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Purpose The aim of this article is to differentiate neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) from biliary atresia (BA) by total hexose. Methods A total of 11 patients with NICCD, 29 patients with BA, and 4,898 children as controls were involved in this study. The blood concentration of amino acids, carnitine, acylcarnitines, and total hexose were measured in dry blood spots (DBS) using tandem mass spectrometry (MS/MS). Results In the patients with NICCD, the blood concentration of the total hexose (15.3 ± 9.0 mmol/L vs. 7.3 ± 2.7 mmol/L; p < 0.001), citrulline (Cit) (197.9 ± 93.7 µmol/L vs. 17.5 ± 7.4 µmol/L; p < 0.001) were higher than those of patients with BA. Using total hexose (> 10 mmol/L), Cit (> 55 µmol/L) to diagnose NICCD, the sensitivity and specificity were 66.7 and 97.8% and 90.0 and 99.1%, respectively, and all of the areas under the receiver-operating characteristic curves were greater than 0.85. Conclusion Elevated total hexose in DBS measured by MS/MS associated with elevated amino acids, especially Cit can be used to diagnose NICCD and differentiate it from BA., (Georg Thieme Verlag KG Stuttgart · New York.)

Icteric Hepatocellular Carcinoma (HCC) is known to cause intraluminal biliary obstruction by one of three mechanisms: hemobilia from the tumour, migration of tumor debris, or continuous growth along the biliary tree. It is however a very rare presentation of HCC and an important differential diagnosis in the approach to obstructive jaundice. We report a case of a recurrent intraductal hepatocellular carcinoma. The patient initially underwent surgical resection of segment five HCC nine months ago with clear margins. The patient now presents with obstructive jaundice and imaging showed a right intraductal tumour involving the confluence, left and common hepatic ducts. He underwent a right hepatectomy and bile duct tumour thrombectomy despite the apparent absence of a parenchymal tumour. Histological examination showed a 2 mm focus of parenchymal tumour with extension of the tumour into the bile duct. In this case report, we reviewed the literature and describe the different surgical approaches to intraductal hepatocellular carcinomas and discuss the pathological aspects of these bile duct tumour thrombus. We report the favourable outcome of surgical resection for intraductal hepatocellular carcinoma and emphasize that intraductal HCC is not a late stage of disease and adequate surgical resection can still provide a reasonable disease free survival.

Background/aims: Management of post-cholecystectomy bile duct injuries may, in certain cases, require hepatectomy. In the literature, indications for hepatectomy in this setting are not clear., Methodology: A retrospective review of our database for patients referred for post-cholecystectomy bile duct injuries from January 2003 to January 2008 was performed., Results: We present three cases of hepatectomy among 45 patients (6.7%) referred for post-cholecystectomy bile duct injuries. Two of these patients had been referred after one or more previous attempts at operative repair in an outside hospital. In one patient, the decision to perform hepatectomy was based on the proximal pattern of biliary stricture aggravated by disruption of the right hepatic artery, leading to irreversible secondary sclerosing cholangitis. Biliary stricture also involved bifurcation and was complicated by liver atrophy in the 2 other patients. Despite complication(s) in 2 out of 3 patients, the long-term outcome was good in all cases., Conclusions: In our experience, the indication for hepatectomy in post-cholecystectomy bile duct injuries, combined with vascular injuries and liver atrophy in symptomatic patients.

Background/aims: Biliodigestive anastomoses to small bile ducts after extensive liver resection or central bile duct remain difficult. The inherent problems associated with this particular procedure have not been widely discussed. In order to demonstrate that reconstruction of segmental or subsegmental bile ducts with mucosa-to-mucosa alignment using transanastomotic stents can reduce morbidity associated with the procedure, we have reviewed our experience with small biliodigestive anastomosis after liver resection., Methodology: Thirteen patients were operated on from 1995 to 1997. Twelve had malignant diagnoses. A comprehensive standardized technique included the use of short or trans-intestinal trans-anastomotic stents and single layer interrupted absorbable sutures with mucosa-to-mucosa alignment. In the patients, in-hospital morbidity and mortality with focus on bile leaks and long-term signs of cholangitis and stricture formation were investigated with a median observation period of 42 months., Results: Eleven underwent extensive liver resections and two had complicated central resections including the bile duct confluence. The procedures included 40 transected bile ducts and 32 anastomoses to the jejunum. One patient died as a result of rupture of a splenic arterial aneurysm caused by a pancreatic pseudocyst. Other complications which occurred in seven patients were intra-abdominal fluid collection (n=5), wound infection (n=1), minor bile leakage from the liver cut surface (n=1), and pleural effusion (n=4). Postoperatively four patients, who had local recurrence with jaundice, showed cholangitis, whereas the remaining nine patients showed no signs of cholangitis., Conclusions: Our technique for reconstructing small bile ducts with mucosa-to-mucosa alignment is considered adequate from the viewpoint of short- and long-term results.

A 45-year-old man was suffering from abdominal pain and vomiting. He was admitted to our hospital with a diagnosis of ileus and obstructive jaundice. He had undergone Roux-en-Y anastomosis for choledocholithiasis 14 years earlier. A computed tomography scan revealed a dilated afferent loop and dilated intrahepatic bile duct. Upper gastrointestinal examination with contrast medium and percutaneous transhepatic cholangiography showed a high intestinal obstruction around the jejunojejunal anastomosis. The patient underwent laparotomy based on a diagnosis of obstructive jaundice due to ileus. During the operation, he was found to have internal herniation of the small bowel through a rent in the mesentery around the Roux-en-Y anastomosis for choledochojejunostomy. The hernia was reduced, and bowel resection was performed due to stenosis of the afferent loop. Jejunojejunal anastomosis was re-performed and the defect in the mesocolon was closed. Internal herniation after Roux-en-Y anastomosis is a rare sequela, but it should be recognized that this complication can occur after Roux-en-Y anastomosis. For prevention of internal herniation around the Roux-en-Y limb, secure closing of the mesenteric defects is important.

We report a case of benign bile duct stricture that could not be differentiated from intrahepatic bile duct carcinoma preoperatively. The patient was a 79-year-old man. Computed tomography showed dilatation of the intrahepatic bile duct in the left lobe. Direct cholangiography showed segmental stricture of the left bile duct. Angiography showed narrowing of the left hepatic artery. Although bile cytology did not show malignant cells, we suspected intrahepatic bile duct carcinoma preoperatively. We performed extended left hepatic lobectomy. Histopathologic examination of the resected duct also showed no malignant cells; fibrosis with infiltration by lymphocytes was seen at the bile duct stricture. In addition, the resected liver specimen showed sclerotic change in the intrahepatic arteries. The postoperative course was uneventful for more than 26 months, without recurrence or cholangitis. We encountered a very rare case of benign segmental bile duct stricture, which was difficult to differentiate from bile duct carcinoma. We think the biliary stricture was secondary to atherosclerosis which may have caused bile duct ischemia.

Background/aims: The aim of this retrospective study was to determine the efficacy and safety of percutaneous T-tube placement for malignant hilar obstruction. Results were compared with self-expanding metal stents., Methodology: Between June 1989 and April 1999, 24 consecutive patients with type II, III, IV hilar obstructions underwent T-tube (n = 12) and metal stent placement (n = 12). A 9-F T-tube was placed percutaneously into the right and left hepatic ducts. Metal stents (diameter: 6-10 mm) were placed in both ducts., Results: Stent placement was successful in all patients. Early complications occurred in 2 patients in the metal stent group (arterio-biliary fistula and cholangitis). In the T-tube group, the 30-day mortality rate were 25%. These were not procedure related. The median patency period in the metal stent group (365.0 +/- 113.3 days) was longer than in the T-tube group (167.0 +/- 71.1 days). There were no significant differences in the median survival rates (224.0 +/- 39.0 days vs. 197.0 +/- 104.8 days)., Conclusions: Internal T-tube placement for drainage of malignant hilar obstruction is safe and associated with few complications. The advantage of this procedure is removability of the T-tube stents.

The palliative treatment of malignant biliary tract obstructions using a metal stent is now an established procedure in clinical practice. An endoscopic, transpapillary approach is the first choice for implantation of the stent. If it is not possible to insert the stent in this way, which is often the case with high obstructions, a percutaneous approach is chosen. It appears to be beneficial to use a metal stent with a fine-meshed net such as, for example, the Wall stent. Metal stents have a higher patency rate than plastic stents so that the primary choice of a metal stent is justified. Coated stents have not yet shown any major advantages. In cases of stent occlusion, the coaxial implantation of a plastic stent seems to be the most efficient. In cases of benign biliary tract stenoses, a metal stent should only be implanted after a careful evaluation of all possible surgical modalities and exploitation of balloon dilatation and long-term splinting methods.

The main complications of endoscopic retrograde cholangiography and sphincterotomy are bleeding, pancreatitis, perforation and sepsis. Two cases of unexplained prolonged cholestatic jaundice in patients who underwent endoscopic retrograde cholangiography (ERC) for biliary obstruction due to choledocholithiasis are reported. The patients were admitted because of right upper quadrant pain, vomiting and jaundice. Laboratory tests showed increased levels of total and conjugated serum bilirubin and increased alkaline phosphatase. Ultrasound examination showed cholelithiasis and choledocholithiasis with bile duct dilatation. ERC with sphincterotomy was performed and gallstones obstructing the common bile duct were removed endoscopically. Following ERC and despite complete patency of the biliary tree, a progressive increase of total and conjugated bilirubin and of alkaline phosphatase was noted, associated with itching and total stool discoloration. The insertion of nasobiliary drain did not improve the jaundice. Prednisolone treatment for 12 days was associated with progressive restoration of serum bilirubin alkaline phosphatase to normal levels. It was postulated that the radiocontrast material used may have acted toxically on the liver with disruption of the canalicular plasma membrane. It is proposed that intrahepatic cholestasis should be added in the list of complications of endoscopic retrograde cholangiography.

A case of intrahepatic cholangiocarcinoma with intrabile duct tumor growth is presented. A 70-year-old female with prolonged obstructive jaundice and cholangitis was diagnosed with a mass lesion in the left lobe of the liver accompanied by an intrabile duct component which caused hilar bile duct obstruction. Percutaneous transhepatic biliary drainage (PTBD) was performed via three routes with intensive care of the drainage catheters over a 2-month period to alleviate jaundice and cholangitis. Extended left hepatic lobectomy with combined resection of extrahepatic bile duct and regional lymph nodes were carried out curatively. Macroscopically, a tumor formed a 8 x 6 cm mass with a scalloped margin in the left lobe and had an intraductal component with a round smooth surface protruding from the lumen of the left hepatic duct into the common bile duct. The histologic type of the tumor was well differentiated tubular adenocarcinoma. The intraluminal tumor grew expansively without invasion to the bile duct wall. The postoperative course was uneventful and the patient has remained in good health without recurrence for 1 year and 3 months since surgery. This case report discusses a rare extension pattern in ICC and the management of malignant hilar obstruction during radical surgery.

This 28-year-old male, a hepatitis B virus (HBV) carrier, received cadaveric renal transplantation and was maintained on cyclosporin A and prednisolone. Jaundice occurred 8 months after the transplantation and he died 2 weeks later due to hepatic failure. The liver histologic findings were compatible with fibrosing cholestatic hepatitis (FCH), which is caused by HBV and has only been reported in liver allografts of orthotopic liver transplantations. This is the first case of FCH developing in a renal transplant recipient. The report illustrates that (1) FCH is also a unique histologic entity in renal transplantations; (2) FCH might occur in a liver chronically infected by HBV without co-existing hepatitis D virus; and (3) FCH can cause fulminant hepatic failure within one year after transplantation while the patient is still in an immunosuppressed state.

Obstructive jaundice due to hilar cholangiocarcinoma is difficult to decompress because of the location of the tumor. We used external radiation alone for biliary decompression and reviewed its efficacy in this study. Subjects comprised 14 patients diagnosed as having inoperable hilar cholangiocarcinoma by ultrasonography, percutaneous transhepatic cholangiography, and CT scanning. The total bilirubin level on admission ranged from 0.4 to 34.6 mg/dl (mean: 11.0 mg/dl). These patients were irradiated with a 4MeV linear accelerator using parallel opposing fields measuring from 7 x 7 cm to 8 x 10 cm. The total radiation dose ranged from 50 Gy to 60 Gy and in fractions of 1.8-2.0 Gy per day. No patient underwent further biliary decompression after percutaneous transhepatic cholangiography, and irradiation was performed immediately after diagnosis. Eleven of the 14 patients received the full dose of external radiation. Three patients discontinued radiotherapy because of severe vomiting and nausea, pneumonia, and a hemorrhagic gastric ulcer. In 10 of the 11 patients, the serum total bilirubin level returned to normal (p < 0.005) and no cholangitis occurred. Obstructive jaundice recurred in one patient, and serum total bilirubin returned to normal again after further irradiation. Eight of the 11 patients could be discharged from hospital and returned to society. The survival time of the 11 patients ranged from 3 to 25 months and the 12-month survival rate was 50% (Kaplan-Meier method). This study suggests that external radiation therapy is an effective treatment for biliary decompression in patients with unresectable hilar cholangiocarcinoma.

In the recent years new avenues have been opened in the treatment of ICP, a complex disorder that seems to represent a maladaptation of some young and otherwise healthy women, to estrogens or other sex hormones. New drugs have been shown capable of providing promising therapeutic effects either on pruritus, the main distressing symptoms of cholestasis (such as epomediol, silymarin) or both on pruritus and some biochemical abnormalities (such as UDCA). Future clinical and experimental studies should provide better insight into the pathogenesis of cholestasis, the mechanisms of bile formation and secretion, and the metabolism of estrogens and other sex hormones and their alteration relationship to cholestasis, a disorder that is highly prevalent in humans.

Within a 4-year period, 24 patients were treated by internal biliary drainage using self-expanding wall stents. In 3 patients, a benign and in 21 patients a malignant obstruction were present. The stents were successfully placed in all cases with an incomplete tumour coverage in three cases. Complete relief of icteric symptoms was seen in 18 out of 24 cases. Acute complications requiring additional treatment occurred in 5 patients. Average survival time was 93 days for malignant disease and 26 months for benign lesions. Cumulative patency after 200 days was 63% for stents placed in malignant obstructions. Stents in benign obstructions remained open in all cases.

Despite many studies on percutaneous transhepatic biliary drainage (PTBD), there are no satisfactory reports of PTBD for malignant obstruction of the hepatic confluence. In this study, the results of PTBD using the direct anterior approach under fluoroscopic guidance are described in 16 patients with malignant biliary obstruction of the hepatic confluence. A total of forty-two drainage catheters were placed in the 16 patients: one catheter was placed in three patients; two catheters in six; three catheters in four; four catheters in two; seven catheters in one. PTBD was successful in 100% of the cases with no mortality or critical complications, and the biliary decompression effect of PTBD was highly satisfactory. Nine patients underwent histologically curative hepatectomies. Two of the nine resected cases died of postoperative hepatic failure, but the postoperative course in the others was satisfactory. The authors stress that selective biliary drainage for each isolated segmental duct is mandatory for advanced malignant obstruction of the hepatic confluence.

In the course of 4 years, among 11,738 admissions there were 245 (2.08%) patients with cholestasis (106 women and 139 men). Intrahepatic cholestasis (i.c.) was detected in 46.5%, and extrahepatic (e.c.) in 53.5%. The most frequent cause of i.c. were alcoholic and nonalcoholic chr. liver disease (fatty liver, chr. hepatitis, cirrhosis) (37% and 30%), acute viral hepatitis (15%) and toxic liver injury (14%) respectively. The causes of e.c. were: choledocholithiasis (44%), cancer of the pancreatic head (15%), cancer of gallbladder and extrahepatic ducts (12%) and cancer of liver (10%). The causes of c. were benigne, in 78.2%, while malignant neoplasms were present in 21.8%. Out of the multitude of laboratory tests two appeared particularly significant: glut, transpeptidase was pathologic in 81% of alcoholic liver disease, in 62% of the cases with obstructive jaundice and in 27.7% of malignant neoplasms. LX-lipoprotein examined in 52 patients was positive in 24% of i.c., and 60% of e.c. Proliferation of bile ducts was the most frequent finding in surgical liver biopsies in choledocholithiasis cases.

The infant who presents with intracellular cholestasis represents a significant diagnostic challenge to the physician. The efficient and complete evaluation of such patients is of utmost importance. Rapid institution of appropriate therapy may be lifesaving in some cases. In others, a definitive diagnosis is essential for appropriate parental counseling.

A marked reduction in bile flow and bile acid excretion was whenever peripheral lymphocytes from patients with drug-induced allergic intrahepatic cholestasis were stimulated with a specific drug in vitro in the presence of a soluble liver-specific antigen fraction, and their culture supernatant injected into the mesenteric vein of rats. A gel filtration study of the active fraction of the supernatant that caused a reduction in bile flow, suggested that the molecular size of this active principle is similar to that of the macrophage migration inhibitory factor (MIF). Histologically, dilated bile canaliculi with decreased microvilli were observed via electron microscopy in rat liver after injection of culture supernatant. No such changes were observed in rats after injection of the supernatant of a lymphocyte culture similarly prepared from normal individuals. These results strongly suggested that sensitized lymphocytes obtained from patients with drug-induced intrahepatic cholestasis produce a factor (of factors) causing cholestasis when stimulated with a specific drug in the presence of liver-specific antigen fractions.

Hepatobiliary abnormalities occur often in patients receiving TPN. Although TPN use may be important in their pathogenesis, many other factors could also be involved. In any individual patient it may be difficult to separate the contribution of TPN itself from that of the other precipitating causes. The most worrisome observation to date is the development of severe progressive liver disease in a small number of patients receiving long-term TPN. Further work is needed to determine whether the use of TPN actually contributes to this condition or is simply an associated finding.