bilirubin-diglucuronide and Jaundice--Neonatal

Topics: Animals; Bile; Bilirubin; Cell Membrane; Fetus; Glucuronates; Glucuronosyltransferase; Glutathione Transferase; Humans; Infant, Newborn; Jaundice, Neonatal; Liver; Phenobarbital; Serum Albumin; Sulfonamides; Vitamin K

Bilirubin conjugates in the serum of cholestatic pediatric patients were investigated with Micronex high-performance liquid chromatography. Serum bilirubin was resolved into four fractions: delta bilirubin (Bd), bilirubin diglucuronide (BDG), bilirubin monoglucuronide (BMG), and unconjugated bilirubin (Bu). The conjugated bilirubin (BDG+BMG) fraction in preoperative patients with biliary atresia (BA) was 48.8 +/- 5.1%, which was significantly higher than that in patients with infantile hepatitis (P < .01). Among postoperative BA patients who recovered from jaundice, the Bd fraction increased during the first month, remained elevated (60% to 80%) for a while, and then gradually decreased. After 6 months, the Bd fraction decreased to 30% in the jaundice-free survivors, but was still higher than that in controls (7%). Even after the total serum bilirubin had normalized (< or = 1.0 mg/dL), distribution of bilirubin fractions remained abnormal, possibly reflecting impaired hepatic excretion of bilirubin. In conclusion, measurement of the conjugated bilirubin fraction enabled BA to be differentiated from infantile hepatitis, and the delta bilirubin fraction proved to be an important indicator of cholestasis in postoperative BA patients with normal serum bilirubin.

Topics: Biliary Atresia; Bilirubin; Child, Preschool; Cholestasis; Chromatography, High Pressure Liquid; Diagnosis, Differential; Hepatitis; Humans; Infant, Newborn; Jaundice, Neonatal; Postoperative Complications

Serum bilirubin fractions were determined by a newly developed, high performance liquid chromatography method in 23 healthy subjects, and in 15 patients with Gilbert's syndrome, five with type 2 Crigler-Najjar syndrome, seven with hemolytic disorders, and 26 with neonatal jaundice. In the healthy subjects, 92.6% of the bilirubin was unconjugated (UCB), 6.2% was bilirubin monoglucoronide (BMG), and 0.5% was bilirubin diglucuronide (BDG). Delta bilirubin (B delta) was not detected. In the patients, the percentage of UCB was significantly higher and that of BMG was significantly lower than in the healthy subjects. The proportion of BDG tended to decrease in Gilbert's and type 2 Crigler-Najjar syndromes; the proportion of B delta tended to increase in hyperbilirubinemia, except in Gilbert's syndrome. In particular, B delta was frequently detected in serum which also contained BDG (mainly in hemolytic disorders) or which presented with high concentrations (above 100 mumol/L) of UCB (mainly in type 2 Crigler-Najjar syndrome and neonatal jaundice). Trace amounts of (Z,E)- and/or (E,Z)-UCB were detected in approximately one fourth of the serum samples analyzed.

Topics: Bilirubin; Chromatography, High Pressure Liquid; Crigler-Najjar Syndrome; Female; Gilbert Disease; Humans; Hyperbilirubinemia; Infant, Newborn; Jaundice, Neonatal; Male