• Journal of Yeungnam Medical Science
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• v.3 no.1
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• pp.351-355
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• 1986

Sclerosing Cholangitis is an uncommon disease which involves either all or part of the extrahepatic biliary duct system and, occassionally, affects the intrahepatic biliary radicles. The disease has also been called "obliterative cholangitis" and "stenosing cholangitis", in reference to a progressive thickening of the bile duct walls encroaching upon the lumen. Several authors have proposed that the temr "primary sclerosing cholangitis" be reserved for cases in which there arew no associated diseases, and that all other cases be classified as "secondary sclerosing cholangitis". Many rigid criterias have been established for the diagnosis of primary sclerosing cholangitis. Recently the authors experienced one case of primary sclerosing cholangitis which was coincided with rigid criterias and was confirmed by operation with histologic examination. We present our case and review the literatures.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.1
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• pp.120-124
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• 2001

Sclerosing cholangitis is a chronic cholestatic disease characterized by inflammation and obliterative fibrosis of the bile ducts, leading to biliary cirrhosis and ultimately to liver failure. In children, sclerosing cholangitis is known to be associated with Langerhans cell histiocytosis, as well as with congenital immunodeficiencies and cystic fibrosis. Secondary sclerosing cholangitis is suspected in Langerhans cell histiocytosis with chronic cholestasis, liver dysfunction and portal hypertension. Unlike primary sclerosing cholangitis, the cholangitis associated Langerhans cell histiocytosis is destructive in nature and progresses more rapidly to biliary cirrhosis, therefore uniformly the prognosis is poor. In this setting, liver transplantation should be considered early in children with sclerosing cholangitis complicating Langerhans cell histiocytosis before end-stage liver failure and variceal bleeding. We experienced a case of secondary sclerosing cholangitis in Langerhans cell histiocytosis in a 2-year-old boy. We report this case with brief review of the related literatures.

• Asian Pacific Journal of Cancer Prevention
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• v.13 no.4
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• pp.1539-1541
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• 2012

Cholangitis is relatively uncommon but associated mortality is high due to the predisposition in people with underlying disease. For this recognition of contributing risk factors is necessary. Therefore, the present descriptive-analytical cross-sectional survey was designed to determine contributing risk factors for post-ERCP cholangitis in patients with pancreatic cancer. From 2005 to 2010, 110 consecutive cases of pancreatic cancer attending to a tertiary referral centre (Taleghani Hospital), Tehran, Iran were recruited. The patients all underwent stenting via endoscopic retrograde cholangiopancreatography (ERCP). On univariate analysis, a metallic stent type (95% confidence interval (CI) 1.025-11.34, P=0.037), having no jaundice (1.44-2.22, P=0.009), having no pain (1.32-1.91, P=0.026), a history of prior ERCP (1.16-10.37, P=0.020), and having a proximal biliary stone (1.002-5.93, P=0.046) were related to cholangitis. However on multivariate analysis, none of these factors were found to be contributing risk factors. Cholangitis is avoidable with adequate biliary drainage. Because success rates are higher and complication rates lower for endoscopists performing large volumes of ERCP, performance of the procedure should be concentrated as much as possible in institutions with endoscopists having adequate experience. Patients with a high risk for complications may be best served by referral to an advanced center.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.4
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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.

• The Journal of Internal Korean Medicine
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• v.26 no.4
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• pp.912-917
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• 2005

Caroli's disease is a non-obstructive dilatation of the intrahepatic bile ducts. It is a rare congenital disorder that classically causes saccular ductal dilatation, which usually it segmental dilatation. Caroli's disease is associated with recurrent bacterial cholangitis and stone formation. A 57 years-old female inpatient suffering from cholangitis due to Caroli's disease was admitted at Wonkwang University Jeonju Oriental Medicine Hospital. The patient was treated with herbal medicine such as Yongdamsagan-tang and acupuncture. As result, there was improvement of symptoms. The process and content of treatment and the patient's recovery over general symptoms is described.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.1
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• pp.91-97
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• 2007

The Overlap syndrome is characterized by a combination of the major hepatobiliary autoimmune diseases such as autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis. It is frequently accompanied by inflammatory bowel disease. Chronic lymphoplasmacellular osteomyelitis is characterized by recurrent episodes of bacterial osteomyelitis and is associated with autoimmune diseases (especially inflammatory bowel disease). We report the case of a girl who was diagnosed with ulcerative colitis and autoimmune hepatitis at 4 years of age and with the overlap syndrome with primary sclerosing cholangitis at 6 years. At 9 years, she was diagnosed with chronic lymphoplasmacellular osteomyelitis.

• The Journal of Internal Korean Medicine
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• v.22 no.4
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• pp.761-764
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• 2001

Recurrent Pyogenic Cholangitis(RPC) is a distinct clinical entity characterized by recurrent attacks of fever, chills, abdominal pain, and jaundice caused by bacterial infection. In 27, July, 2000, We treated a 40-year-old male patient, complained of these symptoms : severe abdominal pain, fatigue, indigestion, interval chilling. We diagnosed as stagnation of the liver-qi and Qi(肝鬱氣滯), gave herbal medicine composited with Herba Lysimachiae(金錢草), Spora Lygodii(海金沙), Herba Artemisiae Scopariae(茵蔯).etc. After treatment, main symptoms were disappeared and stone of common bile duct was removed. As a consequence, herbal medicine treatment was not toxic to liver function and good effect on RPC induced symptoms.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.126-129
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• 1999

A 6 and a half year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst ($4.5{\times}4.0$ cm in size) in the left hepatic lobe and another tubular dilatation ($2.0{\times}0.8$ cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangiodrainage(PTCD) and cystogram showed an ovoid cyst in the left hepatic lobe (Tsuchida type A), measuring $6.6{\times}5.0$ cm in size. Following drainage and administration of parenteral antibiotics she became afebrile and anicteric. However she continued to drain 45-150 cc of bile per day via the tube over the next 2 weeks. The patient successfully underwent intrahepatic cystojejunostomy with intraoperative ultrasonographic guidance. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, successfully managed with PTCD followed by an internal drainage procedure.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.3
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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.

• Advances in pediatric surgery
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• v.18 no.1
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• pp.12-17
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• 2012

The purpose of this study is to analyse clinical impact of specific MRI findings in liver in patients of long-term survivors after Kasai portoenterostomy (KPE). Twenty-eight patients who were underwent KPE were followed up more than 5 years. Macro-regenerative nodule (MRN) and beaded-duct dilatation (BDD) were considered as important findings in liver MRI. The association between these findings in MRI and clinical indicator, serum bilirubin level and history of cholangitis were evaluated. Sixteen patients (57.1%) were shown MRN in liver MRI. Therewere 14 patients(50%) whose MRI showed BDD. Serum total and direct bilirubin were 3.6 mg/dL and 1.8 mg/dL respectively in positive MRN group whereas 1.4 mg/dL and 0.7 mg/dL in negative MRN group (p = 0.427). Serum total and direct bilirubin level were 4.2 mg/dL and 2.1 mg/dL in patients with BDD negative group compare to 1.1 mg/dL and 0.5 mg/dL in BDD positive group (p = 0.281). The odds ratio to have cholangitis in the patient with MRN was 2.3 and 0.53 in patient with BDD in their MRI findings. MRN in liver MRI may suggest high bilirubin level and more chance to have cholangitis, but the findings of BDD may related to low bilirubin level and less change to have cholangitis.