Billette de Villemeur T, Gourmelen M, Beauvais P, Rodriguez D, Vaudour G, Deslys J P, Dormont D, Richard P, Richardet J M
Service de Pédiatrie, Hôpital Trousseau, Paris.
Rev Neurol (Paris). 1992;148(5):328-34.
Creutzfeldt-Jakob disease was diagnosed in four growth hormone recipients at the age of 10, 11, 18 and 19 years. To our knowledge, the two first cases are the first instances of Creutzfeldt-Jakob disease recorded in children. Three of them were still being treated with synthetic hormone at the onset of the disease. Neurological disorders: ataxia and diplopia, appeared first, dementia and myoclonus appeared later. Eighteen cases of Creutzfeldt-Jakob disease in growth hormone recipients are now recorded, and the present risk of Creutzfeldt-Jakob disease in pituitary growth recipients is estimated to be 1/300. Because of the long incubation period, new cases are to be feared. Other causes of iatrogenic Creutzfeldt-Jakob disease are reviewed. These facts incite to consider carefully using products of human origin in human therapy. The interactions between growth hormone, prion and host's genomic make-up are still not clear.
在四名接受生长激素治疗的患者中确诊了克雅氏病，他们的年龄分别为10岁、11岁、18岁和19岁。据我们所知，前两例是儿童中记录的首例克雅氏病病例。其中三人在疾病发作时仍在接受合成激素治疗。神经系统疾病：共济失调和复视首先出现，痴呆和肌阵挛随后出现。目前已记录到18例接受生长激素治疗的患者患克雅氏病的病例，垂体生长激素接受者目前患克雅氏病的风险估计为1/300。由于潜伏期长，令人担忧会出现新病例。对医源性克雅氏病的其他病因进行了综述。这些事实促使人们在人类治疗中谨慎考虑使用源自人类的产品。生长激素、朊病毒和宿主基因组构成之间的相互作用仍不清楚。
