Congenital Biliary Atresia Research Articles

Determination of fetal bile acids and related steroidal compounds and their profile in neonatal biological fluids

The unusual bile acids hydroxylated at 1 beta-, 2 beta-, 4 beta-, 6 alpha- and 19-positions of cholic and chenodeoxycholic acids have been found from the meconium, neonatal bile, blood and urine, and amniotic fluid and pregnant urine by GC-MS analysis. These hydroxylated bile acids and their conjugates were synthesized as their references from the corresponding usual bile acids as starting materials, and the simultaneous and high performance analytical methods were developed by GC-MS, HPLC and enzyme immunoassay. The above mentioned unusual bile acids were identified and determined in significant amounts of the total bile acids in the biological fluids from neonates and pregnant women, but not from normal adults. We, therefore, proposed that they should be called as "fetal bile acids". Application of the developed methods was performed for the studies on the dynamic profile of fetal bile acids in developing fetus and neonates, and the clinical diagnosis of the hepatobiliary diseases of infants and congenital bile acid biosynthetic disorders, Zellweger syndrome, celebrotendinus xanthomatosis, 3-oxo-delta 4-steroid 5 beta-reductase deficiency and congenital biliary atresia. Analyses of steroidal hormones, equine estrogens and 18-hydroxycortisol were also described.

Peri-ductal mesenchymal cells in hepatic tissue of congenital biliary atresia

Peri-ductal mesenchymal cells in hepatic tissue of congenital biliary atresia

Evaluation of antimicrobial system of polymorphonuclear leukocytes and opsonic activity in patients with congenital biliary atresia : M. Kuroiwa, S. Matsuyama, and Y. Nagamachi. J Jpn Soc Pediatr Surg 29:159–164, (February), 1993

Evaluation of antimicrobial system of polymorphonuclear leukocytes and opsonic activity in patients with congenital biliary atresia : M. Kuroiwa, S. Matsuyama, and Y. Nagamachi. J Jpn Soc Pediatr Surg 29:159–164, (February), 1993

Cutaneous Porphyrialike Photosensitivity After Liver Transplantation

Liver disease and cholestasis are often associated with abnormal coproporphyrin levels, but photosensitivity eruptions do not usually occur with these conditions. We present a case of a cutaneous porphyrialike photosensitivity in a liver transplant patient with only mildly elevated coproporphyrin levels. A 5-year-old girl developed a cutaneous porphyria cutanea tarda-like photosensitivity after liver transplantation for congenital biliary atresia. Her cutaneous eruption consisted of fluid-filled vesicles and crusted erosions involving her face and arms. These lesions eventually healed with atrophic scarring and milia. The patient was in a state of chronic hepatic rejection, but her serum and 24-hour urine specimens contained only mildly elevated levels of coproporphyrin. Results of histopathologic examination and direct immunofluorescence of a skin biopsy specimen resembled those of porphyria. Although clinically porphyria cutanea tarda was suspected, her porphyrin profile was not consistent with that diagnosis. Liver transplantation has become relatively common, but porphyria cutanea tarda-like clinical and histopathologic findings associated with coproporphyrinemia in a liver transplant patient have not been reported to date.

Composition of Serum and Erythrocyte Membrane Lipids in Patients with Congenital Biliary Atresia

Composition of Serum and Erythrocyte Membrane Lipids in Patients with Congenital Biliary Atresia

NUMBER OF PATIENTS HAVING AN INDICATION FOR LIVER TRANSPLANTATION IN GUNMA PREFECTURE

This study was designed to assess the need for liver transplantation in Gunma prefecture. To estimate the number of patients who died of end-stage liver disease, we investigated the Gunma version of vital statisitics in Japan from 1985 to 1990. The following result were obtained : Twenty six patients per year died of diseases that were absolute indications for liver transplantation ; these included alcoholic cirrhosis (8.2 persons on average), primary biliary cirrhosis (1.6), congenital biliary atresia (1.2) and acute hepatic failure (15.2). Three hundred and ninety patients per year died of disease that might have responded relatively well to liver transplantation ; these included viral related liver cirrhosis (228) and hepatic malignant neoplasm (162). In Gunma prefecture, the annual number of patients with an absolute indication for liver transplantation was 26 while those with a relative indication for liver transplantation numbered 390.

Radioimmunoassay of Conjugated 1.BETA.-Hydroxycholic Acid in Dried Blood Spots for Diagnosis of Congenital Biliary Atresia.

Radioimmunological measurement of conjugated 1 beta-hydroxycholic acid in dried blood spots obtained from newborns at 4-5 d after birth has been carried out to investigate a diagnostic utility for congenital biliary atresia. The method allowed the determination of this bile acid, with a detection limit of 50 pg in one disc (3 mm i.d.), and showed good reproducibility with inter- and intra-assay coefficients with variations of 3.5-7.7% and 6.3-8.8%. With the analysis, a relatively high concentration of this substance was observed in the patients, as compared to normal subjects, averaging 1.63 ng/disc in the former and 0.97 ng/disc in the latter.

Enzyme Immunoassay for Conjugated 1γ-Hydroxycholic Acid and Its Application to Dried Blood Spotted on Filter Paper

A highly sensitive enzyme immunoassay of conjugated 1γ-hydroxycholic acid in heterologous combination was developed using horse radish peroxidase labeled antigen. The assay method was optimized for quantitation in double antibody-coated microtiter plates. The assay had a lower limit of sensitivity of 12.5 pg/assay. The cross-reactivity for glyco 1γ-hydroxycholic acid was 80.13% and those for conjugated 1γ-hydroxychenodeoxycholic and 1γ-hydroxydeoxycholic acids ranged from 0.13 to 7,58%. The values in dried blood samples obtained by the present method correlated well with those in radioimmunoassay (r=0.964). Concentrations of conjugated 1γ-hydroxycholic acid in dried blood spotted onto filter paper in newborns with congenital biliary atresia (737±264 ng/ml) were significantly higher than those of normal newborns (261±190 ng/ml).

SERUM GH-BINDING PROTEIN BEFORE AND AFTER LIVER TRANSPLANTATION IN CHILDREN WITH CONGENITAL BILIARY ATRESIA

Changes of serum GH-binding protein (GHBP) levels in children with congenital biliary atresia were studied before and after living related liver transplantation (LT). Of 6 children, 3 showed growth retardation (less than −2 SDS of height) before LT. All of them were free from post-operative complication. Steroids were discontinued within the first 3 months. Blood samples were drawn before LT, 1, and 6 months after LT for determination of GHBP levels (ligand mediated immunofunctional assay) and plasma IGF-I levels (RIA, direct method). Preoperative GHBP levels were reduced for their age in 3 of the 6 patients, and IGF-I levels were less than 0.22 U/ml in all 6 children. No correlation was found neither between preoperative height SDS and preoperative GHBP level, nor between the former and preoperative IGF-I level. In the three children with decreased GHBP levels preoperatively, GHBP levels increased markedly a month after LT and stayed normal at 6 months. In the remaining 3 children with normal preoperative GHBP levels, two showed normal values throughout the observation period but one showed reduced serum GHBP level at 6 months after LT. Post-transplantation IGF-I levels were more than 10 times at one month and more than 4 times at 6 months compared with those of pre-transplantation in all but one patient. Height SDS evaluated at 6 months improved in 5 of 6 children. The patient who showed reduced GHBP level could not undergo postoperative catch-up growth in spite of the increased IGF-I level at 6 months. These findings suggest that the increase of GHBP and IGF-I after liver transplantation might reflect the regeneration of transplanted liver as well as the metabolic improvement. Long term observation would be necessary for evaluation of GHBP as an indicator of catch-up growth after liver transplantation.

A CASE OF BILIARY ATRESIA SUCCESSFULLY MANAGED BY ADMINISTRATION OF PROSTAGLANDIN E1 EARLY AFTER OPERATION

This paper describes a successful management of an operated infant with congenital biliary atresia by administering prostaglandin El immediately after Kasai's operation. The infant was a 19-day-old male. He had gray-white stool on the 10th day after birth. On the 19th day he was referred to the hospital because of yellow-stained conjunctiva. Based on closed examination biliary atresia was suspected and laparotomy was carried out on 49th day. Intraoperative findings and cholangiogram were compatible to biliary atresia and hepatic portoenteros-tomy was performed. For the purpose of promotion of bile outflow, the patient was applied to continuous administration of prostaglandin El intravenously. Serum total bilirubin levels (5.8mg/dl) decreased to 2.0mg/dl on the 15th postoperative day and no ascending cholangitis appeared. The patient was discharged from the hospital on 70th day and is strictly followed on an ambulant basis. Prostaglandin El, which is clinically used to preserve the liver, would be expected as a cholagogue in patients with biliary atresia after Kasai's operation.

Multiple pathologic fractures combined with congenital biliary atresia: Report of a case

Multiple pathologic fractures combined with congenital biliary atresia: Report of a case

Efficacy of prostaglandin E2 and ursodeoxycholic acid after portoenterostomy in the prognostically unfavorable type III biliary atresia

Nineteen patients with type III congenital biliary atresia (CBA) were treated by portoenterostomy. In the histological sections, bile ductule diameters below 100 μm and age-dependent liver fibrosis of grade II–IV were found. In group A, 8 patients received no postoperative choleretic therapy; in group B, 6 patients were treated with prostaglandin E2 (PG E2); and in group C, in 5 patients ursodeoxycholic acid (UDCA) was added to the PG E2 therapy. In the first 3 postoperative months a significant beneficial effect of PG E2 was observed as reduced serum bilirubin and aspartate aminotransferase (AST) levels relative to the untreated group. In group C (UDCA and PG E2) significantly lower serum levels of all liver parameters relative to the untreated group and gradually decreased levels of bilirubin, alanine aminotransferase (ALT), g-glutamyl transferase (GGT), and higher cholinesterase (CHE) activity were observed than in the PG E2 group. During episodes of cholangitis steroids were also added, but alterations in serum bilirubin levels, CHE activity, and bile flow did not improve after any adjunctive therapy. Liver enzyme activity was immediately elevated in groups A and B, but significantly lower in group C. The efficacy of both drugs, PG E2 and UDCA, lasted only for the first 3 post-operative months and failed to improve or delay the progression of hepatic damage. After portoenterostomy, 13 patients (68%), died within 6–32 months, 12 from progressive liver failure and 1 after liver transplantation. Six patients (32%) are alive, 3 after liver transplantation; 2 are jaundice-free and 1 jaundiced patient is on a waiting list for transplantation. In these prognostically poor patients with type III CBA, early liver transplantation is the only feasible option as adjuvant Pg E2, UDCA, and corticosteroid therapy demonstrated no long-term improvement after portoenterostomy.

Laminin and procollagen-III-peptide as a serum marker for hepatic fibrosis in congenital biliary atresia

The extracellular matrix (ECM) is a complex of macromolecules that includes collagens, proteoglycans, and complex glycoproteins. In fibrotic liver tissue there is an increase in all of these matrix components, and they increase in serum in the patients with alcoholic hepatitis or liver cirrhosis. These ECM components have been used as a serum marker of hepatic fibrosis. Prolonged obstruction of bile flow results in morphologic and biochemical changes and the development of secondary biliary cirrhosis. In congenital biliary atresia (CBA) there is a close correlation between the degree of the hepatic fibrosis and bile flow after the operation. We estimated that, in CBA, ECM increased in serum, and it would reflect the degree of the hepatic fibrosis. To clarify this we examined the serum procollagen-III-peptide (P-III-P) and laminin in CBA patients. P-III-P was elevated in all preoperative patients but in two of the three postoperative patients whose jaundice disappeared P-III-P was in the normal range. In the all 3 patients whose jaundice continued, P-III-P was in normal range. Serum laminin was elevated in 12 preoperative patients with CBA, but there is no correlation between day of diagnosis and level of laminin. Mean concentration in CBA without jaundice after operation was 3.18 U/mL, 3.226 U/mL in CBA with jaundice and 3.3 U/mL in infantile hepatitis. There were no significant differences among three groups. With the elevation of serum alanine aminotransferase, aspartate aminotransferase, and total bilirubin, serum laminin level was also increased.(ABSTRACT TRUNCATED AT 250 WORDS)

先天性胆道閉鎖症(I Cyst型)術後に肝内結石を併発した1例

先天性胆道閉鎖症(I Cyst型)術後に肝内結石を併発した1例

Operation on Patients with Congenital Biliary Atresia in the Neonatal Period.

Operation on Patients with Congenital Biliary Atresia in the Neonatal Period.

A Complication following Endoscopic Injection Sclerotherapy for Esophageal Varices in Postoperative Congenital Biliary Atresia : A Case of Portal Vein Thrombosis

A Complication following Endoscopic Injection Sclerotherapy for Esophageal Varices in Postoperative Congenital Biliary Atresia : A Case of Portal Vein Thrombosis

Congenital biliary atresia and congenital biliary dilatation in siblings

Congenital biliary atresia or congenital biliary dilatation in siblings have been reported, although the genetic influences in these diseases are considered to be obscure. We report a case of congenital biliary atresia and congenital biliary dilatation in sisters.

Coincidence of Congenital Toxoplasmosis and Biliary Atresia in an Infant

Summary: A 4‐week‐old infant presenting with neonatal cholestasis was found to have congenital toxoplasmosis and biliary atresia. This is the first patient in which their coincidence is reported. Because biliary atresia can coexist with either congenital infection or inborn errors of metabolism, evaluation for an obstructive etiology of jaundice in infants with a recognized cause of intrahepatic cholestasis is necessary.

Coincidence of Congenital Toxoplasmosis and Biliary Atresia in an Infant

A 4-week-old infant presenting with neonatal cholestasis was found to have congenital toxoplasmosis and biliary atresia. This is the first patient in which their coincidence is reported. Because biliary atresia can coexist with either congenital infection or inborn errors of metabolism, evaluation for an obstructive etiology of jaundice in infants with a recognized cause of intrahepatic cholestasis is necessary.

Endoscopic Sclerotherapy for Esophageal Varices After Surgical Procedures for Congenital Biliary Atresia

Abstract: The effectiveness of injection sclerotherapy for esophageal varices due to congenital biliary atresia has not yet been established. Sclerotherapy was performed to treat esophageal varices in four children with variceal bleeding who had received a hepatic portoenterostomy for congenital biliary atresia. Ethanolamine oleate was mixed with a contrast medium for the varicealography in order to determine the flow of the sclerosant by fluoroscopy. Varicealography which was conducted during the injection allowed us to stop the procedure so that mixture would fill up the varix and its feeders but would not enter the systemic circulation. Between 0.5 ml to 2.0 ml of sclerosant was injected at the variceal puncture. There was one case bleeding from the esophageal ulcer. However, it was resolved by conservative treatment. All four children experienced no rebleeding after the therapy. Therefore, injection sclerotherapy using varicealography with sclerosant–contrast medium mixture is recommended for children who develop esophageal varices after surgical procedures for congenital biliary atresia.