IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Autoimmune Diseases/complications/diagnosis ; Bile Ducts, Intrahepatic/pathology/ultrasonography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing/complications/*diagnosis/drug therapy ; Common Bile Duct/pathology/ultrasonography ; Humans ; Immunoglobulin G/*blood ; Immunohistochemistry ; Male ; Pancreatitis/complications/diagnosis ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed

No abstract available.
Bile Duct Diseases/*diagnosis/ultrasonography ; Bile Ducts, Extrahepatic ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Humans ; Hypertension, Portal/diagnosis ; Male ; Middle Aged ; Portal Vein/radiography ; Tomography Scanners, X-Ray Computed

Biliary cast describes the presence of casts within the biliary tree. It is resultant sequel of cholangitis and hepatocyte damage secondary to bile stasis and bile duct injury. Biliary cast syndrome was first reported in patient undergone liver transplantation. The pathogenesis of biliary cast is not clearly identified, but proposed etiologic factors include post-transplant bile duct damage, ischemia, biliary infection, or post-operative biliary drainage tube. Although biliary casts are uncommon, most of biliary cast syndrome are reported in the liver transplant or hepatic surgery patients. A few reports have been published about non-transplant or non-liver surgery biliary cast. We report two cases of biliary cast syndrome in non-liver surgery patients.
Acute Disease ; Ascariasis/diagnosis ; Bile Duct Diseases/*diagnosis/ultrasonography ; Bile Ducts/ultrasonography ; Cholagogues and Choleretics/therapeutic use ; Cholangiopancreatography, Endoscopic Retrograde/adverse effects ; Female ; Gallstones/diagnosis ; Humans ; Liver Cirrhosis, Biliary/diagnosis/drug therapy ; Male ; Middle Aged ; Pancreatitis/etiology ; Tomography, X-Ray Computed ; Ursodeoxycholic Acid/therapeutic use

No abstract available.
Aged ; Common Bile Duct/*pathology ; Common Bile Duct Diseases/*diagnosis/pathology/ultrasonography ; Diagnosis, Differential ; Female ; Humans ; Hyperplasia ; Magnetic Resonance Imaging ; Polyps/*pathology ; Positron-Emission Tomography ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Endoscopic retrograde cholangiopancreatography (ERCP) is the most accurate modality in diagnosis of choledocholithiasis. However, it carries some complications. Endoscopic ultrasonography (EUS) is less invasive than ERCP and used for the diagnosis of choledocholithiasis. Recent studies showed that a usefulness of EUS for the diagnosis of small choledocholithiasis without common bile duct (CBD) dilatation. For such a reason, ERCP is being replaced by EUS in the diagnosis of bile duct stones. The aim of this study was to investigate the accuracy of EUS for the diagnosis of choledocholithiasis without CBD dilatation. METHODS: A total of 66 patients with suspected choledocholithiasis without CBD dilatation were enrolled. EUS were performed in all cases within 48 hours after computed tomography (CT) or ultrasonography (US). Final diagnosis was obtained by ERCP or clinical course (minimum 6 months follow-up). We analyzed the accuracy of US, CT, and EUS, respectively. RESULTS: CT and US were performed in 51 and 15 cases, respectively. CBD stones were detected in 23 (35%) patients by ERCP. EUS showed 100% sensitivity, 95% specificity, 92% positive predictive value, and 100% negative predictive value for identifying CBD stones. CT or US showed 26%, 93%, 67%, and 70%, respectively. There were no EUS-related complications. CONCLUSIONS: EUS was more effective than CT or US and as accurate as ERCP for the diagnosis of small choledocholithiasis without CBD dilatation. Thus, EUS may help to avoid unnecessary diagnostic ERCP and its complication.
Adult ; Aged ; Aged, 80 and over ; Choledocholithiasis/pathology/*ultrasonography ; Common Bile Duct Diseases/diagnosis ; Diagnosis, Differential ; Dilatation, Pathologic/diagnosis ; *Endosonography ; Female ; Gallstones/ultrasonography ; Humans ; Male ; Middle Aged ; Sensitivity and Specificity ; Tomography, X-Ray Computed

Korean autoimmune pancreatitis (AIP) criteria 2007 was aimed to diagnose the wide spectrum of AIP with high sensitivity. The most crucial issue when caring for patients with suspected AIP is to differentiate AIP from pancreatic cancer. Pancreatic cancer can be distinguished from AIP by pancreatic imaging, measurement of serum IgG4 levels, endoscopic ultrasound guided fine needle aspiration and trucut biopsy, and steroid trial. Autoimmune pancreatitis is a rare systemic fibroinflammatory disease which can affect not only the pancreas, but also a variety of organs such as the bile ducts, salivary glands, retroperitoneum, and lymph nodes. Organs affected by AIP have a lymphoplasmacytic infiltrate rich in IgG4-positive cells. This inflammatory process responds dramatically to oral steroid therapy. Granulocytic epithelial lesion (GEL) positive AIP patients differ from GEL negative AIP patients in clinical features such as equal gender ratio, younger mean age, no increase in serum IgG4, no association with extrapancreatic involvement, no relapse, and frequent association with inflammatory bowel disease. Further investigation is needed to clarify the pathogenic mechanisms including more definite serological markers for theses two entities.
Autoimmune Diseases/*diagnosis/pathology/ultrasonography ; Common Bile Duct/radiography ; Fibrosis/pathology ; Humans ; Immunoglobulin G/blood ; Pancreatitis/*diagnosis/pathology/ultrasonography ; Salivary Glands/pathology

Biliary adenoma of the common bile duct is a rare disease found in biliary tract encounterd in biliary mass lesion, and it is difficult to differentiate from their malignant counterparts. Symptoms and signs of these tumors can mimic choleliathiasis and malignant tumors. Therefore, this tumor is rarely diagnosed preoperatively. In addition, there has been no reported case of a tubular adenoma of the common bile duct in Korea, until recently. We experienced a case of tubular adenoma of the common bile duct, which was diagnosed and partially resected by percutaneous transhepatic cholangioscopy. The patient was a 84-year-old male who showed abnormal liver function test. Abdominal ultrasonography showed a dilated common bile duct, and a soft tissue was observed on endoscopic retrograde cholangiography. This was initially thought as a stone, but it became partially detachable from the common bile duct during an endoscopic retrograde cholangioscopic basket removal. The mass lesion was partially resected by a percutaneous transhepatic cholangioscopic snare. This resected tissue was confirmed as a tubular adenoma.
Adenoma* ; Aged, 80 and over ; Biliary Tract ; Cholangiography ; Common Bile Duct* ; Diagnosis* ; Humans ; Korea ; Liver Function Tests ; Male ; Rare Diseases ; SNARE Proteins ; Ultrasonography

BACKGROUND/AIMS: Endoscopic ultrasonography (EUS) is now accepted as an essential tool for the diagnosis and the therapy of various gastrointestinal diseases. With regard to the biliary system, its high diagnostic sensitivity and specificity were reported recently. We assessed the clinical usefulness of EUS for the diagnosis of diseases of the liver, biliary tree, gallbladder and distal common bile duct. METHODS: We sent questionnaires about the clinical usefulness of EUS to the doctors who requested EUS examination for 32 patients with suspected diseases of the liver, gallbladder and around extrahepatic biliary tree from Aug. 2001 to Aug. 2002. We evaluated the answers and clinical characteristics of the patients. RESULTS: Patients were 17 males and 15 females with mean age of 61.8 years old. All received abdominal computed tomography (CT) before EUS and some had abdominal ultrasonography (USG) or endoscopic retrograde cholangiopancreatography (ERCP). EUS was more accurate and informative than other imaging modalities and gave definite final diagnosis for 31.2%. In 40.6%, EUS was helpful as an additional diagnostic tool. CONCLUSIONS: EUS was useful in 71.8% of the cases for the diagnosis of diseases of the liver, biliary tree, gallbladder and around distal common bile duct. EUS can be used as an important adjunct to USG, CT and ERCP.
Biliary Tract* ; Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct* ; Diagnosis* ; Endosonography* ; Female ; Gallbladder* ; Gastrointestinal Diseases ; Humans ; Liver* ; Male ; Sensitivity and Specificity ; Ultrasonography ; Surveys and Questionnaires

BACKGROUNDS: A mucin-producing bile duct tumor is a rare disease causing diffuse bile duct dilatation and intermittent obstructive jaundice due to mucin accumulation in the bile duct. A massive amount of mucin in the dilated bile duct is confirmed during surgery, endoscopic drainage, or percutaneous drainage. These tumors are usually slow-growing, well-differentiated papillary adenocarcinomas that shows intraductal spreading or an intraductal papillary growth pattern. PURPOSE: The purpose of this study was to evaluate the clinical, radiological, and histopathological characteristics of mucin-producing bile duct tumors. METHODS: We treated 10 patients with mucin-producing bile duct tumors during the recent 5 years. Clinical features were reviewed, including symptoms and signs, radiologic characteristics, operative findings, and pathological characteristics. RESULTS: The main clinical symptoms were recurrent abdominal pain, fever, chill, and intermittent jaundice. The characteristic radiologic findings were marked dilatation of the bile ducts distal to the tumors on computed tomography (CT) and ultrasonography and multiple, large, amorphous filling defects on cholangiography. In three cases, percutaneous transhepatic cholangioscopy (PTCS) was useful not only in making a diagnosis but also in delineating the extents of the tumors. In seven of ten cases involved (70%), the tumors were located mainly in the left intrahepatic duct. Pathologically, six (60%) cases were differentiated papillary adenocarcinoma. CONCLUSIONS: A mucin-producing bile duct tumor should be suspected if the patient has diffuse bile duct dilatation without definite evidence of stones, and it can be confirmed by a large amount of mucin secretion. If this type of tumor is suspected, careful preoperative evaluation, including PTCS, should be considered for making an accurate preoperative diagnosis and for determining the extent of the tumor.
Abdominal Pain ; Adenocarcinoma, Papillary ; Bile Duct Neoplasms ; Bile Ducts* ; Bile* ; Cholangiography ; Diagnosis ; Dilatation ; Drainage ; Fever ; Humans ; Jaundice ; Jaundice, Obstructive ; Mucins ; Rare Diseases ; Ultrasonography

BACKGROUND: Choledochal cysts are not so rare in far east Asian countries as in western countries and are found mainly in children. Reflux of pancreatic juice to the bile duct through anomalous pancreaticobiliary ductal union (APBDU) is considered to be an important etiologic factor. METHOD: To study the clinical characteristics of choledochal cysts in children and the significance of APBDU in this disease, we reviewed 80 choledochal cyst cases. APBDU is defined as a long common channel (>1.5 cm). RESULTS: Ages ranged from 19 days to 13 years, and the male to female ratio was 1 to 4 which revealed a predominance among females. The symptoms, in order of frequency, were abdominal pain (63%), jaundice (48%), and abdominal mass (26%); classic triads were present in 3 cases (4%). The diagnosis was made mainly with ultrasonography. Todani type I (88%) and IV (11%) were the two most common types of cysts. An operative cholangiography was performed in 80 cases, among which an analysis of the status of the pancreaticobiliary ductal union was possible in 21 cases. A long common channel (>1.5 cm) was found in all 21 cases, 16 cases being of the P- type and 5 cases of the B- type. A cyst excision and hepaticojejunostomy was performed in 79 cases of type I and type IV cysts, and a liver biopsy only was performed in one case of a type V cyst. A common bile duct stone was associated with one case and cholangitis was associated with two cases as postoperative complications. One patient expired due to heart disease. Seventy-ine patients were followed up for a mean period of 7.2 years after the operation. Seventy-ight patients are healthy now, and liver function tests have returned to normal level. One Carolie's disease patient has been followed up outpatiently without bad liver function. An intraoperative liver biopsy was performed in 73 cases. The results were normal in 3 cases; cholestasis was noted in 2 cases, inflammation in 26, fibrosis in 38, and cirrhosis in 4. CONCLUSIONS: APBDU seems to play an important role in the pathogenesis of choledochal cysts, especially type I and type IV cysts. Excision of the cyst is the procedure of choice for treatment because it eliminates the APBDU and prevents both hepatic cirrhosis and the potential for developing a malignancy.
Abdominal Pain ; Asian Continental Ancestry Group ; Bile Ducts ; Biopsy ; Child ; Cholangiography ; Cholangitis ; Choledochal Cyst* ; Cholestasis ; Common Bile Duct ; Diagnosis ; Far East ; Female ; Fibrosis ; Heart Diseases ; Humans ; Inflammation ; Jaundice ; Liver ; Liver Cirrhosis ; Liver Function Tests ; Male ; Pancreatic Juice ; Postoperative Complications ; Ultrasonography