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At least 19 recordsLinked to original sources
PubMed1991

The differential diagnosis of Crigler-Najjar disease, types 1 and 2, by bile pigment analysis.

Phenobarbital response, bile pigment composition, and the fractional biliary excretion ratio of bilirubin were studied in nine children with Crigler-Najjar disease. In five children, serum bilirubin levels decreased during phenobarbital treatment by 26% or more and the pigment composition in bile changed with a decrease in the proportion of unconjugated bilirubin from 33% +/- 12% to 13% +/- 1% and an increase in monoconjugates and diconjugates from 57% +/- 14% and 10% +/- 2%, respectively, to 72% +/- 4% and 16% +/- 3%. In four children, serum bilirubin levels did not change significantly during phenobarbital treatment. In these patients, bile pigments comprised 91% +/- 10% unconjugated bilirubin, 9% +/- 11% monoconjugates, and 1% +/- 1% diconjugates. On the basis of these differences, the former group can be classified as having type 2 Crigler-Najjar disease and the latter, type 1. Bile pigment analysis in parents of patients with Crigler-Najjar disease showed an increased proportion of monoconjugates in at least one of the partners in three of four couples tested, despite normal serum bilirubin levels. Serum bilirubin levels were about the same in type 1 and 2 patients and amounted to 236 +/- 62 mumol/L and 214 +/- 82 mumol/L, respectively. In addition the fractional bilirubin excretion ratio, calculated as the ratio ([bilirubin in bile]/[bilirubin in serum])/([bile acid in bile]/[bile acid in serum]) could not differentiate between these two groups. However, there was a 10-fold and 100-fold difference of this ratio between patients with Crigler-Najjar disease and those with Gilbert's syndrome and between patients with Crigler-Najjar disease and controls. The fractional bilirubin excretion ratio proved an excellent tool to differentiate between Gilbert's syndrome and Crigler-Najjar disease, whereas Crigler-Najjar disease types 1 and 2 could be differentiated on the basis of bile pigment analysis.

Bile↗
PubMed1993

Anaerobic breakdown of uroporphyrins I and III to bile pigments by extracts of Clostridium tetanomorphum.

Two blue bile pigments were formed under anaerobic conditions from the tetrapyrrole precursor delta-aminolevulinate by cells and cell extracts of Clostridium tetanomorphum. These compounds were also formed by cell extracts from the octacarboxylic tetrapyrrole, uroporphyrin III. Bactobilin, the first bacterial bile pigment to be discovered, is related to uroporphyrin I. The present results hence increase the number of bile pigments related to bactobilin. Bactobilin and its isomers differ markedly from the eukaryotic bile pigments which are all related to the dicarboxylic compound, protoporphyrin IX. The enzyme participating in the formation of the bacterial bile pigments was obligatorily anaerobic, in decided contrast to the only other known bile pigment-forming enzyme, the eukaryotic oxygen-requiring heme oxygenase.

Aminolevulinic Acid↗
PubMed1983

Bactobilin: blue bile pigment isolated from Clostridium tetanomorphum.

A blue bile pigment, possessing four acetic and four propionic acid side chains has been isolated from extracts of the anaerobic microorganism Clostridium tetanomorphum and in smaller amounts from Propionibacterium shermanii. The compound could be prepared in larger amounts by incubation of C. tetanomorphum enzyme extracts with added delta-aminolevulinic acid. The ultraviolet-visible, infrared, and proton magnetic resonance spectra of the pigment indicate a chromophore of the biliverdin type. Field-desorption mass spectrometry of the purified methyl ester showed a strong molecular ion at m/e = 962. This corresponds to the molecular weight expected for the octamethyl ester of a bilatriene type of bile pigment structurally derived from uroporphyrin III or I. Of the five possible structures, two could be eliminated by proton magnetic resonance spectroscopy. The name bactobilin is proposed for this previously unreported bile pigment.

Clostridium↗
PubMed1998

Intraepidermal bile pigment in skin biopsy specimens for graft-versus-host disease versus erythema multiforme.

The differentiation of graft-verus-host disease (GVHD) from erythema multiforme (EM) presents a common diagnostic challenge in skin biopsy specimens from patients who have received patients allogeneic bone marrow transplants. The presence of gastrointestinal involvement might be the only way to make a diagnosis of GVHD in these cases. In the absence of liver function tests, gastrointestinal biopsy, or molecular techniques such as microsatellite DNA analysis, the presence of intraepidermal bile pigment might prove helpful in elucidating hyperbilirubinemia and allowing a pathologist to favor a diagnosis of GVHD over EM. Routinely processed archival tissue from 50 cases of GVHD (42 Caucasian and 8 of unknown race) and 50 cases of EM (31 Caucasian and 19 of unknown race) was examined for pigmentation. Intraepidermal pigmentation was stained for bile pigment and melanin. Among the intraepidermal EM lesions, 4 (8%) stained for intracorneal melanin, but none stained for bile pigment. Among the intraepidermal GVHD lesions, 8 (16%) stained for intracorneal melanin, but 3 (6%) stained for intracorneal bile pigment. In addition, 13 (26%) GVHD lesions and 9 (18%) EM lesions showed melanosis with melanin in all layers of the epidermis as well as within papillary dermal melanophages. Thus, when presented with a differential diagnosis of GVHD versus EM, the presence of intraepidermal bile pigment might suggest liver involvement and a diagnosis of GVHD.

Bile Pigments↗
PubMed1992

Bile pigments in gallbladder and freshly-secreted hepatic duct bile from fed and fasted rainbow trout, Oncorhynchus mykiss.

1. Chromatographic analyses of bile pigments in rainbow trout reveal the presence of primarily unconjugated biliverdin (BV) and bilirubin (BR) glycosyl conjugates. Only trace amounts of unconjugated BR are present in hepatic duct (HD) bile: no beta-glucuronidase activity is detectable. 2. The per cent of BV and BR in HD and gallbladder biles is similar in fasted trout; however, the per cent of BV is significantly increased in HD bile from fed fish. 3. Fasting decreases the rate of choleresis but does not alter the excretory rate of endogenous BV or BR. 4. Erythrocyte life span is estimated to be approximately 500 days.

Animals↗
PubMed1991

Bile pigments in fishes: a review.

Gallbladder (GB) bile of most cyclostomes, elasmobranchs, and teleosts contains appreciable amounts of biliverdin (BV) and bilirubin (BR) conjugates with lesser amounts of unconjugated BR in certain species. Certain elasmobranch and teleost species have been reported to have primarily BV or BR in bile. The appearance of the enzyme BV reductase, which converts BV to BR in mammals, evolved quite early in the evolution of vertebrate species; however, exceptions exist in certain fishes, amphibians, reptiles, and mammals whose bile contains primarily BV. Nearly all analytical studies on bile pigment composition in fishes to date have utilized only GB bile, which may or may not always be representative of the pigments excreted in freshly collected hepatic duct bile. The concentration of BV and BR in GB bile of fishes increases markedly during prolonged fasts. From the limited data currently available, there appears to be no systematic development from primitive to advanced forms in the appearance of certain bile pigments in fishes. While bile of most aquatic species contains appreciable amounts of both BV and BR, it is interesting that the bile of most terrestrial avian and reptilian forms contains primarily BV. The serum of fishes, except for certain bony species such as eels (Anguilleformes) and cottids (Scorpaeniformes), is a light yellow color due to the presence of BR and is similar to that observed in higher vertebrates. Serum from certain eels and cottids is bluish green in color due to the presence of a variety of chromoproteins that contain BV firmly bound as the prosthetic group.

Journal Article↗
PubMed1980

Hepatic bile pigment excretion and erythrocyte turnover in various species.

Endogenous bile pigment excretory rates have now been recorded in fifteen species, and the following review briefly compares the excretion per unit of body weight in animals of varying sizes, attempts to determine if any correlation exists between bile flow and endogenous pigment excretion and discusses bile pigment excretion in relation to mean erythrocyte survival time and metabolic rate. Graphs and one table illustrating pigment metabolism and erythrocyte replacement are presented. Differences between animals excreting primarily biliverdin as opposed to bilirubin are discussed, with special attention being given throughout the article to mammals and their individual differences. Accepted theory concludes that the rate of endogenous bile pigment excretion is primarily dependent upon hemoglobin catabolism. However, further studies will be necessary to clarify basic physiological mechanisms in order to substantiate any theory concerning the correlation of erythrocyte turnover with metabolic rates.

Journal Article↗
PubMed1983

Influence of bile salts on the endogenous excretion of bile pigments.

Effect of the infusion of glycodeoxycholate (GDC), taurocholate (TC) and dehydrocholate (DHC) on bile flow and on bile salt, biliary lipid and bile pigment secretion, has been studied in pentobarbital-anesthetized rabbits. GDC increased bile flow the most, while DHC increased it more than TC. The different choleretic actions of these bile salts cannot be explained by means of variations in their capacity to form micelles. Only GDC and TC were able to stimulate biliary lipid secretion, which suggests that both bile salts increase the formation of mixed micelles. GDC and TC to a lesser extent increased bile pigment excretion, DHC being without effect. These results favour the hypothesis that micellar binding could be an important factor responsible for the effect of bile acids on bile pigment excretion and should not be completely ruled out.

Animals↗