Correction of purine nucleoside phosphorylase deficiency by transplantation of allogeneic bone marrow from a sibling

被引：21

作者：

Broome, CB

Graham, ML

Saulsbury, FT

Hershfield, MS

Buckley, RH

机构：

[1] DUKE UNIV, MED CTR, DEPT PEDIAT, DURHAM, NC 27710 USA

[2] DUKE UNIV, MED CTR, DEPT IMMUNOL, DURHAM, NC 27710 USA

[3] DUKE UNIV, MED CTR, DEPT INTERNAL MED, DURHAM, NC 27710 USA

[4] UNIV VIRGINIA, SCH MED, DEPT PEDIAT, CHARLOTTESVILLE, VA 22908 USA

来源：

JOURNAL OF PEDIATRICS | 1996年 / 128卷 / 03期

关键词：

D O I：

10.1016/S0022-3476(96)70285-8

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Deficiency of the purine salvage pathway enzyme purine nucleoside phosphorylase causes a combined immunodeficiency and neurologic abnormalities and is usually fatal in childhood, We report the first successful transplantation of bone marrow from a sibling with identical class II human leukocyte antigens in this condition, demonstrating correction of both lymphocyte metabolic and functional abnormalities.

引用

页码：373 / 376

页数：4

共 15 条

[1]

BUCKLEY RH, 1994, PEDIATR CLIN N AM, V41, P665

[2]

BUCKLEY RH, 1995, CLIN IMMUNOLOGY PRIN, P1813

[3]

COURNOYER D, 1993, ANNU REV IMMUNOL, V11, P297

[4]

DELUCA EC, 1986, EUR J PEDIATR, V145, P51, DOI 10.1007/BF00441852