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表现为类似白塞病的眼黏膜皮肤综合征的慢性肉芽肿性疾病。

Chronic granulomatous disease presenting as an oculomucocutaneous syndrome mimicking Behçet's syndrome.

作者信息

Kelleher D, Bloomfield F J, Lenehan T, Griffin M, Feighery C, McCann S R

出版信息

Postgrad Med J. 1986 Jun;62(728):489-91. doi: 10.1136/pgmj.62.728.489.

DOI:10.1136/pgmj.62.728.489
PMID:3774683
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2418770/
Abstract

A female patient who presented for the first time at the age of 19 with oculomucocutaneous syndrome was found to have an absolute deficiency of neutrophil peroxide production. Neutrophil peroxide production as measured by chemiluminescence was zero on stimulation with opsonized zymosan. Direct membrane stimulation with FMLP and calcium ionophore also failed to elicit peroxide production. The diagnosis of chronic granulomatous disease should be considered in young patients with oculomucocutaneous syndrome.

摘要

一名19岁首次出现眼黏膜皮肤综合征的女性患者被发现绝对缺乏中性粒细胞过氧化物生成。通过化学发光法测量，用调理酵母聚糖刺激时中性粒细胞过氧化物生成量为零。用N-甲酰甲硫氨酰亮氨酰苯丙氨酸（FMLP）和钙离子载体进行直接膜刺激也未能引发过氧化物生成。对于患有眼黏膜皮肤综合征的年轻患者，应考虑慢性肉芽肿病的诊断。

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Chronic granulomatous disease presenting as an oculomucocutaneous syndrome mimicking Behçet's syndrome.表现为类似白塞病的眼黏膜皮肤综合征的慢性肉芽肿性疾病。
Postgrad Med J. 1986 Jun;62(728):489-91. doi: 10.1136/pgmj.62.728.489.
2
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Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease.白塞病诊断标准。白塞病国际研究小组。
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本文引用的文献

1
A fatal granulomatosus of childhood: the clinical study of a new syndrome.一种儿童期致命性肉芽肿病：一种新综合征的临床研究。
Minn Med. 1957 May;40(5):309-12.
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The measurement of opsonic and phagocytic function by Luminol-dependent chemiluminescence.通过鲁米诺依赖的化学发光法测定调理吞噬功能。
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Clinical and laboratory criteria for the diagnosis of Behçet's disease.白塞病诊断的临床及实验室标准。
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4
Absence of cytochrome b-245 in chronic granulomatous disease. A multicenter European evaluation of its incidence and relevance.慢性肉芽肿病中细胞色素b - 245缺失。一项关于其发病率及相关性的欧洲多中心评估。
N Engl J Med. 1983 Feb 3;308(5):245-51. doi: 10.1056/NEJM198302033080503.
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NIH conference. Recent advances in chronic granulomatous disease.美国国立卫生研究院会议。慢性肉芽肿病的最新进展。
Ann Intern Med. 1983 Nov;99(5):657-74. doi: 10.7326/0003-4819-99-5-657.
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Behçet's disease. Report of twelve cases with three manifesting as papilledema.白塞病。12例报告，其中3例表现为视乳头水肿。
Am J Med. 1970 Dec;49(6):823-9. doi: 10.1016/s0002-9343(70)80064-x.
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N Engl J Med. 1974 Apr 18;290(16):915-6.
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J Clin Pathol. 1977 Mar;30(3):250-3. doi: 10.1136/jcp.30.3.250.