Biomaterial Database

Biliary excretion of conjugated sulfobromophthalein (BSP) in constitutional conjugated hyperbilirubinemias. 1975

H Abe, and K Okuda

Sulfobromophthalein (BSP) and its three major forms of conjugates were determined in bile or duodenal aspitate, plasma and urine following intravenous administration of free BSP and synthetic BSP-glutathione (BSP-GSH) in five patients with the Dubin-Johnson syndrome and two patients with the Rotor syndrome, using alumina column chromatography. It was found that in Dubin-Johnson patients the biliary excretion of conjugated BSP was selectively impaired, conjugated BSP increased in plasma replacing free BSP after 30 min, and plasma retention of BSP-GSH was greater than that of free BSP when administered intravenously. In contrast, biliary excretion of BSP and its conjugates was not impaired and regurgitation of conjugated BSP into plasma was minimal in the Rotor syndrome. Thus, these two constitutional hyperbilirubinemias can be separated by their basic defects in BSP metabolism.

UI	MeSH Term	Description	Entries
D007566	Jaundice, Chronic Idiopathic	A benign, autosomally recessive inherited hyperbilirubinemia characterized by the presence of a dark pigment in the centrilobular region of the liver cells. There is a functional defect in biliary excretion of bilirubin, cholephilic dyes, and porphyrins. Affected persons may be asymptomatic or have vague constitutional or gastrointestinal symptoms. The liver may be slightly enlarged, and oral and intravenous cholangiography fails to visualize the biliary tract.	Dubin-Johnson Syndrome,Hyperbilirubinemia 2,Hyperbilirubinemia II,Chronic Idiopathic Jaundice,Chronic Idiopathic Jaundices,Dubin Johnson Syndrome,Hyperbilirubinemia 2s,Hyperbilirubinemia IIs,Idiopathic Jaundice, Chronic,Idiopathic Jaundices, Chronic,Jaundices, Chronic Idiopathic,Syndrome, Dubin-Johnson
D008099	Liver	A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.	Livers
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D005260	Female		Females
D005978	Glutathione	A tripeptide with many roles in cells. It conjugates to drugs to make them more soluble for excretion, is a cofactor for some enzymes, is involved in protein disulfide bond rearrangement and reduces peroxides.	Reduced Glutathione,gamma-L-Glu-L-Cys-Gly,gamma-L-Glutamyl-L-Cysteinylglycine,Glutathione, Reduced,gamma L Glu L Cys Gly,gamma L Glutamyl L Cysteinylglycine
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006933	Hyperbilirubinemia, Hereditary	Inborn errors of bilirubin metabolism resulting in excessive amounts of bilirubin in the circulating blood, either because of increased bilirubin production or because of delayed clearance of bilirubin from the blood.	Rotor Syndrome,Hyperbilirubinemia, Rotor Type,Hereditary Hyperbilirubinemia,Hereditary Hyperbilirubinemias,Hyperbilirubinemias, Hereditary,Rotor Type Hyperbilirubinemia,Syndrome, Rotor
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults