• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.15 no.2
• /
• pp.122-126
• /
• 2012

Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive inherited disorder characterized by multiple recurrent episodes of severe cholestatic jaundice without obstruction of extrahepatic bile duct. We present the case of a 7-year-old boy with BRIC confirmed by mutation analysis in the ATP8B1 gene and typical clinical manifestation. Despite inheritance of BRIC, we detected a mutation on only one allele. To our knowledge, this is the first report of BRIC with a confirmed single heterozygote novel mutation in the ATP8B1 gene in Korea.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.7 no.2
• /
• pp.274-277
• /
• 2004

The heterotopic pancreas is defined as the presence of pancreatic tissue lacking anatomical and vascular continuity from the main body of pancreas. Its incidence has been reported as widely ranging from 0.55~13.7% in autopsy studies and 0.2% in upper abdominal laparotomies. The most common sites are the antrum of stomach, duodenum and proximal jejunum. But, lesions have also been found in the ileum, Meckel diverticulum, common bile duct and the esophagus. Most cases are incidentally encountered during surgery, and on rare occasions, epigastric pain, hemorrhage, gastric outlet obstruction and intussusception have been directly attributable to the presence of the heterotopic pancreas. A 3-month-old boy presented with 1-day history of vomiting and irritability. Intussusception was confirmed on ultrasound scan. At laparotomy there was an irreducible ileoileal intussusception, the intussuscepted portion of ileum was resected and end to end anastomosis was performed. Histologically, the mass was found to be composed of pancreatic tissue.

• The Korean Journal of Cytopathology
• /
• v.3 no.1
• /
• pp.1-11
• /
• 1992

From the one hundred forty eight patients with evidence of biliary tract obstruction, 275 bile samples were obtained from percutaneously placed biliary drainage catheters. Of the 148 patients, ova of Clonorchis sinensis were demonstrated in 17 patients (11.5%), with the epithelial cells. Among them, one case also demonstrated coexisting adenocarcinoma. In 105 patients, the medical records were available for review and the clinical diagnoses were malignancy in 99 patients and benign lesion in 6 patients. Of the 99 patients in which clinico-radiologic diagnosis were malignant, cytologic results were positive in 23.2%. Dividing the patients Into two groups, the ones with tumor of bile duct origin (group I) and the others with tumors producing extrinsic compression of bile duct, such as periampullary carcinoma, pancreas head carcinoma or metastatic carcinoma in lymph nodes from tumors of adjacent organs (group II), the cytologic results were positive in 37% and 11.6%, respectively. In patients with histologic confirmation, the positive correlation was found in 50% and 20% in group I and group II, respectively, with remarkable difference between two groups. There were no false positives in cytologic diangosis. The overall concordance rate of cytologic diagnosis with diagnosis of clinical investigation in both benign and malignant lesions was 27.6% and the diagnostic specificity was 100%.

• Journal of Yeungnam Medical Science
• /
• v.18 no.1
• /
• pp.51-58
• /
• 2001

Background: Cholestatic hepatitis is failure of bile to reach the duodenum with hepatocellular damage and no demonstable obstruction of the major bile ducts. The prognosis is usually good with recovery in less than 4 weeks after withdrawal of the offending drug. However, a prolonged course lasting over 3 months is possible and, in rare cases, progression to ductopenia with development of a vanishing bile duct syndrome occurs. A differential diagnosis with other causes of Chronic liver disease is needed. Materials and Methods: From January 1991 through January 2000, 14 patients diagnosed as cholestatic hepatitis by liver biopsy were included. The possible causative drug, clinical features, laboratory findings, and progression of cholestatic hepatitis were evaluated. The semiquantitative study of liver lesions was performed by two independent observers. Results: Causes of cholestatic hepatitis are 5 cases of oriental medicine, 3 cases of anti-tuberculosis medication, 1 case of ticlopidine and antibiotics and 4 cases of unknown causes. The clinical features of cholestatic hepatitis were jaundice, itching, urine color change, and general weakness. During 6 to 30 months, LFT of 5 patients showed prolonged elevation. Elevated total cholesterol ${\geq}$250 mg/dL in 6 patients, pheripheral blood eosinophilia in 5 patients, auto-antibody positive in 6 patients were observed respectively. The biopsies showed intralobular bilirubinostasis with a mixed portal inflammatory infiltration. Conclusion: In cholestatic hepatitis, durations of abnormal LFT are variable regardless of causative drugs. If cholestatic hepatitis progresses toward chronic course, viral hepatitis, primary biliary cirrhosis, and autoimmune hepatitis should be differentially diagnosed and sequential liver biopsies are needed.

• Journal of Digestive Cancer Reports
• /
• v.7 no.1
• /
• pp.18-21
• /
• 2019

Serine protease inhibitor Kazal-type 1 (SPINK1) is a gene expressed from pancreatic acinar cell which its mutation is known to be associated with chronic pancreatitis (CP) and pancreatic cancer. We report a case of a 47-years-old female with nausea and weight loss with yellow discoloration of skin. Initial imaging and endoscopic study led us to an impression of chronic pancreatitis with pancreatic cancer with common bile-duct dilation. Biopsy result was confirmed with pancreatic adenocarcinoma and additional imaging revealed lymph node and bone metastasis. Our genetic analysis revealed 194+2T>C mutation of SPINK1. Biliary obstruction was successfully decompressed by stent insertion and underwent chemotherapy and radiotherapy. Although there is accumulating evidence of association between SPINK1 mutation and CP, the relationship between SPINK1 mutation and pancreatic cancer in CP patient is an emerging concept. Genetic analysis should be considered in patients with young age especially when diagnosed with both CP and pancreatic cancer.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.3 no.1
• /
• pp.63-67
• /
• 2000

Purpose: Biliary atresia, one of the major causes of neonatal cholestais, is an idiopathic, serious disorder, affecting the newborn that results in complete obstruction of biliary tract. Successful reestablishment of bile flow is dependent on early surgical intervention, early diagnosis is imperative. The authors evaluate the utility of Tc-99m-labeled diisoprpyliminodiacetic acid (DISIDA) hepatobiliary scintigraphy in the diagnosis of biliary atresia. Methods: From January, 1995 to August, 1999, total 60 patients with neonatal cholestasis underwent Tc-99m DISIDA hepatobiliary scintigraphy at Asan Medical Center. Results: The undelying causes of neonatal cholestasis were biliary atresia in 14, neonatal hepatitis in 33, intrahepatic bile duct paucity in 9, and total parenteral nutrition induced cholestasis in 4. All patient with biliary atresia were interpreted correctely in DISIDA hepatobiliary scintigraphy, showing 100% sensitivity. Of the 46 patients with neonatal hepatitis and other causes, 37 patients had intestinal radioactivity showing 80% specificity. Conclusion: Visualization of DISIDA in the intestinal tract indicates patency of the biliary ducts and excludes the diagnosis of biliary atresia. But the absence of intestinal excretion on the DISIDA hepatobiliary scintigraphy dose not necessarily indicate biliary atresia.

• The Korean Journal of Nuclear Medicine
• /
• v.16 no.2
• /
• pp.71-80
• /
• 1982

In the present communication, the results will be reported on a clinical study of how well scintigraphic visualization of the hepatobiliary elements and several commonly used clinical liver function tests correlate each other in various diseases oft hepatobiliary system. The demonstrability of the biliary tract, gallbladder (GB) and duodenum was rather closely paralleled to serum bilirubin level and less closely to alkaline phosphatase and rather poorly to SGOT and SGPT. The biliary tree could not be visualized scintigraphically when bilirubin exceeded 10.5mg/dl. The usefulness of Tc-99m EHIDA [N-(2,6-diethylacetanilido) iminodiacetic acid, made by Amersham, England] hepatobiliary scintigraphy (Tc EHIDA HBS) in settling diagnostic controversy and ambiguity raised by oral cholecystography, intravenous cholangiography and ultrasonography in many hepatobiliary diseases is well known. The purpose of this investigation was to semiquantitatively evaluate the scintigraphic demonstrability of the hepatobiliary tract, GB and duodenum following intravenous injection of Tc-99m EHIDA in normal subjects and in patients with a disturbed liver function from various hepatobiliary diseases. The hepatobiliary scintigraphy was performed in 10 normal subjects and 39 patients with various hepatobiliary diseases (Table 1) at the Dept. of Radiology, St. Mary's Hospital Catholic Medical College, Seoul, Korea during 2 years period from September 1979. Scintigraphic examination was started at end of 3 minutes after intravenous injection of Tc-99m EHIDA in the amount of $50{\mu}Ci/kg$ and was continued until after 30 minutes at 5 minutes interval. The imaging was usually terminated when the tracer could be seen in the duodenum. Late scintigrams were obatained at 1 1/2, 3, 4 and 6 hours when reeded. Scintigrams were analyzed in terms of promptness and clarity of visualization of the biliary tree, GB and duodenum and demonstrability of these anatomical landmarks was correlated with the values of liver function tests. The demonstrability of the common hepatic duct, common bile duct, GB and duodenum was closely paralleled to the level of serum bilirubin when it is less than 10.5 mg/dl as shown in figure 1. However when the bilirubin exceeded 10.5 mg/dl the time of visualization between protracted reaching a flat curve or plateau around 10.5 mg/dl. The biliary tract could not be visualized when the bilirubin was higher than 10.5 mg/dl. The correlability between scintigraphic demonstration and serum alkaline phosphatase was less strong and between scintigraphic demonstration and SGOT and SGPT was rather poor. The present clinical study confirmed the usefulness and limitation of Tc-99m EHIDA hepatobiliary scintigraphy in visulizing and diagnosing the biliary system and duodenum when radiogrpahy and ultrasonography failed to provide useful informations. Scintigraphy was very helpful in the diagnosis of neonatal hepatitis, biliary atresia, cholecystitis and extrahepatic biliary obstruction. The hepatobiliary system and duodenum were visualized when serum bilirubin level was less than 10.5 mg/dl, SGOT 135 units, SGPT 114 units and alkaline phosphatase 52.2 KAU.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.2 no.2
• /
• pp.194-203
• /
• 1999

Purpose: Childhood pancreatitis has more various and somewhat different etiology than adult. Until now the analysis of severity in childhood pancreatitis were not well-known, although several studies have been made. Therefore, we studied the etiology and complications in childhood pancreatitis and analyzed whether Ranson and CT criteria could be applicated to evaluate the severity of childhood pancreatitis patients. Methods: The records of 30 patients with pancreatitis under 15 years of ages who were diagnosed in Asan medical center were reviewed. Age, sex, history, etiology, clinical features and treatment was reviewed in all patients but complications, Ranson and CT criteria were available in only 12 patients. Correlation between the number of complications and both Ranson and CT criteria were calculated with Spearman correlation coefficient. Results: 1. Median age at diagnosis was 7.3 years of age. 28 cases were acute pancreatitis and 2 cases were chronic pancreatitis. 2. Etiology: choledochal cyst(8 cases), drug (7 cases), trauma (4 cases), infection (3 cases), biliary stone or bile sludge (3 cases), idiopathic (2 cases) Hemolytic uremic syndrome, pancreatic duct obstruction, iatrogenic (1 case). 3. Local complications were ascites (5 cases), pseudocyts (4 cases) and systemic complications were hyperglycemia (4 cases), hypocalcemia (3 cases), pleural effusion (3 cases), etc. 4. Positive correlation was found between the number of complication and Ranson creteria (r=0.78, P=0.0016) and between the number of complication and CT criteria (r=0.65, P=0.015) in 13 cases. Conclusion: A trial to search the biliary duct anomaly may help to find the causes of childhood idiopathic pancreatitis, and both Ranson and CT criteria can be applicated to pediatric patients to evaluate the severity of childhood pancreatitis.