Diagnosis Of Congenital Biliary Atresia Research Articles

Prenatal diagnosis with chromosome microarray and pregnancy outcomes of fetuses with biliary tract system abnormalities

To investigate the prenatal diagnostic value of chromosome microarray analysis (CMA) in fetuses presenting with ultrasound-based biliary tract system (BTS) anomalies. Amniocentesis was performed and CMA was applied in 271 pregnant women carrying fetuses with BTS abnormalities between April 2015 and December 2020. Pregnancy outcomes and fetal prognosis were followed from 1 to 6years. Sixteen cases (5.9%, 16/271) of chromosomal anomalies were detected. The detection rate of chromosomal abnormalities was significantly higher for fetuses with nonisolated BTS anomalies than for those with isolated BTS anomalies (9.0% vs. 0%, p=0.0017). Follow-up results were obtained from 267 fetuses, including 25 cases of termination of pregnancy (9.4%), 237 live births (88.8%), and 5 (1.9%) neonatal demises. The incidence of congenital biliary atresia in the small gallbladder and nonvisualized gallbladder groups was 3.0% (1/33) and 9.5% (7/74), respectively; however, none was detected on postnatal ultrasound reexamination in the gallbladder enlargement or the other BTS groups. An isolated BTS abnormality is not an indication for invasive prenatal chromosomal analysis. When combined with other ultrasonographic abnormalities, prenatal CMA should be provided. When a small or nonvisualized gallbladder is found prenatally, ultrasonography is limited in the differential diagnosis of congenital biliary atresia.

Diagnostic value of HA, PC-III, IV-C, and LN in infants with congenital biliary atresia.

Hyaluronic acid (HA), type III procollagen III (PC-III), type IV collagen IV (IV-C), and laminin (LN) have certain diagnostic value for hepatobiliary diseases. No published studies have compared the diagnostic accuracy of these 4 indicators for the diagnosis of congenital biliary atresia (CBA) in infants. This study aimed to investigate the diagnostic value of HA, PC-III, IV-C, and LN in infants with CBA.From January 2017 to December 2020, 185 infants with nonphysiological jaundice in the Second Department of General Surgery at the Children’s Hospital of Hebei were enrolled in this study. Forty-six infants with CBA (CBA group) and 139 infants without CBA (noncongenital biliary atresia group) were diagnosed using ultrasonography, magnetic resonance imaging, intraoperative exploration, and intraoperative cholangiography. The levels of HA, PC-III, IV-C, and LN in the 2 groups were statistically analyzed. The diagnostic accuracy was determined using receiver operating characteristic curves and by calculating the area under the curve. Univariate and multivariate logistic regression analyses were performed to identify the independent risk factors.Compared to the noncongenital biliary atresia group, the levels of HA, PC-III, IV-C, and LN were significantly increased in the CBA group (P <.05). The receiver operating characteristic analysis showed the optimal cutoff values for HA, PC-III, IV-C, and LN were 162.7, 42.5, 199.7, and 101.2 ng/mL, and the area under the curves were 0.892, 0.762, 0.804, and 0.768, respectively. The sensitivity values for the diagnosis of CBA were 76.82%, 71.61%, 70.32%, and 72.28%, and the specificity values for the diagnosis of biliary atresia were 70.22%, 70.44%, 66.34%, and 68.71%, respectively. In the multivariate model, HA ≥162.7 ng/mL (odds ratio [OR] = 5.28, 95% confidence interval [CI]: 3.15–8.37), PC-III ≥42.5 ng/mL (OR = 4.61, 95% CI: 2.54–7.16), IV-C ≥199.7 ng/mL (OR = 5.02, 95% CI: 2.98–7.64), and LN ≥101.2 ng/mL (OR = 6.25, 95% CI: 2.41–10.07) remained associated with the occurrence of CBA.HA, PC-III, IV-C, and LN have high accuracy for the diagnosis of CBA in infants, and these factors are potential diagnostic biomarkers for CBA.

Role of a liver pathology standardized scoring system in the diagnosis of congenital biliary atresia and its relationship with prognosis

Objective: To evaluate the diagnostic value of a histologic scoring system in congenital biliary atresia and its prognostic relevance. Methods: From January 2017 to June 2018 at Children's Hospital of Fudan University, 172 wedge liver biopsy specimens were obtained from infants with neonatal cholestasis [119 patients with congenital biliary atresia (CBA) and 53 patients with non-obstructive cholestasis as control]. A pathologist, single-blinded to the final diagnosis, made the histological diagnosis individually based on an 8-feature (portal ductal proliferation, bile duct reaction, bile plugs in portal ductules, liver fibrosis, edema in portal region, cholestasis, inflammatory cells infiltration in portal region, and ductal plate malformation), 21-point scoring system. Results: The main pathologic changes of biliary atresia were hepatocyte cholestasis, hyperplasia of bile ducts, fibrosis and infiltration of inflammatory cells in the portal area. There were significant difference in the degree of portal edema, bile duct hyperplasia and fibrosis between two groups (P<0.01). In addition, there were characteristic bile duct thrombosis in 97.5%(116/119) of the cases and abnormal development of bile duct plate in 9.2%(11/119) of the cases. Compared with non-CBA infant cholestasis group, the difference was statistically significant (P<0.05). The scoring system has high sensitivity, specificity (both 94.1%) and accuracy (94.3%) in the diagnosis of CBA. A score equal to or more than 11 points supported a diagnosis of CBA; whereas a score less than 11 points might suggest cholestasis. The degree of hepatic fibrosis and ductal plate malformation were related to prognosis. Conclusions: The liver pathology scoring system (8-feature, 21-point) is more accurate in diagnosing CBA than previous methods, which may guide the clinicopathological diagnosis. This histological scoring system also helps to assess the prognosis of CBA.

Clinical value of hepatobiliary imaging at 6 h combined with serum ALP/.GAMMA.-GT ratio in the diagnosis of biliary atresia

Objective To investigate the clinical value of hepatobiliary imaging at 6 h combined with serum ALP/γ-GT ratio in the diagnosis of congenital biliary atresia (BA). Methods From March 2013 to June 2014, 187 infants (111 males, 76 females, mean age 58.9 d ) who were admitted to the Children′s Hospital of Jiangxi Province were retrospectively analyzed for persistent jaundice more than two weeks, deranged liver function and hepatosplenomegaly. They were diagnosed pathologically as BA (118 cases) and infant hepatitis syndrome (IHS, 69 cases). Patients underwent 99Tcm-EHIDA hepatobiliary imaging after treatment with ursodeoxycholic acid and other medications for 7-10 d. The serum γ-GT, ALP and ALP/γ-GT ratio between two groups were analyzed by two-sample t test and ROC curve. The diagnostic efficiency of BA by hepatobiliary imaging at 6 h, serum γ-GT content, ALP/γ-GT ratio and combined use of hepatobiliary imaging at 6 h and serum ALP/γ-GT ratio were compared using χ2 test. Results Serum γ-GT, ALP and ALP/γ-GT ratio between the two groups were all significantly different (t=8.694, 7.233, 2.635, all P 0.05). Conclusions The diagnostic value of combined use of hepatobiliary imaging at 6 h and serum ALP/γ-GT ratio is significantly higher than that of hepatobiliary imaging alone. It is a simple, cost-effective, safe and clinically practical diagnostic tool for BA. Key words: Biliary atresia; Hepatitis; Infant; Radionuclide imaging; Alkaline phosphatase; Gamma-glutamyltransferase; EHIDA

Effect of different time phases of radionuclide hepatobiliary scintigraphy on the differential diagnosis of congenital biliary atresia

To investigate the value and essentiality of 6- and 24-h delay hepatobiliary scintigraphy in the differential diagnosis of biliary atresia (BA), we retrospectively analyzed 197 infants (121 boys/76 girls; age range, 3-205 days; average age, 63.9 days) admitted to Jiangxi Children's Hospital for persistent jaundice (> 2 weeks), hepatosplenomegaly, and abnormal liver function. After receiving anti-inflammatory treatment and cholagogic pre-treatment for 7-10 days without a clear diagnosis, the children underwent 99mTc-labeled diethylacetanilide-iminodiacetic acid hepatobiliary scintigraphy. BA and infant hepatitis syndrome were diagnosed in 107 and 90 infants, respectively after laparoscopic cholangiography, surgical pathology, or 6-month clinical follow-up. The diagnostic efficiencies of hepatobiliary scintigraphy for BA were evaluated within 50 min and at 6 and 24 h. The areas under the receiver operating characteristic curves within 50 min, at 6 and 24 h were 0.696, 0.829 , and 0.779 , suggesting poor diagnostic value within 50 min, but improvement at 6 and 24 h. The compliance rate of 6- and 24-h imaging for BA diagnosis was 89.34% (176/197; paired chi-square test Kappa value, 0.77; P > 0.05), signifying high consistency. The diagnostic efficiency values of 6-/24-h imaging for BA diagnosis were sensitivity (90.65/89.72%), specificity (74.44/78.89%), accuracy (83.25/84.77%), positive and negative predictive values (80.83/83.48% and 87.01/86.59%), with no significant difference (P > 0.05). To provide optimal treatment in early BA, the- 6-h hepatobiliary scintigraphy had practical value, especially when combined with tomographic or dynamic imaging; 24-h delay imaging was deemed unnecessary because it was not significantly superior.

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.

Ultrasonography (US) in Diagnosis of Congenital Biliary Atresia (CBA)

Ultrasonography (US) in Diagnosis of Congenital Biliary Atresia (CBA)

Congenital biliary atresia in a cat: a case report

ABSTRACTA case of congenital biliary atresia in a six‐month‐old cat is presented. The cat had recurrent bouts of depression, anorexia, vomiting and pyrexia, since it was acquired as a three‐month‐old kitten. Physical examination showed the cat to be jaundiced. Abdominal palpation revealed several smooth, round masses, confluent with the liver in the right cranial quadrant. A tentative diagnosis of congenital biliary atresia was made based on history, clinical and laboratory examination and radiography. This was confirmed at necropsy.

Duodenal bile acids in diagnosis of congenital biliary atresia

This diagnostic study was useful in distinguishing between complete and partial biliary obstruction in a small number of cases. It has been used widely in Japan and is published here to bring it to the attention of other investigators.

Diagnosis of congenital biliary atresia by duodenal tubing : S. Hashimoto et al. J Jpn Soc Pediatr Surg 14:889–892, (October), 1978

Diagnosis of congenital biliary atresia by duodenal tubing : S. Hashimoto et al. J Jpn Soc Pediatr Surg 14:889–892, (October), 1978

A radiopharmaceutical in the diagnosis of congenital biliary atresia

Diethyl-HIDA, a new hepatobiliary radiopharmaceutical labeled with 99mTc, was used in the preoperative diagnosis of biliary atresia and in the postoperative evaluation of hepatic-porto-enterostomy function. The good results obtained in the four first cases suggest extensive use of the method, with the aim of a more precocious and more attendable diagnosis.