PEDIATRICS Vol. 81 No. 1 January 1988, pp. 85-92
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Human Growth Hormone Therapy and Creutzfeldt-Jakob Disease: A Drama in Three Acts

Paul Brown MD1

1 From the Laboratory of Central Nervous System Studies, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, Maryland

PROLOGUE

In 1963, at about the time when research on human growth hormone had reached the point at which large-scale clinical trials could be envisioned, a child was born who within the first 2 years of life was found to have deficiencies of thyroid hormone, insulin, and growth hormone. He was begun on replacement therapy and, among other attributes of growth during his childhood, accumulated several pounds of medical records, first in Los Angeles and later at Palo Alto, CA. According to his mother, he was always a happy, optimistic, and talkative youngster; perhaps because of his tenuous medical condition, he was also somewhat of a stoic. Therefore, when he complained, it behooved people to listen.

ACT I

Scene I

In May 1984, the young man and his family flew from San Francisco to Atlanta en route to Maine to visit his grandparents. As he rose from his seat to change planes, he complained of dizziness. His mother, who was experienced in the diagnosis of hypoglycemia, gave him some candy and watched him closely for the rest of the trip. Nothing more happened, and the incident was forgotten. Several days later, however, in Maine, he turned down an offer to go for a spin on the lake in his grandfather's motorboat, saying that "he didn't need to go for a spin because he was already dizzy." On his return from Maine, the patient went back to school, but his dizziness persisted, and now his speech seemed slightly changed. He was seen by a local practitioner, who was unable to find any physical basis for the complaints but was concerned about a possible middle ear problem and suggested that he be seen at Stanford, where he was already being followed for his endocrine problems.

Submitted on May 18, 1987
Accepted on July 9, 1987




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