期刊
CLINICAL NEUROPATHOLOGY
卷 31, 期 3, 页码 127-134出版社
DUSTRI-VERLAG DR KARL FEISTLE
DOI: 10.5414/NP300441
关键词
growth hormone treatment; iatrogenic CJD; transitional form of panencephalopathic CJD
资金
- CJD Foundation
- NIH [RO1 NS062787]
- foundation Alliance Biosecure
- University Center on Aging and Health
- McGregor Foundation
- Case Western Reserve University
We report a case of iatrogenic Creutzfeldt-Jakob disease(iCJD) in a child with a neonatal growth hormone (GH) deficiency that was treated with native human growth hormone (hGH) between the ages of 9 months and 7 years. Three years after the end of treatment a progressive neurological syndrome consistent with Creutzfeldt-Jakob disease (CJD) developed, leading to death within a year, at age 11. Neuropathological examination showed an unusual widespread form of CJD, notably characterized by (i) involvement of the cerebellar white matter, (ii) cortico-spinal degeneration and (iii) ballooned neurons. A transitional form of the disease between common iatrogenic and panencephalopathic CJD is suggested.
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