• 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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• 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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• 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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• 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.

• 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.

• Korean Journal of Radiology
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• 제24권11호
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• pp.1167-1171
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• 2023

• 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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• 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.

• 한국간담췌외과학회지
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• 제27권3호
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• pp.241-250
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• 2023

Helicobacter pylori is a gram-negative pathogen commonly associated with peptic ulcer disease and gastric cancer. H. pylori infection has also been reported in cholelithiasis, cholecystitis, gallbladder polyps, and biliary tract cancers. However, the association between H. pylori and gallbladder and biliary tract pathologies remains unclear due to the paucity of literature. In response to the current literature gap, we aim to review and provide an updated summary of the association between H. pylori with gallbladder and biliary tract diseases and its impact on their clinical management. Relevant peer-reviewed studies were retrieved from Medline, PubMed, Embase, and Cochrane databases. We found that H. pylori infection was associated with cholelithiasis, chronic cholecystitis, biliary tract cancer, primary sclerosing cholangitis, and primary biliary cholangitis but not with gallbladder polyps. While causal links have been reported, prospective longitudinal studies are required to conclude the association between H. pylori and gallbladder pathologies. Clinicians should be aware of the implications that H. pylori infection has on the management of these diseases.

• Tuberculosis and Respiratory Diseases
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• 제67권4호
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• pp.359-363
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• 2009

Immunoglobulin G4 (IgG4) related autoimmune diseases are characterized by high serum IgG4 concentrations, sclerosing inflammation of numerous IgG4-positive lymphoplasma cells of varying origin, and a positive response to steroid treatment. Autoimmune pancreatitis, sclerosing cholangitis, and retroperitoneal fibrosis are representative presentations of IgG4 related autoimmune disease. Herein, we describe 2 patients (40-years-old woman and 47-years-old man) diagnosed with pulmonary involvement of IgG4-related autoimmune disease. The patients were admitted for an evaluation of the lung mass or multiple lung nodules found on chest radiography. Surgical lung biopsies were performed and pathologic finding revealed lymphoplasmacytic sclerosing inflammation with numerous IgG4 positive cells. The patients had elevated serum total IgG and IgG4 levels. Treatment consisted of high dose methylpredinisolone (1 mg/kg/day) and demonstrated good responsiveness. However, one patient experienced 2 relapses while being tapered off of steroid treatment.