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慢性肉芽肿病;我们对慢性肉芽肿病理解的基础阶段。

Chronic Granulomatous Disease; fundamental stages in our understanding of CGD.

作者信息

Assari Tracy

机构信息

Molecular Immunology Unit, The Institute of Child Health, University College London and Great Ormond Street Hospital for Children NHS Trust, 30 Guilford Street, London WC1N 3EH, UK.

出版信息

Med Immunol. 2006 Sep 21;5:4. doi: 10.1186/1476-9433-5-4.

DOI:10.1186/1476-9433-5-4
PMID:16989665
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1592097/
Abstract

It has been 50 years since chronic granulomatous disease was first reported as a disease which fatally affected the ability of children to survive infections. Various milestone discoveries from the insufficient ability of patients' leucocytes to destroy microbial particles to the underlying genetic predispositions through which the disease is inherited have had important consequences. Longterm antibiotic prophylaxis has helped to fight infections associated with chronic granulomatous disease while the steady progress in bone marrow transplantation and the prospect of gene therapy are hailed as long awaited permanent treatment options. This review unearths the important findings by scientists that have led to our current understanding of the disease.

摘要

自慢性肉芽肿病首次被报道为一种致命影响儿童抵抗感染能力的疾病以来,已经过去了50年。从患者白细胞破坏微生物颗粒能力不足到该疾病遗传所基于的潜在遗传易感性等各种里程碑式的发现都产生了重要影响。长期抗生素预防有助于对抗与慢性肉芽肿病相关的感染,而骨髓移植的稳步进展以及基因治疗的前景被誉为期待已久的永久性治疗选择。这篇综述揭示了科学家们的重要发现,这些发现使我们对该疾病有了目前的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78b8/1592097/259b510d2448/1476-9433-5-4-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78b8/1592097/259b510d2448/1476-9433-5-4-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78b8/1592097/259b510d2448/1476-9433-5-4-1.jpg

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本文引用的文献

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Progress toward effective gene therapy for chronic granulomatous disease.慢性肉芽肿病有效基因治疗的进展。
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