Humans ; Infant ; Biliary Atresia/diagnosis* ; Choledochal Cyst/diagnosis* ; RNA, Circular ; Diagnosis, Differential

PURPOSE: Ascending cholangitis is the most common complication after Kasai operations. The aim of this study is to evaluate the therapeutic efficacy of cefotaxime as an empirical antibiotic on ascending cholangitis after Kasai operations. METHODS: Thirty-nine episodes of cholangitis in twenty-nine children who underwent Kasai operations at Seoul National University Children's Hospital from January 1991 to December 2000 were included in this study. Empirical cefotaxime treatments were divided into three groups: cefotaxime and amikacin treatment group(CA group), cefotaxime and gentamicin treatment group(CG group) and cefotaxime treatment group(C group). A diagnosis of cholangitis was made on the basis of unexplained fever(>38degrees C) and either development of acholic stool or elevation of serum total bilirubin (>1.5 mg/dL). Therapeutic efficacy was judged by elimination of fever up to 72 hours, 120 hours, and 168 hours after antibiotic treatment. RESULTS: There were therapeutic responses in 51%(20/39) up to 72 hours after antibiotic treatment : 54%(13/24) in CA group, 43%(3/7) in CG group and 50%(4/8) in C group. There were therapeutic responses in 69%(27/39) up to 120 hours, in 79%(31/39) up to 168 hours and in 82%(32/ 39) up to 2 weeks. There were no differences in therapeutic efficacy among the three regimens. In 12 of 39 episodes, the etiologic pathogens including Escherichia coli and enterococcus were cultured from the blood. CONCLUSION: Cefotaxime can be tried as an initial antibiotic in Korean children with ascending cholangitis after Kasai operation prior to the identification of microorganism on culture. However, further evaluation of pathogen and its resistant strain to cefotaxime should be done.
Amikacin ; Biliary Atresia* ; Bilirubin ; Cefotaxime* ; Child ; Cholangitis* ; Diagnosis ; Enterococcus ; Escherichia coli ; Fever ; Gentamicins ; Humans ; Seoul

This paper includes our 9-year experience of 34 infants with biliary atresia with introduction of a new non-invasive diagnostic method, that is, ultrasonographic "triangular cord" (TC) sign. TC sign was defined as visualization of a triangular or a band-like echogenicity just cranial to the portal vein. Ultrasonographic TC sign seemed to be a simple, non-invasive, time-saving and useful tool in the diagnosis of biliary atresia, representing 84% sensitivity. Active bile excretion was restored in 90% of the patients who were treated between 31-60days, 78% of those between 61-90 days, and 33% of those being 91days or older. The incidence of postoperative cholangitis was 36%, and construction of antireflux valve in the Roux-en -Y loop did not affect the incidence of postoperative cholangitis (P=0.18). As for the surgical outcome, of 34 infants with biliary atresia, 23 (68%) are alive for 2-102 months period, and 12 of them are alive for more than 5 years . Five-year estimate survival by Kaplan-Meier method was 66 %.
Bile ; Biliary Atresia* ; Cholangitis ; Diagnosis* ; Humans ; Incidence ; Infant ; Portal Vein ; Ultrasonography

PURPOSE: The most common causes of neonatal cholestasis are neonatal hepatitis (NH) and extrahepatic biliary atresia (EHBA). Since neonatal cholestasis presents with variable expression of same pathologic process and has similar clinical, biochemical, and histologic features between EHBA and idiopathic neonatal hepatitis (NH), differential diagnosis is often difficult. We reviewed the differences of clinical characteristics and laboratory data to find out any correlation between the results of Tc(99m) DISIDA scan and presence of acholic stool. METHODS: Between June 1993 and January 2001, total 29 infants younger than 4 month-old underwent Tc(99m) DISIDA scan. Their biochemical tests and clinical course were reviewed retrospectively. RESULTS: Patients who had negative intestinal activity on Tc(99m) DISIDA scan showed acholic stool and revealed higher serum direct bilirubin and urine bilirubin level. 18.2% of patients with acholic stool showed intestinal activity on Tc(99m) DISIDA scan and 81.8% of them did not. All the patients without acholic stool showed positive intestinal activity on Tc(99m) DISIDA scan. The result of Tc(99m) DISIDA scan and the presence of acholic stool showed high negative correlation (r :-0.858). Patients with acholic stool and negative intestinal activity on Tc(99m) DISIDA scan showed higher serum total bilirubin level. Patients without acholic stool and positive intestinal activity on Tc(99m) DISIDA scan showed higher serum level of ALT. CONCLUSION: Patients with acholic stool and negative intestinal activity showed high correlation, but 18.2% of patients with acholic stool showed positive intestinal activity. So operative cholangiogram or transcutaneous liver biopsy should be performed for confirmation.
Biliary Atresia ; Bilirubin ; Biopsy ; Cholestasis* ; Diagnosis, Differential ; Hepatitis ; Humans ; Infant ; Liver ; Radionuclide Imaging* ; Retrospective Studies

Malassezia folliculitis is characterized by pruritic follicular papules and pustules by Malassezia which are inhabitants of normal skin. We report a case of Malassezia folliculitis in a 12-month-old female, with a history of liver cirrhosis due to congenital biliary atresia. She presented with erythematous papules and pustules on the face 4 months ago. The diagnosis of Malassezia folliculitis was established by Parker ink-KOH direct microscopy. The lesions were successfully treated with topical ketoconazole cream for 2 weeks.
Biliary Atresia ; Diagnosis ; Female ; Folliculitis* ; Humans ; Infant* ; Ketoconazole ; Liver Cirrhosis* ; Liver* ; Malassezia* ; Microscopy ; Skin

OBJECTIVE: To study the value of serum gamma-glutamyl transpeptidase (GGT) combined with direct bilirubin (DB) in the diagnosis of biliary atresia. METHODS: A total of 667 infants with cholestasis who were hospitalized and treated from July 2010 to December 2018 were enrolled as subjects. According to the results of intraoperative cholangiography and follow-up, they were divided into biliary atresia group with 234 infants and cholestasis group with 433 infants. The two groups were compared in terms of age of onset, sex, and serum levels of total bilirubin (TB), DB, alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bile acid (TBA), and GGT. A receiver operating characteristic (ROC) curve analysis was performed for indices with statistical significance, and the area under the ROC curve (AUC) and the optimal cut-off value for diagnosis were calculated. RESULTS: The biliary atresia group had a significantly younger age of onset than the cholestasis group (P<0.001). There were no significant differences in sex, ALT, and AST between the two groups (P>0.05), while the biliary atresia group had significantly higher serum levels of TB, DB, TBA, and GGT than the cholestasis group (P<0.05). GGT combined with DB had the highest AUC of 0.892 (95% confidence interval: 0.868-0.916) in the diagnosis of biliary atresia. At the optimal cut-off values of 324.0 U/L for GGT and 115.1 μmmol/L for DB, GGT combined with DB had a sensitivity of 79.8% and a specificity of 83.2% in the diagnosis of biliary atresia. CONCLUSIONS GGT combined with DB has high sensitivity and specificity in the diagnosis of biliary atresia and can be used as an effective indicator for diagnosis of biliary atresia in infants.
Alanine Transaminase ; Aspartate Aminotransferases ; Biliary Atresia ; diagnosis ; Bilirubin ; Humans ; Infant ; gamma-Glutamyltransferase ; blood

PURPOSE: To assess the usefulness of (99m)Tc-DISIDA scanning in the early evaluation of neonatal cholestasis and to verify the diagnostic value of this test in the differential diagnosis of biliary atresia. METHODS: DISIDA scannings were performed and analyzed in 87 children(58 males and 29 females; age, 18-139 days, mean, 59.1 days) with neonatal cholestasis. Five groups according to the final diagnosis and the results of DISIDA scanning were analyzed by scatter plots using the parameters of age and the level of liver function tests(direct bilirubin, AST, ALT, ALP, GGT). The diagnostic sensitivity, specificity and accuracy of DISIDA scanning in the diagnosis of biliary atresia were compared between a higher bilirubin group and a lower bilirubin group(direct bilirubin level >5 mg/dL vs. <5 mg/dL) decided by the pattern of scatter plots. RESULTS: DISIDA scannings in the diagnosis of biliary atresia were analyzed by high sensitivity(100 percent, 16/16) but lower specificity(70.4 percent, 50/71) and accuracy(75.9 percent, 66/87). False positivity(29.6 percent, 21/71) was higher in patients with a higher direct bilirubin level(42.5 percent for >5 mg/dL vs. 9.7 percent for <5 mg/dL, P<0.01). The age and the level of liver function tests(AST, ALT, ALP, GGT) analyzed by scatter plots revealed neither diagnostic value in predicting final diagnosis nor estimated the accuracy rate of DISIDA scanning in the evaluation of neonatal cholestasis. CONCLUSION: We suggest that DISIDA scannings should not be routinely used in evaluating neonatal cholestasis with elevated direct bilirubin level(>5 mg/dL), especially if it delays early diagnosis and surgical intervention.
Biliary Atresia ; Bilirubin ; Cholestasis* ; Diagnosis ; Diagnosis, Differential ; Early Diagnosis ; Female ; Hepatitis ; Humans ; Infant, Newborn ; Liver ; Male ; Sensitivity and Specificity

PURPOSE: Many diagnostic modalities for neonatal cholestasis have shown features that are helpful, however until recently none of them are not pathognomonic of biliary atresia (BA). We carried out a prospective study of infants with cholestatic jaundice with the aim of establishing an efficient method of diagnosing BA. METHODS: Twenty-seven consecutive infants with cholestatic jaundice were enrolled in this study and ranged from 7 to 152 days in age (mean; 51.2+/-34.2 days). Gastroduodenoscopy was carried out using a fiberscope (Olympus N30). All the babies were fasted for at least 4 hours before the procedure and 20 ml of 10% dextrose solution was given at the time of endoscopy. The endoscopic examination focused on the 5 minutes observation of the evidence of biliary secretion. If there was lack of the evidence of the biliary secretion, endoscopy was removed and repeated the examination with some pause. RESULTS: There are lack of the evidence of biliary secretion in all infants with BA. In non-BA group, 8 out of the 10 infants showed biliary secretion on the first trial, however one (Alagille syndrome) of the two infants without evidence of biliary secretion, finally exhibited biliary secretion on the second trial. The above observations resulted in the diagnostic accuracy of 96.3% with 100.0% sensitivity and 90.0% specificity. CONCLUSION: In light of the results from our relatively small study, endoscopy is a convenient, and relative inexpensive procedure. we strongly support the use of endoscopy for the diagnosis of BA in the screening and evaluation of infantile cholestasis.
Biliary Atresia* ; Cholestasis ; Diagnosis ; Diagnosis, Differential* ; Endoscopy* ; Glucose ; Humans ; Infant ; Jaundice, Obstructive ; Mass Screening ; Prospective Studies ; Sensitivity and Specificity

Biliary atresia (BA) is very difficult to distinguish from neonatal hepatitis (NH) and its prognosis depends on the age at the time of Kasai operation. Therefore early differentiation between these two conditions is very important. Although various clinical and laboratory tests have been reported to differentiate between them, they are still of limited value. From 1980 to 1999, forty-five infants were referred to our pediatric surgical unit for operation for suspected BA. Eight patients underwent Kasai operation immediately because late diagnosis. These were excluded from the study. The clinical history, physical findings, radiologic and laboratory examinations of 37 cases were analyzed retrospectively. The average age of BA (n=20) was 55.1+/-6.7 days, and that of NH (n=17) was 55.8+/-5.6 days. The sex ratio of BA was 13:7, and that of NH was 14:3. All the patients had obstructive jaundice and acholic stool except 4 BA and 6 NH patients. Acholic stool with yellow component was more frequent in NH. Onset of jaundice was within 2 weeks after birth in 85% of BA, and in 65% of NH. The onset of acholic stool was within 2 weeks after birth in 60% of BA, and in 23.5% of NH. The duration of jaundice and acholic stool of BA were 50.9+/-6.6 days and 41.3+/-8.4 days and those of NH were 40.1+/-3.1 days and 26.6+/-5.4 days respectively. The ultrasonogram and hepatobiliary scan were useful, but not a definitively reliable method for the differentiation of these two diseases. There was no difference in laboratory data. Seventeen cases had NH among 45 referred cases for Kasai operation with the clinical impression of BA, and 4 cases of 17 NH cases needed to be explored to rule out BA. In conclusion, false positive rate of clinical impression of BA was 37.8%, and negative exploration rate was 8.9%. Therefore, careful clinical observation for 1-2 weeks by an experienced pediatric surgeon was very important to avoid unnecessary operation to rule out NH up to the age of 8 - 10 weeks, so long as the stool had yellow component.
Biliary Atresia* ; Delayed Diagnosis ; Diagnosis, Differential ; Hepatitis ; Humans ; Infant ; Jaundice ; Jaundice, Obstructive ; Parturition ; Prognosis ; Retrospective Studies ; Sex Ratio ; Ultrasonography

When jaundice persists for more than 14 days postnatally, the early diagnosis of surgical jaundice is important for the prognosis in extrahepatic biliary atresia after draining procedure. The role of diagnostic laparoscopy to differenctiate medical causes of jaundice from biliary atresia is evaluated in this report. Four patients with prolonged jaundice have been included in this study. When the gallbladder was not visualized we proceeded to laparotomy. In patients with enlarged gallbladder visualized at laparoscopy, laparoscopic guided cholangiogram was performed, and laparoscopic liver biopsy was done for those who had a patent biliary tree. Two patients had small atretic gallbladder and underwent a Kasai hepato-portoenterostomy. One patients showed a patent gallbladder and common bile duct with atresia of the common hepatic and intrahepatic ducts, and they underwent a Kasai hepatic-portoenterostomy. One patient showed an enlarged gallbladder and laparoscopic-guided cholangiogram were normal. Laparoscopic liver biopsy was performed. There were no complications. Laparoscopy wth laparoscopic-guided cholangiogram may be a valuable method in accurate and earlier diagnosis in an infant with prolonged jaundice.
Biliary Atresia ; Biliary Tract ; Biopsy ; Common Bile Duct ; Diagnosis ; Early Diagnosis ; Gallbladder ; Humans ; Infant ; Jaundice ; Jaundice, Obstructive* ; Laparoscopy* ; Laparotomy ; Liver ; Prognosis