Hurler syndrome: a case report of a 5-year follow-up of dental findings after bone marrow transplantation

被引：10

作者：

Wadenya, Rose ^{[1
]}

Stout, Angela ^{[2
]}

Gupta, Avin ^{[3
]}

Monge, Janet ^{[4
]}

机构：

[1] Univ Penn, Sch Dent Med, Dept Prevent & Restorat Sci, Div Pediat & Community Oral Hlth, Philadelphia, PA 19104 USA

[2] Private Pediat Dent Practice, Chesheim Dent Associates, Erdenheim, PA USA

[3] Univ Calif Los Angeles, Sch Dent, Residency Pediat Dent, Los Angeles, CA 90024 USA

[4] Univ Penn, Dept Anthropol & Penn Museum, Philadelphia, PA 19104 USA

来源：

SPECIAL CARE IN DENTISTRY | 2010年 / 30卷 / 01期

关键词：

Hurler syndrome; mucopolysaccharidosis; dental development;

D O I：

10.1111/j.1754-4505.2009.00115.x

中图分类号：

R78 [口腔科学];

学科分类号：

1003 ;

摘要：

Hurler syndrome is a rare autosomal recessive disorder of mucopolysaccharide metabolism. It results from a deficiency in lysosomal enzymes responsible for the breakdown of glycosaminoglycans. Affected individuals may show progressive physical and mental deterioration as glycosaminoglycans are deposited in the organs of the body. Bone marrow transplantation (BMT) is effective in improving some of the clinical manifestations of Hurler syndrome. Death is caused by cardiorespiratory failure and usually occurs before the second decade of life. In this case report, the course of dental development was followed over 5 years, from the primary dentition into the permanent dentition, of a child who was successfully treated with a bone marrow transplant in infancy. The timing of bone marrow therapy has significant and variable effect on the stages of tooth development with implications for the long-term maintenance of the dentition.

引用

页码：14 / 17

页数：4