Toyohiko Watanabe

Effect of Taurine on the Fatty Liver of Children with Simple Obesity

This study elucidated the effect of taurine on fatty liver in simple obesity. Taurine was orally administered to 10 children with fatty liver. During taurine administration, the CT numbers of the liver, which were low in the beginning, increased. Serum ALT levels were improved, especially in those children whose weight was well controlled. Even in those who failed in weight control, serum ALT levels were slightly recovered. Ratios of glycine/taurine-conjugated bile acids were decreased. Thus, taurine was effective in treating fatty liver of children with simple obesity regardless of the success/failure of weight control. Taurine administration is considered to be helpful as an adjuvant therapy for fatty liver.

Ursodeoxycholic acid therapy in the treatment of biliary atresia.

The prognosis of operated biliary atresia in the cases with bile excretion chiefly depends upon the prevention of ascending cholangitis. An antibiotic is therefore intravenously administered during the early postoperative phase, but cannot be used over a long period. In the cases showing satisfactory bile excretion after operation, ascending cholangitis is rare because of rapid disappearance of jaundice. Regarding this, the authors prescribed ursodeoxycholic acid (UDCA) at 10-15 mg/kg/day to 6 infants with biliary atresia for several weeks after operation, and then determined the effects of UDCA in improving jaundice and bile excretion. As a result, serum bilirubin and serum total bile acid (STBA) levels were decreased in 4 of the 6 infants. In the remaining 2 infants, their STBA levels showed no decrease, but were rather increased; these infants subsequently died of hepatic failure. These results suggested that UDCA is useful in the treatment of cholestasis associated with biliary atresia in the cases attaining postoperative bile excretion. It was also suggested that the treatment with UDCA should be stopped when the STBA levels increased after the beginning of the treatment. Therefore, it was thought that STBA levels measured during UDCA therapy could serve as a good indicator of the choleretic effect of UDCA.

Sulfated and Nonsulfated Bile Acids in Urine of Patients with Biliary Atresia: Analysis of Bile Acids by High-Performance Liquid Chromatography

Summary To elucidate urinary bile acid patterns in patients with biliary atresia (BA), 15 sulfated and nonsulfated bile acids in urine were separately measured by high-performance liquid chromatography. This relatively simple technique for fluorescence detection utilizes the enzyme 3α-hydroxysteroid dehydrogenase (3α-HSD) to reveal urinary bile acid patterns. By this method, recovery rates of sulfated and nonsulfated bile acids in urine were satisfactory, and this analysis was shown to be applicable to clinical situations. In 10 patients with BA, the mean level of total bile acids in urine (23.35 ± 18.51 μmol/day) was seven times higher than the mean level in eight normal infants (3.05 ± 2.05 μmol/day). In the infants with BA, the mean level of total sulfated bile acids was about half of the total bile acid level. The main components of urinary nonsulfated bile acids in BA were glycocholic acid (6.21 ± 5.55 μmol/day) and taurocholic acid (2.28 ± 1.33 μmol/day), whereas the main components of the urinary sulfated bile acids were glycochenodeoxycholic acid (4.58 ± 6.97 μmol/day) and taurochenodeoxycholic acid (3.67 ± 3.54 μmol/day). Chenodeoxycholic acid, which is relatively toxic to the liver, may more easily be conjugated with sulfate and, hence, excreted into urine at a faster rate than cholic acid. Marked individual variations in urinary bile acid patterns were observed not only in BA patients but also in normal controls.

Investigation of serum bile acids; seven patients with Alagille syndrome

To clarify whether an abnormal bile acid pattern has a role in the pathogenesis of Alagille syndrome, we compared serum bile acid patterns in seven with Alagille syndrome with those of patients with congenital biliary atresia (CBA), neonatal hepatitis (NH) and normal infants.Of the seven patients with Alagille syndrome, four patients were younger and three were older than 1 year. The mean total serum bile acid level in the infants was higher than in older subjects. There was a dissociation between the levels of serum total bile acid and bilirubin in three of the seven cases. The mean total bile acid levels in serum were in the following decreasing order: CBA, Alagille syndrome, NH and controls.The ratio of cholate to chenodeoxycholate in the younger patients with Alagille syndrome was significantly higher than CBA (P<0.001). However, no specific bile acid pattern was found in Alagille syndrome by high-performance liquid chromatography (HPLC).