CHONDROOSSEOUS DYSPLASIA IN SEVERE COMBINED IMMUNODEFICIENCY DUE TO ADENOSINE-DEAMINASE DEFICIENCY (CHONDROOSSEOUS DYSPLASIA IN ADA DEFICIENCY SCID)

被引：19

作者：

CHAKRAVARTI, VS

BORNS, P

LOBELL, J

DOUGLAS, SD

机构：

[1] UNIV PENN, SCH MED,CHILDRENS HOSP PHILADELPHIA, DIV INFECT DIS & IMMUNOL,IMMUNOL SECT, PHILADELPHIA, PA 19144 USA

[2] UNIV PENN, SCH MED, CHILDRENS HOSP PHILADELPHIA, DEPT RADIOL, PHILADELPHIA, PA 19104 USA

[3] GEISINGER MED CTR, DEPT HEMATOL ONCOL, DANVILLE, PA 17822 USA

来源：

PEDIATRIC RADIOLOGY | 1991年 / 21卷 / 06期

关键词：

D O I：

10.1007/BF02026688

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Adenosine deaminase (ADA) deficiency may manifest as severe combined immunodeficiency (SCID) in early infancy. Some of these children develop radiologic changes which may be in part related to effects of this enzyme deficiency on the bony epiphysis [1]. We describe the radiologic changes in a neonate with ADA deficiency and their resolution with polyethylene glycol conjugated adenosine deaminase (PEG-ADA, ADAGEN; Enzon, Inc., South Plainfield, NJ) enzyme replacement therapy.

引用

页码：447 / 448

页数：2

共 6 条

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