Percutaneous Cholecystocholangiography-A Tool to Conclusively Exclude Biliary Atresia.

The objective of this study was to evaluate the feasibility, safety, and diagnostic accuracy of percutaneous cholecystocholangiography (PCC) in cases of conjugated hyperbilirubinemia in which biliary atresia (BA) could not be diagnosed or ruled out based on clinical, radiological, and histopathological findings. This was a retrospective, chart review of all cholestatic infants who underwent PCC within the last 5 y. PCC was performed via the transhepatic route using 23-g needle. The patency of both the proximal and distal biliary trees was assessed. PCC was technically feasible in 12/13 (92.3%) of infants without any procedure-related complications. PCC demonstrated proximal and distal biliary patencyin 7/12 (58.3%) infants, thereby avoiding unnecessary laparotomy in them. PCC failed to demonstrate biliary patency in 5 infants; of which, 4 were confirmed as cases of BA on laparotomy. PCC can correctly differentiate BA from non-BA cases of conjugated hyperbilirubinemia preoperatively, reducing the negative laparotomy rates.

Clinical Value of Inflammation-Based Prognostic Scores to Predict the Resectability of Hyperbilirubinemia Patients with Potentially Resectable Hilar Cholangiocarcinoma.

BACKGROUND: We aimed to examine whether inflammation-based prognostic scores could predict tumor resectability in a cohort of hilar cholangiocarcinoma patients with preoperative hyperbilirubinemia. We also sought to investigate the prognostic factors associated with overall survival in the subgroup of patients with an R0 resection. METHODS: A total of 173 patients with potentially resectable hilar cholangiocarcinoma, as judged by radiological examinations, were included. The potential relationship of the Glasgow prognostic score (GPS), modified GPS, platelet lymphocyte ratio (PLR), neutrophil lymphocyte ratio (NLR), prognostic nutritional index (PNI), and prognostic index (PI) with tumor resectability were investigated using univariate and multivariate analysis. RESULTS: Among the 173 patients, 134 had R0 resection margins. Univariate analysis identified that patients with PLR ≥ 150, NLR ≥ 3, PNI ≥ 45, GPS (0.1/2), modified GPS (0.1/2), preoperative CA 125 > 35 U/mL, and a tumor size ≥ 3 cm were more likely to have unresectable tumors. Multivariate analysis indicated that tumor size ≥ 3 cm (OR = 2.422, 95% CI: 1.053-5.573; P = 0.037), PLR ≥ 150 (OR = 3.324, 95% CI: 1.143-9.667; P = 0.027), preoperative CA 125 > 35 U/mL (OR = 3.184, 95% CI: 1.316-7.704; P = 0.010), and GPS (0.1/2) (OR = 2.440, 95% CI: 1.450-4.107; P = 0.001) were independent factors associated with tumor resectability. In selected patients with an R0 resection in this cohort, nodal status (P = 0.010) and tumor differentiation (P = 0.025) were predictive of poor survival outcome. CONCLUSION: Patients with higher GPS, CA 125, and PLR levels, and a larger tumor size, tend to have unresectable tumors even if they were judged as potentially resectable using preoperative radiological examinations.

Does Hyperbilirubinemia Contribute to Adverse Patient Outcomes Following Pancreatoduodenectomy?

BACKGROUND: Jaundice due to biliary obstruction leads to multiple physiologic derangements and a decline in performance status that may result in unfavorable intra- and postoperative outcomes following a Whipple procedure. While preoperative biliary decompression may improve synthetic function, this strategy has been reported to increase the incidence of infectious complications following surgery. We hypothesized that hyperbilirubinemia at the time of pancreatoduodenectomy (PD) would be a risk factor for increased morbidity and mortality postoperatively. METHODS: The American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) Pancreatectomy Demonstration Project and the 2014 Procedure Targeted Pancreatectomy databases were queried for patients with a bilirubin level obtained within 7 days of PD. Results were compared among patients with bilirubin level percentiles <80th (0-2.9 mg/dL), 80-90th (3-7.3 mg/dL), and >90th (>7.3 mg/dL). Data were further evaluated between those with a bilirubin ≥10 mg/dL and those with a normal level and by utilizing bilirubin as a continuous variable. Outcomes included 30-day mortality and overall and serious morbidity as previously defined by ACS-NSQIP. Categorical variables were compared using chi-squared, Fisher's exact, Kruskal-Wallis, or Wilcoxon rank sum tests with a p = 0.05 considered significant. RESULTS: The combined databases yielded 2556 patients who had PD and a preoperative bilirubin level for analysis. When comparing patients with bilirubin levels at the 80th (n = 2055), 80-90th (n = 273), and >90th percentiles (n = 228), no difference was observed among groups with respect to overall and serious morbidity or mortality. Similarly, no difference in postoperative outcomes was observed between the 147 patients who had a bilirubin ≥10 mg/dL and those with normal levels or when bilirubin increased when levels were analyzed as a continuous variable. CONCLUSION: Modest degrees of hyperbilirubinemia were not shown to affect morbidity and mortality following pancreatoduodenectomy. The indication and need for preoperative biliary decompression should be reserved, and utilized selectively, only for those with symptomatic, elevated bilirubin levels.

[Cholecystolithiasis as a cause of local hepatitis].

In an acute inflammation of gallbladder inflammatory process spreads on surrounding tissues, including hepatic tissue, what causes the regional hepatitis occurrence. In some patients, suffering calculous cholecystitis on background of transition of inflammatory process from gallbladder to hepatic tissue likewise a regional hepatitis, hyperbilirubinemia, the skin yellowness are revealed, what simulates choledocholithiasis and obturation jaundice.

[Surgical tactics in patients with cholecystitis complicated by bilirubinemia].

Basing on experience of treatment of more than 11 000 patients there were analyzed its results in 248, who were admitted to the hospital in emergency for an acute cholecystitis and raising of a bilirubin level from 29.54 to 167.16 micromol/l. Miniinvasive tactic was applied, surgical treatment was divided on the stages: laparoscopic cholecystectomy with the common biliary duct (CBD) draining, postoperative transdrainage cholangiography (in 184 patients any calculi or other obstacles to the bile outflow were not revealed), endoscopic papillosphincterotomy--in accordance with the indications established. An acute intervention on CBD using miniaccess was needed in 4 patients only. The results were estimated as good and excellent.

Dental treatment of an adult patient with a history of biliary atresia.

OBJECTIVE: Biliary atresia is a congenital disease treated by liver transplantation. Adults may have oral consequences of the medical therapy. Green teeth are oral manifestations of the induced hyperbilirubinemia. Gingival enlargement is associated with the immunosuppressive drug. This case report describes the successful treatment of an 18-year-old patient displaying severe green teeth and gingival enlargement. METHOD AND MATERIALS: The gingival enlargement was treated by reducing the pathogenic oral microflora through scaling and root planing of the teeth, gingival excision surgeries, and conversion from cyclosporin to tacrolimus. RESULTS: Gingival enlargement and inflammation had completely disappeared after nonsurgical treatment for the maxilla and after surgical treatment for the mandible. The green coloration of the teeth was masked using composite restorations. CONCLUSION: This case report indicates that a patient's quality of life can be improved by a team approach combining pharmacologic and dental therapies.

[Correction of hepatic dysfunction as pre-treatment before systemic chemotherapy in patients with bile duct neoplasms].

This prospective randomized study incorporates 141 surgical department patients with hepatobiliary tumors. The 1st group patients received 800 ml/day of remaxol. The 2nd1 group patients received Ringer solution and 10% glucose at 1:1 ratio. The subgroups included: 1 subgroup-with pre-operative cholecysto- or choledochostomia and B-subgroup-without pre-operative interventions. The combined surgical and pharmaceutical correction of bile passage, bilirubinemia, cholestasis and cytolysis by remaxole leads to better hepatic dysfunction correction and allows better timing of chemotherapy in bile passage tumors.

Tc-99m-BrIDA hepatobiliary (HIDA) scan has a low sensitivity for detecting biliary complications after orthotopic liver transplantation in patients with hyperbilirubinemia.

BACKGROUND: Tc-99m-BrIDA hepatobiliary scans are noninvasive tests for detecting biliary leaks and obstructions. However, there is low sensitivity and specificity in patients with hyperbilirubinemia. Biliary complications (BC) are the Achilles heel of orthotopic liver transplantation (OLT). We questioned whether hyperbilirubinemia in liver transplant recipients rendered HIDA scanning less dependable. METHODS: HIDA findings were compared to endoscopic retrograde cholangiopancreatography, laparotomy, and clinical course. Results were categorized as follows: true positive (TP), true negative (TN), false positive (FP), false negative (FN), or nondiagnostic/inconclusive. We searched for variables associated with erroneous or nondiagnostic tests which we defined as all examinations determined to be FP, FN and/or nondiagnostic/inconclusive. RESULTS: Thirty-four patients underwent a HIDA scan. The sensitivity and specificity were 70 and 100%. The sensitivity of HIDA improved to 100% in patients with a total bilirubin (TB) <5 mg/dl. Inconclusive and FN patients had a total bilirubin >5 mg/dl. One FN had a TB <5 mg/dl, but was determined inconclusive due to the roux-en-Y. CONCLUSION: HIDA scans performed when the total bilirubin was <5 mg/dl had a high sensitivity and specificity for detecting biliary complications after OLT. However, when the total bilirubin exceeded 5 mg/dl, the specificity was still 100% but the numbers of nondiagnostic/inconclusive and FN exams were increased.

The optimal timing of referral to an intestinal failure program: the relationship between hyperbilirubinemia and mortality.

PURPOSE: Multidisciplinary treatment of pediatric intestinal failure has shown promising results. However, there are limited data as to the optimal time frame for referral of patients to intestinal failure programs. The aim of this study was to explore the relationship of hyperbilirubinemia at referral with patient outcomes in a multidisciplinary program. METHODS: A retrospective analysis was performed of a prospectively collected database from a multidisciplinary intestinal failure program. Multivariable logistic regression adjusted for age at referral was used to model the association between the conjugated bilirubin at referral and risk of mortality. Median values with range are reported. RESULTS: Sixty-two patients were referred from 2005 to 2009. Patients presented at age 6.4 months (0.4-261.4 months) and were followed up for 16.8 (0.3-53.0) months. Nine subjects (14.5%) died, and 12 subjects (19.4%) were listed for combined liver-intestine transplant. A 50% mortality was seen in patients referred with a conjugated bilirubin ≥ 7.2 mg/dL (n = 12), whereas mortality at referral bilirubin levels <7.2 mg/dL was 6%. After adjusting for age at referral, patients with a conjugated bilirubin ≥ 7.2 mg/dL at referral were 15.4 times more likely to die than patients who presented with lower bilirubin levels (P = .001; 95% confidence interval, 2.8-83.4). CONCLUSION: Within a pediatric intestinal failure program, mortality is associated with the degree of hyperbilirubinemia at time of referral. These data strongly suggest that these patients should be referred to a multidisciplinary program early in the evolution of their liver disease.

[Small-for-size syndrome. Report of one case]. / "Síndrome pequeño para su tamaño", (small-for-size), como complicación de un trasplante hepático con donante vivo adulto relacionado.

Although the use of cadaveric split or living donor liver transplantation is a valid option for liver transplants, they have several complications, being the "small-for-size syndrome" one of the most frequent. This entity is mainly due to the incapacity that the graft has to meet the blood drainage demands. We report a 61 year-old patient with sub-acute liver failure, transplanted with a partial liver graft that developed hyperbilirubinemia, ascites and liver function deterioration. A meso-caval shunt was performed, after which the ascites resolved, serum bilirubin normalized and the synthetic function of the liver improved. After one month, a follow-up CT seen showed the absence of blood flow in the shunt, possible due to the reduction of the hyper-perfusion of the liver. The clinical and biochemical condition of the patient continued improving despite the lack of flow through the shunt.

"Síndrome pequeño para su tamaño", (small-for-size), como complicación de un trasplante hepático con donante vivo adulto relacionado / Small-for-size syndrome: report of one case

Although the use of cadaveric split or living donor liver transplantation is a valid option for liver transplants, they have several complications, being the "small-for-size syndrome" one of the most frequent. This entity is mainly due to the incapacity that the graft has to meet the blood drainage demands. We report a 61 year-old patient with sub-acute liver failure, transplanted with a partial liver graft that developed hyperbilirubinemia, ascites and liver function deterioration. A meso-caval shunt was performed, after which the ascites resolved, serum bilirubin normalized and the synthetic function of the liver improved. After one month, a follow-up CT seen showed the absence of blood flow in the shunt, possible due to the reduction of the hyper-perfusion of the liver. The clinical and biochemical condition of the patient continued improving despite the lack of flow through the shunt.

De novo autoimmune hepatitis after living donor liver transplantation in a 25-day-old newborn baby: a case report.

De novo autoimmune hepatitis (AIH) has been described recently as a new type of graft dysfunction in pediatric patients receiving liver transplantation. Herein we have reported the case of a boy, diagnosed as neonatal hemochromatosis, who received a reduced left lateral graft 25 days after birth. Pretransplantation autoantibodies and serological tests were negative. The postoperative course was smooth. No episode of vascular or biliary complication or acute cellular rejection was observed. The maintenance immunosuppressant was tacrolimus only. Liver dysfunction occurred 13 months after living donor liver transplantation. Liver biopsies showed no acute cellular rejection, but severe apoptosis and regeneration of liver cells at the centrolobular area. At that time, various autoantibodies including anti-nuclear, anti-double-stranded DNA, and anti-smooth muscle antibodies were positive. In addition, serum immunoglobulin G (IgG) was elevated. Based on these findings, he was diagnosed as de novo AIH. The treatment consisted of reducing the tacrolimus dose and reintroduction of steroids. After 12 months of treatment, liver dysfunction improved, serum autoantibodies became negative, and serum IgG level normalized. Currently his immunosuppressive therapy consists of low-dose tacrolimus and prednisolone. In conclusion, the present case demonstrated that de novo AIH can appear in living donor liver transplant patients despite appropriate immunosuppression. Reducing the tacrolimus dose and reintroduction of prednisolone sustained the graft and prevented retransplantation.

Acute decompensation and absence of brain and kidney dysfunction predict long-term efficacy of plasma exchange in hyper-bilirubinemic cirrhotic patients awaiting liver transplantation.

Various artificial liver support systems are currently used in patients with decompensated chronic liver disease or acute liver failure as a bridge to recovery or to orthotopic liver transplantation (OLT). Between June 2004 and September 2006, 9 subjects were treated with plasma exchange (PE) for acute decompensation on chronic liver disease or chronic decompensation in end-stage liver disease. All of them were awaiting OLT or were listed at the moment of decompensation. Grade II to III hepatic encephalopathy (HE) was present in 4 patients, significant renal dysfunction in 3 patients, and ascites in 6 patients. Baseline serum total bilirubin was 35.1+/-11.2 mg/dL (mean value+/-SD). The patients underwent a mean of 12.1 2-hour exchanges over 1 to 8 weeks. The 3 who recovered were alive after a mean follow-up of 22.7+/-10.3 months. There were 3 patients who underwent transplantation and 3 who died due to liver failure during treatment. Only subjects with acute decompensation and without HE or significant renal dysfunction survived without OLT. PE did not significantly modify the grade of HE or the renal function. PE seemed to be a safe, long-term, effective therapeutic option for acute decompensation among subjects with chronic liver disease without brain or renal dysfunction.