Corbeel L, Ceuppens J L, Van den Berghe G, Claeys H, Casteels-Van Daele M
Eur J Pediatr. 1984 Nov;143(1):45-8. doi: 10.1007/BF00442747.
A girl, 13 months of age, presented with generalised granulomatous skin, hair and mucosal candidiasis. Her lymphocytes failed to respond in vitro to Candida antigen (CA); the intradermal test with CA was also negative. Serum immunoglobulins, complement components, granulocyte functions (phagocytic and fungicidal), T-cell subsets, mitogenic and allogenic lymphocyte stimulation, natural killer cell activity and immune interferon production were all found to be normal. No circulating immune complexes were detected. Ketoconazole, an antimycotic drug, 5 mg/kg twice daily for 1 month and 2.5 mg/kg twice daily for another month spectacularly cleared all lesions. Afterwards, 4-monthly injections with transfer factor (TF) were given. Intradermal reactivity to CA was observed after the second TF injection. The lymphocyte responsiveness to CA in vitro became strongly positive 3 months after the last TF injection. The level of CA precipitins in serum, which was very high (11 lines) before ketoconazole treatment, decreased to 4 lines. No serum inhibitor of lymphocyte proliferation to CA could be demonstrated in the patient's serum before or after treatment. This specific CA unresponsiveness was not due to an excess of OKT8 + (suppressor) cells; macrophage migration inhibiting factor (MIF) production was normal. The nonresponsiveness might be due to antigenic overload or to suppressor cell induction not demonstrable in the present studies. The child has remained free of lesions during 3 years of follow-up without any further treatment.
一名13个月大的女童出现全身性肉芽肿性皮肤病变、毛发及黏膜念珠菌病。她的淋巴细胞在体外对念珠菌抗原(CA)无反应;CA皮内试验也呈阴性。血清免疫球蛋白、补体成分、粒细胞功能(吞噬和杀菌)、T细胞亚群、有丝分裂原及同种异体淋巴细胞刺激、自然杀伤细胞活性和免疫干扰素产生均正常。未检测到循环免疫复合物。抗真菌药物酮康唑,每日两次,每次5mg/kg,持续1个月,之后每日两次,每次2.5mg/kg,再持续1个月,显著清除了所有病变。此后,每4个月注射一次转移因子(TF)。第二次注射TF后观察到对CA的皮内反应性。最后一次注射TF后3个月,淋巴细胞对CA的体外反应性变为强阳性。酮康唑治疗前血清中CA沉淀素水平很高(11条带),降至4条带。治疗前后患者血清中均未显示出对CA的淋巴细胞增殖血清抑制剂。这种对CA的特异性无反应并非由于OKT8 +(抑制)细胞过多;巨噬细胞移动抑制因子(MIF)产生正常。无反应可能是由于抗原过载或本研究中未证实的抑制细胞诱导。该患儿在3年随访期间未再接受任何治疗,病变未再出现。