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髓过氧化物酶缺乏症:患病率及临床意义。

Myeloperoxidase deficiency: prevalence and clinical significance.

作者信息

Parry M F, Root R K, Metcalf J A, Delaney K K, Kaplow L S, Richar W J

出版信息

Ann Intern Med. 1981 Sep;95(3):293-301. doi: 10.7326/0003-4819-95-3-293.

DOI:10.7326/0003-4819-95-3-293
PMID:6267975
Abstract

Leukocyte differential counting by flow cytochemistry has shown 28 subjects with partial or complete neutrophil myeloperoxidase (MPO) deficiency in a population of about 60 000 patients screened at a general hospital. Partial (13 patients) or complete (13 patients) MPO deficiency was confirmed by examination of cytochemical stains in 26, biochemical measurement of total enzymatic activity in eight, and flow cytometry in six patients. None had apparent hematologic disorders. Only four patients had infections; of these, two had major systemic infections (one, candidiasis; one, bacteremia). In assays of leukocyte function only minor defects in killing of Staphylococcus aureus by MPO-deficient cells were noted whereas killing of Candida albicans was much more impaired. Family studies in eight patients have shown various degrees of partial or complete MPO deficiency in first-degree relatives of six. The findings indicate that the incidence of MPO deficiency is much higher than previously suspected. Although MPO appears to be necessary for killing of Candida species by neutrophils, the importance of its role in normal antibacterial defense must be re-evaluated.

摘要

通过流式细胞化学进行白细胞分类计数显示,在一家综合医院筛查的约60000名患者中,有28名受试者存在部分或完全性中性粒细胞髓过氧化物酶(MPO)缺乏。通过对26名患者进行细胞化学染色检查、对8名患者进行总酶活性的生化测定以及对6名患者进行流式细胞术检测,证实了13名患者为部分性(13例)或完全性(13例)MPO缺乏。所有患者均无明显血液系统疾病。只有4名患者发生感染;其中,2名患者发生严重全身感染(1例为念珠菌病;1例为菌血症)。在白细胞功能检测中,仅发现MPO缺乏细胞对金黄色葡萄球菌的杀伤存在轻微缺陷,而对白色念珠菌的杀伤受损更为严重。对8名患者的家族研究显示,6名患者的一级亲属存在不同程度的部分或完全性MPO缺乏。这些发现表明,MPO缺乏的发生率比之前怀疑的要高得多。虽然MPO似乎是中性粒细胞杀伤念珠菌属所必需的,但其在正常抗菌防御中的作用重要性必须重新评估。

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Myeloperoxidase deficiency: prevalence and clinical significance.髓过氧化物酶缺乏症:患病率及临床意义。
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Cytochemical distinction between azurophils and catalase-containing granules in leukocytes. I. Studies in developing neutrophils and monocytes from patients with myeloperoxidase deficiency: comparison with peroxidase-deficient chicken heterophils.白细胞中嗜天青颗粒与含过氧化氢酶颗粒的细胞化学鉴别。I. 对髓过氧化物酶缺乏症患者发育中的中性粒细胞和单核细胞的研究:与过氧化物酶缺乏的鸡异嗜性粒细胞的比较。
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