• 대한영상의학회지
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• 제83권5호
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• pp.1003-1013
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• 2022

담도폐쇄증은 원인은 알 수 없으나 간외 담도의 섬유화로 내강이 막히면서 간문부에 '섬유성 담도잔유물'의 형태로 남아있는 질환이다. 'Triangular cord' (이하 TC) sign은 간문부에 남아있는 섬유성 담도잔여물을 초음파 검사에서 나타내는 소견이다. 본 종설에서는 담도폐쇄증의 수술 및 초음파 소견의 비교, TC sign의 정의, 객관적인 진단기준, 진단의 정확도 및 감별진단에 대하여 기술하고자 한다.

• Korean Journal of Radiology
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• 제24권5호
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• pp.465-475
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• 2023

Objective: To evaluate the feasibility of ultrasound shear wave elastography (SWE) for predicting hepatic fibrosis and native liver outcomes in patients with biliary atresia. Materials and Methods: This prospective study included 33 consecutive patients with biliary atresia (median age, 8 weeks [interquartile range, 6-10 weeks]; male:female ratio, 15:18) from Severance Children's Hospital between May 2019 and February 2022. Preoperative (within 1 week from surgery) and immediate postoperative (on postoperative days [PODs] 3, 5, and 7) ultrasonographic findings were obtained and analyzed, including the SWE of the liver and spleen. Hepatic fibrosis, according to the METAVIR score at the time of Kasai portoenterostomy and native liver outcomes during postsurgical follow-up, were compared and correlated with imaging and laboratory findings. Poor outcomes were defined as intractable cholangitis or liver transplantation. The diagnostic performance of SWE in predicting METAVIR F3-F4 and poor hepatic outcomes was analyzed using receiver operating characteristic (ROC) analyses. Results: All patients were analyzed without exclusion. Perioperative advanced hepatic fibrosis (F3-F4) was associated with older age and higher preoperative direct bilirubin and SWE values in the liver and spleen. Preoperative liver SWE showed a ROC area of 0.806 and 63.6% (7/11) sensitivity and 86.4% (19/22) specificity at a cutoff of 17.5 kPa for diagnosing F3-F4. The poor outcome group included five patients with intractable cholangitis and three undergoing liver transplantation who showed high postoperative liver SWE values. Liver SWE on PODs 3-7 showed ROC areas of 0.783-0.891 for predicting poor outcomes, and a cutoff value of 10.3 kPa for SWE on POD 3 had 100% (8/8) sensitivity and 73.9% (17/23) specificity. Conclusion: Preoperative liver SWE can predict advanced hepatic fibrosis, and immediate postoperative liver SWE can predict poor native liver outcomes in patients with biliary atresia.

• Advances in pediatric surgery
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• 제5권2호
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• pp.111-115
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• 1999

The results of hepatic portojejunostomy in 34 patients with biliary atresia operated upon by one surgeon between May 1989 and December 1997 were analyzed. Eleven (32.3 %) patients were 60 days or younger, 14 patient (41.2 %) were between 60 and 90 days, and 9 (26.5 %) were over 90 days of age. Jaundice cleared in 20 cases (58.8 %). Three patients died of liver insufficiency, 2 were anicteric but died from esophageal variceal bleeding. Three patients died as a result of sepsis, heart failure and left kidney agenesis. Five patients were lost to follow-up. The five-year survival rate was 73.8 %. Two patients over 90 days of age, survived more than 5 years. Survival rates were not significantly related to the age at operation. We conclude that hepatic portojejunostomy should be considered as a primary surgical modality for biliary atresia, even at age 90 days or more. Early detection of esophageal varices and sclerotherapy may be necessary. Liver transplantation is necessary if hepatic failure develops.

• Advances in pediatric surgery
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• 제13권1호
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• pp.1-12
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• 2007

Biliary atresia (BA) is the result of fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Kasai portoenterostomy has been the standard operative procedure in biliary atresia. Recently, there has been remarkable increase in the survival rate in cases of BA. However, long-term survivors are not clearly evaluated in Korea. To define long-term prognosis factors of patients who underwent surgery for BA, a retrospective study was undertaken of 10 (37 %) patients surviving more than 10 years among 27 patients who underwent one of Kasai procedures between 1981 and 1995. Hepatomegaly was present in 4 and splenomegaly in 7 patients. Serum bilirubin was normalized at 1 year after operation. Aspartate aminotransferase (AST, GOT), Alanine aminotransferase(ALT, GPT) were normalized at 12 years and alkaline phosphatase(ALP) was normalized at 13.5 years. Cholangitis developed mainly within 5 years after operation so close follow up is needed. Life long follow-up is needed because of progressive deterioration of liver function even after 10 years.

• 대한소아치과학회지
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• 제31권3호
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• pp.400-405
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• 2004

담도폐쇄증은 간장외 담관의 부분적이거나 완전한 파괴 또는 부재로 인한 담즙흐름의 완전한 폐쇄로 정의된다. 이 질환은 대략 10,000명당 1명 정도 비율로 발생하며 여성에서 약간높은 빈도를 보인다. 원인인자는 명확히 밝혀지지 않았으며 증상은 과빌리루빈혈증과 함께 황달, 점토색변, 지방설사, 어두운 갈색뇨, 간비대 등을 보인다. 최근의 담도폐쇄증의 치료는 간문장문합술 단독 혹은 간이식술을 병행하는 경향이다. 본 증례의 환자에서는 다양한 정도의 치아변색을 보였으며 색상은 노란색에서부터 짙은 초록색까지 다양했다. 법랑질의 저형성증이 모든 치아에 나타났고 구강위생이 좋지 않았으며 다발성 우식증을 가지고 있었다.

• Advances in pediatric surgery
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• 제10권2호
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• pp.136-141
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• 2004

1988년 9월부터 1999년 4월까지 가톨릭 대학교 의과대학 부속병원 외과에서 황달, 무담즙성 변 및 복부 종괴를 주소로 내원하여 간 외 담관낭이 동반된 담도폐쇄증으로 진단받은 7예의 환자의 임상상을 검토하였다. 전체 선천성 담도폐쇄증 환아 중 9.6 %에서 간 외 담관낭을 동반한 III 형의 담도폐쇄증을 보였다. 남여 성비는 2:5로 여아에서 높았다. 모두 카사이 수술을 시행하였으며 수술 시 평균연령은 57일이었다. 7예 중 3예 (42.9 %)에서 긴 생존기간을 보였다. 예후에 미치는 인자에 대해서는 지속적인 연구관찰이 필요할 것으로 사료된다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제24권4호
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• pp.366-376
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• 2021

Purpose: There is no consensus regarding adjuvant therapies following Kasai portoenterostomy (KP) for biliary atresia (BA). This study aimed to analyze the effect of extended perioperative intravenous antibiotics (PI-Abx) and adjuvant corticosteroid on cholangitis and jaundice clearance rates in the 3 years post-KP in children with BA. Methods: Data of patients who underwent KP between 1999-2018 at a single center were retrospectively analyzed. Group A (1999-2010) received PI-Abx for 5 days, Group B (2010-2012) received PI-Abx for 5 days plus low-dose prednisolone (2 mg/kg), and Group C (2012-2017) received PI-Abx for 14 days plus high-dose prednisolone (5 mg/kg). Results: Fifty-four patients were included with groups A, B, and C comprising 25, 9, and 20 patients, respectively. The number of episodes of cholangitis was 1.0, 1.6, and 1.3 per patient (p=NS) within the first year and 1.8, 2.3, and 1.7 (p=NS) over 3 years in Groups A, B, and C, respectively. The jaundice clearance rate at 6 months was 52%, 78%, and 50% (p=NS), and the 3-year native liver survival (NLS) rate was 76%, 100%, and 80% (p=NS) in Groups A, B, and C, respectively. A near-significant association was observed between the incidence of cholangitis within the first year and decompensated liver cirrhosis/death at 3 years post KP (p=0.09). Persistence of jaundice at 6 months was significantly associated with decompensated cirrhosis/death at 3 years (p<0.001). Conclusion: The extended duration of PI-Abx and adjuvant corticosteroids was not associated with improved rates of cholangitis, jaundice clearance, or NLS in patients with BA.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제27권3호
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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.