Life-threatening diarrhea in atypical course of legionellosis

After eating a meal of poultry a 41-year-old man fell ill with severe diarrhoea, persistent high fever of around 39 degrees C, dehydration and somnolence. On admission to hospital physical examination was normal except for signs of dehydration. The blood count showed a leukocytosis (13,300/microliters) with 60% stab-form neutrophils. The erythrocyte sedimentation rate was raised to 49/82 mm. Also increased were the serum concentrations of creatinine (3.5 mg/dl), creatine kinase (179 U/l), lactate dehydrogenase (298 U/l) and C-reactive protein (16.8 mg/dl). Bacteriological and virological examinations of blood and stool were negative. A normal fluid and electrolyte balance was re-established. But as there was no improvement, ampicillin was administered, 2 g three times daily, then ciprofloxacin, 500 mg two times daily, and finally combined with metronidazole, 500 mg three times daily. Despite this treatment a chest radiograph on the tenth day revealed an infiltration in the left basal lung segment, and the legionella titre became positive at 1:2045. The antibiotic treatment was changed to 150 mg roxithromycin two times daily. The fever fell within 3 days and the diarrhoea stopped after 5 days. He was discharged free of symptoms after 24 days.     

A case of inflammatory pseudotumor of the liver causing elevated serum CA19-9 levels

Inflammatory pseudotumor of the liver is a rare lesion characterized by proliferating fibrovascular tissue admixed with inflammatory cells. A 50-yr-old Japanese man was hospitalized because of upper abdominal pain and high fever. Computed tomography revealed a poorly demarcated, low density mass in the left lobe of the liver, and abnormal laboratory findings included WBC 9340/mm3, CRP 10.5 mg/dl, and marked elevation of CA19-9 to 1167.9 U/ml. Endoscopic retrograde cholangiography showed irregularity of the intrahepatic bile duct of the left lateral segment, and the lateral segmental branches of the portal vein were not visualized on the venous phase of abdominal angiography. Ultrasound-guided liver biopsy was performed, but malignant disease, including intrahepatic cholangiocarcinoma, could not be completely ruled out. The patient underwent left hepatic lobectomy with lymph node dissection. Histopathological examination yielded a definitive diagnosis of inflammatory pseudotumor. The lesion was immunohistochemically stained for CA19-9 by the ABC method, and the biliary epithelium in severely inflamed portal canals was found to be positive. The markedly elevated preoperative level of CA 19-9 decreased to almost within the normal range and the patient remains well 2 yr 9 months after surgery, without any complications.     

Acute cholecystitis and persistent liver necrosis in mice provoked by isothiocyanate

Two known cholangiotoxic agents, alpha-naphthylisothiocyanate (ANIT) and p-phenylenediisothiocyanate (PDT), were administered in single doses to mice to study their effects on the gallbladder. Both compounds caused maximal bile duct necrosis and periportal hepatocytic necrosis at 24 hours. In contrast, the gallbladders were edematous but not necrotic at 24 hours after treatment. At 48 hours, and in some animals up to four days, severe cholecystitis was present, while bile ducts revealed progressive regeneration. The delay in the onset of gallbladder lesions was assumed to be the result of the toxic agent concentration in gallbladder bile after hepatic bile secretion was suppressed for 24 hours. The lesions provoked by PDT were similar to those induced by ANIT, except for a hemorrhagic component.     

Von Recklinghausen type I neurofibromatosis and neuroendocrine tumor (somatostatinoma) in a 50-year-old woman

HISTORY AND CLINICAL FINDINGS: A 50-year-old woman has had diffuse abdominal symptoms for approximately 2 weeks. For 30 years a von Recklinghausen's neurofibromatosis has been known. INVESTIGATIONS: Clinically and chemically there was a cholestasis (alkaline phosphatase 244 U/l, gamma GT 83 U/l) with uneventful values for transaminases and bilirubin. The hepatitis serology (A, B, C) as well as the AMA were negative. Somatostatin with 73 ng/l was slightly increased. Ultrasonography revealed a low-grade intrahepatic cholestasis, the ductus pancreaticus was extended to 9 mm, while endoscopic retrograde cholangiopancreatography showed an extended pancreatic duct without inflamed changes as well as an extended intra- and extrahepatic gall duct system without detecting a stone. The oesophagogastroduodenoscopy showed a polypoid tumor 3 cm above the Papilla Vateri which is part of a neuroendocrine tumor of the carcinoid type, immunoreactive towards somatostatin. TREATMENT AND COURSE: In the framework of the surgical intervention carried out by extirpation of the polypoid tumor above the Papilla Vateri by segment excision with a duodeno-duodenostomy. Within a period of 15 months, the patient was free from a tumor relapse or metastasis. CONCLUSION: Carcinoid tumors should always be considered in patients who have von Recklinghausen's neurofibromatosis in combination with abdominal pain in the duodenal area, especially if cholestasis parameters and bilirubin are high or if gastrointestinal bleeding occurs.     

Traumatic biliary stricture successfully treated by percutaneous transhepatic bile duct dilatation: a case report

A 24 year-old woman complained of obstructive jaundice 24 days after blunt abdominal trauma due to a traffic accident. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a stricture, 15 mm in length, at the common bile duct associated with upper bile duct dilatation. Jaundice was reduced by percutaneous transhepatic cholangio-drainage (PTCD). A 7 Fr-sized PTCD tube was exchanged for a larger-sized catheter for percutaneous transhepatic cholangioscopy expecting gradual dilatation of the stricture. Following the confirmation of satisfactory dilatation of the stricture, the catheter was removed. There was no recurrence of jaundice 16 months later in a follow-up study.     

K+ Channel density increases selectively in the endfoot of retinal glial cells during development of Rana catesbiana

BACKGROUND: We investigated the bile duct wall thickness measured on intraductal US in patients who had not undergone biliary drainage, with special attention to the influence of cancer at the distal bile duct, bile duct stones, obstructive jaundice, longitudinal cancer extension, and primary sclerosing cholangitis on wall thickness. METHODS: The study included 183 patients. Patients who had undergone previous biliary drainage were excluded. Intraductal US was performed by the transpapillary route with use of a thin-caliber ultrasonic probe (2.0 mm diameter, 20 MHz frequency). The bile duct wall thickness (width of the inside hypoechoic layer) was retrospectively measured on US images. RESULTS: Bile duct wall thicknesses of the common hepatic duct for the control group (n = 95), cancer at the distal bile duct group (n = 9), bile duct stone group (n = 56), and obstructive jaundice group (n = 17) were 0.6 +/- 0.3 mm (mean +/- SD), 0.8 +/- 0.5 mm, 0.8 +/- 0.6 mm, and 0.8 +/- 0. 5 mm, respectively. No significant differences (p > 0.05) were found between them. However, wall thickness for the cancer extension to the common hepatic duct group (n = 4, 2.0 +/- 0.4 mm) and sclerosing cholangitis group (n = 2, 2.5 +/- 0.4 mm) were significantly greater than in the other groups (p < 0.005). CONCLUSIONS: In patients who have not undergone previous biliary drainage, the bile duct wall thickness was not thicker in patients with obstructive jaundice. However, the duct wall was significantly thicker in patients with either longitudinal cancer extension or primary sclerosing cholangitis compared with that of other groups.     

Hepatic adenine nucleotides and microsomal cholesterol 7 alpha-hydroxylase activity in the obstructed and freely draining lobes of the liver after selective bile duct obstruction

BACKGROUND: The effect of selective bile duct obstruction (SBDO) on hepatic reserve function of the bile duct obstructed (BDO) and nonobstructed freely draining (FD) lobes of the liver is obscure. METHODS: The bile duct branches draining from the left lateral and median lobes of the liver were ligated for 4 and 10 days in rats, and hepatic reserve functions in BDO and FD lobes were assessed by microsomal cholesterol 7 alpha-hydroxylase activities and by hepatic adenine nucleotides and energy charge levels. The values were compared with those in the sham-operated control liver. Cholesterol 7 alpha-hydroxylase activities were determined by gas-liquid chromatography--mass spectrometry, and hepatic adenosine triphosphate (ATP), adenosine diphosphate (ADP), and adenosine monophosphate (AMP) levels with high-pressure liquid chromatography. RESULTS: The histological examination of the BDO lobes showed proliferation and formation of new bile ductules and fibrous connective tissues linking portal areas. Microsomal cholesterol 7 alpha-hydroxylase activities, hepatic energy charge and each adenine nucleotide level did not differ between FD and BDO lobes, and the values were similar to those in the sham-operated liver. CONCLUSIONS: Selective bile duct obstruction shows no adverse effects on microsomal and mitochondrial functions in both the BDO and FD lobes of the liver.     

13/14 Translocation in a man with reproductive failure. Mitotic and meiotic studies

Sixty-seven asymptomatic patients underwent repeat ultrasonography four to 16 months following cholecystectomy with or without common bile duct exploration. Fifty-six patients (84%) had common hepatic ducts with a normal internal diameter of 4 mm or less. Four patients had common hepatic ducts measuring 5 mm, and seven patients had common hepatic ducts measuring 6 to 10 mm. It is concluded that, although the common hepatic duct generally is not dilated following cholecystectomy, there is a significant minority (16%) of asymptomatic patients who have common hepatic ducts larger than 4 mm, and in these patients the duct may measure up to 10 mm.     

DHF with complication of acute pancreatitis related hyperglycemia: a case report

DHF is endemic in Indonesia, with incidence of 9.72/100,000 population and CFR of 2.5%. Acute pancreatitis is a rare complication in DHF, usually without hyperglycemia. We report here 1 patient of DHF grade II with complication of acute pancreatitis, and hyperglycemia which occured as a result of pancreatitis. A 24 years old female was referred from Santa Jusuf Hospital, with 5 days of fever and hematemesis. On physical examination we found slight fever and hematoma on her left leg. Laboratory examination revealed Hb 13.4 g%, WBC 8,500/mm3, Ht 42%, platelets 22,500/mm3, amylase 317 U/l, lipase 1,198 U/l and blood glucose 397 mg%. CT scan result of pancreas was consistent with acute pancreatitis. Diagnosis of dengue infection was made after the finding of positive IgM and IgG for dengue virus. After 18 days clinical symptoms and signs and laboratory results returned to normal.     

Multiple hepatic cysts along the intrahepatic bile duct--case report

A case of multiple hepatic cysts of the periductal gland located along the left intrahepatic bile duct is described. Ultrasonography and computed tomography disclosed many cystic lesions along the left portal vein in the left lateral segment of the liver. Percutaneous transhepatic cholangiography showed many compressed lesions. The resected specimen revealed multiple cysts of 2-7mm in diameter along the intrahepatic bile duct. Microscopically, cysts within the large Glisson's capsule were intermixed with lobuli of the periductal glands, thus suggesting periductal gland origin. Histopathological features of these cysts were similar to those of "multiple hilar cysts" reported by Nakanuma, but lack of portal hypertension and underlying chronic liver disease is the significant characteristics in this case which is different from "multiple hilar cysts".     

MR cholangiography of congenital biliary malformations in infancy

We presented MR cholangiography (MRC) of congenital biliary malformations in infancy. MRC was obtained during induced sleeping. In two cases of congenital dilation of bile duct, MRC revealed cystic or spindle dilatation of intra- and extra hepatic bile ducts. In one biliary atresia, MRC revealed the serpentine gall bladder and cystic dilatation of the extrahepatic bile duct without connection to the dilated hilar bile duct. MR cholangiography, which can be obtained noninvasively, is useful for the diagnosis and the preoperative assessment of congenital biliary malformations in infancy.     

Composition of biliary lipids and kinetics of bile acids after cholecystectomy in man

Postcholecystectomy biliary lipid composition and bile acid kinetics were studied in 24 women and 4 men. Hepatic bile was collected periodically for as long as 4 months without interrupting the enterohepatic circulation and without infecting the biliary system. In 23 patients with cholesterol gallstones, fasting biliary cholesterol made up 10.2% of total lipids in the steady state; in 5 patients with bilirubinate stones, saturation of fasting hepatic bile with cholesterol was lower (8.7% of total lipids). The percentage of deoxycholic acid after cholecystectomy was not higher than that of seven healthy, noncholecystectomized controls. Postcholecystectomy studies of diurnal variation of biliary lipids (7 patients) showed that postprandial hepatic bile had a significantly lower cholesterol saturation than fasting bile. Pool sizes of cholic and chenodeoxycholic acids were low (average 0.4 g/70 kg, each); total synthesis for both bile acids was normal (average 460 mg/day/70 kg), but fractional turnover rates of the two primary bile acids increased after cholecystectomy, probably due to more frequent recycling of the small bile acid pool.     

Yamakawa prosthesis with prolonged placement as a therapy concept exemplified by benign biliary stricture

AIM: As an alternative method to the operative revision of benign bile duct strictures, we report the use of a large-bore bile duct prosthesis (Yamakawa prosthesis) in one patient. METHODS: Bilateral percutaneous transhepatic implantation of Yamakawa prostheses (14 F right side, 12 F left side) was performed without adjunctive balloon dilatation. The prostheses were exchanged every 8 weeks under continuous antibiotic treatment and were finally removed after 8 months. RESULTS: Control cholangiography showed a normal intrahepatic biliary system on the right side and a 50% stenosis at the orifice of the left hepatic duct. Follow-up over 18 months showed no signs of recurrent disease. CONCLUSIONS: In comparison to balloon dilatation and implantation of metallic stents, prolonged bilateral splinting with large-bore Yamakawa prostheses seems to be an alternative for the treatment of benign bile duct strictures.     

Needs, demands and manpower balance. Dentists/populations. FDI Commission, 1996

BACKGROUND:The cause of extrahepatic portal vein obstruction in childhood is unknown. We investigated the anatomical features of extra hepatic portal vein obstruction to clarify its cause. STUDY DESIGN: We studied portal venous anatomy in 10 patients with extrahepatic portal vein obstruction without hepatic disturbances ranging in age from 1 to 7 years (mean age, 4.2 years) using ultrasonography, portal venography, cholangio-computed tomography, and magnetic resonance imaging. RESULTS: The extrahepatic portal vein was not obliterated, but it crossed over the common bile duct from the left to the right side at the cranial level of the pancreas and ran in a cranial direction along the right side of the common bile duct or coiled itself around the bile duct. Thus, the extrahepatic portal vein formed a tortuous eta-shape. CONCLUSIONS: The portal vein was not obstructed in patients with extrahepatic portal vein obstruction but formed a characteristic eta-shape by coiling itself around the common bile duct, suggesting that extrahepatic portal vein obstruction has an embryological cause.     

Effect of bile duct ligation on bile acid metabolism in rats

The effect of bile duct ligation on the quantitative and qualitative changes of bile acids in serum, liver, urine, and feces, and the concentration of cholesterol and phospholipids in serum and liver were examined in male rats. The concentration of bile acids in serum increased over 100-fold on day 5 but was lower than the 5-day level on days 10 and 15. The concentration in the liver also increased about 10-fold. beta-Muricholic acid predominantly increased but the secondary bile acids, deoxycholic acid and hyodeoxycholic acid, decreased. The urinary excretion of bile acids increased to about 40 mg/day per rat on the first day of bile duct ligation but this increase was reduced on day 2 to about half and remained at that level until day 24. These values exceeded that of fecal bile acids, 12 mg/day per rat, before bile duct ligation. The amount of bile acid sulfates in the urine was as low as 1% of the total. The urinary non-sulfated bile acids consisted mainly of beta-muricholic acid (60%) and cholic acid (20%), while the sulfates contained a considerable amount of unidentified acidic substances (40%) in addition to cholic acid and beta-muricholic acid. The concentration of cholesterol and phospholipids in serum markedly increased on day 5 but declined gradually thereafter. The liver cholesterol concentration did not change but the phospholipid concentration decreased. Fecal sterols did not change in both the total amount and composition. These data indicated that daily synthesis of bile acids, especially beta-muricholic acid, was accelerated in bile duct-ligated rats.     

Familial occurrence of congenital bile duct cysts

Congenital bile duct cysts are now a well-documented anomaly of the biliary tree, and have become more common in Japan. Familial occurrence of congenital bile duct cysts, however, is extremely rare, with only six reported cases in the literature. We report a familial pattern of congenital bile duct cysts in a mother and her daughter. A 33-year-old female was admitted for evaluation of right upper quadrant abdominal pain and fever 6 days after an uneventful delivery of her second child. A computed tomography (CT) and ultrasound scan (US) revealed an obstructed biliary tract. Percutaneous transhepatic biliary drainage was then performed, and a cholangiogram revealed a Scholtz type B choledochocele without an anomalous connection of the pancreaticobiliary ducts. Endoscopic US demonstrated that the choledochocele was associated with a stone in the cyst. A pylorus-preserving pancreatoduodenal resection was performed, and a histological study revealed that the choledochocele was lined by biliary mucosa without evidence of malignancy. The newborn infant had an abdominal tumour. An US and CT revealed a congenital bile duct cyst. An operation was performed and the intraoperative cholangiogram showed an Alonso-Lej type I congenital bile duct cyst with an anomalous connection of the pancreaticobiliary ducts. Whether congenital bile duct cysts are hereditary remains to be elucidated.     

Reversibility of hepatic mitochondrial damage in rats with long-term cholestasis

BACKGROUND/AIMS: Long-term bile duct ligation in rats is associated with secondary biliary cirrhosis and metabolic alterations, e.g. mitochondrial dysfunction. We performed the current studies to characterize the reversibility of hepatic mitochondrial dysfunction after reversing biliary obstruction by Roux-en-Y anastomosis. METHODS: Rats were studied after 4 weeks of bile duct ligation, and after 5 or 14 days of reanastomosis. Control rats were pair-fed to treated rats and all rats were studied after starvation for 24 h. Mitochondria were isolated by differential centrifugation and enzyme activities determined by spectrophotometric methods. RESULTS: In comparison to controls, plasma beta-hydroxybutyrate concentrations were decreased in bile duct ligated rats (200+/-70 vs. 790+/-200 micromol/l) and remained decreased after relief of biliary obstruction. In contrast, plasma free fatty acids were not different between controls and treated rats. Oxidative metabolism of L-glutamate, succinate and duroquinol was decreased in liver mitochondria from bile duct ligated rats. After relief of biliary obstruction, the metabolism of L-glutamate and duroquinol normalized quickly, whereas succinate metabolism remained impaired. Similar results were obtained for the mitochondrial oxidases in disrupted mitochondria. The activities of complex I, II, III and V of the respiratory chain were reduced in bile duct ligated rats. After relief of biliary obstruction, complex I and III normalized quickly, whereas complex II and V remained impaired. Oxidative metabolism of long-chain fatty acids by isolated liver mitochondria was decreased in bile duct ligated rats and did not recover after relief of biliary obstruction. CONCLUSIONS: Long-term cholestasis in the rat is associated with a decrease in specific functions of liver mitochondria which recover only partially after Roux-en-Y anastomosis. The persistence of decreased mitochondrial fatty acid metabolism cannot be explained by impaired activity of the respiratory chain, but is more likely due to alterations in mitochondrial beta-oxidation.     

Mechanical removal of the smear layer

Complications after nonoperative management of hepatic trauma are rare but include persistent biliary fistula in 4% of cases. Therapy usually involves surgical drainage or hepatic resection to control the fistula. The authors present a case of hepatic biliary fistula treated nonoperatively with percutaneous drainage and endoscopic sphincterotomy. A 16-year-old girl suffered a grade III parenchymal liver fracture to the right lobe in an automobile accident. She was hemodynamically stable with no coexistent injuries and was treated nonoperatively. Over 2 weeks her total bilirubin rose to 3.2 mg/dL, and alkaline phosphatase was 463 U/L. Ultrasound showed free intraperitoneal fluid, and 2 L of bilious fluid were retrieved by paracentesis. A radionuclide scan confirmed massive extravasation of isotope from the right lobe. Two drains percutaneously placed over the parenchymal fracture produced 500 to 600 mL of bile daily. Endoscopic retrograde cholangiopancreatography (ERCP) 1 week later showed a normal extrahepatic biliary system. A 1-cm sphincterotomy was performed without difficulty. Within 72 hours, percutaneous drains produced only 35 mL per day. The follow-up radionuclide scan showed no evidence of extrahepatic biliary extravasation, and 3 weeks later the drains were removed. Six months after the accident, results of the computerized tomography scan and liver function tests were normal. It was believed that endoscopic sphincterotomy reduced fistula output by decreasing the intrabiliary pressure caused by the ampulla, thus favoring internal drainage. This case demonstrates the effectiveness of endoscopic sphincterotomy as an alternative to direct surgical intervention for the management of posttraumatic biliary fistula.     

Spontaneous rupture of an intrahepatic bile duct with biloma treated by percutaneous drainage and endoscopic sphincterotomy

A case of spontaneous rupture of an intrahepatic bile duct with biloma formation treated by percutaneous drainage and endoscopic sphincterotomy is reported. A 73-yr-old woman was admitted with fever and abdominal pain. There was no past history of abdominal surgery, instrumentation, or trauma. Ultrasound and computed tomography revealed a massive fluid collection in the abdominal cavity. Endoscopic retrograde cholangiography demonstrated extravasation of contrast medium from a distal biliary radicle in the left lobe of the liver. After successful treatment by percutaneous drainage and endoscopic sphincterotomy, the patient did well. Ultrasound and computed tomography showed resolution of the biloma. Nontraumatic bilomas are very rare: in fact, only 24 cases of spontaneous biloma have been reported. Endoscopic treatment for patients with spontaneous bilomas can be safe and effective, and should be considered.     

The lymph nodes of the porta hepatis

The area under the liver was dissected in 27 human autopsy specimens to search for lymph nodes in the fissures. Nodes were present in all instances. They were in the transverse fissure, posterior to the portal vein, posterior to pars transversus of the left portal vein and associated with the left hepatic artery. The size varied from 2 millimeters to 2 centimeters. Each node was histologically confirmed. Nodes were infrequent and small on the right. Nodes were not found between the portal vein, hepatic artery and bile ducts in the fissures. Nodes were found outside the fissures in the fascia between the bile duct and hepatic artery. Occlusion of the portal vein and hepatic artery could be expected before occlusion of the bile duct. Node enlargement in the transverse fissure is anticipated as a rare cause of jaundice.