Unconjugated, Glycine‐conjugated, Taurine‐conjugated Bile Acid Nonsulfates and Sulfates in Urine of Young Infants with Cholestasis

Abstract

A direct assay system for conjugated bile acids using an enzymatic procedure and high-performance liquid chromatography was used for the analysis of urinary bile acid profiles in young infants with intrahepatic cholestasis (idiopathic neonatal hepatitis syndrome) or extra-hepatic biliary atresia. The major urinary bile acids were cholate and chenodeoxycholate conjugates, but a small amount of deoxycholate and 3 beta-hydroxy-5-cholenate conjugates were detected. Although there was no significant difference in total bile acid excretion between patients with intrahepatic cholestasis and extrahepatic biliary atresia, mean ratios of cholate to chenodeoxycholate and sulfated to total urinary bile acids were different between the two groups examined (5.63 +/- 2.83 vs. 2.50 +/- 1.25, p less than 0.05, 15.8 +/- 9.9 vs. 34.5 +/- 9.9%, p less than 0.005). The proportion of taurine-conjugated chenodeoxycholate in the sulfate fraction to the total bile acid was lower in intrahepatic cholestasis, compared with that in biliary atresia (7.7 +/- 7.5 vs 22.7% +/- 7.8%, p less than 0.005). The greater ratio of cholate to chenodeoxycholate and the reduced excretion of sulfated urinary bile acids in intrahepatic cholestasis was due to decreased taurine-conjugated chenodeoxycholate sulfate excretion.

Simple and quantitative analysis of urinary sulfated tauro- and glycodihydroxycholic acids in infant with cholestasis by electrospray ionization mass spectrometry

“…There are three possible sulfated forms of bile acids: 3-sulfated bile acid, or 3-sulfated taurineor glycine-conjugated forms. Although, there are several reports of direct enzymatic assays for USBA [9,10], these assays do not determined which bile acid molecules show increased sulfation.…”

Screening and outcomes in biliary atresia: Summary of a National Institutes of Health workshop

“…The large-scale application of screening using serum bile acids with tandem mass spectrometry has shown that this approach is feasible with dried blood spots, but there is overlap in the levels between controls and infants with cholestatic liver diseases, including BA. 108 Smaller studies using isotopic dilution mass spectrometry, 109 high-performance liquid chromatography, 110 and enzymatic methods [111][112][113][114] have also shown that urinary and serum bile acids are elevated in children with BA, but the levels overlap with those of control newborns during the critical first several weeks of life. Thus, bile acid levels may be helpful in differentiating breast milk jaundice or prolonged physiologic jaundice from jaundice caused by liver disease but cannot be used alone for the screening and early detection of BA.…”

Κινητικη Μελετη Και Αναλυτικες Εφαρμογες Ενζυμικων Συστηματων Για Τον Προσδιορισμο Χολικων Οξεων Σε Βιολογικα Υγρα

“…CDCA, αποτελούν το μεγαλύτερο ποσοστό των συζευγμένων χολικών οξέων. Στο ποσοστό των μη εστεροποιπμένων χολικών οξέων υπερέχει το GCA, ενώ το TCDCA αποτελεί το μεγαλύτερο ποσοστό των βειοεστέρων (301).…”

“…Τα πρωτογενή χολικά οξέα, CA και CDCA σε όλες τους τις μορφές, ελεύθερα, συζευγμένα με ταυρίνη ή γλυκίνη, θειοεστέρες ή γλυκουρονίδια, αποτελούν το μεγαλύτερο ποσοστό («80%) των χολικών οξέων που απεκκρίνεται στα ούρα (299)(300)(301).…”

“…2) Τα πρωτογενή χολικά οξέα αποτελούν το μεγαλύτερο ποσοστό («80%) των χολικών οξέων των ούρων (299)(300)(301).…”