GASTROINTESTINAL SYMPTOMS AND DELAYED GASTRIC-EMPTYING IN FABRYS-DISEASE - RESPONSE TO METOCLOPRAMIDE

The pathophysiology of gastrointestinal symptoms in patients with Fabr y's disease is uncertain, despite the demonstration of histological an d radiographic abnormalities of the gastrointestinal tract. The aims o f this study were to determine if the gastrointestinal symptoms report ed by patients with Fabry's disease are associated with abnormal gastr ic emptying and, if they are, whether prokinetic drug therapy would be beneficial. Ten patients with Fabry's disease had radionuclide gastri c emptying studies performed to determine if gastrointestinal symptoms correlated with objective evidence of abnormal gastric emptying. A se cond study was performed in seven patients who received 5 days of ther apy with oral metoclopramide. The mean percent solid gastric emptying for Fabry's patients was significantly less than that of a normal cont rol group (40 vs 67%, P < 0.005). Five of seven patients with symptoms had abnormal gastric emptying. Six of seven symptomatic patients repo rted clinical improvement with metoclopramide. Of four symptomatic pat ients who had a repeat study after treatment, two showed improved empt ying. In conclusion, our results suggest that gastrointestinal symptom s in Fabry's disease are frequently associated with delayed gastric em ptying and that treatment with metoclopramide produces symptomatic and sometimes functional improvement. ((C) 1998 Lippincott Williams & Wil kins).