Mühlebach T J, Feickert H J, Welte K, Seger R A
Division of Immunology-Haematology, Childrens Hospital, University of Zürich, Switzerland.
Eur J Pediatr. 1991 Jun;150(8):575-8. doi: 10.1007/BF02072210.
Variant X-linked chronic granulomatous disease (CGD) is characterised by a decreased but still measurable respiratory burst and cytochrome b content of phagocytes resulting in a clinically milder form of the disease. We examined the in vivo effect of recombinant human granulocyte-macrophage colony stimulating factor (rh-GM-CSF) on the neutrophil functions of a patient treated for liver abscess. The number of white blood cells was markedly increased at the highest dose of GM-CSF injected (30 micrograms/kg per day). This was mainly due to a large increase in eosinophils and to a lesser extent in neutrophils. No change in the deficient neutrophil respiratory burst nitroblue tetrazolium (NBT)-reduction, superoxide (O2-)-production and cytochrome b content was observed during 6 weeks of therapy with increasing doses of GM-CSF. No significant clinical improvement of the liver abscess was observed during treatment with GM-CSF.
X连锁慢性肉芽肿病（CGD）变异型的特征是吞噬细胞的呼吸爆发和细胞色素b含量降低，但仍可测量，导致该疾病的临床症状较轻。我们研究了重组人粒细胞-巨噬细胞集落刺激因子（rh-GM-CSF）对一名因肝脓肿接受治疗的患者中性粒细胞功能的体内作用。在注射GM-CSF的最高剂量（每天30微克/千克）时，白细胞数量显著增加。这主要是由于嗜酸性粒细胞大幅增加，中性粒细胞增加程度较小。在使用递增剂量的GM-CSF进行6周治疗期间，未观察到缺陷中性粒细胞的呼吸爆发、硝基蓝四氮唑（NBT）还原、超氧化物（O2-）产生和细胞色素b含量有变化。在使用GM-CSF治疗期间，未观察到肝脓肿有明显的临床改善。
