• Advances in pediatric surgery
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• 제9권2호
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• pp.81-88
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• 2003

Congenital dilatation of the common bile duct (choledochal cyst) is an uncommon disease. Although the etiology is unknown yet, various theories such as distal obstruction of the common bile duct, congenital weakness of the duct and anomalous union of the pancreaticobiliary duct have been offered to explain the occurrence of choledochal cyst. Thirty - six cases of choledochal cyst over 22 years were analyzed clinically and classified according to Todani's classification and Kimura's anomalous union of pancreaticobiliary duct type. Todani type 1 consisted with 22 cases which were subdivided into 19 cases of type Ia, 1 case of type Ib and 2 of type Ic. Type IVa consisted with 14 cases including one case of Caroli's disease. There were 25 type BP cases and 10 type PB cases and 1 normal pancreatobiliary junction. Serum alkaline phosphatase was increased significantly in almost all cases. Seven patients (19.4%) had associated congenital anomalies such as double gallbladder, left - sided gallbladder, common bile duct web, biliary atresia, accessory hepatic duct, heterotopic pancreas, cleft lip and 2 cases of intestinal malrotation. All patients underwent cyst excision and Roux - en - Y hepaticojejunostomy and cholecystectomy. There was one death due to methicillin resistant Staphylococcus aureus sepsis.

• Advances in pediatric surgery
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• 제4권2호
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• pp.100-109
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• 1998

The anomalous pancreaticobiliary duct union (APBDU) might cause the formation of choledochal cyst and malignancies of hepatopancreaticobiliary system. The purpose of this study is to make an experimental animal model of APBDU similar to that of human. One to two-month-old Mongrel dogs (n=12) were divided into two groups; the control group (n=2) had a sham operation performed, and in the experimental group (n=10) the end of distal ' common bile duct (CBD) was anastomosed to the side of the dorsal pancreatic duct making APBDD. Serum was obtained for chemical analysis on the 10th postoperative day. The dogs were sacrificed at the 5th week (n=3), the 6th week (n=3), the 7th week (n=2), the 8th week (n=2) and the 6th month (n=2) after the experimental surgery. With sacrifice, operative cholangiogram was taken, and bile juice was obtained for chemistry and bacterial culture. The en-bloc specimens of the hepatopancreaticobiliary system were removed for microscopic examination. Serum and bile juice amylase levels were elevated in the experimental group(n=10), but not in the control group(n=2). Operative cholangiograms of control group revealed no evidence of bile duct dilatation.. On the other hand, the bile duct in the experimental group was markedly dilated without any evidence of stenosis at the anastomosis site (n=10). Histologic examination of the hepatopancreaticobiliary system in the experimental group resembled the findings of choledochal cyst in human. The APBDU of this animal model can produce bile duct dilatation by pancreaticobiliary reflux. We think that this animal model can be potentially promising for the research about the APBDU associated hepatopancreaticobiliary diseases.

• Journal of Digestive Cancer Research
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• 제3권1호
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• pp.42-44
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• 2015

Anomalous union of the pancreaticobiliary duct (AUPBD) has been shown to predispose to carcinomas of the biliary tract (bile duct and gallbladder) and pancreatic cancer because of chronic recurrent inflammatory reaction due to pancreatic or bile juice refluxes. However, pancreatic duct would be less affected by the bile because of the relatively higher intraductal pressure of the pancreatic duct. We report a case of metachronous pancreatic cancer in AUPBD patient without choledochal cyst who underwent cholecystectomy because of gallbladder cancer.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권3호
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• pp.170-177
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• 2014

Purpose: Choledochal cyst is a cystic dilatation of common bile duct. Although the etiology is presently uncertain, anomalous pancreaticobiliary ductal union (APBDU) is thought to be a major etiology of choledochal cyst. In this study, we analyzed the clinical and anatomical characteristics and pathologies of patients diagnosed with choledochal cyst in a single institute for 25 years. Methods: A total of 113 patients, diagnosed with choledochal cyst and who received an operation in Severance Children's Hospital from January 1988 to May 2013, were included. Medical records were reviewed, including clinical and demographic data, surgical procedures. Abdominal ultrasonography, magnetic resonance cholangiopancreatography, and intraoperative cholangiography were used as diagnostic tools for evaluation and classification of choledochal cyst and the presence of anomalous pancreaticobiliary ductal union. Todani's classification, and relationship between APBDU and surgical pathology. Results: Among 113 patients, 77 patients (68.1%) presented symptoms such as hepatitis, pancreatitis and/or cholecystitis. Eighty three patients (73.5%) had APBDU, and 94 patients (83.2%) showed inflammatory pathologic changes. APBDU, pathologic inflammation, and serological abnormalities such as hepatitis or pancreatitis showed a statistically significant correlation to one another. Conclusion: APBDU is thought to be one of the etiologic factors of choledochal cyst. It is related to the inflammatory changes in bile duct that can lead to the cystic dilatation.

• Clinical and Experimental Pediatrics
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• 제45권11호
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• pp.1381-1388
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• 2002

목 적: MRCP는 간담도췌장 질환을 진단하는데 있어서 비침습적인 방법으로 매우 유용한 것으로 알려져 있다. 본 연구의 목적은 간담도췌장 질환이 의심되는 소아에서 MRCP의 진단적 유용성에 대해 알아보고자 한다. 방 법: 1996년 10월부터 2001년 5월까지 연세의대 세브란스병원 소아과에 간담도췌장 질환으로 입원하여 복부 초음파 및 MRCP를 시행한 환아 70례를 대상으로 하였다. 환아의 최종 진단은 수술, 조직검사 소견, 임상 소견, 기타 검사 등을 토대로 하였다. 결 과 : 1) 대상 환아는 총 70례로 남녀비는 1 : 1.2였으며 평균 연령은 $2.6{\pm}3.3$세였다. 2) 간기능 검사상 혈청 AST, ALT 치는 각각 $192.7{\pm}139.6IU/L$, $182.7{\pm}143.8IU/L$이었고, 총 빌리루빈치, 직접 빌리루빈치는 각각 $6.9{\pm}3.7mg/dll$, $4.8{\pm}2.4mg/dll$이었다. 3) 간담도 췌장질환의 원인은 담도폐쇄증 25례(35.7%), 신생아 간염 18례(25.7%), 담관낭종 16례(23%)로 이 중 APBDU를 동반한 경우가 7례(10.0%), APBDU를 동반하지 않은 경우가 6례(12.9%)였으며, 전염성 단핵구증, 전격성 간염, 콕시엘라증(coxiellosis) 등으로 인한 담즙 정체성 간염이 5례(7.1%), 경화성 담도염이 2례(2.8%), 이차성 담도 경화증은 1례(1.4%), 만성 재발성 췌장염 환아가 3례(4.3%)였다. 4) 수술 소견과 병리 조직학적 소견을 기준으로 할 때 간담도췌장질환에 대한 복부 초음파의 진단의 정확도 75.7%에 비해 MRCP는 97.1%로 높았다. MRCP의 예민도와 특이도는 담도폐쇄증의 경우 각각 100%, 98%였고, APBDU가 동반된 담관낭종의 경우 87.5%, 100%였으며, APBDU가 동반되지 않은 담관낭종, 경화성 담도염, 만성 반복성췌장염은 양자 모두 100%였다. 결 론 : 본 연구에서 MRCP는 간담도췌장 질환을 진단하는데 있어서 편리하고 안전하며 비침습적인 방법으로서 그 진단율 또한 다른 검사에 비해 뛰어나 소아에서 간담도췌장질환이 의심될 때 우선적으로 시행할 수 있는 매우 유용한 검사임을 알 수 있었다.

• Journal of Digestive Cancer Research
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• 제1권2호
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• pp.104-107
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• 2013

담낭암은 진행된 상태에서 발견되었을 경우 예후가 매우 불량한 암으로 전신항암화학요법으로 치료를 하게 되나 일반적으로 고식적인 역할에 그치는 경우가 대부분이다. 본 증례는 원위부 림프절 전이가 있어서 수술적 절제가 불가능한 담낭암 환자에서 gemcitabine과 cisplatin을 병합한 전신항암화학요법으로 여섯 주기 치료 후 부분 관해를 얻어서 확대 담낭절제술이 가능하였던 증례로 매우 드문 증례를 경험하였기에 보고하는 바이다. 담낭암에서 수술 전 항암화학요법의 효과는 아직까지 확립되어 있지 않으나, 최근 양호한 종양 반응률과 종양 억제율을 보이는 항암화학요법이 개발됨에 따라서 그 가능성이 기대되고 있다.