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February 20, 1991, Vol 265, No. 7 >
ARTICLE | February 20, 1991

Creutzfeldt-Jakob Disease in Pituitary Growth Hormone Recipients in the United States FREE

Judith E. Fradkin, MD; Lawrence B. Schonberger, MD, MPH; James L. Mills, MD, MS; Walter J. Gunn, PhD; Joyce M. Piper, DrPH; Diane K. Wysowski, PhD; Ruth Thomson; Stephen Durako; Paul Brown, MD
[+] Author Affiliations

Reprint requests to Endocrinology and Metabolic Diseases Programs Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Westwood Bldg, Room 603, Bethesda, MD 20892 (Dr Fradkin).


JAMA. 1991;265(7):880-884. doi:10.1001/jama.1991.03460070062043
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References

To assess the magnitude of Creutzfeldt-Jakob disease (CJD) occurrence among recipients of pituitary-derived human growth hormone (HGH), we conducted an epidemiologic follow-up of 6284 recipients of HGH distributed through the National Hormone and Pituitary Program. Seven neuropathologically confirmed cases of CJD have occurred in this population to date: six patients with clinical CJD presented with ataxia and imbalance, rather than with altered mentation, which is the most common initial manifestation in sporadic CJD, and one patient died in the preclinical incubation state of the disease. All seven cases occurred among the nearly 700 HGH recipients who started therapy before 1970. Since only 10% of the cohort has been followed up for the 15-year average incubation interval from midpoint of HGH treatment to onset of symptoms, the great majority of potentially exposed patients have not yet attained the requisite incubation period for expression of CJD. The median duration of HGH therapy of 100 months in the CJD cases was significantly longer than 41 months for all patients starting treatment before 1970; thus, the duration of pituitary HGH therapy is a major risk factor for CJD.

(JAMA. 1991;265:880-884)

REFERENCES

1 +
Koch TK, Berg BO, De Armand SJ, et al.  Creutzfeldt-Jakob disease in a young adult with idiopathic hypopituitarism: possible relationship to administration of cadaveric human growth hormone . N Engl J Med. 1985;;313:731-733.
2 +
Gibbs CJ Jr, Joy A, Heffner R, et al.  Clinical and pathological features and laboratory confirmation of Creutzfeldt-Jakob disease in a recipient of pituitary-derived human growth hormone . N Engl J Med. 1985;;313:734-738.
3 +
Tintner R, Brown P, Hedley-Whyte ET, et al.  Neuropathologic verification of Creutzfeldt-Jakob disease in the exhumed American recipient of human growth hormone: epidemiologic and pathogenetic implications . Neurology. 1986;;36:932-936.
4 +
Brown P, Gajdusek DC, Gibbs CJ Jr, et al.  Potential epidemic of Creutzfeldt-Jakob disease from human growth hormone therapy . N Engl J Med. 1985;;313:728-731.
5 +
Mills JL, Fradkin J, Schonberger L, et al.  Status report on the U.S. human growth hormone recipient follow-up study . Horm Res. 1990;;33:116-120.
6 +
Brown P, Cathala F, Castaigne P, et al.  Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases . Ann Neurol. 1986;;20:597-602.
7 +
New MI, Brown P, Temeck JW, Owens C, Hedley-Whyte ET, Richardson EP.  Preclinical Creutzfeldt-Jakob disease discovered at autopsy in a human growth hormone recipient . Neurology. 1988;;38:1133-1134.
8 +
Marzewski DJ, Towfighi J, Harrington MG, Merril CR, Brown P.  Creutzfeldt-Jakob disease following pituitary-derived human growth hormone therapy: a new American case . Neurology. 1988;;38:1131-1133.
9 +
Powell-Jackson J, Weller RO, Kennedy P, et al.  Creutzfeldt-Jakob disease after administration of human growth hormone . Lancet. 1985;;2:244-246.
10 +
Croxson M, Brown P, Synek B, et al.  A new case of Creutzfeldt-Jakob disease associated with human growth hormone therapy in New Zealand . Neurology . 1988;;38:1128-1130.
11 +
Cochius JI, Burns RJ, Blumbergs PC, Mack K, Alderman CP.  Creutzfeldt-Jakob disease in a recipient of human pituitary-derived gonadotropin . Aust N Z J Med. 1990;;20:592-594.
12 +
Brown P, Cathala F, Raubertas RF, Gajdusek DC, Castaigne P.  The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15-year investigation in France and review of the world literature . Neurology. 1987;;37:895-904.
13 +
Gibbs CJ Jr, Gajdusek DC.  Infection as the etiology of spongiform encephalopathy in Creutzfeldt-Jakob disease . Science. 1969;:165:1023-1025.
14 +
Brown P.  Human growth hormone therapy and Creutzfeldt-Jakob disease: a drama in three acts . Pediatrics. 1988;;81:85-92.
15 +
Goujard J, Entat M, Maillard F, Mugnier E, Rappaport R, Job JC.  Human pituitary growth hormone (hGH) and Creutzfeldt-Jakob disease: results of an epidemiological survey in France, 1986 . Int J Epidemiol. 1988;;17:423-427.
16 +
Raben MS.  Human growth hormone . Recent Prog Horm Res. 1959;;15:71.
17 +
Wilhelmi AE.  Fractionation of human pituitary glands . Can JBiochem Physiol. 1961;;39:1659-1668.
18 +
Saxena BB, Henneman PH.  Isolation and properties of the electrophoretic components of human growth hormone by Sephadex-gel filtration and preparative polyacrylamide-gel electrophoresis . Biochem J. 1966;;100:711-717.
19 +
Taylor DM, Dickinson AG, Fraser H, et al.  Preparation of growth hormone free from contamination from unconventional slow viruses . Lancet. 1985;;2:260-262.
20 +
Brown P.  Virus sterility for human growth hormone . Lancet. 1985;;2:729-730.
21 +
Pocchiari M, Peano S, Conz A, et al.  Successful decontamination of native human pituitary-derived growth hormone . Horm Res. In press.
22 +
Brown P.  The clinical neurology and epidemiology of Creutzfeldt-Jakob disease, with special reference to iatrogenic cases . In: Novel Infectious Agents and the Central Nervous System . New York, NY: John Wiley & Sons Inc; 1988;:3-23.
23 +
Hsiao K, Baker HF, Crow TJ, et al.  Linkage of a prior protein missense variant to Gerstmann-Straussler syndrome . Nature. 1989;;338:342-345.
24 +
Tateishi J, Kitamoto T, Doh-ura K, et al.  Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Straussler-Scheinker syndrome . Neurology. 1990;;40:1578-1581.
25 +
Owen F, Poulter M, Shah T, et al.  An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease . Mol Brain Res. 1990;;7:273-276.
26 +
Goldfarb LG, Mitrova E, Brown P, Toh BH, Gajdusek DC.  Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia . Lancet . 1990;;336:514-515.
27 +
Goldfarb LG, Korczyn AD, Brown P, Chapman J, Gajdusek DC.  Mutation in codon 200 of scrapie amyloid precursor gene liked to Creutzfeldt-Jakob disease in Sephardic Jews of Libyan and non-Libyan origin . Lancet. 1990;;336:637-638.
28 +
Goldfarb LG, Haltia M, Brown P, et al.  A new mutation in the scrapie amyloid precursor gene (at codon 178) is linked to the occurrence of Creutzfeldt-Jakob disease in a large Finnish kindred . Lancet . In press.

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1 +
Koch TK, Berg BO, De Armand SJ, et al.  Creutzfeldt-Jakob disease in a young adult with idiopathic hypopituitarism: possible relationship to administration of cadaveric human growth hormone . N Engl J Med. 1985;;313:731-733.
2 +
Gibbs CJ Jr, Joy A, Heffner R, et al.  Clinical and pathological features and laboratory confirmation of Creutzfeldt-Jakob disease in a recipient of pituitary-derived human growth hormone . N Engl J Med. 1985;;313:734-738.
3 +
Tintner R, Brown P, Hedley-Whyte ET, et al.  Neuropathologic verification of Creutzfeldt-Jakob disease in the exhumed American recipient of human growth hormone: epidemiologic and pathogenetic implications . Neurology. 1986;;36:932-936.
4 +
Brown P, Gajdusek DC, Gibbs CJ Jr, et al.  Potential epidemic of Creutzfeldt-Jakob disease from human growth hormone therapy . N Engl J Med. 1985;;313:728-731.
5 +
Mills JL, Fradkin J, Schonberger L, et al.  Status report on the U.S. human growth hormone recipient follow-up study . Horm Res. 1990;;33:116-120.
6 +
Brown P, Cathala F, Castaigne P, et al.  Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases . Ann Neurol. 1986;;20:597-602.
7 +
New MI, Brown P, Temeck JW, Owens C, Hedley-Whyte ET, Richardson EP.  Preclinical Creutzfeldt-Jakob disease discovered at autopsy in a human growth hormone recipient . Neurology. 1988;;38:1133-1134.
8 +
Marzewski DJ, Towfighi J, Harrington MG, Merril CR, Brown P.  Creutzfeldt-Jakob disease following pituitary-derived human growth hormone therapy: a new American case . Neurology. 1988;;38:1131-1133.
9 +
Powell-Jackson J, Weller RO, Kennedy P, et al.  Creutzfeldt-Jakob disease after administration of human growth hormone . Lancet. 1985;;2:244-246.
10 +
Croxson M, Brown P, Synek B, et al.  A new case of Creutzfeldt-Jakob disease associated with human growth hormone therapy in New Zealand . Neurology . 1988;;38:1128-1130.
11 +
Cochius JI, Burns RJ, Blumbergs PC, Mack K, Alderman CP.  Creutzfeldt-Jakob disease in a recipient of human pituitary-derived gonadotropin . Aust N Z J Med. 1990;;20:592-594.
12 +
Brown P, Cathala F, Raubertas RF, Gajdusek DC, Castaigne P.  The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15-year investigation in France and review of the world literature . Neurology. 1987;;37:895-904.
13 +
Gibbs CJ Jr, Gajdusek DC.  Infection as the etiology of spongiform encephalopathy in Creutzfeldt-Jakob disease . Science. 1969;:165:1023-1025.
14 +
Brown P.  Human growth hormone therapy and Creutzfeldt-Jakob disease: a drama in three acts . Pediatrics. 1988;;81:85-92.
15 +
Goujard J, Entat M, Maillard F, Mugnier E, Rappaport R, Job JC.  Human pituitary growth hormone (hGH) and Creutzfeldt-Jakob disease: results of an epidemiological survey in France, 1986 . Int J Epidemiol. 1988;;17:423-427.
16 +
Raben MS.  Human growth hormone . Recent Prog Horm Res. 1959;;15:71.
17 +
Wilhelmi AE.  Fractionation of human pituitary glands . Can JBiochem Physiol. 1961;;39:1659-1668.
18 +
Saxena BB, Henneman PH.  Isolation and properties of the electrophoretic components of human growth hormone by Sephadex-gel filtration and preparative polyacrylamide-gel electrophoresis . Biochem J. 1966;;100:711-717.
19 +
Taylor DM, Dickinson AG, Fraser H, et al.  Preparation of growth hormone free from contamination from unconventional slow viruses . Lancet. 1985;;2:260-262.
20 +
Brown P.  Virus sterility for human growth hormone . Lancet. 1985;;2:729-730.
21 +
Pocchiari M, Peano S, Conz A, et al.  Successful decontamination of native human pituitary-derived growth hormone . Horm Res. In press.
22 +
Brown P.  The clinical neurology and epidemiology of Creutzfeldt-Jakob disease, with special reference to iatrogenic cases . In: Novel Infectious Agents and the Central Nervous System . New York, NY: John Wiley & Sons Inc; 1988;:3-23.
23 +
Hsiao K, Baker HF, Crow TJ, et al.  Linkage of a prior protein missense variant to Gerstmann-Straussler syndrome . Nature. 1989;;338:342-345.
24 +
Tateishi J, Kitamoto T, Doh-ura K, et al.  Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Straussler-Scheinker syndrome . Neurology. 1990;;40:1578-1581.
25 +
Owen F, Poulter M, Shah T, et al.  An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease . Mol Brain Res. 1990;;7:273-276.
26 +
Goldfarb LG, Mitrova E, Brown P, Toh BH, Gajdusek DC.  Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia . Lancet . 1990;;336:514-515.
27 +
Goldfarb LG, Korczyn AD, Brown P, Chapman J, Gajdusek DC.  Mutation in codon 200 of scrapie amyloid precursor gene liked to Creutzfeldt-Jakob disease in Sephardic Jews of Libyan and non-Libyan origin . Lancet. 1990;;336:637-638.
28 +
Goldfarb LG, Haltia M, Brown P, et al.  A new mutation in the scrapie amyloid precursor gene (at codon 178) is linked to the occurrence of Creutzfeldt-Jakob disease in a large Finnish kindred . Lancet . In press.

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