A pathophysiological study of the intestinal manifestations of a vasoactive intestinal peptide, calcitonin, and catecholamine-secreting tumour.
A three year old girl with severe watery diarrhoea and a vasoactive intestinal peptide, calcitonin, and catecholamine-secreting supra-renal ganglioneuroblastoma is reported. Steady-state perfusion studies showed the jejunum to be in a net secretory state with respect to water, sodium, and chloride at low concentrations (2 mmol/l) of glucose whereas higher concentrations (56 mmol/l) reversed secretion to absorption; transmural rectal potential difference was increased (lumen negative); Na+ absorption by the rectum was impaired and secretion of potassium and bicarbonate excessive. Motility studies showed prolonged, slowly propagated migrating motor complexes with abnormal runs of non-propagated contractions in the fasting state. During perfusion with glucose, no postprandial activity occurred. These results suggest that diarrhoea results from small intestinal secretion with impaired colonic function and that tumour products may have a direct effect on intestinal motility.
D000310:Adrenal Gland Neoplasms / Q000503:physiopathology*; D001692:Biological Transport; D002116:Calcitonin / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D003967:Diarrhea / Q000503:physiopathology; D005260:Female; D005729:Ganglioneuroma / Q000503:physiopathology*; D005769:Gastrointestinal Motility; D006801:Humans; D007223:Infant; D007421:Intestine, Small / Q000503:physiopathology; D008297:Male; D009638:Norepinephrine / Q000378:metabolism*; D012007:Rectum / Q000503:physiopathology; D014660:Vasoactive Intestinal Peptide / Q000378:metabolism*
D014660:Vasoactive Intestinal Peptide; D002116:Calcitonin; D009638:Norepinephrine