Prediction of Survival Following Percutaneous Biliary Drainage for Malignant Biliary Obstruction.

BACKGROUND AND OBJECTIVES: Percutaneous transhepatic biliary drain is an intervention used to relieve malignant biliary obstruction. This study aims to explore survival after biliary drain insertion, predictive factors of survival and effectiveness to reduce total bilirubin level. METHODS: We conducted a retrospective analysis of 72 patients who had malignant biliary obstruction and received biliary drain during the time period between March 2005 and February 2015. RESULTS: Median patients' age was 56 years. 38 (52.7%) were males, 34(47.2%) were females. Median survival post biliary drain insertion was 46 days, 95% C/I (37.92-54.02), range (2-453 days). 1, 3, and 6 month survival rates were 64.7%, 26.5%, and 7.4% respectively. Multivariate analysis by Cox proportional hazards regression model showed the presence of ascites to be significant predictors of survival, other factors analyzed were: total bilirubin, serum creatinine, international normalization ratio, serum albumin, pleural effusion and liver metastasis. CONCLUSION: Survival after biliary drain insertion can vary from few days to few months. Presence of ascites is an independent predictor of survival after this intervention.

Presenting Pattern of Pediatric Celiac Disease.

OBJECTIVES: Celiac disease (CD) is an autoimmune disease that can be complicated by impaired nutrition and growth. With the development of sensitive serologic tests, safe endoscopy, and efforts to educate primary care physicians, more children are diagnosed as having CD. The aim of this study is to evaluate the pattern of the presentation of pediatric CD in western New York. METHODS: Chart review of pediatric patients with CD was undertaken. Patients' demographics, presenting features, disaccharidase assay (DA), celiac serology, and Marsh score were reviewed from patients seen at the Digestive Diseases and Nutrition Center, State University of New York at Buffalo from January 2003 through March 2013. RESULTS: A total of 165 patients with CD were evaluated. Mean age was 10.7 ± 4.3 years, 76 male patients. The presenting features were abdominal pain (n = 87, 52.7%), constipation (n = 65, 38.9%), diarrhea (n = 52, 31.1%), family history of first-degree relative (n = 47, 28.1%), diabetes mellitus type 1 (n = 37, 22.2%), failure to thrive (n = 36, 21.8%), reflux (n = 25, 15.1%), vomiting (n = 24, 14.5%), fatigue (n = 15, 9%), short stature (n = 9, 5.4%), thyroid disease (n = 9, 5.4%), Down syndrome (n = 8, 4.8%). We found no correlation between Marsh score and serum tissue transglutaminase (tTG) immunoglobulin (Ig) A level at diagnosis and no correlation between DA and serum tTG IgA level, presenting feature and tTG IgA level, presenting feature and Marsh score, tTG IgA and DA, or between the age and the presenting feature. CONCLUSIONS: Children newly diagnosed as having CD in western New York presented most frequently with abdominal pain and constipation and were older at the time of diagnosis than those described in the classical presentation of CD. We speculate that our patients may have a different long-term natural history and risk factors than originally described for patients with CD.

Increased apolipoprotein A5 expression in human and rat non-alcoholic fatty livers.

Apolipoprotein A5 (apoA5) is a potent regulator of triglyceride (TG) metabolism and therefore may contribute to the pathogenesis of non-alcoholic fatty liver disease (NAFLD), a disease characterised by excessive TG-rich lipid droplets in hepatocytes. To test this hypothesis, we examined the mRNA expression of apoA5 in paediatric NAFLD livers in comparison to healthy controls. According to microarray and quantitative real-time PCR, human NAFLD livers exhibited elevated apoA5 expression compared to healthy controls. The apoA5 expression levels were positively correlated with hepatic TG storage and a marker for lipid droplets (perilipin), but were not correlated with plasma TG levels. These observations were confirmed with a NAFLD rat model. Interestingly, apoA5 expression was not altered in cultured fat-laden HepG2 cells, demonstrating that fat storage does not induce apoA5 in NAFLD livers. Therefore, the correlation between apoA5 and intracellular fat storage is likely explained by the potent effect of apoA5 in promoting intracellular fat storage. Our NAFLD patients and rats had elevated insulin resistance, which may have a role in elevating apoA5 expression in NAFLD livers. Our data support the hypothesis that apoA5 promotes hepatic TG storage and therefore contributes to the pathogenesis of NAFLD, and may represent a potential target for therapeutic intervention.

Pediatric Burkitt's lymphoma: CT findings.

PURPOSE: To review and analyze all CT scans of the cases of Burkitt's lymphoma (BL) in children diagnosed in our institution. MATERIALS AND METHODS: A retrospective analysis of 33 children with BL between the years 2003 and 2005 seen in our institution was undertaken. Twenty-nine male and four female patients from age 3 to 16 years (with a mean age 5.9 years) were reviewed. RESULTS: The gastrointestinal tract was involved in 19 patients (57.5%), kidneys in 9 (27.2%), peritoneum in 8 (24.2%), liver in 4 (12.1%), spleen in 3 (9%), adrenals in 3 (9%), and pancreas in 1 patient (3%). Extra-nodal head and neck involvement was seen in eight patients (24.2%). Bone involvement in four (12.1%), lung in three (9%), heart in two (6%), skin in two (6%), and testis in one (3%) of these patients. Abdominal lymph nodes were enlarged in 21 children (63.6%), while cervical lymph nodes were enlarged in 8 (24.2%). CONCLUSION: CT proved to be an invaluable tool in the characterization of the disease processes in these children. In addition, it provided us with useful information about the anatomical distribution, patterns of involvement, as well as complications of BL.