CHRONIC GRANULOMATOUS-DISEASE PRESENTING IN A 69-YEAR-OLD MAN

被引：92

作者：

SCHAPIRO, BL

NEWBURGER, PE

KLEMPNER, MS

DINAUER, MC

机构：

[1] TUFTS UNIV, SCH MED, BOSTON, MA 02111 USA

[2] UNIV MASSACHUSETTS, SCH MED, DEPT PEDIAT, WORCESTER, MA 01605 USA

[3] CHILDRENS HOSP MED CTR, DIV HEMATOL ONCOL, BOSTON, MA 02115 USA

[4] HARVARD UNIV, SCH MED, DANA FARBER CANC INST, BOSTON, MA 02115 USA

[5] HARVARD UNIV, SCH MED, DEPT PEDIAT, BOSTON, MA 02115 USA

来源：

NEW ENGLAND JOURNAL OF MEDICINE | 1991年 / 325卷 / 25期

关键词：

D O I：

10.1056/NEJM199112193252506

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

CHRONIC granulomatous disease is a rare inherited disease characterized by severe recurrent bacterial and fungal infections beginning in childhood.1 Phagocytes from patients with chronic granulomatous disease exhibit defective killing of ingested microorganisms because of deficient production of Superoxide and related microbial oxidants by the NADPH oxidase of the phagocyte. The active oxidase complex is composed of both membrane and cytosolic proteins that catalyze the one-electron transfer from NADPH to oxygen, thus forming Superoxide.2 It is now recognized that chronic granulomatous disease can result from genetic defects in at least four different oxidase polypeptides. The most common form of chronic granulomatous. © 1991, Massachusetts Medical Society. All rights reserved.

引用

页码：1786 / 1790

页数：5

共 42 条

[11] A MISSENSE MUTATION IN THE NEUTROPHIL CYTOCHROME-B HEAVY-CHAIN IN CYTOCHROME-POSITIVE X-LINKED CHRONIC GRANULOMATOUS-DISEASE [J].