, Tae Hun Kim, Kwon Yoo, Ye Ji Han, Jeong Eun Choi, Ji Yoon Kim, Min-Sun Cho Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease that may progress to end stage liver cirrhosis. Benefits of ursodeoxycholic acid (UDCA) treatment has been investigated through large clinical studies. However, most of the studies were done in western countries and recent increase in prevalence of this relatively uncommon chronic liver disease draws attention in Korea. As early UDCA treatment effectively prevent the grave consequences of PBC progression, early diagnosis and lifelong management with UDCA is important. This study was designed to investigate the clinical features of PBC and response rates of UDCA treatments in Ewha Womans University Medical Center.
Clinical data of PBC patients diagnosed between 2001 and 2014 at Ewha Womans University Medical Center were analyzed retrospectively.
A total of 35 patients with mean follow-up duration of 42 months were enrolled. At the diagnosis, 72.7% of the patients were asymptomatic, 5.7% had decompensated liver cirrhosis. The mean serum alkaline phosphate (ALP) level was 2.65 times upper limit of normal. UDCA was prescribed in 91.4% of the patients (n=32), among which 77.4% exhibited biochemical responses defined as serum ALP less than 2 upper limit of normal at 6 months (Mayo criteria).
Most PBC patients were asymptomatic at the time of diagnosis and the average biochemical responses rate to UDCA treatment were ranged from 60.0% to 78.9% according to various response criteria. To elucidate the clinical features and courses of Korean PBC patients in detail, larger scale investigations and longer clinical follow up studies are warranted.
, Jin Young Choi, Ho Jun Kang, Ji Hee Sung, Sang Jong Park, Sun Hong Yoo, Young Min Park Vitamin A deficiency can occur as a result of malnutrition, malabsorption, or poor vitamin metabolism due to liver disease and night blindness might develop as the first symptom. Although there have been foreign reports about night blindness due to vitamin A deficiency which was derived from liver cirrhosis, primary biliary cirrhosis, intestinal bypass surgery or bariatric operation, it is hard to find reports about night blindness after percutaneous transhepatic biliary drainage for external bile drainage. We report a case of night blindness derived from fat-soluble vitamin A deficiency developed after long-term (18 months) external bile drainage for benign biliary stricture occurred after left hepatic lobectomy and hepaticojejunostomy due to the Klatskin tumor (IIIb). Her night blindness and low serum retinol level (0.02 mg/L) was dramatically improved after vitamin A supplementation. We recommend lipid-soluble vitamin supplementation on the case of long-term external bile drainage.
The role of Helicobacter pylori(HP) in benign and malignant pancreatico-biliary tract disease is concerned in recent papers. The urease gene of Hp were found in human bile, and bacteria morphologically resembling Hp were found in resected gallbladder mucosa from patients with gallbladder disease. It was hypothesized that there is an association between the presence of Hp in bile and pancreatico-biliary disease. The aims of this study are to examine if Hp exist in the bile juice and to investigate whether Hp plays a role in the pancreatico-biliary disease.
Thirty-eight patients (18 males and 20 females, mean age 71 ?27yr ; range 45-92yr) with gallstone and malignant pancreatico-biliary disease were enrolled in this study ; 23 cases were gallstone diseases, 10 cases were cholangiocarcinomas, and 5 cases were pancreatic cancers. Thirty-eight controls were age- and sex-matched and enrolled from subject attending routine medical check-up. The presence of Hp in stomach was confirmed by ?4C-breath test. The polymerase chain reaction (PCR) assay was used to detect the Hp in bile.
The Hp-positive rate in stomach was much higher in control (26/38,68.4%) than the patients with pancreatico-biliary disease(l1/38, 28.9%) (p<0.01). The Hp-positive rate in bile of pancreatico-biliary disease was 18.4% but, there is no relation between of the presence of Hp in the stomach and in the bile (p=0.33). Also there was no significant difference of the presence of Hp in bile (p>0.05) and stomach (p=0.28) between benign and malignant disease.
The Hp-positive rate in bile was similar in benign and malignant pancreatico-biliary disease. But Hp may not be important risk factor in pancreatico-biliary disease in Hp-prevatent country like south Korea.
, Sun Young Yi, Sung Ae Jung, Kwon Yoo Magnetic resonance cholangiography(MRC) has emerged as an accurate and noninvasive modality for the evaluation of the biliary diseases. We performed this study to access the accuracy and efficacy of MRC for the diagnosis of obstructive biliary diseases.
From March 1999 to September 2000, 87 patients with obstructive biliary diseases(24 malignant cancers, 55 cholelithiasis, 8 other obstructive biliary diseases) underwent MRC.
MRC correctly revealed the obstructing biliary lesions and the cause of obstructions in 100% and 92% of the cases of malignancy respectively. The sensitivity and specificity of MRC were 94%, and 82% for extrahepatic bile duct stones, 94%, 100% for intrahepatic bile duct stones, and 84%, 100% for gallbladder stones retrospectively. In the other cause of biliary obstructions(3 benign biliary stricture, 2 acalculous cholecystitis, 2 xanthogranulomatous cho-lecystitis, and 1 extrinsic compression due to lymphadenopathy), MRC correctly showed the level of obstructions in all patients. But, MRC could not diagnose accurate causes of the obstruction except 3 patients(1 acalculous cholecystitis, 1 xanthogranulomatous cholecystitis, and 1 extrinsic compression due to lymphadenopathy).
This study demonstrated that MRC was able to accurately identify the level and the cause of biliary obstructions in both malignancy and cholelithiasis. MRC was easily performed for a short duration and was a noninvasive diagnostic modality for assessing the biliary tree. Therefore, MRC was very efficacious clinically for the diagnosis of obstructive biliary diseases.
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