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先天性铁粒幼细胞贫血患儿的牙科管理:一例报告。

Dental management of a child with congenital sideroblastic anemia: a case report.

作者信息

Lindemeyer Rochelle G, Goldberg Erick, Pinto Andres

机构信息

Pediatric Dental Residency Program, School of Dental Medicine, The University of Pennsylvania, Pa, USA.

出版信息

Pediatr Dent. 2007 Jul-Aug;29(4):315-9.

Abstract

The sideroblastic anemias comprise a heterogeneous group of acquired and congenital disorders which that have in common: (1) anemia; (2) the presence of ring sideroblasts in the bone marrow; and (3) impaired heme biosynthesis. Elevation in iron levels is secondary to a deficiency in mitochondrial physiology. Patients exhibit mild to moderate hemolysis due to peripheral red blood cell destruction, and often require regular blood transfusions. The purpose of this case report was to describe the dental management of a child with congenital sideroblastic anemia--a rare hematologic disorder--and to emphasize how important it is for pediatric dentists to be cognizant of a patient's hematologic profile before attempting to perform any dental procedure. The timing of the patient's transfusions regarding the scheduling of dental care is crucial for successful management and anticipation of possible complications. Preventive dentistry should be a hallmark of dental management for these children.

摘要

铁粒幼细胞性贫血包括一组异质性的获得性和先天性疾病,其共同特征为:(1)贫血;(2)骨髓中存在环形铁粒幼细胞;(3)血红素生物合成受损。铁水平升高是线粒体生理功能缺陷的继发表现。患者因外周红细胞破坏而出现轻度至中度溶血,常需定期输血。本病例报告的目的是描述一名患有先天性铁粒幼细胞性贫血(一种罕见的血液系统疾病)儿童的牙科治疗,并强调儿科牙医在尝试进行任何牙科手术之前了解患者血液学状况的重要性。患者输血时间与牙科护理安排对于成功管理和预测可能的并发症至关重要。预防性牙科应成为这些儿童牙科治疗的标志。

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