Myrup B, Valerius N H, Mortensen P B
Department of Medicine A, Rigshospitalet, Denmark.
Gut. 1998 Jan;42(1):127-30. doi: 10.1136/gut.42.1.127.
In several diseases there is a relation between deficiency of neutrophil granulocytes and granulomatous lesions. Recently, in glycogen storage disease type Ib, this relation has been supported by the beneficial effect of treatment of enteritis with granulocyte-macrophage colony stimulating factor.
To investigate whether chronic granulomatous disease could be treated according to the same principle.
Inflammatory lesions were monitored in two brothers with chronic granulomatous disease demonstrated by very low superoxide production in neutrophil granulocytes. The two patients were treated with recombinant human granulocyte colony stimulating factor on three occasions when the disease was active.
In one patient, remission of an inflamed stenosis of the colon sigmoideum was shown by granulocyte scintigraphy after one month of treatment with granulocyte colony stimulating factor. In the other patient, remission of colon disease of later of a non-malignant tumor in the right lung hilum was shown by colonoscopy and computed tomography scans respectively.
Remission of inflammatory lesions in two brothers with chronic granulomatous disease was induced by granulocyte colony stimulating factor on three occasions. The mechanism for this effect is now known. The result is similar to the response found in patients with leucocyte deficiency due to glycogen storage disease type Ib.
在几种疾病中，中性粒细胞缺乏与肉芽肿性病变之间存在关联。最近，在I型糖原贮积病中，粒细胞-巨噬细胞集落刺激因子治疗肠炎的有益效果支持了这种关联。
研究慢性肉芽肿病是否可根据相同原则进行治疗。
对两名患有慢性肉芽肿病的兄弟的炎性病变进行监测，他们的中性粒细胞超氧化物生成极低。在疾病活动的三个阶段，这两名患者接受了重组人粒细胞集落刺激因子治疗。
一名患者在接受粒细胞集落刺激因子治疗一个月后，粒细胞闪烁显像显示乙状结肠炎性狭窄缓解。另一名患者，分别通过结肠镜检查和计算机断层扫描显示右肺门非恶性肿瘤后期的结肠疾病缓解。
粒细胞集落刺激因子在三个阶段诱导了两名患有慢性肉芽肿病的兄弟的炎性病变缓解。这种效应的机制目前尚不清楚。结果与I型糖原贮积病所致白细胞缺乏患者的反应相似。
