• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.1
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• pp.51-61
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• 2002

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.

• Advances in pediatric surgery
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• v.7 no.2
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• pp.95-104
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• 2001

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 of 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 $55.1{\pm}16.7$ days, and that of NH (n=17) was $55.8{\pm}15.6$ 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 $50.9{\pm}16.6$ days and $41.3{\pm}18.4$ days and those of NH were $40.1{\pm}23.1$ days and $26.6{\pm}25.4$ 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.27 no.3
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• pp.168-175
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• 2024

Purpose: The presence of alcoholic stool in biliary atresia (BA) patients is the basis of a stool color card (SCC), a screening tool that has led to more patients receiving Kasai portoenterostomy earlier. This study aimed to evaluate the color image processing of stool images captured using smartphones. We propose that measuring digital color parameters is a more objective method for identifying BA stools and may improve the sensitivity of BA screening. Methods: A prospective study was conducted in five hospitals in Thailand between October 1, 2020, and December 31, 2021. Stools from infants presenting with jaundice, acholic stool, or dark-colored urine were photographed. Digital image color analysis was performed, and software was developed based on the color on the original SCC. Sensitivity and specificity for predicting BA stools were compared between the SCC and the software. Results: Of 33 infants eligible for data collection, 19 were diagnosed with BA. Saturation and blue were two potential digital color parameters used to differentiate BA stools. The receiver operating characteristic curve was used to determine the optimum cutoff point of both values, and when saturation ≤56 or blue ≥61 was set as a threshold for detecting BA stool, high accuracy was achieved at 81.8% and 78.8%, respectively. Conclusion: Digital image processing is a promising technology. With appropriate cutoff values of saturation in hue, saturation, value and blue in red, green, blue color models, BA stools can be identified, and equivocal-colored stools of non-BA patients can be differentiated with acceptable accuracy in infants presenting with jaundice.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.17 no.2
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• pp.121-124
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• 2014

Omega (${\omega}$)-3 polyunsaturated fatty acids appear to be effective in preventing and treating parenteral nutrition-associated liver disease, and several mechanisms were proposed for this observation. An 8-week-old male infant with cholestasis and acholic stool was diagnosed non-syndromic intrahepatic interlobular bile duct paucity by open-wedge liver biopsy. Initially he was treated with usual supportive medical therapy, including ursodeoxycholic acid. However, the clinical status and laboratory tests did not improve. Omega (${\omega}$)-3 polyunsaturated fatty acids (initially intravenous administration and oral administration later), were started and his liver function, including aminotransferase level and bilirubin levels normalized, and the ivory stool color turned green. We report the possible effectiveness of ${\omega}$-3 polyunsaturated fatty acids as a potent choleretic agent for non-syndromic intrahepatic interlobular bile duct paucity, a very rare structural pediatric hepatic disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.284-288
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• 2004

Spontaneous perforation of the biliary duct is a rare disease that must be treated immediately when diagnosed in infancy. This disease must be suspected in a previously healthy infant who suddenly has abdominal distension, ascites, and intermittent jaundice. The best way to diagnose the leakage of bile in a clinically stable infant is to use radionuclide hepatobiliary scan. In most cases, however, the diagnosis of the perforation of common bile duct is frequently made during the procedure of operative cholangiogram. The prognosis is usually good with early diagnosis. We experienced an infant of 4 months of age who presented with sudden abdominal distension, jaundice and acholic stool, and confirmed the diagnosis of perforation of common bile duct through operative cholangiogram with t-tube insertion. We report a case of spontaneous perforation in an infant with review of the literatures.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.1
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• pp.65-69
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• 2008

A two-month-old baby had acholic stool, neonatal hyperbilirubinemia and congenital heart disease. Atresia of the hepatic duct was confirmed by open cholangiography, which showed a non-opacified intrahepatic bile duct. Liver biopsy and the Kasai operation were performed. Because the liver biopsy pathology revealed a paucity of intrahepatic bile ducts, the patient was diagnosed with the Alagille syndrome. We report the case of an infant diagnosed with the Alagille syndrome with atresia of the hepatic duct.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.4
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• pp.286-290
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• 2016

Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks. However, a liver biopsy was suggestive of biliary atresia. This finding forced us to perform intraoperative cholangiography, which revealed a patent common bile duct with impacted thick bile. We performed normal saline irrigation and the symptom was improved, the final diagnosis was IBS. Thus, we herein report that IBS can be treated with omega-3 PUFAs as an alternative to surgical intervention.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.257-262
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• 2005

Clinical findings in neonates and infants with cholestasis characteristically include prolonged jaundice, acholic stool, pruritus and failure to thrive. We report two cases of cholestasis presenting with spontaneous bleeding due to vitamin K deficiency. Laboratory studies on admission revealed moderate liver dysfunction and a bleeding tendency due to vitamin K deficiency. After administration of vitamin K, the bleeding tendency disappeared. Vitamin K deficiency was resulting from a combination of cholestasis-induced fat malabsorption and low vitamin K supplementation due to breast-milk feeding. Cholestasis should be considered in neonate and infant with a bleeding tendency.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.473-481
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• 2002

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(>$38^{\circ}C$) 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.