Normal Serum Bilirubin Research Articles

Hodgkin's lymphoma-related vanishing bile duct syndrome: A case report and literature review

We report the case of a 38-year-old man who developed vanishing bile duct syndrome in association with Hodgkin's lymphoma. He was noted to have cervical lymphadenopathy and marked elevation of total serum bilirubin at diagnosis. He achieved complete remission with normalization of serum bilirubin after eight courses of Adriamycin, bleomycin, vinblastine, and dacarbazine chemotherapy followed with autologous hematopoietic cell transplantation. Consecutive liver biopsies performed at diagnosis and at the stage of complete remission revealed the disappearance and regeneration of interlobular bile ducts, respectively. Our case provides pathological evidence that Hodgkin's lymphoma-related vanishing bile duct syndrome is a reversible bile duct injury disease. Bilirubin is a reliable serum marker to monitor the treatment response of these cases. The mechanism to develop hyperbilirubinemia with vanishing bile duct in such a case of Hodgkin's lymphoma remains to be studied. A literature review was carried out.

Hypernatremia-associated myelinolysis following the management of sepsis in a patient with glioblastoma treated with radiotherapy and temozolomide

Dear Editor, A 58-year-old male presented with neutropenic sepsis a week after the completion of a 6-week course of temozolomide chemotherapy and conformal radiotherapy (CT?RT). At admission, pancytopenia and pneumonia were managed with tazocin, growth factor filgrastim, platelets transfusion, and intravenous saline. Blood results during CT?RT showed only mild liver enzymes elevation. Comorbidities were diabetes mellitus, hypertension, and non-alcoholic hepatosis with a normal baseline serum bilirubin, minimally increased alkaline phosphatase (ALP 130 IU/L, normal range 30–125 IU/L), and less than five times raised alanine aminotransferase (ALT 135 IU/L, normal range 5–45 IU/L). Six months previously, he had undergone total thyroidectomy with the removal of two parathyroid glands followed by a course of radioiodine ablation for papillary thyroid carcinoma. Regular medications were atenolol, lisinopril, metformin, and levothyroxine. On increasing dexamethasone dose to 4 mg/day, GCS improved from 14 to 15. Blood culture was sterile. On day 6, with sodium increasing to 161 mmol/L, GCS decreased to 13 (Fig. 1). At presentation, the temperature was 38.5 C

Clinical Predictors of Pancreatic Carcinoma Causing Acute Pancreatitis

Acute pancreatitis may be the first presentation of pancreatic carcinoma (PaCa). The present study was designed to identify clinical findings suggestive of PaCa in patients with nonalcoholic nongallstone-related (NANG) acute pancreatitis and evaluate accuracy of endoscopic ultrasound for diagnosing PaCa in this setting. This is a retrospective analysis of 332 consecutive patients who underwent endoscopic ultrasound-fine-needle aspiration after acute pancreatitis. Patients with gallstones or common bile duct stones, who were heavy or binge alcohol drinkers, or who had post-endoscopic retrograde cholangiopancreatography pancreatitis were excluded. Among 218 patients with NANG acute pancreatitis, 38 patients had PaCa. Age more than 50 years (P = 0.008), history of smoking (P < 0.001), weight loss of 10 lb or greater (P = 0.003), serum bilirubin levels of higher than 2 mg/dL (P = 0.035) or serum alkaline phosphatase level of higher than 165 U/mL (in patients with normal serum bilirubin levels) (P = 0.003), and radiological findings of an identifiable pancreatic mass (P = 0.001) or distal pancreatic atrophy (P = 0.006) had significant association with an underlying PaCa on multivariate analysis. Of the 38 patients with PaCa in this cohort, 37 had 2 or more of these findings. Endoscopic ultrasound-fine-needle aspiration had 99.5% accuracy (98.6, 100%) for diagnosing carcinoma in this clinical setting. The clinical criteria defined previously potentially can help select patients with NANG acute pancreatitis with a higher likelihood of an underlying pancreatic neoplasm for further imaging.

1550 Phototherapy and DNA Changes in full term Neonates with Hyperbilirubinemia

BackgroundOxidative effects of phototherapy on cell membranes and cell components may have a wide range of potential adverse effects, including .DNA damage. Apoptosis is an indispensable mechanism for maintaining many...

Hepatobiliary and Pancreatic: Extrahepatic biliary atresia in an adult

A teenager, aged 18, was referred for evaluation because of elevated serum levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST) for 16 years. There were no significant abdominal or other symptoms. However, on physical examination, he had a large spleen, 8 cm below the left costal margin. Routine blood tests showed anemia, leucopenia and thrombocytopenia. Liver function tests revealed a normal serum bilirubin but elevated levels of ALT, AST, alkaline phosphatase (ALP) and gamma-glutamyl transpeptidase (GGT). Various investigations for viral hepatitis, autoimmune disease and Wilson's disease were unhelpful. An abdominal ultrasound study and computed tomography (CT) scan showed dilatation of intrahepatic ducts and splenomegaly. Magnetic resonance cholangiopancreatography (MRCP) showed cystic dilatation of the hilar bile duct and moderate dilatation of intrahepatic ducts. The gallbladder (GB) and common bile duct (BD) were clearly shown and the gallbladder appeared to be linked to intrahepatic ducts (Figure 1). Endoscopic retrograde cholangiopancreatography (ERCP) was also performed and showed contrast passing from the common bile duct into the cystic duct and gallbladder (Figure 2). The common hepatic duct was not outlined. Endoscopy revealed esophageal and gastric varices while CT angiography showed a normal hepatic artery, portal vein and inferior vena cava. Figure 1 Figure 2 At laparotomy, the patient had features of cirrhosis. An operative cholangiogram was performed by injection of contrast into the gallbladder and only showed contrast in the common bile duct, similar to findings at ERCP. In the process of mobilizing the gallbladder, bile ducts in the gallbladder bed were shown to communicate with the gallbladder. The common hepatic duct could not be identified but dissection revealed a hilar pouch containing bile. Two hepaticojejunostomies were performed to drain bile from the gallbladder bed and from the hilar pouch. Splenectomy was also performed. The anastomoses in the gallbladder bed were shown to be patent by passage of contrast through a stent. Liver function tests returned to normal after 8 months. This may be the first report of biliary atresia diagnosed in an adult. In this case, he had an unusual variant characterized by absence of the common hepatic duct (type II). The diagnosis was delayed because of the development of anastomoses between branches of the right hepatic duct and the gallbladder. Despite this, there was persistent cholestasis with the development of biliary cirrhosis and portal hypertension. The surgical procedure appears to have been helpful in the short-term but the longer-term outcome remains unclear. Contributed by

Stent patency using competing risk model in unresectable pancreatic cancers inserted with biliary self‐expandable metallic stent

Biliary self-expandable metallic stents (SEMS) play an important role in the quality of life and palliative treatment in unresectable pancreatic cancer patients. We aimed to determine the factors affecting the patency of biliary SEMS and the survival in unresectable pancreatic cancer with obstructive jaundice. Considering the competing risk and survival, we retrospectively evaluated the patency in 107 unresectable pancreatic cancer patients with obstructive jaundice who were successfully treated with biliary SEMS from January 2000 to April 2010. There were 107 incidents of biliary drainage that were clinically successful and the overall survival period was a median of 133 days. Stent occlusion before death was observed in 36 (33.6%) of 107 patients. Cumulative stent obstruction rates were 4.7%, 16.8%, and 24.4% at 1, 3, and 6 months, respectively. Lower cancer stage (<5 month's hazard ratio [HR] = 2.327, >5 month's HR = 0.108) was only associated with the longer patency of the stents in a multivariable analysis using a Fine and Gray model that considered competing risk. In multivariable analysis, lower cancer stage, uncovered stent and normalized serum bilirubin level were associated with a longer survival period (HR = 2.335, 1.906 and 1.795 respectively, P < 0.05). The patency of biliary SEMS in unresectable pancreatic cancers might be affected by the stage. Lower cancer stage and normalized bilirubin are associated with longer survival.

A comparative study of surgery and endoscopy for the treatment of bile duct stricture in patients with chronic pancreatitis

This study aimed to compare the outcomes of endoscopic treatment (ET) and surgical treatment (ST) for common bile duct (CBD) stricture in patients with chronic pancreatitis (CP). From 2004 to 2009, 39 patients (35 men and 4 women; median age, 52 years; range, 38-66 years) were referred for CBD stricture in CP. Of these 39 patients, 33 (85 %) underwent primary ET, and 6 underwent primary ST. Treatment success was defined in both groups as the absence of signs denoting recurrence, with normal serum bilirubin and alkaline phosphatase levels after permanent stent removal in ET group. The follow-up period was longer than 12 months for all the patients. For the patients treated with ET, the mean number of biliary procedures was 3 (range, 1-10) per patient including extractible metallic stents in 35 % and multiple plastic stents in 65 % of the patients. The mean duration of stent intubation was 11 months. The surgical procedure associated with biliary drainage (4 choledochoduodenostomies, 1 choledochojejunostomy, and 1 biliary decompression within the pancreatic head) was a Frey procedure for five patients and a pancreaticojejunostomy for one patient. The overall morbidity rate was higher in the ST group. The total hospital length of stay was similar in the two groups (16 vs 24 days, respectively; p = 0.21). In terms of intention to treat, the success rates for ST and ET did not differ significantly (83 % vs 76 %; p = 0.08). Due to failure, 17 patients required ST after ET. Event-free survival was significantly longer in the ST group (16.9 vs 5.8 months; p = 0.01). The actuarial success rates were 74 % at 6 months, 74 % at 12 months, and 65 % at 24 months in the ST group and respectively 75 %, 69 %, and 12 % in the ET group (p = 0.01). After more than three endoscopic procedures, the success rates were 27 % at 6 months and 18 % at 18 months. For bile duct stricture in CP, surgery is associated with better long-term outcomes than endoscopic therapy. After more than three endoscopic procedures, the success rate is low.

Reversal of jaundice in two patients with inoperable cholangiocarcinoma treated with Cisplatin and gemcitabine combination.

Two patients are presented with severe jaundice, due to inoperable cholangiocarcinoma. The chemotherapeutic approach in patients with severe jaundice is discussed. Many schedules of chemotherapy were developed in this tumor type with normal serum bilirubin. We report here the first successful use of cisplatin and gemcitabine combination chemotherapy in these patients. Tolerability was good and liver function tests gradually improved.

Prosthetic valve, fever and arthropathy--Still a difficult diagnosis

Adult-onset Still’s disease (AOSD) is a rare multisystem inflammatory condition of uncertain aetiology. The annual incidence is approximately 0.16 cases per 100 000 persons.1 The predominant manifestations are those of a spiking fever, arthropathy, an evanescent salmon-pink rash and elevated inflammatory markers. Diagnosis is often delayed given the non-specific clinical and laboratory findings. We describe a man with a febrile illness, rapidly deteriorating clinical course and recent prosthetic mitral valve replacement. A 67-year-old Caucasian male gave a 4-week history of fever, malaise and a cough productive of muco-purulent sputum. Over the week immediately preceding admission he had also noticed a sore throat and a patchy rash. Three months earlier he had undergone a metallic mitral valve replacement and coronary artery bypass grafting for ischaemic mitral regurgitation. On clinical examination he was afebrile with no peripheral stigmata of bacterial endocarditis or lymphadenopathy. He had a prosthetic first heart sound with a soft systolic murmur and a pink macular rash across his trunk and upper limbs. Laboratory investigations at presentation revealed a C-reactive protein (CRP) of 45 mg/l, serum alanine transferase 86 U/l with normal serum bilirubin, alkaline phosphatase and albumin concentrations. Renal function was also normal. Haemoglobin concentration was measured at 13.2 g/l, total leucocytes 9.9 × 109/l and platelet count 214 109/l. Erythrocyte sedimentation rate was 14 mm/h. Multiple blood and urine cultures were taken but no specific treatment instigated. His antistreptolysin O titre was 13 U/ml. A chest radiograph demonstrated …

Phototherapy and DNA changes in full term neonates with hyperbilirubinemia

BackgroundPhototherapy has remained the standard therapeutic approach for neonatal hyperbilirubinemia. Oxidative effects of phototherapy on cell membranes and cell components may have a wide range of potential adverse effects, including enhanced lipid peroxidation and DNA damage. Apoptosis is an indispensable mechanism for maintaining many cellular functions, including cell replication, and removal of damaged cells with high burden of genetic mutations. Many genes function as apoptosis regulatory genes. Examples of these genes include the BCL2 gene which is an anti-apoptotic oncogene, and the BAX gene which acts as a promoter of apoptosis. ObjectivesAssess the effect(s) of phototherapy on DNA and on the rate of apoptosis in full term neonates with hyperbilirubinemia. It comprised 35 neonates with indirect hyperbilirubinemia who received phototherapy for 48h, and 20 apparently healthy full term neonates with normal serum bilirubin level, as a control group. DNA damage was assessed by DNA fragmentation and micronucleus assay. Determination of the anti-apoptotic effect(s) of BCL2 gene was achieved by quantitative assay of its product, (BCL2) protein, by ELISA and BAX gene expression status was assessed by PCR. ResultsThe frequency of micronuclei in circulating lymphocytes of neonates who received phototherapy has significantly increased before and after phototherapy compared to controls, (p<0.001; p<0 0.00001), respectively. DNA fragmentation in circulating lymphocytes, was significantly higher among cases before and after phototherapy compared to controls (p<0.0001; p<0 0.00001). The plasma BCL2 protein was significantly lower in the cases before and after phototherapy compared to controls (p<0.01; p<0.01), respectively, and BAX gene expression was significantly high among cases before and after phototherapy compared to controls. ConclusionsGeno toxic effects of bilirubin and phototherapy, induce more DNA damage and enhances apoptosis of exposed cells, probably through down regulation of BCL2 expression and upregulation of BAX gene expression in neonates with hyperbilirubinemia.

Outcomes of Patients Undergoing Percutaneous Biliary Drainage to Reduce Bilirubin for Administration of Chemotherapy

To describe outcomes in patients undergoing percutaneous biliary drainage to reduce total serum bilirubin level for administration of chemotherapy. A total of 647 consecutive patients underwent percutaneous biliary drainage between September 2001 and December 2008. In 168, the indication for biliary drainage was to decrease total serum bilirubin level to permit administration of chemotherapy. Of these, 20 were excluded because they had hepatic arterial infusion pumps, leaving 148 patients as the study group. The primary diagnoses for these patients were gallbladder cancer (n = 23), cholangiocarcinoma (n = 21), pancreatic cancer (n = 36), and other metastatic cancers (n = 68). Medical records and imaging studies were reviewed for demographic data, procedural information, pre- and postdrainage total serum bilirubin level levels, 30-day complications, and subsequent biliary procedures. The probability of attaining a total serum bilirubin level of 1 mg/dL or lower by 100 days was 31% (95% CI, 23%-39%). Predrainage total serum bilirubin level of 9 mg/dL or lower (hazard ratio [HR], 3.27; 95% CI, 1.86-5.75; P < .001), 100% liver drainage (HR 2.73, 95% CI, 1.56-4.78; P <.001), and lower predrainage International Normalized Ratio (INR; HR, 0.80; 95% CI, 0.70-0.92; P = .002) were associated with an increased likelihood of attaining a total serum bilirubin level of 1 mg/dL or lower. The most common indication for follow-up was pericatheter leakage, which occurred in nearly one third of cases. During follow-up, patients required three visits per 100 catheter-days, or approximately one per month. Median overall survival in this population was approximately 3.5 months. Only 31% of patients attained a normal serum bilirubin level by 100 days, and median overall survival was 107 days. Careful patient selection is warranted before biliary drainage for this indication. Maximal biliary drainage, a preprocedure total serum bilirubin of less than 9 mg/dL, and a lower INR were factors associated with serum bilirubin normalization in this cohort.

Lessons from an incidental diagnosis of paroxysmal nocturnal haemoglobinuria

Dear Editor, The diagnosis of paroxysmal nocturnal haemoglobinuria (PNH) is usually suspected in patients who develop unexplained haemolytic anaemia, thrombotic episodes or pancytopenia. The report describes an incidental finding of this rare condition during the workup of a young adult with stroke and discusses the clinical implications of such a diagnosis. A 29-year-old male presented with sudden onset of right-sided weakness and speech difficulties. Clinical findings were consistent with a left partial anterior circulation stroke. There were no obvious vascular risk factors or family history of cardiovascular disease although there was history of cocaine abuse 3 years earlier and heavy alcohol intake. He had had an admission 3 years earlier with abdominal pain and was noted to have abnormal liver function tests. This was attributed to excessive alcohol consumption following unremarkable clinical assessment and liver ultrasound. Although he had been unwell with non-specific symptoms, an episode of transient limb weakness, 3 months preceding the admission, could have been related to a transient ischaemic attack. On this admission, full blood count revealed a haemoglobin of 11.4 g/dl, white cell count of 4.8×10/l and platelet count of 95×10/l. Renal screen was normal although the liver function tests showed some abnormalities [gamma glutamyl transferase of 183 U/l (normal range less than 40 U/l) and alanine transaminase of 47U/l (normal range less than 35U/l), normal serum bilirubin]. Computerised tomography (CT) and magnetic resonance imaging confirmed a cerebral infarct. CT angiogram identified a left middle cerebral artery (MCA) primary bifurcation stenosis. There was visible thrombus in the left carotid artery and left MCA. Initially it was thought there was a carotid artery dissection but it was difficult to prove on imaging. A follow-up scan revealed normal blood vessels. The patient was appropriately anticoagulated. This would also be consistent with previous dissection. Other investigations into the potential aetiology of the stroke excluded antiphospholipid syndrome (IgG and IgM anticardiolipin antibodies within normal limits and lupus anticoagulant tests negative) and inherited thrombophilic factors (normal antithrombin, protein C and S levels and negative for prothrombin gene and factor V Leiden mutations). Liver ultrasound to identify the cause of his persistent abnormal liver function tests showed an unexpected finding of large portal venous thrombus, later confirmed with a dual-phase CT abdomen. The finding of non-cirrhotic portal vein thrombosis prompted tests for paroxysmal nocturnal haemoglobinuria and myeloproliferative disorder, two under-recognised causes for this unusual site thrombosis [1]. PNH screen revealed 55% clonal population in the red cells and 80% in the granulocytes by flow cytometry confirming the diagnosis of the acquired haemolytic condition. Janus kinase-2V617F mutation was however not detected. Further laboratory workup for PNH revealed normal lactate dehydrogenase (LDH), reticulocyte count J. Thachil (*) :V. Martlew Department of Haematology, Roald Dahl Haemostasis and Thrombosis Centre, Royal Liverpool University Hospital, L7 8XP, Liverpool, UK e-mail: jeckothachil@yahoo.co.uk

Single Dose of 50 mg/kg Clofibrate in Jaundice of Healthy Term Neonates: Randomised Clinical Trial of Efficacy and Safety

To evaluate the effect of single oral dose of 50 mg/kg clofibrate in hyperbilirubinemia of term healthy neonates in Yazd, Iran. A parallel single- blinded randomized clinical trial, conducted on 60 healthy term neonates admitted between July and December 2007 to Shahid Sadoughi Hospital. Inclusion criteria were neonates with gestation age of 38-42 wk, birth weight of 2500-4000 g, product of normal vaginal delivery, breast-fed and total serum bilirubin (TSB) level of 17-29.9 mg/dL. Neonates with sepsis, anemia, severe asphyxia, hemolytic diseases, major congenital anomalies, indirect hyperbilirubinemia and underlying hepatic disorders were excluded. Selection of patients was based on random allocation via table of random numbers and the patients distributed into two groups. In group one, 30 neonates were treated with phototherapy alone and in 30 of other group treatment done with single dose of 50 mg/kg clofibrate and phototherapy. The primary endpoint with respect to efficacy in reducing of TSB was achieving TSB to less than 14 mg/dL as measured at the beginning, 12, 24 and 48 h after the start of phototherapy. Secondary outcomes were hospital stay days, duration of phototherapy and side effects of treatments during hospital stay and on the second day after discharge. No significant differences were seen from the viewpoint of rout of delivery, gender, gestational age, birth weight, hemoglobin and bilirubin level at time of admission and weight in discharge time in the two groups. After 48 h of intervention, 27 (90%) neonates in clofibrate group and 15 (56.7%) in control group had TSB of less than 14 mg/dL (p 0.02). Mean TSB 12 h after treatment (mean ± SD: 14.82 ± 1.7 mg/dL vs. 16.67 ± 1.77 mg/dL, P 0.001), 24 h after treatment (mean ± SD: 11.97 ± 2.92 mg/dL vs. 14.61 ± 2.52 mg/dL, P 0.001) and 48 h after treatment (mean ± SD: 7.91 ± 2.45 mg/dL vs. 12.74 ± 2.21 mg/dL, P 0.0001), mean of hospital stay days (mean ± SD: 1.7 ± 0.7 days vs. 3.2 ± 1.2 days, P 0.03) and duration of phototherapy (mean ± SD: 30.2 ± 13.99 h vs. 46.2 ± 15.58 h, P 0.001] were significantly lower in clofibrate group. Only loose stool was seen in two patients of clofibrate group and no significant difference was seen from view of safety of the treatments. A single dose of 50 mg/kg clofibrate in treatment of neonatal hyperbilirubinemia is effective, safe and cost effective in view of reducing hospital stay days.

Decompensated alcoholic liver disease and birth weight

IntroductionOnly a minority of heavy drinkers develop serious alcoholic liver disease (ALD). Low birth weight has been associated with a several adult-onset diseases such as ischemic heart disease, hypertension and...

P01-107 - Sphincter of oddi dysfunction: sonographic examination in opiate addicts

ObjectiveTo benefit from the usefulness of ultrasonography in measurement of common bile duct in opiate addicts and to correlate between the common bile duct (CBD) diameter and the duration of addiction.Materials and methodFifteen addicts, diagnosed according to DSM IV diagnostic criteria, were examined by abdominal ultrasound. The common bile duct is measured and the results were analyzed with other factors like age, the period of addiction, laboratory findings.ResultsAccording to the findings, there is a significant increase in the range of the CBD diameter in comparison with normal bile ducts. Also, the mean diameter of the CBD in the different age groups showed a significant difference (p &lt; 0.01) and there was a positive significant correlation between the CBD diameter and the period of addiction (p &lt; 0.05, r = 0.875); the multiple comparisons shows that the significant difference between the 3 age groups were due to a significance between group (1) &amp; (2), and group (1) &amp; (3) but no significance between group (2) &amp; (3). So, with the increased length of the addiction period, the mean CBD diameter increases.ConclusionIn opiate addicts, the common bile duct is found to be dilated; however with normal serum bilirubin and alkaline phosphatase level and no obstructive causes by ultrasound examination, no need for any further assessment.

How I manage my patients with hepatocellular carcinoma

In the late 1990s, the development of a standardized staging system for hepatocellular carcinoma (HCC) (Barcelona clinical liver cancer) using evidence-based data made it possible to stratify HCC patients to receive appropriate treatment with substantial clinical benefit. In the past, treatment failures were often because of faulty staging of tumour disease. This was the case for hepatic resection, as patient survival after surgery was endangered by tumour recurrence (70% at 5 years) as well as the risk of unresolved post-surgical hepatic decompensation, a complication that can be avoided by restricting resection to patients with low portal hypertension and normal serum bilirubin. It was also true for liver transplantation, whose outcome clearly improved when stringent patient selection criteria were adopted based on the volume and number of tumour nodules and venous invasion (so-called Milan criteria), resulting in <15% risk of tumour recurrence and a 75% 5-year survival rate. Switching from an empirical to an evidence-based patient selection approach improved the outcome of local ablation therapies, mainly because ideal candidates were identified, resulting in>50% 5-year survival rates, similar to those obtained with hepatic resection. While the prognosis is still poor in patients with advanced HCC, the recent availability of multikinase inhibitors has generated hope for improving survival in these patients.

Normal levels of serum pancreatic enzymes in patients with progressive familial intrahepatic cholestasis type 2.

High prevalence of elevated serum pancreatic enzymes in children with cholestasis with normal fecal elastase-1 concentrations has been documented. However, this state is related predominantly to biliary atresia. Therefore, we aimed to assess pancreatic damage by measuring serum pancreatic enzymes in patients with progressive familial intrahepatic cholestasis type 2 (PFIC type 2). Twenty PFIC type 2 patients with normal serum bilirubin and bile acid concentrations were included in the study. Thirty pancreatic insufficient cystic fibrosis (PI-CF) patients, thirty patients with acute pancreatitis (AP) and thirty healthy subjects (HS) served for the purpose of comparison. In all subjects, serum lipase and elastase-1 levels were measured. In all but one PFIC type 2 patients and all HS normal lipase activities were found. Serum elastase-1 concentrations were normal in all PFIC type 2 patients and HS. The enzyme levels were very similar in both groups studied. Lipase activities in PFIC type 2 patients were significantly higher than in PI-CF patients (p < 0.00001) and lower than in patients with AP (p < 0.00001). Serum elastase-1 levels in PFIC type 2 patients were significantly lower than in patients with AP (p < 0.00001) and not different from those in PI-CF patients. In conclusion. serum pancreatic enzymes in patients with PFIC type 2 are normal. No pancreatic damage in these patients could be detected.

Thermal Ablation for Primary and Metastatic Hepatic Malignancies

Abstract Surgical resection offers the best hope for long-term survival in both hepatocellular carcinoma patients and those with metastatic liver disease. Patients most likely to benefit are those with a single tumor and well-preserved liver function, who have normal serum bilirubin and normal portal flow. Patients are often ineligible for resection for a variety of reasons. Ablative options, such as cryoablation and radiofrequency ablation (RFA), are minimally invasive procedures particularly suited for those patients who are felt to be poor surgical risks. These options are based on the principle that decreasing the volume of viable tumor or preventing new tumor growth can lead to longer survival and potential cure in selected patients. Complications related to these procedures are minimal compared with open surgery. The most common complications are bleeding and infection.

Outcomes in Lymphoma Patients with Obstructive Jaundice: A Cancer Center Experience

Little information is available to guide clinicians on the optimal approach to managing obstructive jaundice in lymphoma patients. The aim of this study was to review our experience in treating lymphoma patients with obstructive jaundice in order to develop guidelines as to the best interventional approach. We reviewed the medical records of all lymphoma patients who underwent endoscopic retrograde cholangiopancreatography (ERCP) and/or percutaneous biliary drainage (PBD) for obstructive jaundice between June 2002 and October 2008. We identified 35 lymphoma patients who underwent ERCP and/or PBD for obstructive jaundice. The mean age was 57.6 years. Most patients (66%) had diffuse large B-cell lymphoma. Stents were placed by ERCP in 25 patients and PBD in nine. Serum bilirubin levels normalized following ERCP or PBD in 29 of 33 (85%) patients with stricture. Stricture resolution occurred in 12 cases. Patients who had obstructive jaundice at the time of their lymphoma diagnosis had significantly longer mean overall survival following intervention than patients in whom obstructive jaundice developed later in the course of their disease (21.3 months vs. 4.5 months, P = 0.0001). ERCP and/or PBD effectively normalized serum bilirubin levels. Plastic stents should be used in patients who have obstructive jaundice at the time of lymphoma diagnosis because these strictures tend to resolve before stent exchanges are necessary. For patients in whom obstructive jaundice develops later in the course of their disease, the limited prognosis often makes a single intervention with insertion of a plastic stent sufficient to provide adequate biliary decompression.

Jaundice as a Presenting Manifestation of Pediatric Non-Hodgkin Lymphoma

There are few data available on the management and outcome of pediatric patients with non-Hodgkin lymphoma (NHL) presenting with jaundice. Controversies exist on biliary drainage and chemotherapy dose modifications to be carried out in such patients. The aim of this study was to analyze the causes of jaundice in, the various modalities of treatment administered to, and outcome of patients aged 18 years or less, with de novo NHL and jaundice treated at our center. A retrospective analysis show 9 such cases (11.2% of all NHL cases), all of whom were treated by a uniform protocol. Chemotherapy dose modifications were based on serum bilirubin and clinical status of the patients. Obstructive jaundice was the most common cause (7 of 9). Seven patients achieved normal serum bilirubin with chemotherapy alone. Only one had a biliary drainage procedure. Four patients were alive and disease-free at last follow-up. Although biliary obstruction is the commonest most cause of jaundice in pediatric and adolescent NHL, biliary drainage may be unnecessary and chemotherapy alone may suffice as initial treatment for these patients.