• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.2
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• pp.114-123
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• 2017

Purpose: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. Methods: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling. Whole exome sequencing and Sanger sequencing were performed on the patients and the family members. Results: Idiopathic or viral hepatitis was diagnosed in 34%, metabolic disease in 20%, total parenteral nutrition induced cholestasis in 16%, extrahepatic biliary atresia in 14%, genetic disease in 10%, neonatal lupus in 2%, congenital syphilis in 2%, and choledochal cyst in 2% of the patients. The patient with progressive familial intrahepatic cholestasis had novel heterozygous mutations of ABCB11 c.11C>G (p.Ser4*) and c.1543A>G (p.Asn515Asp). The patient with benign recurrent intrahepatic cholestasis had homozygous mutations of ABCB11 c.1331T>C (p.Val444Ala) and heterozygous, c.3084A>G (p.Ala1028Ala). Genetic confirmation of ABCB11 spectrum liver disorder led to early liver transplantation in the progressive familial intrahepatic cholestasis patient. In addition, the atypically severe benign recurrent intrahepatic cholestasis patient was able to avoid unnecessary liver transplantation after genetic analysis. Conclusion: ABCB11 spectrum liver disorders can be clinically indistinguishable as they share similar characteristics related to acute episodes. A comprehensive genetic analysis will facilitate optimal diagnosis and treatment.

• Journal of Yeungnam Medical Science
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• v.23 no.2
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• pp.152-161
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• 2006

Carcinoma of the gallbladder is an uncommon but highly malignant tumor with a poor five year survival rate. Early detection is very important for successful treatment because this tumor is very hard to cure in cases where it has advanced beyond the reach of surgical treatment. The purpose of this review was to evaluate risk factors for carcinoma of the gallbladder and determine the best management approach.. Solitary polys, more than one cm are considered to be predisposing factors for gallbladder carcinoma when they are found to be echopenic, sessile, and with a high cell density. Anomalous union of the pacreato-biliary duct(AUPBD) without a choledochal cyst is also considered to increase risk for gallbladder carcinoma. A polyps size of more than one cm and an AUPBD are indications for prophylactic cholecystectomy. The presence of gallstones is a well-established risk factor for the development of gallbladder carcinoma; risk appears to correlate with the stone size and the duration of chronic cholecystitis. Metaplastic changes of the gallbladder epithelium present with chronic cholecystitis and may indicate a premalignant lesion. Abnormal forms of cholecystitis such as xanthogranulomatous or a porcelain gallbladder also have malignant potential; cholecystoenteric fistula as well as bacterial infection of the gallbladder(typhoid, helicobacter species) also has malignant potential. In this review, the risk factors associated with carcinoma of the gallbladder are summarized with special attention to gallstones, polyps, AUPBD, and chronic inflammation.

• The Korean Journal of Nuclear Medicine
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• v.21 no.1
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• pp.17-24
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• 1987

Authors classified 161 cases of photon defects due to the gall bladder on hepatic parenchymal scintigraphy on $^{99m}Tc-phytate$ and $^{99m}Tc-DISIDA$ according to the position of the gall bladder, the pattern of photon defects and the hepatobiliary diseases. The results were as follows; 1) Conocordance of $^{99m}Tc-DISIDA$ and $^{99m}Tc-phytate$ hepatic parenchymal images in photon defect due to the gall bladder was 94% of 32 cases. 2) The frequency according to the position of the gall bladder was in order to 68% of the gall bladder of the lower margin of the liver, 30% of the intrahepatic gall bladder and 2% of the extrahepatic gall bladder, and the frequency of the photon defects due to the gall bladder was in order to 81% of the intrahepatic gall bladder, 71% of the gall bladder of the lower margin of the liver and 20% of the extrahepatic gall bladder. 3) The pattern of the photon defects due to the gall bladder was 47% of funnel shape in the intrahepatic gall bladder, 69% of semilunar shape in the gall bladder of the lower margin of the liver and 100% of semilunar shape in the extrahepatic gall bladder. 4) All of 9 cases of the intrahepatic gall bladder at the lateral area of the right lobe and the gall bladder of the lower margin of the liver at the right hepatic angle were associated with liver cirrhosis with the right lobe atrophy and the left lobe hypertrophy, 2 cases of the gall bladder of the lower margin of the liver at just-left side of the porta hepatis with hepatoma in the right lobe and 1 case of the intrahepatic gall bladder at the central portion of the right lobe with choledochal cyst.

• Advances in pediatric surgery
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• v.18 no.1
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• pp.1-11
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• 2012

담관 낭종은 흔하지는 않지만, 외과적 절제가 필요한 주요 질환이다. 절제하지 않고 남겨두면 다른 질환으로의 이환이나 다양한 합병증에 의한 사망까지도 야기할 수 있기 때문에 적절한 수술적 치료가 필수적이다. 최근 수술에 따른 다양한 문제들이 계속 보고되고 있으며, 출생 전이나 건강 검진 시 발견되는 무증상의 담관 낭종의 수술 시기에 대해서도 아직 논란이 되고 있다. 저자들은 1995년부터 2009년까지 담관 낭종으로 수술 받은 환자 중 수술 받을 당시의 연령이 18세 이하인 32명 환자의 임상 양상과 수술 기록을 후향적으로 분석하여, 수술 성적에 영향을 주는 요인들을 알아보고자 하였다. 전체 32명 중 남자 10명, 여자 22명이었으며, 수술 당시의 평균 연령은 5.4세였다. 췌담관 합류이상은 9명(28.1%)이었으며, 평균 추적 관찰 기간은 34.6개월이었다. 술 전 증상을 호소한 환자는 30명(93.8%)이었으며, 복통(63.3%), 황달(40.0%), 구토(23.3%), 복부종물(16.7%), 발열(16.7%)등의 순서였다. 술 전 혈액 검사에서 AST/ALT의 상승이 18명(56.3%), 고빌리루빈혈증이 8명, 백혈구 증가증이 7명(21.9%)이었다. 술 후 합병증은 5명(15.6%)에서 나타났는데, 2명은 담관염이었고, 나머지 3명은 문합부 협착, 술 후 출혈, 장 폐쇄가 각각 1명 있었다. 술 후 평균 재원 기간은 12.2일 이었으며, 추적 관찰 기간 중 3명(9.0%)이 재입원 하였다. 수술 성적에 영향을 미치는 요인으로, 수술 당시의 나이가 많을수록 평균 수술 시간과 재원 기간이 증가하였다(p=0.004, p=0.028). 증상이 있었던 환자군에서 무증상 환자군보다 재원 기간이 더 길었다(p=0.001). 또한 췌담관 합류이상이 있었던 환자군에서 재입원율이 유의하게 높았다(p=0.005). 소아의 담관 낭종은 진단이 되면 증상 발현 전이라도 조기에 수술하는 것이 수술 시간 및 재원 기간을 단축시킬 수 있으며, 췌담관 합류이상이 동반된 경우에는 술 후에 재입원할 가능성이 높으므로 주의깊게 추적 관찰하는 것이 바람직할 것이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.2
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• pp.262-266
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• 1999

Obstruction of the extrahepatic bile ducts is the most common cause of conjugated hyperbilirubinemia in early infancy. More than 90% of such obstructive lesions are accounted for by extrahepatic biliary atresia. A rare lesion is obstruction of the common duct by impacted, thickened secretions and bile. Bile plug syndrome is defined as extrahepatic obstruction of the bile ducts by bile sludge in term infants without anatomic abnormalities, congenital chemical defects of bile, or hepatocellular lesions. Obstruction of extrahepatic ducts by plugs of biliary material apperas to be due to the inspissation and precipitation of bile and mucus within the lumen of the ducts. Cholestasis and precipitation of bile develop in association with abnormal composition of bile in cystic fibrosis, hepatocellular damage, prolonged erythroblastic jaundice, altered biliary dynamics with total parenteral nutrition, gut dysfunction, diuretic therapy, exchange transfusions and perinatal hemolysis. In those cases, the term inspissated bile syndrome is used. The clinical and laboratory findings in bile plug syndrome are identical to those observed in biliary atresia and choledochal cyst. The diagnosis can be suspected based on the findings of clinical and laboratory examinations together with hepatobiliary imaging, ultrasonography, radionuclide scan and liver biopsy. We experienced a case of spontaneous resolution of bile plug syndrome in a 4-year-old girl. We report this case with brief review related literatures.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.1
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• pp.70-78
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• 2022

Purpose: Cholestasis resulting from cytomegalovirus (CMV)-induced hepatitis manifests in 40% of patients with a CMV infection. Ganciclovir treatment in children with CMV infections has proven to be highly effective. Until now, there are very few studies have identified predictive factors for liver biochemistry improvement after ganciclovir therapy. This study aimed to identify the predictors of liver biochemistry improvement in patients with CMV cholestasis after ganciclovir treatment. Methods: A retrospective cohort study was conducted using medical records from Dr. Sardjito General Hospital Yogyakarta, Indonesia from 2013 to 2018. CMV cholestasis was confirmed based on serum CMV IgG and IgM positivity and/or blood and urine CMV antigenemia positivity. Incomplete medical records and other etiologies for cholestasis, such as biliary atresia, choledochal cyst, metabolic diseases, and Alagille syndrome, were excluded. Patient age at cholestasis diagnosis and ganciclovir treatment, duration of CMV cholestasis, history of prematurity, central nervous system involvement, and nutritional status were analyzed and presented as an odds ratio (OR) with a 95% confidence interval (95% CI). Results: CMV cholestasis with ganciclovir therapy was found in 41 of 54 patients. Multivariate analysis showed that a shorter duration of CMV cholestasis (OR: 4.6, 95% CI: 1.00-21.07, p=0.04) was statistically significant for liver biochemistry improvement after 1 month of ganciclovir treatment. The remaining factors that were analyzed were not significant predictors of liver biochemistry improvement in patients with CMV cholestasis after ganciclovir treatment. Conclusion: A shorter duration of CMV cholestasis is the predictor of liver biochemistry improvement after 1 month gancyclovir treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.1
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• pp.46-58
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• 1999

Purpose: The aim of the present study was to evaluate the long-term clinical profile including the underlying etioligy and the prognostic factors of the neonatal cholestasis. Method: We studied the 190 infants presented with neonatal cholestasis for the last 12 years (from 1981 to 1992). The underlying causes, clinical findings and long-term outcomes were evaluated. And the prognostic factors were also analyzed. Result: Underlying disease were neonatal hepatitis in 101 (idiopathic in 77 and infectious in 24), intrahepatic bile duct paucity in 5, biliary atresia in 79, choledochal cyst in 5. Metabolic disease was not observed in this study. The important clinical problems during follow-up were persistent high fever, gastrointestinal bleeding, hepatic encephalopathy and ascites. The main causes of the death were hepatic encephalopathy and gastrointestinal bleeding. While three fourth of infants with idiopathic and infectious neonatal hepatitis recovered usually within a year, five-year survival rate for biliary atresia was just 40%, the mortality observed usually within the first year after Kasai operation and prognostic factor was the time of operation. Underlying disease was the most important prognostic factor of neonatal cholestasis. Conclusion: This study showed that most common causes of neonatal cholestasis were biliary atresia and idiopathic neonatal hepatitis, infectious neonatal hepatitis, choledochal cyst and Alagille syndrome, but few neonatal cholestasis of genetic or metabolic liver disease was observed. The most important long-term prognostic factor of neonatal cholestasis was the underlying disease.

• Water for future
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• v.7 no.2
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• pp.75-82
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• 1974

본시험은 관개 및 배수 설계를 위한 기초자료 제공을 목적으로 토양수분 및 지하수위의 변화상태를 구명하고자 하는 것으로 카나다 퀴백(Quebec) 지방 저지대의 대표적인 2종의 태양인 점토와 사질로움 토양에 대해서 일차적으로 지표, 지하배수간의 토양수분과 지하수위의 변화를 연구분석한 것으로 그 결과를 요약하면 다음과 같다. 1. 2종의 토양(점토, 사질로움)에 있어서 공히 토양수분은 지표로부터 지하로 내려갈수록 증가되는 상태를 나타냈으면 이는 하층토로 내려감에 따라 팽창된 치밀한 토양 조직을 가지고 있음을 시사하며 2. 동기에 있어서는 지표로부터 12inch 깊이의 토양수분이 더 깊은 18inch나 24inch 깊이의 토양수분보다 증가된 상태를 유지하였다. 이는 토양내의 빙결막이나 지표면의 눈 또는 어름에 영향으로 생각되며 이 경향은 점토질 토양에서 보다 토양내 빙결막으로의 수분 이종을 조장해주는 투수 계수가 큰 사질로움 토양에서 더 높았다. 3. 양식험구 공히 지표에서 얕은 지하 0∼3inch 이내의 토양수분은 항우를 전후해서 급격한 변화를 가져왔다. 여기에서 수분의 급상승은 호우시나 표층토가 하층토의 토양수분에 영향을 주는 항우이전에 포장용수량(Field capacity)에 달했기 때문에 급항하는 지표면의 물 분자가 열에너지에 의해 증발산되고 천층에 산재한 초, 수근에 의해 소비되는 때문인 것으로 사료된다. 4. 지하수위하의 토양수분은 포화에 달하여 거의 일정할 것으로 기대되었지만 실제로 많은 변화를 가져왔다. 이들 변화에는 부분적으로 토양사극이 지하수위하이라도 채워지지 않았거나(Capillary pressure가 작을 시) 혹은 관측기간 중에 토양의 융기와 수축에 기인된다고 생각된다. 5. 지하배수구가 지표배수구에 비해 지하수위항하가 빨랐음은 물론 사질로움 토양에서는 보다 높은 투수계수로 인해서 지하수위의 항하가 점토질 토양에서 보다 훨씬 빨랐음을 보여주고 있다.표시할 수 있다.된다.acid $0.41{\sim}0.65%$, 오미자는 malic acid $1.51{\sim}3.90%$, citric acid $2.40{\sim}3.92%$로 주요 유기산이 있다. 타닌은 물보다 에탄올 추출물이 다소 함량이 높았으며 특히 오갈피는 $3.35{\sim}3.85%$로 매우 높은 함량이었다.a 6 cases, etc. 2. The retroperitoneal space including kidneys were the most commonly involved site (43.5%), of which Wilms' tumor was the commonest. 3. About 2/3 of tumors developed under the age of 6 and the commonest lesion was Wilms' tumor and the next being neuroblastoma, teratoma, ovarian cyst and so forth. 4. In all tumors except ovarian tumor and choledochal cyst, male was more frequently affected. 5. In plain abdomen 75% of neuroblastoma crossed the mid line while in Wilms' tumor only 2 cases (14%) showed midline cross. Calcific density was 입력불가 in all 6 cases of teratoma (100%) as dense and discrete ossification, in 3 case of neuroblatoma (38%) characteristically in diffuse stippled appearance and in one case

• Journal of the Korean Society of Radiology
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• v.79 no.5
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• pp.271-275
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• 2018

The falciform ligament is a hepatic suspensory ligament that extends from the umbilicus to the diaphragm, containing the ligamentum teres and a vestigial remnant of the umbilical vein. Among the rarely-occurring pathologies of the falciform ligament, which include ligament cyst, tumor, abnormal vascularization, and congenital ligament defect, a falciform ligament abscess is even more sporadic. Accordingly, the definitive diagnosis of the falciform ligament abscess is rather challenging and may easily be misinterpreted as an infected choledochal cyst or a liver abscess. We present a 25-day-old infant with the falciform ligament abscess, which developed after the umbilical venous catheter insertion and was successfully treated with percutaneous drainage and antibiotic administration.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.2
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• pp.141-147
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• 2011

Purpose: Ultrasonography (US) is widely used as a screening test in patients with abdominal pain (AP). We investigated the usefulness of US by a pediatrician in children with AP. Methods: We retrospectively analysed the medical records of children with AP who undertook US from December, 2008 to July, 2010. Results: A total of 628 patients (325 male, 303 female) were enrolled in this study. The mean age of patients was $8.08{\pm}4.61$ years. Duration of AP was acute in 427 and chronic in 201 patients. Localization of AP was diffuse (36.9%), periumbilical (24.4%), epigastric (21.0%), and right lower quadrant (8.1%). On the examination, there were no abnormal findings in 327 patients (52.1%). Abnormal ultrasonographic findings were mesenteric lymphadenitis (27.1%), intestinal mural thickening (10.0%), intussusception (3.0%), appendicitis (2.6%), choledochal cyst (1.6%), and pancreatitis (0.3%). We performed additional imaging studies such as computed tomography (CT) or magnetic resonance imaging (MRI) in 39 patients who showed obscure findings on the US. In 33 patients (84.6%), the same results were obtained from CT or MRI. Two cases of appendicitis, one case of pancreatitis and one case of Henoch-Sh$\ddot{o}$nlein purpura were diagnosed by the CT examination. However, there were two cases of appendicitis diagnosed by US thathad no evidence of appendicitis on the CT. Diagnostic accuracy of initial US in children with abdominal pain was 99.4%. Conclusion: US by a pediatrician as a screening test in children with AP provides a rapid and accurate diagnostic indication and has non-invasive and radiation-free advantages.