INTRODUCTION:

The 5-year survival of patients with gallbladder cancer remains low. However, patients can be stratified into prognostic categories based on established factors such as T, N, and R status. New concepts regarding prognostic significance of lymph node disease, the importance of residual gallbladder fossa disease, and the gravity of presentation with jaundice are reviewed. In addition, a number of new prognostic factors proposed in recent years are considered.

METHODS:

PubMed was searched for "gallbladder cancer" with builder "date-completion" 2008 to present. A total of 1,490 articles were screened from which 168 were retrieved. From this, 40 articles specifically related to prognosis form the basis for this review.

DISCUSSION:

Key factors of prognostic significance remain T and N stage and R0 resection. Residual disease either in the gallbladder fossa, lymph nodes, or cystic duct margin dictates hepatectomy, lymphadenectomy and bile duct resection, respectively. Adequate lymphadenectomy requires removal of six nodes, and hepatectomy must be sufficient to achieve R0. Subtleties regarding lymph node ratio, significance of pathological features such as dedifferentiation, and budding may hold value for stratifying patients with early stage disease, but require further investigation.

In severe and rapidly increasing jaundice, the use of intensive phototherapy provides greater effectiveness and a faster decrement in bilirubin levels compared to conventional phototherapy. The aim of this study was to compare the effectiveness of two types of intensive phototherapy: intensive compact fluorescent tube (CFT) and intensive light-emitting diode (LED) phototherapy. Forty-three infants over 35 weeks of gestation with severe non-hemolytic hyperbilirubinemia were enrolled in the prospective study. All infants received multidirectional (circular-shaped) intensive phototherapy. Of these, 20 infants received CFT while 23 infants received LED phototherapy. Bilirubin levels and body temperatures were measured periodically, and the rates of bilirubin decrement were calculated. Mean serum bilirubin level of the 43 infants was 20.5±1.5 mg/dl at the beginning of the therapy and mean duration of phototherapy was 20.6±1.1 hours. The rate of mean bilirubin decline was 47.2% and the decrease was more prominent in the first four hours (0.84 ± 0.41 mg/dl/h). The rates of bilirubin decrement were comparable between the LED and CFT groups. Slightly elevated mean body temperature (37.1ºC) was determined in the CFT group (p<0.05). Intensive phototherapy units with both LED and CFT were effective, showing a decline of half the initial value of bilirubin during the study period in infants with non-hemolytic jaundice. This study shows that intensive phototherapy with either CFT or LED can provide rapid decrease in bilirubin levels in the first few hours. This rapid decline is important in cases that have high risk of bilirubin encephalopathy.

Biliary rhabdomyosarcoma (RMS) is a rare malignancy of childhood. The radiological findings and clinical presentation of the tumor can mimic an entirely different pathology. The incidence of RMS has impeded the development of a standardized form of treatment. A four-year-old child with botryoid RMS in the common bile duct is reported herein to emphasize the role of surgery in the small-sized tumor and the role of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis and relief of biliary obstruction before total excision.

To discuss the relationship between advanced schistosomiasis and HBV infection.A total of 250 advanced schistosomiasis patients were examined with ultrasound, and their serum samples were detected for liver function, HBsAg, etc. The correlations between HBV infection and advanced types, abnormal liver function, liver cancer and mortality were analyzed, respectively.The positive rate of HBsAg was 58.4% (146/250). The rates of abnormal liver function (46.6%), jaundice (23.3%), cancer (17.8%) and mortality (23.3%) were significantly higher in the advanced schistosomiasis patients with HBsAg than in the advanced schistosomiasis patients without HBsAg (P values < 0.05 or 0.01).Advanced schistosomiasis combined with HBV infection aggravates the liver damage.

BACKGROUND:

This study presents our 11-year surgical experience with choledochal cysts (CDC), focusing on the complexity in the diagnosis and management of large-size CDCs.

METHODS:

Medical records of 70 patients who underwent surgery for CDC between 2000 and 2011 were retrospectively reviewed. Of them, ten patients were found with cyst size >10 cm (group A), and we compared the differences in the clinical presentation, radiological diagnosis and surgical management with those with cysts ≤10 cm in size (group B).

RESULTS:

Abdominal pain and lump were noted in all group A patients, but only 67 % and 14 % in group B patients, respectively. Sixty percent of group A, but only 14 % of group B patients, had a triad of abdominal pain, mass and jaundice. Cholangitis was more common in group A patients. Most of group B patients were correctly diagnosed with ultrasound alone, but misdiagnosis occurred more frequently in group A. Magnetic resonance cholangiopancreatography correctly achieved the diagnosis in all. Operative time (5 vs. 3 h) and blood loss (300-500 vs. 50-100 mL) were more in group A in comparison to group B.

CONCLUSIONS:

The clinical presentation and surgical management of giant choledochal cysts differ from their smaller counterparts. Diagnostic dilemma was common with giant CDC especially with ultrasound and CT.

Many substances, drugs or not, can be responsible for acute hepatitis. Nevertheless, toxic etiology, except when that is obvious like in acetaminophen overdose, is a diagnosis of elimination. Major causes, in particular viral etiologies, must be ruled out. Acetaminophen, antibiotics, antiepileptics and antituberculous drugs are the first causes of drug-induced liver injury. Severity assessment of the acute hepatitis is critical. Acute liver failure (ALF) is defined by the factor V, respectively more than 50% for the mild ALF and less than 50% for the severe ALF. Neurological examination must be extensive to the search for encephalopathy signs. According to the French classification, fulminant hepatitis is defined by the presence of an encephalopathy in the two first weeks and subfulminant between the second and 12th week after the advent of the jaundice. During acetaminophen overdose, with or without hepatitis or ALF, intravenous N-acetylcysteine must be administered as soon as possible. In the non-acetaminophen related ALF, N-acetylcysteine improves transplantation-free survival. Referral and assessment in a liver transplantation unit should be discussed as soon as possible.

We conducted a hospital-based study from June 2002 to December 2006 of Thai children aged 1-15 years with acute hepatic failure (AHF) to determine the causes and outcomes. Eleven children were included in the study. Hepatitis B virus was the cause of AHF in one child, infection-associated hemophagocytic syndrome was the cause in 1 child, Wilson's disease was the cause in 1 child and dengue fever was suspected to be the cause in 2 children. In 6 children the cause of AHF was unknown. Jaundice was reported in 9 of 11 children. Ten of 11 children had mild to moderate encephalopathy on admission. Five of 11 children died due to AHF. No liver transplantations were performed among the children in this study. Further studies into the relationship between dengue infection and AHF are needed.

Hemophagocytic syndrome (HPS) is an uncommon hematological disorder that manifests as fever, splenomegaly, and jaundice, with hemophagocytosis in the bone marrow and other tissues pathologically. Secondary HPS is associated with malignancy and infection, especially viral infection. The prevalence of cytomegalovirus (CMV) infection in ulcerative colitis (UC) patients is approximately 16%. Nevertheless, HPS in UC superinfected by CMV is very rare. A 52-year-old female visited the hospital complaining of abdominal pain and hematochezia for 6 days. She was diagnosed with UC 3 years earlier and had been treated with sulfasalazine, but had stopped her medication 4 months earlier. On admission, her spleen was enlarged. The peripheral blood count revealed pancytopenia and bone marrow aspiration smears showed hemophagocytosis. Viral studies revealed CMV infection. She was treated successfully with ganciclovir. We report this case with a review of the related literature.

Congenital hypothyroidism (CH) is the most common congenital endocrine disorder, with an incidence of 1:2,000 to 1:4,000 live births and it is a leading preventable mental retardation. Neonatal Screening Programs allow early identification of the disease and the adequate treatment of affected children can avoid the complications related to deprivation of the hormone. Most cases of primary congenital hypothyroidism (85%) are due to thyroid dysgenesis (ectopia, hypoplasia or agenesis) while the remaining result from defects in hormone synthesis. Affected children (> 95%) usually have no symptoms suggesting the disease at birth. The most frequent symptoms and signs are prolonged neonatal jaundice, hoarse cry, lethargy, slow movements, constipation, macroglossia, umbilical hernia, large fontanelle, hypotonia and dry skin. Around the world, various strategies are used for the screening of the CH. In Brazil, screening for CH is mandatory by law and usually done by serum TSH in dried blood collected from the heel. The recommended age for performing this test is after 48 hours of life until the 4th day. Diagnostic confirmation is required dosing TSH and free T4 or total T4 in serum.