History Of Jaundice Research Articles

2205 Bupivacaine-Induced Liver Injury: A Diagnostic Dilemma

INTRODUCTION: Bupivacaine is local anesthetic (1). Very few cases of bupivacaine induced liver injury have been noted in the literature; we report a case of this very rare side effect (2). CASE DESCRIPTION/METHODS: A 69-year-old male patient underwent a right inguinal herniorrhaphy. One week after the surgery, patient began to have nausea, vomiting, fever, and dark brown urine. Stools were normal and there was no pruritis. There was no previous history of jaundice, chronic liver disease or malignancy. New medication use, alcohol, illicit drug use, risky sexual behavior was negative. On examination, vitals were all within normal limits. Icterus was present but otherwise his skin was only remarkable for the left lower quadrant scar, which was healing well without surrounding erythema or purulent discharge. Liver function tests were notable for a total bilirubin of 17.4 mg/dL, direct bilirubin was 10.8 mg /dL,. AST was 355 U/L, ALT was 570 U/L, alkaline phosphatase was 944 U/L, LDH was 315 U/L. Viral, autoimmune and structural causes of acute hepatitis were excluded. Given the negative evaluation, a retrospective review of medications received during and after surgery was performed, and the patient was found to have received bupivacaine via PCA post-operatively. As all the other options were excluded, the patient was diagnosed with liver injury from bupivacaine. The patient was managed conservatively and recovered fully in about four weeks. DISCUSSION: Bupivacaine is a local anesthetic which can be given through elastomer pumps for pain control (1). There are case reports in the literature that suggested bupivacaine can cause liver injury when given for more than one week (2,3). DILI is a diagnosis of exclusion (2,4). The common causes of liver injury such as drugs, alcohol, viruses, autoimmune diseases and biliary tract abnormalities need to be excluded (4). The diagnosis can be established with the help of Naranjo criteria (5) (Table 1). Most of these liver injuries respond to conservative management. There have been no reports of any acute or chronic liver failure in such cases. If the patient is at high risk of liver injury then they should receive local anesthetic agent other than bupivacaine (3).

2485 An Unusual Case of a Primary Mass in the Porta Hepatis

INTRODUCTION: Burkitt's Lymphoma is an aggressive subtype of B-cell non- Hodgkin Lymphoma which can present in three forms: endemic, sporadic, and immunodeficiency- associated. It commonly presents in the pediatric population and occurs in less than 1% of adult non-Hodgkin lymphomas. It classically presents with abdominal symptoms with ascites and can commonly involve the distal ileum, stomach, cecum, and mesentery. We describe an unusual case of Burkitt's lymphoma presenting as a mass in the porta hepatis, associated with obstructive jaundice. CASE DESCRIPTION/METHODS: A 74 y/o male presented with a two-week history of jaundice and intermittent epigastric pain. Physical exam findings were scleral icterus, jaundiced skin, and soft nontender abdomen with normal bowel sounds. Labs were pertinent for alkaline phosphatase 1,264, AST 166, ALT 95, total bilirubin 18.8, and direct bilirubin of 15.1. Ultrasound and MRI/MRCP demonstrated a 10.3 × 6.8 × 7.2 mass infiltrating the porta hepatis, peripancreatic, and periduodenal tissues. There were no other masses or adenopathy. At ERCP there was a friable mass replacing the ampulla. Cholangiogram showed dilated intrahepatic ducts proximal to an obstruction at the level of the common bile duct and a metal expandable stent was placed. Biopsy of the ampullary mass revealed high grade, mature B-cell lymphoma. Endoscopic ultrasound revealed a heterogenous mass and fine needle aspiration yielded necrotic cells. Tumor cells were positive for CD20, PAX5, BCL6, CD10, CD20, and LEF1. Pathology was positive for Myc rearrangement and translocation t(8;14). Combination chemotherapy was then initiated. DISCUSSION: Primary tumors of the porta hepatitis are uncommon as it is more often the site of metastatic disease from breast, pancreas, gastric, and colon cancer. This case is particularly unusual in that the primary tumor is a Burkitt lymphoma, which did not arise from lymph nodes or from the pancreas and which presented with obstructive jaundice.

2441 Hepatotoxicity by Methoxsalen: The Innocuous Culprit

INTRODUCTION: Drug-induced liver injury (DILI) is the second most common cause of acute liver failure; 15-20% cases of DILI can be attributed to damage caused by herbal or dietary supplements. Methoxsalen, commonly known as psoralen, is the active ingredient in Bakuchi seeds and is used for treatment of vitiligo and psoriasis. DILI caused by Psoralen is rare, with only three cases reported in literature. This case demonstrates the importance of eliciting a history of herbal supplement use in patients with acute liver failure and the need for regulation of herbal supplements. CASE DESCRIPTION/METHODS: A 60-year-old female presented with two-week history of jaundice and right upper quadrant pain (RUQ). She started taking Bakuchi seeds four weeks prior to presentation and would apply it topically and consume it daily. Patient denied history of alcohol use, blood transfusions, or illicit drug use. Physical exam was notable for scleral icterus and jaundice. Labs revealed abnormal liver function tests (LFTs) (Figure 1) with a peak total bilirubin of 19 mg/dL and a MELD-Na on admission of 19. Work up revealed positive anti-smooth muscle antibodies and AFP elevation to 15.9 ng/mL. Pertinent negatives included viral hepatitis serologies, HSV, EBV, factor V activity, and hemochromatosis. A RUQ ultrasound showed no abnormalities. An MRI abdomen was done which revealed mild periportal edema and mildly dilated common bile duct without choledocholithiasis or obvious mass. Given concerns for DILI, patient was started on acetylcysteine. Hospital course was complicated by episodes of confusion; an ammonia level was elevated at 75 umol/l. Patient was started on lactulose. Because patient's LFTs improved with a stabe PT/INR of 12/1.2, she did not meet criteria for a liver transplant. A liver biopsy was performed which revealed submassive necrosis and collapse; hepatitis with plasma cells and eosinophils with significant cholestasis. Given biopsy results consistent with DILI, patient was discharged on oral prednisone and lactulose to follow up with hepatology in one week. DISCUSSION: LFT abnormalities were seen in 2% to 12% of subjects taking psoralen; the elevations were mild-moderate in severity, asymptomatic and self-limited in course with recovery of LFT seen in 6 to 8 weeks. However, severe jaundice and death from hepatic failure has been described in patients with preexisting cirrhosis. This case emphasizes the need for increased surveillance to formulate guidelines regarding regulation and use of herbal supplements.

2230 Benign Intermittent Intestinal Alkaline Phosphatase Elevation

INTRODUCTION: Liver function tests (LFTs) are commonly checked during routine clinical encounters without specific signs or symptoms. An elevated alkaline phosphatase (AP) level is followed by investigative algorithms to identify the etiology. Total AP comprises liver, bone and intestinal fractions. Elevated intestinal alkaline phosphatase (IAP) can occur as a rare benign condition or due to intestinal pathologies. Unlike previously published reports of benign persistent IAP elevation, our patient had intermittent elevation of IAP. CASE DESCRIPTION/METHODS: A 48-year-old obese female was seen in liver clinic for evaluation of elevated AP for the last two years. She had undergone work-up for elevated AP on several occasions but the exact etiology was not found. She did not report any prior history of jaundice, pruritis, nausea, vomiting, diarrhea, abdominal pain, arthralgias or arthritis. She denied any family history of elevated IAP. She was found to have elevated total AP on 6 separate occasions over the last 18 months ranging from 186 to 239 U/l (normal < 117). Her fasting isozyme fractionation was checked 4 times. She had elevated total AP and normal liver/bone fractions on all occasions but twice had elevated IAP of 62 and 93 U/l (normal < 15 U/l), 26-33% of total AP (normal < 14%). Her other LFTs, TFT, PTH, Ca, CBC, BMP, GGT, viral hepatitis serologies, AMA and Vit D were unremarkable. Abdominal imaging revealed a fatty liver while a bone scan was normal. Our patient remained asymptomatic and did not require any treatment. DISCUSSION: Elevated AP can be physiological during rapid bone growth and pregnancy or pathological due to hepatobiliary disease or abnormal bone metabolism. Non-hepatic causes of elevated AP include heart failure, renal failure, hyperthyroidism, hyperparathyroidism and certain malignancies. IAP normally accounts for up to 14% of total AP. IAP can be elevated in conditions such as intestinal perforation, intestinal ischemia, extensive erosions/ulceration in GI tract, liver cirrhosis, familial benign elevation of IAP or post-prandial rise after a fatty meal particularly in patients with blood group B or O. Our patient did not have any of the above-listed conditions. Although she was blood group B, the blood draws were done after an overnight fast. While investigating elevated AP clinicians should be aware of pathological and benign causes of IAP elevation including benign intermittent IAP elevation which may require repeated testing of AP isozymes.

The impact of preoperative biliary stenting in pancreatic cancer: A case-matched study from the German nationwide pancreatic surgery registry (DGAV StuDoQ|Pancreas)

Background/ObjectiveThe impact of preoperative biliary stenting (PBS) before pancreatoduodenectomy (PD) for pancreatic ductal adenocarcinoma (PDAC) is controversial. MethodsPatients undergoing PD with or without PBS for PDAC were identified from the German DGAV-StuDoQlPancreas registry. The impact of PBS on perioperative complications was analyzed. Results1133 patients undergoing PD for PDAC were identified from the registry. After matching, 480 PBS patients vs. 480 patients without PBS were analyzed. Postoperative complications Clavien-Dindo classification (CDC) grade IIIa-IVb were higher in PBS patients (PBS 27% vs. no PBS 22%, p = 0.027). 320 PBS patients (66%) had no history of jaundice. In these patients, PBS was associated with higher morbidity. In contrast, PBS was not associated with higher complication rates in patients with history of jaundice. Serum bilirubin levels of 15 mg/dl and higher lead to more CDC IIIa-IVb (24% vs. 28%, p = 0.053) and higher mortality (3% vs. 7%, p < 0.001). PBS in patients with serum bilirubin levels of >15 mg/dl increased CDC IIa-IVb complications (21% vs. 50%, p = 0.001), mortality was equivalent. ConclusionMost PBS procedures were performed in patients with no history of jaundice and increased morbidity. Serum bilirubin levels >15 mg/dl lead to higher morbidity and mortality. PBS correlated with higher complication rates in these patients.

EFFICACY OF LIVER FUNCTION TEST IN PATIENTS WITH SYMPTOMATIC GALL STONE DISEASE

Background: Cholelithiasis is a common clinical problem in surgical practice all over the world. Liver function tests (LFT’s) are routinely performed in patients undergoing cholecystectomy for symptomatic gall stones.&#x0D; Objective: To evaluate the effectiveness of routine LFTs in patients with symptomatic gall stones attending Shridev Suman Subharti Medical College attached with Dr. K.K. Bhatnagar Memorial Subharti Hospital, Dehradun, Uttarakhand, India&#x0D; Material and Methods: The present prospective observational study was conducted from December 2016 to November 2017 in the Dept. of Surgery in collaboration with Dept. of Biochemistry at Shridev Suman Subharti Medical College attached with Dr. K.K. Bhatnagar Memorial Subharti Hospital, Dehradun, Uttarakhand, India. All patients clinically diagnosed with symptomatic cholelithiasis were included in the study. Patients with past and present history of jaundice, pancreatitis, cholangitis and abnormal calibre common bile duct on ultrasonography (more than 8mm) were excluded from the study. Data regarding relevant history, clinical examination findings, LFT’s and ultrasound examination findings were analyzed and noted in pre-designed proforma.&#x0D; Results: Study composed of 120 patients comprising 100(83.33%) female and 20(16.67%) male with the mean age of 45±25 years. Out of 120 patients, 85(70.83%) presented as chronic calculous cholecystitis, 29(24.17%) as biliary colic and 6(5.00%) with acute calculous cholecystitis. The LFT’s were within normal range in 107(89.16%) patient, which labeled as group A. In group A, 80(74.76%) patients were presented as chronic calculous cholecystitis, 24 (22.42%) as biliary colic and 3(2.8%) as acute calculous cholecystitis. The levels of LFT’s were found abnormal in 13 patients (8.33%) which is labeled as group B. In group B, clinical presentation of 2 (40.00%) patients were as chronic calculous cholecystitis, 8(30.00%) patients as biliary colic &amp; 3(30.00%) patients as acute calculous cholecystitis. Parameters of LFTs, Bilirubin were elevated in 2, AST in 3 and ALP in 8 patients. All patients from either group had normal Common Bile Duct (CBD) caliber on preoperative ultrasound examination and per operatively (in open surgery), no CBD dilatation or palpable calculi.&#x0D; Conclusion: Routine LFT’s in preoperative assessment of symptomatic cholelithiasis usually reveals normal findings and is not recommended as a good predictive marker for the detection of silent CBD calculi.&#x0D; Keywords: Cholelithiasis, Choledocholithiasis, Common bile duct (CBD), LFT, Cholecystectomy, Laparoscopic

Symptomatic giant cavernous hemangioma of the liver in a 42-year-old man

Most cases of liver hemangioma are asymptomatic and discovered incidentally on liver ultrasound or computed tomography scan. Giant cavernous hemangioma (GCH) are however clinically distinct from smaller asymptomatic ones and may be confused with primary or metastatic malignancy. Symptomatic GCH of the liver are rare. The aim of the study was to show the rare case of symptomatic giant cavernous hemangioma of the liver which may be confuse with primary or metastatic malignancy. The patient’s images (abdominal ultrasound and computed tomography scans) and case file were reviewed and summarized. The subject matter of giant cavernous hemangioma was reviewed in the literature. The index case was discussed and compared with literature. We report a 42-year-old man who presented with a 2-year history of right upper abdominal pain and 6-month history of chest pain. No history of jaundice, body swelling or previous history of blood transfusion. No history of smoking or alcohol consumption. The patient is a known diabetic. Abdominal ultrasound scan showed hepatomegaly with a huge well defined oval shaped mixed echogenic mass lesion with lobulated margins occupying 4th and 5th segments of liver, measuring 84.9×111 mm in size suggestive of adenoma. The remaining hepatic parenchyma was normal. No intrahepatic biliary dilatation was seen. Initial histological examination revealed adenoma. Repeat histology done later however revealed hepatitis. Abdominal CT scan was later carried out and showed hepatomegaly but with no discernible mass lesion on precontrast images. Contrast enhanced images however, showed a fairly rounded mass lesion with peripheral enhancement and delayed filling-in at the venous phase, seen in the superior aspect of the right lobe extending to the dome. The intrahepatic vasculature and biliary ducts are not dilated. A diagnosis of cavernous hemangioma was made based on criteria of delayed (centripetal) filling-in. The patient however was lost to follow up. Hepatic hemangioma can be giant and symptomatic and despite its rarity, may still be encountered in practice.

Spontaneous perforation of cystic duct in a 3-month-old Nigerian boy

Introduction: Biliary ascites is a rare condition in infancy and is commonly caused by the perforation of the extrahepatic bile duct. The cystic duct is the part of extrahepatic biliary tract most rarely affected. This is likely to be the first case of biliary ascites of infancy, secondary to spontaneous cystic duct perforation in our centre. Methods: Consent was obtained from the parents of the child. Ethical approval was also obtained from the research and ethics committee of Ahmadu Bello University Teaching Hospital, Shika, Zaria. The case notes of the child were summarized and the relevant literature was reviewed to give the report a context. Results: A three-month-old boy presented with ten days history of jaundice, a week history of abdominal distention, and a day’s history of fever. There was associated bilateral inguinal hernia, excessive cry, refusal to feed, and weight loss. Pregnancy history was normal. He was acutely ill-looking, irritable, febrile with an axillary temperature of 37.7°C, mildly pale, jaundiced with a greenish tinge, had a weight of 4.7kg. He had gross ascites and bilateral reducible inguinal hernia. Bedside abdominal paracentesis reveals thick yellow and foamy fluid. Abdominal ultrasound scan revealed gross ascites, clotting profile was deranged, there was hyperbilirubinaemia of 228 umol/L, other laboratory results including liver function test were normal. He was commenced on medical management for presumed sepsis with obstructive jaundice. Five days into the admission, there was no significant improvement, and the diagnosis was reviewed to a ruptured choledochal cyst and the child was prepared for an explorative laparotomy. Intra-operatively, the cystic duct was perforated. Cholecystectomy and repair of the common bile duct were performed and the child did well postoperatively. Conclusion: Perforated cystic duct should be suspected in infants presenting with jaundice abdominal distension, acholic stools with or without fever, vomiting, and inguinal hernias. Early diagnosis and prompt surgical intervention greatly reduce morbidity and mortality from the disease.

Seroprevalence and risk factors associated with HBV and HCV infection among subjects with type 2 diabetes from South India

This is a brief summary of the prevalence on Hepatitis C (HCV) and Hepatitis B (HBV) viral infections and associated risk factors in Type 2 diabetes subjects. Prevalence of HBV (9%) was higher compared to HCV (2%) infection in the screened 388 subjects. Results showed that these infections are independent of the liver damage. Risk factors prominently observed among positive HCV and HBV cases were longer duration of diabetes, hospital admission, history of jaundice and history of surgeries which enlightened the importance of hepatitis vaccination once the subject is diagnosed with diabetes.

Relationship between Neonatal Skin Bilirubin Level and Severe Jaundice with Maternal, Childbirth, and Neonatal Characteristics

Background: Neonatal jaundice is the most common cause of newborns' hospitalization. This study aimed to examine various maternal, childbirth, and neonatal factors affecting the neonatal skin bilirubin level and severe jaundice. Methods: This cross-sectional study was conducted on 1066 healthy neonates with a gestational age of 35 weeks or over and birth weight of ≥2000 g at teaching hospitals of Alzahra and Taleghani in Tabriz, Iran, during 2016-2017. The participants were selected using convenience sampling. Data were collected using a three-part researcher-made questionnaire assessing maternal, childbirth, and neonatal characteristics based on the medical histories of mothers and newborns as well as interviews with mothers. Neonatal jaundice was measured utilizing the Kj-8000 device on days 3-6 after birth by measuring the skin bilirubin level. Moreover, the need to phototherapy (severe jaundice) was determined based on the American Academy of Pediatrics guideline. The generalized linear model and multivariate logistic regression were employed for data analysis. Results: Out of the followed-up neonates, 94 (9.96%) cases developed severe jaundice and 850 (90.04%) newborns did not show any symptoms of this complication. The results of the adjusted generalized linear model showed that infant’s bilirubin level correlated significantly with a history of jaundice in previous children, infant’s age at first meconium excretion, frequency of feeding, gestational age, mother’s blood type, and a number of maternal ultrasound during pregnancy. Moreover, the results of adjusted logistic regression revealed an association between severe jaundice and variables, such as the place of residence, history of jaundice in previous children, feeding the infant with water or sugar water during breastfeeding intervals, frequency of feeding the infant, gestational age, mother’s blood type, time of discharge from the hospital, and number of pregnancies. Conclusion: Some factors related to neonatal jaundice were identified to raise the awareness of healthcare personnel for the early identification and treatment of neonatal jaundice in order to prevent its complications.

IL-28B genetic variation, gender, age, jaundice, hepatitis C virus genotype, and hepatitis B virus and HIV co-infection in spontaneous clearance of hepatitis C virus.

Spontaneous viral clearance observed in some patients is one of the variants of the hepatitis C virus (HCV) infection natural history. We aimed to look at the complexity of factors affecting the spontaneous clearance of HCV (SC HCV). A total of 357 anti-HCV positive patients (309 with chronic hepatitis C and 48 patients with SC HCV) were included into the study. We studied the effects of the interleukin-28B (IL-28B) gene polymorphism, gender, age, the routes of virus transmission, past hepatitis C with jaundice, HCV genotype, and hepatitis B virus (HBV) and HIV co-infection on the outcome of HCV infection. Based on the study results, the SC HCV was found in 48 individuals (13.4%). The most significant positive factors affecting the SC HCV included IL-28B single nucleotide polymorphism (SNP) rs12979860 (CC) and SNP rs8099917 (TT) (OR 4.03, p<0.001) and (OR 3.14, p<0.002), female gender (OR 2.72, p<0.001), young age (OR 2.30, p<0.008), and past history of jaundice (OR 5.12, p<0.001). The markers of a past HBV infection were found significantly more often in SC. Positive predictors of the SC HCV include favorable IL-28B genotype, female gender, young age, a history of jaundice, markers of a past HBV infection, the absence of HIV infection, but not the viral genotype.

The Association between Neonatal Icterus or Neonatal Phototherapy and the Likelihood of Childhood Asthma among Iranian Children

Background: There might be a close link between neonatal icterus and/or neonatal phototherapy and childhood asthma. The present study aimed to assess the relationship between neonatal icterus and/or phototherapy and the likelihood of childhood asthma among Iranian population. Materials and Methods The present case-control study was performed on 102 consecutive asthmatic children hospitalized at Children’s Medical Center, Tehran, Iran. Asthma was diagnosed by a pediatric asthma and allergy specialist based on clinical manifestations and/or spirometry results for children older than 5 years. A total of 113 sex and age-matched children without asthma who were admitted to other wards during the same period of time were selected as the control group. Data were collected by a researcher-made checklist including data regarding participants’ age, gender, and gestational age, history of neonatal icterus and history and duration of phototherapy, filled by participants’ parents/guardians. Results: There was a significant difference between cases and controls with respect to preterm birth, history of phototherapy and duration of phototherapy. History of icterus was not associated with childhood asthma. In the multivariable logistic regression model, both history of phototherapy (P=0.029), and duration of phototherapy (P=0.03) were considered as determinants for occurrence of childhood asthma. Conclusion According to the results, history of neonatal icterus was not associated with childhood asthma, but history and duration of phototherapy were both determinants of childhood asthma.

Prevalence of Autistic Spectrum Disorders in Preschool Children in Qalyubiya Governorate

Autism spectrum disorder is a neurodevelopmental disorder with a strong genetic basis. This study has been conducted to determine prevelance and the possible risk factors of autism. . This studty is a retrospective study that included101 children (67 males and 34 female). Their ages ranged from 26 years with mean age 3.87±1.03 (26) . Cases was collected from KGs, special centers and psychiatry clinic, Pediatric Department , Benha univeristiy hospital and Benha specialized Hospital . The included child was subjected to: the followings tools: Confirmation of diagnosis using DSMV criteria,, and assessment of severity of autistic symptoms using childhood autism rating scale (CARS). A small structured questionnaire and complete medical history stressing on developmental, therapeutic and behavioral histories were also developed and administered . ASD prevalence was 1 in51 childrenThe current study was conducted on 101 autistic children, their ages range from2 – 6 years with a mean value of 3.87±1.03 years, 66.3 of autistic children were males and 33.7 were females, with a male/female ratio of 2:1; (p > 0.05 all).Advanced paternal age (father, P35 years) at birth was found in 91% of cases and the difference was statistically significant. Positive family history was found to be statistically unsignificant. As regards natal factors, a history of low birth weight, delivery by ceserian section were significantly higher among cases . Also postnatal factors as history of hypoxia, resuscitation and history of jaundice were considered significantly risk factors for autism(p=0.000).

Spontaneous bacterial peritonitis among adult patients with ascites attending Korle-Bu Teaching Hospital.

Spontaneous bacterial peritonitis (SBP) is one of the most common and life-threatening complications of ascites, mostly in patients with cirrhotic ascites and children with nephrotic syndrome. Recognition and prompt treatment of this condition is essential to prevent serious morbidity and mortality. It is therefore important to determine the prevalence of SBP among in-patients with ascites attending our facility and to determine the clinical characteristics associated with SBP among these patients. A cross-sectional study was conducted involving 140 patients with ascites irrespective of the underlying cause from 25th March 2016 to 25th November 2016. Demographic information and clinical data were collected using a standardized questionnaire. Ascitic fluid culture, the gold standard for SBP diagnosis and ascitic fluid cell count was done. Positive ascitic fluid culture and/ or ascitic polymorpho nuclear leukocyte ≥250cells/mm3 were diagnostic for SBP. Of the 140 patients with ascites the mean age was 44.7±13.2 years. There were seventy six (76) male and sixty four (64) female patients. The prevalence of SBP was 21.43% (30/140). Majority, (41.7%) of the bacteria isolated from ascitic fluid with SBP was Escherichia coli. History of jaundice, low arterial blood pressure on admission and encephalopathy were found to be independent predictors of SBP. SBP is common among patients with ascites admitted at the Korle-Bu Teaching Hospital. Jaundice, encephalopathy and low blood pressure are highly suggestive of SBP and diagnostic paracentesis should be done immediately on admission to confirm the diagnosis. None.

ATYPICAL PRESENTATION OF CAROLI’S SYNDROME: A CASE REPORT

Caroli’s disease and Caroli’s syndrome are rare congenital disorders. Caroli’s disease is characterized by multiple sequential cystic or saccular dilatations of the large intrahepatic biliary ducts while Caroli’s syndrome has small bile duct involvement and congenital hepatic fibrosis. The incidence of Caroli’s disease is as low as 1/1,000,000 people. The average age of presentation is early adolescence. Magnetic resonance cholangiopancreatography is a most valuable investigation in diagnosis. Here, we report the case of Caroli’s Type II without renal involvement as late as 6 years of age with severe portal hypertension and hypersplenism. The child had no history of jaundice or recurrent abdominal pain in the past.

Patient reported abdominal pain as a surrogate of the clinical benefit of tipifarnib in pancreatic cancer patients.

275 Background: Tumor CXCL12 expression identifies patients who may benefit from tipifarnib therapy. In pancreatic cancer, CXCL12 is known to decrease abdominal pain by inducing the migration of pain suppressing Schwann cells to tumor lesions. Given the association between CXCL12 and pain suppression, we investigated whether the absence of patient reported abdominal pain could be a surrogate of clinical benefit from tipifarnib in pancreatic cancer patients. Methods: We conducted a retrospective analysis of a randomized placebo-controlled trial comparing gemcitabine plus tipifarnib (GT) versus gemcitabine plus placebo (GP) in pancreatic adenocarcinoma patients. Tipifarnib was given at 200 mg bid orally continuously; gemcitabine at standard dosing. Primary study end point was overall survival. Results: 688 patients were enrolled of whom 155 (22.5%) reported no abdominal pain at study entry, 81 received GT and 74 GP. Baseline characteristics were balanced in the subset of patients with no reported abdominal pain (GT,GP): median age (63,59), female gender (35%,43%), ECOG 0-1 (89%,90%), metastatic disease (64%,70%), 6-month weight loss &gt;10% (48%,39%), 6-month jaundice history (51%,45%), and prior Whipple surgery (16%,13%). Subjects receiving GT who did not report abdominal pain at study entry had higher frequency of locally advanced disease (36% vs 24%) and 6-month jaundice (51% vs 38%), and lower frequency of lung (6% vs 14%) and peritoneum (4% vs 13%) metastases than those reporting pain. No differences in survival were observed in the overall study. Median overall survival for GT was 193 vs 182 days for GP. Notably, absence of abdominal pain at study entry was associated with higher median survival in the GT arm (no pain, pain): 305 vs 176 days, HR=0.52, p&lt;0.0001, whereas no significant effect was observed in the control arm: 184 vs 182 days. A trend for better survival with GT was observed in subset of patients with no abdominal pain at study entry (GT,GP): 305 vs 184 days, p=0.058. Conclusions: Absence of abdominal pain may serve as a surrogate of clinical benefit from tipifarnib in pancreatic cancer. (Van Cutsem et al. JCO 2004;22:1430-8; NCT00005648; Trial Sponsor: J&amp;J PRD). Clinical trial information: NCT00005648.

Novel compound heterozygote mutations of TJP2 in a Chinese child with progressive cholestatic liver disease

BackgroundProgressive familial intrahepatic cholestasis (PFIC) is a group of genetic autosomal recessive disorders that predominantly affects young children and results in early-onset progressive liver damage. Several types of PFIC were defined based on different genetic aetiologies in last decades.Case presentationHere, we report a Chinese young child diagnosed as PFIC with variants in tight junction protein 2 (TJP2). The patient was affected by a long history of jaundice, pruritus, and failure to thrive. Highly elevated level of serum total bile acid (TBA) and normal levels of gamma-glutamyltransferase (GGT) were observed at hospitalization. The patient’s clinical symptoms could be alleviated by administration of ursodeoxycholic acid. Genetic testing by next generation sequencing (NGS) found novel compound heterozygote mutations c.2448 + 1G > C/c.2639delC (p.T880Sfs*12) in TJP2, which were inherited from her mother and father, respectively. Both mutations were predicted to abolish TJP2 protein translation, and neither has previously been identified.ConclusionWe report a Chinese female PFIC child with novel compound heterozygous mutations of TJP2. Genetic testing by NGS is valuable in the clinical diagnosis of hereditary liver disease.

A Documentation of Hepatitis Outbreak in Chittagong

Introduction: Hepatitis E Virus (HEV) causes outbreaks of jaundice and it is associated with morbidity and higher maternal mortality. There is a recent outbreak of hepatitis in the Chittagong city and present study is aimed to observe the clinical and serological trends along with outcome of hepatitis cases visiting two tertiary care hospitals Chittagong, Bangladesh.&#x0D; Methods: It was an observational study done in two tertiary care hospital of Chittagong in a period of 3 months (May, June &amp; July 2018) among 230 patients of hepatitis. After inclusion with written informed consent patients were introduced a questionnaire. Their demographic data, risk behaviors were noted, history related to hepatitis were recorded. Examination was done and evaluation regarding presence of hepatic encephalopathy at bed site was noted. Later serological findings were evaluated. After collection of all data it were compiled and analyzed by SPSS- 20.&#x0D; Results: Among 230 cases age group, gender and locality of the study patients were analyzed where younger age groups specially 21-30 years 114(49.6%) and 31-40 years 38(16.5%) were the most affected group. Gender distribution was found mostly same. Halishahar area which is located in western part of the Chittagong city was found mostly affected 196(85.2%). Use of water from WASA (Water and Sewerage Authority) supply was 168(73%) and deep tubewel water was 56(24.3%). Among them 80(34.8%) subjects do not boil water Anorexia (74.8%) nausea (77.4%) vomiting (83.5%) history of fever (89.6%) passage of dark color urine (99.1%) and weakness (97.4%) were some common presenting complaints. Family history of jaundice was found in 40.9% of cases. Among all 216(93.91%) had clinical jaundice, 91(39.56%) had right upper quadrant of the abdomen pain, 41(17.8%) had hepatomegally, 14(6.1%) had spleenomegally, 24(10.4%) had signs of hepatic failure and 34(14.8%) female were pregnant. Anti HEV was found positive in 164(71.3%) cases, 12(5.2%) were positive for anti HAV, 6(2.6%) were positive for HBsAg. None was found anti HCV positive. Among all 4(1.8%) cases died due to hepatic failure or multi-organ failure with AKI. All of them were pregnant. Two pregnant lady had missed abortion. Among all 11(4.7%) cases loosed follow and 213(92.60%) cases had uneventful recovery.&#x0D; Conclusion: This outbreak of HEV was of usual pattern of morbidity and mortality, and therefore points to water supply and sanitation issues.&#x0D; Chatt Maa Shi Hosp Med Coll J; Vol.17 (2); Jul 2018; Page 2-5

Administration Frequencies of Hepatitis A and Hepatitis B Vaccines at Two Schools with Different Socioeconomic Status Before Admission to National Immunization Program

Amac: Hepatit A ve hepatit B asilarinin ulkemiz Genisletilmis Bagisiklama Programi’na girmeden once, sosyoekonomik duzeyleri farkli iki okulda, 7-15 yas arasi cocuklarda her iki etkene yonelik asilama oranlarinin ve seroprevelanslarin karsilastirilmasi amaclanmistir. Yontem: Calismamiz, 2003 yilinda, Istanbul Bakirkoy ilcesinde, sosyoekonomik duzeyi farkli iki okulda ogrenim goren 546 cocuk arasinda yapilmistir. Ebeveynlere calisma oncesi bilgilendirme toplantisi yapilmis, calismaya gonullu ebeveynlerin cocuklari calismaya dâhil edilmistir. Cocuklarin hepatit A ve hepatit B asilari uygulanma durumu, cocukta sarilik gecirme oykusu sorgulandi. Cocuklardan alinan venoz kanlardan Anti HAV IgG, HbsAg, Anti Hbs belirtecleri calisilmistir. Bulgular: Sosyoekonomik duzeyin daha yuksek oldugu okulda hepatit A ve hepatit B asisi ile asilanma oranlari ve bu etkenlerin seroprevalanslari istatistiksel anlamli olarak yuksek saptanmistir. Sonuc: Ulusal Asilama Programi’nda yer almayan asilarin uygulanmasi, sosyoekonomik duzeye gore farklilik gosterebilmektedir. Gunumuzde hepatit A ve hepatit B asilarinin Ulusal Bagisiklama Programi’nda yer almasi, cocuk sagliginda var olan bir sosyal esitsizligin kaldirilmasi acisindan onemli bir halk sagligi girisimidir.

CLINICAL AND EPIDEMIOLOGICAL MANIFESTATION OF HEPATOCELLULAR CARCINOMA IN PATIENTS BELONGING TO ETHNIC GROUPS OF CAUCASIANS AND ASIANS OF NORTH-EAST ASIA

Aim. The current study is aimed at determining differences of epidemiological and clinical profiles associated with HCC in patients belonging to ethnic groups of Asians from Mongolia and Caucasians from Asian region of Russia. Materials and methods. The studies were carried out in the cross-border regions of Mongolia and Asian part of Russia (Irkutsk region). 300 patients with hepatocellular carcinoma (HCC) of the Caucasian and Mongolian races were enrolled in the study. The level of alpha-fetoprotein (AFP) in the serum was determined by the chemiluminescence technique. Results. The long-term dynamics of the HCC incidence shows more unfavourable trends in the territory of Mongolia compared to Irkutsk region. In both groups, male patients over 60 years of age predominated. Patients from Mongolia often have a history of jaundice and alcohol abuse. Out of the etiological factors, HCC is more often associated with the hepatitis B virus in Mongolia than in the Asian part of Russia. At the same time, in Caucasians, HCC develops primarily on the background of liver cirrhosis. In patients with HCC, AFP level higher than 20 ng / ml were significantly more frequent in the ethnic group of Caucasoids than in Mongoloids. Conclusions. Mongolia in terms of the incidence of HCC belongs to the hyperendemic regions of the world. In this country, among the risk factors for the development of the disease, hepatitis B virus plays a major role, which significantly differs from the Asian part of Russia. For the purpose of early diagnosis of HCC, it is necessary to search for new molecular markers or their combinations due to the insufficient diagnostic efficiency of AFP determination.