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A familial glucagonoma syndrome
Abstract
A family with multiple endocrine neoplasia type I (MEN-I) is described in which three members had A-cell pancreatic tumors. Two of these members had classic glucagonoma syndromes. The proband, a 62 year old woman, had a high (less than or equal to 9.2 ng/ml) basal plasma glucagon level, most of which eluted in the 3,500 dalton fraction. Plasma glucagon increased following the ingestion of mixed meals and arginine. Secretin, which, in the dog, has been reported to inhibit normal glucagon secretion, provoked a twofold increase in 3,500 dalton plasma glucagon concentration. Increased plasma glucagon in the proband was associated with mild hyperglycemia and insulin resistance. Somatostatin infusion suppressed peripheral glucagon and insulin levels, and increased blood glucose levels. The unique responses to secretin and somatostatin observed in this patient may be diagnostically important in syndromes of inappropriate or autonomous glucagon secretion.
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Because the glucagon antiserum crossreacts with species in addition to 3,500-dalton glucagon and somatostatin suppresses 3,500-dalton glucagon preferentially, biologically active glucagon was likely suppressed substantially.
“However, since the antiserum used crossreacts with species in addition to 3,500dalton glucagon (25), and somatostatin suppresses 3,500-dalton glucagon preferentially (26–28), biologically active glucagon was likely suppressed substantially.”
From Epinephrine supports the postabsorptive plasma glucose concentration and prevents hypoglycemia when glucagon secretion is deficient in man (1984) · cites this paper as [28]
Somatostatin preferentially suppresses the secretion of 3,500-D glucagon in dogs, a glucagonoma patient, and normal humans.
“Since somatostatin preferentially suppresses the secretion of 3,500-D glucagon in dogs (20, 21), a glucagonoma patient (22), and normal humans¹”
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References 43 · Cited by 42
Research works printed but not linked: 35
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Metadata
- Authors
- Stacpoole, P. W., Jaspan, J., Kasselberg, A. G., Halter, S. A., Polonsky, K., Gluck, F. W., Liljenquist, J. E., & Rabin, D.
- ORCID
- Stacpoole, Peter W.
- Venue
- The American Journal of Medicine
- Publisher
- Elsevier BV
- Published
- 1981-05
- Volume
- 70
- Issue
- 5
- Pages
- 1017-1026
- ISSN
- 0002-9343 (ISSN-L)
- OpenAlex
- W1989222547
- Cited by
- 42
- References
- 43
- Citations by year
- 2024: 1
- FWCI
- 7.07
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The American Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Oncology › Neuroendocrine Tumor Research Advances
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pancreatic function and diabetes
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
- Keywords
- glucagonoma syndrome, multiple endocrine neoplasia type 1, plasma glucagon, secretin, somatostatin, insulin resistance, hyperglycemia, arginine stimulation
- MeSH terms
- Adolescent, Arginine, Blood Glucose, Female, Glucagon, Humans, Insulin, Adenoma, Islet Cell, Male, Middle Aged, Pancreatic Neoplasms, Secretin, Somatostatin
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