Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nineteen cases of choledochal cyst are reviewed. Two distinct groups of patients were identified. Patients under one year of age, initially diagnosed as having biliary atresia, had a higher mortality rate, a higher incidence of severe cirrhosis with portal hypertension, and associated atresia or stenosis in the biliary tree. The second group, presenting between 3 and 20 years of age with more classic symptoms, had mild cirrhosis without portal hypertension and had associated choledocholithiasis and pancreatitis. It is suggested that the younger patients had a congenital form of cystic bile duct dilatation and that the older patients had an acquired form, perhaps related to a common channel with reflux of pancreatic juice into the common bile duct. Postoperative follow-up supports the current view that choledochocyst-jejunostomy with choleystectomy has a lower rate of long-term complications than does choledochocyst-duodenostomy.
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PMID:Choledochal cyst: a review of 19 cases. 99 19

In a review of pediatric autopsies from 1951 to 1985, we identified 40 cases in which pancreatitis was diagnosed pathologically. Twenty-six of these patients were under 4 years of age, and the male-to-female ratio was 1.5. Six groups of patients were identified: 10 with hepatobiliary disease, including 9 with biliary atresia; 7 with immunosuppressive therapy for tumors (n = 2), leukemia (n = 4) and aplastic anemia (n = 1); 6 with viral infections; 8 with congenital anomalies, including congenital heart disease (n = 3); and 9 with miscellaneous problems. Several patients had surgery and various intercurrent complications. Clinical features attributable to the pancreatitis included vomiting or excessive nasogastric drainage (60%), pleural effusions (40%), and abdominal pain (25%). However, the diagnosis was suspected clinically in only 5 of 40 patients. Our findings suggest several pathogenic mechanisms exist for childhood pancreatitis: biliary obstruction, infections, drug toxicity, immunosuppression (acting in synergy with drug toxicity, trauma, and low-flow states resulting from shock, heart failure, and vasculopathy.
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PMID:Clinicopathologic studies in childhood pancreatitis. 334 10

Primary sclerosing cholangitis (PSC) is a rare disease for which the etiology is unknown. Because abnormalities of the choledochopancreatic duct junction (CDPDJ) have been implicated in the pathogenesis of other rare biliary problems such as choledochal cysts and biliary atresia, we reviewed the cholangiographic anatomy of the CDPDJ in our patients with PSC. From 1955 through July 1983, 46 patients with PSC were seen at the University of California at Los Angeles Medical Center. In 20 of these 46 patients anatomy of the CDPDJ could be evaluated by review of endoscopic retrograde cholangiopancreatography (11 patients) or by reflux into the pancreatic duct during transhepatic, operative, or postoperative tube cholangiography (nine patients). Roentgenograms were evaluated for the length of common channel of the biliary and pancreatic ducts and the degree of reflux into and anatomic abnormalities of the pancreatic duct. All bile ducts were abnormal, and pancreatic duct reflux occurred in 14 of 42 patients (33%) who underwent transhepatic or tube cholangiography. An abnormally long (greater than 15 mm) common channel was present in two of 20 patients (10%), pancreatic duct reflux of more than 100 mm occurred in six of 14 patients (43%), and pancreatic duct abnormalities were seen in nine of 18 patients (50%). These data suggest that in patients with PSC abnormal pancreatic duct reflux and anatomy are common but variations in the anatomy of the CDPDJ are rare. However, altered function of the CDPDJ may contribute to excessive reflux into and anatomic changes of the pancreatic ducts and the moderate incidence of pancreatitis observed in patients with PSC.
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PMID:Anatomy of the choledochopancreatic duct junction in primary sclerosing cholangitis. 396 27

The authors present a review of the classification, aetiology, presentation, treatment and long-term outcome of children and adults with biliary atresia and choledochal cyst disease. Biliary atresia should be suspected in any infant with jaundice beyond the second week of life. Although the aetiology and pathogenesis remain unclear, early management with portoenterostomy has significantly improved the course of this disease. Recent advances in immunosuppression have made liver transplantation a valuable and necessary adjunct to biliary bypass. With choledochal cyst disease, adults, unlike children, often present with acute biliary tract symptoms or pancreatitis. The treatment of choice remains extrahepatic cyst excision and biliary bypass. This treatment has excellent long-term results that minimize the development of malignancy.
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PMID:Biliary atresia and biliary cysts. 951 2

Ascites is a poorly understood postoperative complication of orthotopic liver transplantation (OLT). It is associated with additional morbidity and can prolong hospitalization considerably. The incidence, the factors predictive of occurrence and the etiology of this complication are not known. The charts of 118 patients with 138 OLT were analyzed according to the following criteria: ascites lasting longer than the first 10 postoperative days, assessed by loss of ascitic fluid through drainage tubes, surgical wounds or paracentesis, with a peak volume of > or =10 mL/kg/day. Patients were divided into three groups: Group 1, no ascites; Group 2, ascites associated with postoperative complications, including chylus ascites; and Group 3, ascites not associated with postoperative complications. Postoperative ascites occurred in 43 of 138 OLT (31.2%). Patients with biliary atresia, preoperative portal hypertension, postoperative pleural effusion or at retransplantation had ascites significantly more often. In 32 of 138 (23.2%) OLT, ascites was associated with postoperative complications, including thrombosis, abdominal infections, intestinal perforation, biliary leak, pancreatitis, and chylus ascites. In 11 of 138 (7.9%) OLT, ascites was the only postoperative complication (group 3). Group three patients were significantly older, and had lower preoperative platelet counts and preoperative ascites more often than group 1 patients. The primary liver diseases were mainly cystic fibrosis of the pancreas, congenital hepatic fibrosis, and North American Indian childhood cirrhosis. The serum-ascites albumin gradient suggested a hepatic origin of ascites. Postoperative ascites is associated with the duration and degree of preoperative portal hypertension. We speculate that the mechanism involved includes a disproportion between venous blood volume and liver uptake capacity of the donor organ.
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PMID:Ascites after orthotopic liver transplantation in children. 1566 4

The purpose of this analysis is to study the clinical features, liver histology and the outcome after surgery in infants with choledochal cysts (CDCs), and to draw conclusions on the management strategy accordingly. The clinical features, liver histology and the outcome after surgery in 14 cases of CDCs treated before 1 year of age (the "infantile group") were compared with 52 cases that were diagnosed and treated between 1 and 12 years of age (the "classical pediatric group"). The differentiation of the "infantile choledochal cysts" from "biliary atresia with cystic dilatation" was based on the configuration of intrahepatic bile ducts on cholangiogram. Jaundice was the universal presenting feature in 14 patients in the "infantile group", the clinical presentation in 6 infants was indistinguishable from biliary atresia. On the other hand, pancreatitis as a presenting complication was seen exclusively in those presenting later, i.e. the "classical pediatric group". Fibrosis, as early as 4 weeks of age, was seen in all nine (100%) infants in whom a liver histology was available. Seven of these nine infants had bridging fibrosis (n = 4) or cirrhosis (n = 3) on liver histology. The outcome was satisfactory in 9 of the 14 (64.3%) infants despite bridging fibrosis (n = 4) or cirrhosis (n = 1) in 5 of these 9 babies. The CDCs presenting in infancy differ from the classical variety of later presenting pediatric CDCs in terms of their presentation, liver histology and outcome. They merit an emergent surgical treatment following which the outcome is satisfactory. Delay results in progression to end stage liver disease.
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PMID:Experience with choledochal cysts in infants. 1694 25

Chylous ascites are the accumulation of chylomicron-rich lymphatic fluid within the peritoneal cavity, resulting from obstruction or disruption of abdominal lymphatic channels. This rare condition may be associated with neoplastic or infectious infiltration of lymphatics, with pancreatitis, and with abdominal surgery. It may occur spontaneously in 0.5% of patients with cirrhosis; but only among a few liver transplantation cases. The management of chylous ascites is controversial; the variety of described treatments include repeated paracentesis, dietary control, peritoneovenous shunting, and surgical ligation of the disrupted lymphatic channels. In this article, we report 2 cases of rapid resolution of chylous ascites after liver transplantation following 5 days of treatment using a somatostatin analog and total parenteral nutrition (TPN). A 3.5-year-old girl and a 5-year-old girl underwent living related liver transplantation for biliary atresia and hepatoblastoma, respectively. Chylous ascites, diagnosed by ascitic fluid examination, developed within the 2 weeks after transplantation in the 2 cases. Treatment by fasting, TPN, and somatostatin analog resulted in rapid resolution of the ascites within 1 week. The prevalence of chylous ascites was noted in 1.6% of children (2/119) after liver transplantation. These cases highlight the use of somatostatin analog and parenteral nutrition in chylous ascites after liver transplantation.
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PMID:Chylous ascites after living related liver transplantation treated with somatostatin analog and parenteral nutrition. 1826 17

Recent studies demonstrated that viremia and extraintestinal rotavirus infection are common in acutely infected humans and animals, while systemic diseases appear to be rare. Intraperitoneal infection of newborn mice with rhesus rotavirus (RRV) results in biliary atresia (BA), and this condition is influenced by the host interferon response. We studied orally inoculated 5-day-old suckling mice that were deficient in interferon (IFN) signaling to evaluate the role of interferon on the outcome of local and systemic infection after enteric inoculation. We found that systemic replication of RRV, but not murine rotavirus strain EC, was greatly enhanced in IFN-alpha/beta and IFN-gamma receptor double-knockout (KO) or STAT1 KO mice but not in mice deficient in B- or T-cell immunity. The enhanced replication of RRV was associated with a lethal hepatitis, pancreatitis, and BA, while no systemic disease was observed in strain EC-infected interferon-deficient mice. In IFN-alpha/beta receptor KO mice the extraintestinal infection and systemic disease were only moderately increased, while RRV infection was not augmented and systemic disease was not present in IFN-gamma receptor KO mice. The increase of systemic infection in IFN-deficient mice was also observed during simian strain SA11 infection but not following bovine NCDV, porcine OSU, or murine strain EW infection. Our data indicate that the requirements for the interferon system to inhibit intestinal and extraintestinal viral replication in suckling mice vary among different heterologous and homologous rotavirus strains, and this variation is associated with lethal systemic disease.
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PMID:Role of interferon in homologous and heterologous rotavirus infection in the intestines and extraintestinal organs of suckling mice. 1849 62

Monoclonal antibodies against cell surface markers are powerful tools in the study of tissue regeneration, repair, and neoplasia, but there is a paucity of specific reagents to identify stem and progenitor cells in tissues of endodermal origin. The epitope defined by the GCTM-5 monoclonal antibody is a putative marker of hepatic progenitors. We sought to analyze further the distribution of the GCTM-5 antigen in normal tissues and disease states and to characterize the antigen biochemically. The GCTM-5 epitope was specifically expressed on tissues derived from the definitive endoderm, in particular the fetal gut, liver, and pancreas. Antibody reactivity was detected in subpopulations of normal adult biliary and pancreatic duct cells, and GCTM-5-positive cells isolated from the nonparenchymal fraction of adult liver expressed markers of progenitor cells. The GCTM-5-positive cell populations in liver and pancreas expanded greatly in numbers in disease states such as biliary atresia, cirrhosis, and pancreatitis. Neoplasms arising in these tissues also expressed the GCTM-5 antigen, with pancreatic adenocarcinoma in particular showing strong and consistent reactivity. The GCTM-5 epitope was also strongly displayed on cells undergoing intestinal metaplasia in Barrett's esophagus, a precursor to esophageal carcinoma. Biochemical, mass spectrometry, and immunochemical studies revealed that the GCTM-5 epitope is associated with the mucin-like glycoprotein FCGBP. The GCTM-5 epitope on the mucin-like glycoprotein FCGBP is a cell surface marker for the study of normal differentiation lineages, regeneration, and disease progression in tissues of endodermal origin.
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PMID:The GCTM-5 epitope associated with the mucin-like glycoprotein FCGBP marks progenitor cells in tissues of endodermal origin. 2276 Oct 39