Finn A, Hadzić N, Morgan G, Strobel S, Levinsky R J
Department of Immunology, Institute of Child Health, London.
Arch Dis Child. 1990 Sep;65(9):942-5. doi: 10.1136/adc.65.9.942.
The records of 28 patients with chronic granulomatous disease born over a 32 year period were reviewed. The characteristics of the group, and the frequency with which various clinical and laboratory features had been recorded, was assessed. Nine patients were known to have died, in most cases of progressive suppurative infection. Actuarial analysis showed 50% survival through the third decade of life. The long term survival of patients developing symptoms after the end of the first year of life was significantly better than that of patients whose illness started in infancy. Our data confirm that the severity of chronic granulomatous disease is not uniform, and that the prognosis for long term survival is better than that suggested in earlier reports. Early onset may be a poor prognostic sign and invasive aspergillosis is a life threatening complication. In the absence of curative treatment, trials to assess the effectiveness of interferon gamma are necessary and early antenatal diagnosis should be offered to as many affected families as possible.
回顾了32年间出生的28例慢性肉芽肿病患者的记录。评估了该组患者的特征以及各种临床和实验室特征的记录频率。已知9例患者死亡，多数死于进行性化脓性感染。精算分析显示，活到第三个十年的生存率为50%。出生后第一年末出现症状的患者长期生存率明显高于婴儿期发病的患者。我们的数据证实，慢性肉芽肿病的严重程度并不一致，长期生存预后比早期报告提示的要好。早发可能是预后不良的迹象，侵袭性曲霉病是一种危及生命的并发症。在缺乏治愈性治疗的情况下，有必要进行评估干扰素γ疗效的试验，并应尽可能为更多受影响的家庭提供早期产前诊断。
