Endocrine cells in the upper gastrointestinal tract in relation to gastrointestinal dysfunction in patients with familial amyloidotic polyneuropathy

Gastrointestinal (GI) dysfunction is a common complication of familial amyl oidotic polyneuropathy (FAP). In previous reports, a decreased content of s mall and large intestinal endocrine cells has been found in patients with F AP and it has been suggested that this may contribute to the development of GI disturbances, The aim of the present study was to investigate the endoc rine cell content in the stomach and duadenum of FAP patients, and to corre late the findings with gastric emptying. Fifteen patients with FAP were included in the study. Twenty-eight subjects with macroscopically and histologically normal mucosa were used as control s for endocrine cell contents and 14 healthy subjects for gastric scintigra phy. The endocrine cells were identified by immunohistochemistry and quanti fied with image analysis. Gastric emptying time was detected by scintigraph y and endoscopy. The number of chromogranin A-immunoreactive (IR) cells war reduced in all i nvestigated parts of the GI tract except bulbus duodeni. Gastrin/CCK cell c ontent was reduced in duodenum, but fended to be increased in antrum of the stomach (P=0.07). Otherwise, the content of all other endocrine cells type s in the upper GI tract was reduced compared with controls. A correlation w ith malnutrition tvas found for gastric inhibitory polypeptide and secretin cell content in bulbus duodeni. Gastric scintigraphy disclosed delayed gas tric emptying of solid food, but the finding was not correlated to the decr eased content of neuroendocrine cells, The severity of endocrine cell deple tion was not correlated to duration of GI disturbances. The present study showed that the endocrine cells of the stomach are affect ed in FAP patients and that the abnormalities in the upper GI endocrine cel ls occur early during the course of the disease.