• Journal of Yeungnam Medical Science
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• 제3권1호
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• pp.351-355
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• 1986

저자들은 최근 영남대 의대 부속병원 외과에서 치험한 원발 경화성 담관염 1예를 보고하고 아울러 문헌을 고찰하였다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제10권1호
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• pp.91-97
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• 2007

저자들은 4세에 진단된 자가면역간염과 궤양성 대장염 그리고 6세에 진단된 원발성 경화담관염을 가진 중 복증후군 환아에서 9세에 만성 림프형질세포성 골수염이 발병한 뒤 면역억제 치료에 잘 반응하지 않는 증례를 경험하였기에 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제4권1호
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• pp.120-124
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• 2001

저자들은 복부 팽만과 지속적인 황달을 주소로 내원한 랑게르한스세포 조직구증 환아에서 간조직 검사 및 내시경적 역행성 담도조영술로 진단한 속발성 경화성 담관염 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제19권3호
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• pp.210-213
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• 2016

Primary sclerosing cholangitis (PSC), a rare progressive liver disease characterized by cholestasis and bile duct fibrosis, has no accepted, effective therapy known to delay or arrest its progression. We report a 15 year old female patient diagnosed with PSC and moderate chronic active ulcerative colitis (UC) who achieved normalization of her liver enzymes and bile ducts, and resolution of her UC symptoms with colonic mucosal healing, after treatment with a single drug therapy of the antibiotic oral vancomycin. We postulate that the oral vancomycin may be acting both as an antibiotic by altering the intestinal microbiome and as an immunomodulator. Oral vancomycin may be a promising treatment for PSC that needs to be further studied in randomized trials.

• Clinical and Experimental Pediatrics
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• 제52권4호
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• pp.504-507
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• 2009

자가면역성 간염, 원발성 담관성 간경화증, 원발성 경화성 담관염은 대표적인 자가 면역성 간질환이다. 이 각 질환의 임상적, 조직학적, 혈청학적 특징이 혼재되어 어느 한 질환으로 진단하기 어려운 경우를 중복증후군(overlap syndrome)이라 하고, 최근 소아에게서도 드물게 보고되고 있다. 한편 경화성 담관염은, 염증성 장질환에서 가장 흔히 동반되는 간담도계질환으로 알려져 있다. 저자들은 궤양성 대장염으로 진단된 환아에게서 간조직 검사, 혈청 검사, 방사선 검사 등을 통해 자가면역성 간염-원발성 경화성 담관염의 중복증후군을 진단하였기에 보고하는 바이다. 염증성 장질환 환아에게서 자가 면역성 간질환 또는 중복증후군 동반여부를 염두에 두어 임상적, 조직학적, 혈청학적 검사를 통해 적절한 진단과 치료가 필요하겠다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제24권2호
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• pp.154-163
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• 2021

Purpose: In children overlap of autoimmune hepatitis (AIH) and primary sclerosing cholangitis is labelled as autoimmune sclerosing cholangitis (ASC). The only prospective pediatric study showed a high prevalence of ASC by using endoscopic retrograde cholangiopancreatography. Aims of our study were to find the prevalence of ASC by using magnetic resonance cholangiography (MRC) in AIH and in non-AIH cirrhosis and to compare clinical presentation and outcome of AIH and ASC. Methods: Prospectively we did MRC in 38 children with AIH (cases) and 19 disease controls (Wilson disease). Multiple biliary strictures with proximal dilatation on MRC were taken as definitive changes of ASC. Detail clinical, laboratory parameters, liver histopathology and treatment outcome were recorded. Results: The median age of cases was 11.5 (3-18) years, 22 (57.9%) were girls and 28 (73.7%) were diagnosed as type 1 AIH. MRC was done in 11 children (28.9%) at the time of diagnosis and in 27 (71.1%) after a median follow-up of 2.5 (0.3-10) years. Abnormal MRC changes were seen in 14/38 (36.8%) of AIH and 8/19 (42.1%) of controls. However, definite changes of ASC were present in four (10.5%) children in AIH and none in controls. None of the clinical, laboratory, histological parameters and treatment response were significantly different between ASC and AIH groups. Conclusion: The prevalence of ASC in children with AIH was just 10.5%. We suggest MRC in select group with cholestatic features, inflammatory bowel disease and in those who showed poor response to immunosuppression instead of all children with AIH.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제18권4호
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• pp.268-275
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• 2015

Purpose: Primary sclerosing cholangitis (PSC) is a rare condition that can be associated with inflammatory bowel disease (IBD). The aim of this study was to evaluate PSC and its association with IBD in children. Methods: We retrospectively enrolled 13 pediatric patients (<18 years) with PSC treated at Asan Medical Center between June 1989 and December 2013. Clinical findings and long-term outcomes were investigated. During the same period, the incidence of PSC among IBD patients was evaluated among 600 Crohn disease (CD) and 210 ulcerative colitis (UC) patients. Results: All 13 study patients diagnosed with PSC also presented with IBD. Eleven boys and two girls with a median age of 15.0 years old (9.0-17.8 years) were included. The cumulative incidence of PSC for UC was 5.7% (12 of 210) and 0.2% for CD (1 of 600), respectively. PSC occurred during follow-up for IBD for five patients (38.5%) whereas, IBD developed during follow-up for PSC for two patients (15.4%), and was diagnosed during the initial work-up for PSC for 6 patients (46.2%). For the 77.3 month median follow-up period, 9/13 patients (69.2%), neither the clinical symptoms nor blood test results worsened. Two cases (15.4%) developed liver cirrhosis and underwent liver transplantation. Among 13 PSC patients with IBD, two (15.4%) developed colorectal cancer, and no one developed cholangiocarcinoma. Conclusion: All patients with PSC in this study had associated IBD. The incidence of PSC was not rare compared to reports in adults. PSC should be considered during the management of IBD and vice versa in children.

• Journal of Yeungnam Medical Science
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• 제38권1호
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• pp.1-9
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• 2021

Gallbladder (GB) polyp is a mucosal projection into the GB lumen. With increasing health awareness, GB polyps are frequently found using ultrasonography during health screening. The prevalence of GB polyps ranges between 1.3% and 9.5%. Most patients are asymptomatic and have benign characteristics. Of the nonneoplastic polyps, cholesterol polyps are most common, accounting for 60%-70% of lesions. However, a few polyps have malignant potential. Currently, the guidelines recommend laparoscopic cholecystectomy for polyps larger than 1 cm in diameter due to their malignant potential. The treatment algorithm can be influenced by the size, shape, and numbers of polyps, old age (>50 years), the presence of primary sclerosing cholangitis, and gallstones. This review summarizes the commonly recognized concepts on GB polyps from diagnosis to an algorithm of treatment.

• Asian Pacific Journal of Cancer Prevention
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• 제13권11호
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• pp.5381-5384
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• 2012

Background and Aim: Cholangiocarcinoma (CCA) is an uncommon malignancy of the bile duct, occurring in nearly 2 out of 100,000 people. It is a type of adenocarcinoma that originates in the mucous glands of the epithelium, or surface layers of the bile ducts. The aim of this study was to evaluate the clinical features, diagnostic results and factors associated with survival, morbidity and mortalityof cholangiocarcinoma cases in Iranian patients. Method: In this retrospective study the hospital medical records of 283 patients with a primary or final diagnosis of cholangiocarcinoma who had been admitted to gastroenterology ward of our hospital from 2004 to 2011 were retrospectively reviewed. Results: 283 patients (180 male, 63%, and 103 female, 38.6%) with a mean age of $59.7{\pm}14.4$ years were studied. The most frequent symptoms were painless jaundice (190, 66.9%), abdominal pain (77, 27%), pruritus 133 (46.8%) and weight loss (169, 59.5%). The most frequent associated risk factors and diseases were as follows: gallstones (72, 25.4%), diabetes (70, 24.6%), HBV infection (52 (18.3%), HCV infection 43 (15%), primary sclerosing cholangitis (16, 5.6%) and smoking (120, 42.3%). The most frequent type of cholangiocarcinoma in ERCP and MRCP was hilar. The mean survival time was $7.42{\pm}5.76$ months. Conclusion: The mean survival time in our study was lower than one year. Moreover the most frequent risk factors and associated diseases were smoking, gallstones and diabetes. Painless jaundice, abdominal pain and weight loss were the most clinical features related to cholangiocarcinoma. Additionally survival time did not correlate with risk factors, associated diseases and clinical presentations, but was linked to biliary metallic stenting and surgery.

• Asian Pacific Journal of Cancer Prevention
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• 제15권18호
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• pp.7673-7677
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• 2014

Background: Patients with long-standing inflammatory bowel disease (IBD) have an increased risk of developing colorectal cancer (CRC). Colon cancer risk in IBD increases with longer duration and greater anatomic extent of colitis, the presence of primary sclerosing cholangitis, family history of CRC and degree of inflammation of the bowel. This study aimed to characterize the histopathological pattern of benign colorectal diseases among Saudi patients and to highlight age and gender variations of lesions as base line data for future studies to investigate the link between benign/IBD and colorectal cancers in the local population. Materials and Methods: The materials consisted of 684 biopsies, reported as benign (excluding malignancies and polyps) at the Department of Pathology, King Fahad Hospital, Madinah, Saudi Arabia from January 2006 to December 2013. Data collected and entered in MS-Excel and were analyzed using SPSS-20. Results: Of 684 colorectal tissues reviewed, 408 specimens (59.6%) were from male patients and 276 specimens (40.4%) were from females giving a male: female ratio of 1.5:1. Age of the patients ranged from 4 to 75 years with a mean of 39.6 years. The most frequent histologic diagnosis was a chronic non specific proctocolitis followed by ulcerative colitis, accounting respectively for 52.6% and 31.7% of all cases. These were followed by Crohn's disease 22 (3.2%), ischemic bowel disease 20 (2.9%), diverticular disease 14 (2%), eosinophilic colitis 12 (1.7%) and solitary rectal ulcer 12 (1.7%). A minority of 21 patients (3.1%) were cases of acute nonspecific proctocolitis, schistosomiasis, tuberculosis, volvulus and pseudomembranous colitis. Conclusions: These data show that although chronic non specific proctocolitis and ulcerative colitis were the dominant diagnoses, Crohn's disease, ischemic bowel disease and diverticular disease also existed to a lesser extent and should be considered in the differential diagnosis of benign colorectal diseases. This study provides a base line data for future studies which would be taken up to investigate the link between benign/IBD and colorectal cancers in the local population.