Skip to main content
Gut logoLink to Gut
. 1994 Oct;35(10):1413–1418. doi: 10.1136/gut.35.10.1413

Impact of familial amyloid associated polyneuropathy on duodenal endocrine cells.

M el-Salhy 1, O Suhr 1, R Stenling 1, E Wilander 1, L Grimelius 1
PMCID: PMC1375016  PMID: 7959197

Abstract

Duodenal endocrine cells in 11 patients with familial amyloid associated polyneuropathy (FAP) were compared with those in 12 healthy volunteers by means of immunohistochemistry and morphometry. The total endocrine cell content, determined by the argyrophilic reaction and chromogranin A immunoreactivity, was significantly reduced in FAP patients compared with controls. There was a significant reduction in the serotonin, cholecystokinin/gastrin, and secretin immunoreactive cell content. A decreased cell content was also noted for somatostatin and gastric inhibitory polypeptide immunoreactive cells but this was not statistically significant. Amyloid deposits were noted in seven of the 11 biopsy specimens from FAP patients, but otherwise the duodenum was histologically normal in both groups. The reduction in endocrine cell content was not correlated with the degree of amyloid deposit in the duodenum. These findings indicate that patients with FAP have reduced intestinal endocrine cells. This does not seem to be related to amyloid deposits in the mucosa or to villous or crypt abnormalities. The observed changes in endocrine cells may contribute to the development of intestinal motility dysfunction and maldigestion in these patients.

Full text

PDF
1413

Images in this article

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Andersson R. Familial amyloidosis with polyneuropathy. A clinical study based on patients living in northern Sweden. Acta Med Scand Suppl. 1976;590:1–64. [PubMed] [Google Scholar]
  2. Buchan A. M., Polak J. M. The classification of the human gastroenteropancreatic endocrine cells. Invest Cell Pathol. 1980 Jan-Mar;3(1):51–71. [PubMed] [Google Scholar]
  3. Cetin Y. Chromogranin A immunoreactivity and Grimelius' argyrophilia. A correlative study in mammalian endocrine cells. Anat Embryol (Berl) 1992;185(3):207–215. doi: 10.1007/BF00211819. [DOI] [PubMed] [Google Scholar]
  4. Chang C. M., Yu Y. L., Wong M., Ng T. H., Woo E., Huang C. Y. Type I familial amyloid polyneuropathy in a Chinese family. Acta Neurol Scand. 1989 May;79(5):391–396. doi: 10.1111/j.1600-0404.1989.tb03806.x. [DOI] [PubMed] [Google Scholar]
  5. Debas H. T., Mulvihill S. J. Neuroendocrine design of the gut. Am J Surg. 1991 Feb;161(2):243–249. doi: 10.1016/0002-9610(91)91139-a. [DOI] [PubMed] [Google Scholar]
  6. Dwulet F. E., Benson M. D. Polymorphism of human plasma thyroxine binding prealbumin. Biochem Biophys Res Commun. 1983 Jul 29;114(2):657–662. doi: 10.1016/0006-291x(83)90831-8. [DOI] [PubMed] [Google Scholar]
  7. El-Salhy M., Grimelius L., Wilander E., Ryberg B., Terenius L., Lundberg J. M., Tatemoto K. Immunocytochemical identification of polypeptide YY (PYY) cells in the human gastrointestinal tract. Histochemistry. 1983;77(1):15–23. doi: 10.1007/BF00496632. [DOI] [PubMed] [Google Scholar]
  8. Feurle G. E. Pathophysiology of diarrhea in patients with familial amyloid neuropathy. Digestion. 1987;36(1):13–17. doi: 10.1159/000199393. [DOI] [PubMed] [Google Scholar]
  9. Grimelius L., Wilander E. Silver stains in the study of endocrine cells of the gut and pancreas. Invest Cell Pathol. 1980 Jan-Mar;3(1):3–12. [PubMed] [Google Scholar]
  10. Harvey R. F. Hormonal control of gastrointestinal motility. Am J Dig Dis. 1975 Jun;20(6):523–539. doi: 10.1007/BF01074937. [DOI] [PubMed] [Google Scholar]
  11. Holmgren G., Ericzon B. G., Groth C. G., Steen L., Suhr O., Andersen O., Wallin B. G., Seymour A., Richardson S., Hawkins P. N. Clinical improvement and amyloid regression after liver transplantation in hereditary transthyretin amyloidosis. Lancet. 1993 May 1;341(8853):1113–1116. doi: 10.1016/0140-6736(93)93127-m. [DOI] [PubMed] [Google Scholar]
  12. Holmgren G., Haettner E., Nordenson I., Sandgren O., Steen L., Lundgren E. Homozygosity for the transthyretin-met30-gene in two Swedish sibs with familial amyloidotic polyneuropathy. Clin Genet. 1988 Nov;34(5):333–338. doi: 10.1111/j.1399-0004.1988.tb02887.x. [DOI] [PubMed] [Google Scholar]
  13. Holmgren G., Holmberg E., Lindström A., Lindström E., Nordenson I., Sandgren O., Steen L., Svensson B., Lundgren E., von Gabain A. Diagnosis of familial amyloidotic polyneuropathy in Sweden by RFLP analysis. Clin Genet. 1988 Mar;33(3):176–180. doi: 10.1111/j.1399-0004.1988.tb03434.x. [DOI] [PubMed] [Google Scholar]
  14. Holmgren G., Steen L., Ekstedt J., Groth C. G., Ericzon B. G., Eriksson S., Andersen O., Karlberg I., Nordén G., Nakazato M. Biochemical effect of liver transplantation in two Swedish patients with familial amyloidotic polyneuropathy (FAP-met30). Clin Genet. 1991 Sep;40(3):242–246. doi: 10.1111/j.1399-0004.1991.tb03085.x. [DOI] [PubMed] [Google Scholar]
  15. Holt I. J., Harding A. E., Middleton L., Chrysostomou G., Said G., King R. H., Thomas P. K. Molecular genetics of amyloid neuropathy in Europe. Lancet. 1989 Mar 11;1(8637):524–526. doi: 10.1016/s0140-6736(89)90068-8. [DOI] [PubMed] [Google Scholar]
  16. Ikeda S., Yanagisawa N., Hongo M., Ito N. Vagus nerve and celiac ganglion lesions in generalized amyloidosis. A correlative study of familial amyloid polyneuropathy and AL-amyloidosis. J Neurol Sci. 1987 Jun;79(1-2):129–139. doi: 10.1016/0022-510x(87)90267-x. [DOI] [PubMed] [Google Scholar]
  17. Olofsson B. O., Eriksson P., Eriksson A. The sick sinus syndrome in familial amyloidosis with polyneuropathy. Int J Cardiol. 1983 Aug;4(1):71–73. doi: 10.1016/0167-5273(83)90217-6. [DOI] [PubMed] [Google Scholar]
  18. Rubenstein A. E., Yahr M. D., Mytilineou C., Bajaj K. Peripheral catecholamine depletion in amyloid autonomic neuropathy. Mt Sinai J Med. 1978 Nov-Dec;45(6):782–789. [PubMed] [Google Scholar]
  19. Saraiva M. J., Costa P. P., Birken S., Goodman D. S. Presence of an abnormal transthyretin (prealbumin) in Portuguese patients with familial amyloidotic polyneuropathy. Trans Assoc Am Physicians. 1983;96:261–270. [PubMed] [Google Scholar]
  20. Sjölund K., Sandén G., Håkanson R., Sundler F. Endocrine cells in human intestine: an immunocytochemical study. Gastroenterology. 1983 Nov;85(5):1120–1130. [PubMed] [Google Scholar]
  21. Southern E. M. Detection of specific sequences among DNA fragments separated by gel electrophoresis. J Mol Biol. 1975 Nov 5;98(3):503–517. doi: 10.1016/s0022-2836(75)80083-0. [DOI] [PubMed] [Google Scholar]
  22. Staunton H., Dervan P., Kale R., Linke R. P., Kelly P. Hereditary amyloid polyneuropathy in north west Ireland. Brain. 1987 Oct;110(Pt 5):1231–1245. doi: 10.1093/brain/110.5.1231. [DOI] [PubMed] [Google Scholar]
  23. Steen L., Ek B. Familial amyloidosis with polyneuropathy. A long-term follow-up of 21 patients with special reference to gastrointestinal symptoms. Acta Med Scand. 1983;214(5):387–397. [PubMed] [Google Scholar]
  24. Suhr O., Danielsson A., Steen L. Bile acid malabsorption caused by gastrointestinal motility dysfunction? An investigation of gastrointestinal disturbances in familial amyloidosis with polyneuropathy. Scand J Gastroenterol. 1992;27(3):201–207. doi: 10.3109/00365529208999949. [DOI] [PubMed] [Google Scholar]
  25. Tawara S., Nakazato M., Kangawa K., Matsuo H., Araki S. Identification of amyloid prealbumin variant in familial amyloidotic polyneuropathy (Japanese type). Biochem Biophys Res Commun. 1983 Nov 15;116(3):880–888. doi: 10.1016/s0006-291x(83)80224-1. [DOI] [PubMed] [Google Scholar]
  26. WEIBEL E. R. Principles and methods for the morphometric study of the lung and other organs. Lab Invest. 1963 Feb;12:131–155. [PubMed] [Google Scholar]
  27. el-Salhy M., Wilander E., Lundqvist M., Nilsson G., Moody A. J., Imagawa K., Grimelius L. Glucagon and glucagon-like immunoreactive cells in mid- and hindgut carcinoids. APMIS. 1988 Jun;96(6):537–542. doi: 10.1111/j.1699-0463.1988.tb05341.x. [DOI] [PubMed] [Google Scholar]

Articles from Gut are provided here courtesy of BMJ Publishing Group

RESOURCES