Scintigraphic documentation of an improvement in hepatobiliary excretory function after treatment with ursodeoxycholic acid in patients with cystic fibrosis and associated liver disease

Abstract

We have previously documented that ursodeoxycholic acid exerts a beneficial effect on liver function and bile acid metabolism in patients with cystic fibrosis. We hypothesized that the mechanism of action may be related in part to the choleretic properties of the administered bile acid. We therefore compared hepatobiliary scintigraphic images obtained before and 1 yr after initiation of ursodeoxycholic acid therapy to document an improvement in bile flow in 13 patients with cystic fibrosis and hepatobiliary involvement. Before therapy, hepatobiliary scintigraphy documented biliary stasis with retention of the isotope in intrahepatic and extrahepatic bile ducts in nine patients; during therapy, duct dilatation decreased substantially in eight patients, with decreased intrahepatic retention and more rapid biliary outflow of the tracer. The time of appearance of isotope in the intestine decreased (from a mean of 36.9 +/- 17.8 min to 18.8 +/- 9.0 min; p less than 0.01) in all patients in whom it had been abnormal, and the half-time of hepatic washout decreased from a mean of 35 +/- 20.7 min to 26 +/- 15.6 min (p less than 0.05). During ursodeoxycholic acid administration enrichment of bile was achieved, with the mean ursodeoxycholic acid percent composition increasing from 5.8% +/- 2.9% to 35.7% +/- 8.5%. Ursodeoxycholic acid became the predominant bile acid in serum. Liver function improved in all 10 of the patients with abnormal values at baseline. We conclude that hepatobiliary scintigraphy is of value in monitoring the therapeutic responses of cystic fibrosis patients with liver disease to ursodeoxycholic acid therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

A two-year prospective study of the effect of ursodeoxycholic acid on urinary bile acid excretion and liver morphology in cystic fibrosis-associated liver disease

“…8,10 In CF, UDCA has been shown to improve LFT and to ameliorate the hepatic wash-out of isotope at hepatobiliary scintigraphy. [11][12][13][14] No long-term study of the effects of UDCA treatment on liver morphology in patients with CF has been presented thus far. In this study, the influence of 2 years of treatment with UDCA on CF-associated liver disease was evaluated prospectively by assessment of LFT, the pattern of urinary bile acid excretion, and liver morphology.…”

In liver transplantation, T tube bile represents total bile flow: Physiological and scintigraphic studies on biliary secretion of organic anions

“…The changes in bile secretion and handling of 99m Tc-BRIDA during acute allograft rejection suggest that canalicular transport mechanisms involving bile acids or different organic anions are similarly sensitive. Furthermore, studies by Colombo et al 43 have documented improvement in hepatobiliary excretory function by hepatobiliary scintigraphy in patients with cystic fibrosis and associated liver disease after treatment with the bile acid UDCA. Similarly, Jazrawi et al 44 examined kinetics of hepatic bile acid handling in cholestatic liver and the effect of UDCA scintigraphically.…”

Tratamientos de la colestasis crónica en niños

“…El AUDC estimula la capacidad secretora biliar en los hígados de pacientes con FQ, tal como se ha determinado por gammagrafía hepatobiliar [10] . En varios estudios piloto se ha observado que el AUDC es responsable de una mejoría en las pruebas bioquímicas, principalmente una reducción de los niveles séricos de aminotransferasas (ALT y AST) y ␥ -glutamiltransferasa (GGT) [11] .…”