Lonsdale D
Dev Pharmacol Ther. 1980;1(4):254-64.
The 2 children whose cases are reported here both had recurrent episodes of fever and cervical lymphadenopathy. The conventional approach had been unsuccessful in identifying the cause or therapy. In neither case was there an infectious agent demonstrated, and biopsy of a pathologically enlarged lymph gland revealed only reactive hyperplasia in each case. Abnormal metabolism was revealed in the first patient by detecting a substance in urine which is reported to be diagnostic for a form of subacute necrotizing encephalomyelopathy. In the second case, red cell transketolase indicated thiamine pyrophosphate deficiency. Both children had elevated concentrations of folate and B12 in serum. Neither of the 2 patients had further episodes when given a clinical trial with large doses of thiamine hydrochloride. Recurrent episodes of febrile lymphadenopathy are extemely frequent in children and spontaneous resolution occurs, while in others there is either proven or assumed infection. Although final proof of therapeutic efficacy is lacking, the rapid improvement and maintenance of health in both children was striking after conventional therapy had failed.
在此报告病例的2名儿童均有反复发热和颈部淋巴结病发作。传统方法未能成功找出病因或进行治疗。两例均未发现感染病原体，对病理肿大的淋巴结进行活检，结果显示每例均只有反应性增生。在第一例患者中，通过检测尿液中的一种物质发现了代谢异常，据报道该物质可诊断一种亚急性坏死性脑脊髓病。在第二例中，红细胞转酮醇酶表明焦磷酸硫胺素缺乏。两名儿童血清中的叶酸和维生素B12浓度均升高。在给予大剂量盐酸硫胺素进行临床试验时，这两名患者均未再发作。发热性淋巴结病反复发作在儿童中极为常见，有些会自行缓解，而在其他儿童中则存在已证实或疑似的感染。尽管缺乏治疗效果的最终证据，但在传统治疗失败后，两名儿童均迅速好转并保持健康，这一点令人瞩目。
