毫克,占其输血年铁摄入量的47%。静脉输注是该患者治疗的重要组成部分,对于其他皮下注射去铁胺不足以防止持续铁蓄积的慢性输血患者可能也有用。一些患者已成功在家中接受静脉去铁胺治疗,从而缩短了住院时间并降低了费用。去铁胺促使尿液中铝适度排泄,但对铜或锌的排泄没有影响。

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