Yangyang Ma

Early surgical outcomes and pathological scoring values of older infants (≥90 d old) with biliary atresia

PURPOSE This study aimed to analyze the impact of age at Kasai operation on the short-term outcome of type III biliary atresia (BA) and to discuss if pathological scoring can be the prediction of effect in the Kasai procedure for the older (≥ 90 days) infant. METHODS During the period 2004 through 2010, 452 infants with type III BA were reviewed. The relationship between ages at Kasai operation and jaundice clearance rates and two year native liver survival rates were assessed, retrospectively. Pathological slides were analyzed with a histological scoring system. RESULTS All of the patients were divided into 3 groups according to their ages at operation (group A: aged 60 days or less (n=146), group B: age between 60 and 90 days (n=222) , and group C: age on or over 90 days (n=84)). The worst outcome of clearance of jaundice was found in group A but not in group C 2 weeks after the operation (P<0.05). Jaundice clearance rates showed no difference among the three groups either at 3-months or 6-months after operation. Moreover, in group C patients, the pathological scores showed no difference between the jaundice clearance group and jaundice persistence group 6 months after surgery. In group C, two year survival rate of patients with native livers was 36.1%. CONCLUSION Some patients over 90 days of age at surgery can still benefit from a Kasai procedure. The pathological scoring system does not play a role in predicting jaundice clearance in patients over 90 days of age at surgery.

A pathological scoring system in the diagnosis and judgment of prognosis of biliary atresia

OBJECTIVE The purpose of this study was to evaluate the diagnostic and prognostic value of a histological scoring system in biliary atresia (BA). METHODS From June 2013 to July 2014, 86 wedge liver biopsy specimens were obtained from infants with neonatal cholestasis (58 patients with biliary atresia and 28 patients with non-obstructive cholestasis as control) in our center. A pathologist, single-blinded to the final diagnosis, made the histological diagnosis individually based on an 8-feature (liver fibrosis, portal ductal proliferation, bile plugs in portal ductules, cholestasis, hepatocellular changes inflammatory cells infiltration in portal region, extramedullary hematopoiesis, and ductal plate malformation), 21-point (0 to 21) scoring system. RESULTS In this retrospective study, ductular reaction (bile ductular proliferation) and liver fibrosis in the portal area, bile plugs, and ductal plate malformation were the best indicators of BA. With the scoring system, a score of ≥8 had the best diagnostic utility to differentiate BA from other intrahepatic cholestasis histologically (sensitivity 94.7%, specificity 86.2%, accuracy 91.9%). Liver fibrosis and ductal plate malformation were confirmed to be related with the prognosis. CONCLUSIONS An 8-feature, 21-point histological scoring system has a good diagnostic accuracy in the interpretation of liver histology in neonatal cholestasis. The use of liver fibrosis and ductal plate malformation are also feasible to assess the prognosis.

Knockdown of β-catenin expression inhibits neuroblastoma cell growth in vitro and in vivo ☆

OBJECTIVE The purpose of this study was to investigate the inhibitory effect of β-catenin gene silencing on regulation of the biological behavior of neuroblastoma BE(2)C cells in vivo and in vitro. METHODS A lentivirus, carrying β-catenin siRNA, was used to stably knockdown β-catenin expression in neuroblastoma BE(2)C cells for assessing tumor cell viability, colony formation, cell cycle distribution, apoptosis, xenograft formation, and growth in nude mice. RESULTS Levels of β-catenin expression were markedly decreased in BE(2)C cells. Downregulation of β-catenin was concomitantly accompanied by reductions in colony formation and invasion capacity and by growth inhibition of BE(2)C cells in vitro. The mechanism appears to be a G0/G1 phase arrest and induction of apoptosis. In vivo, both tumor volume and weight of β-catenin knockdown cells were obviously reduced compared to the control and parental cells. CONCLUSION β-Catenin knockdown could effectively control growth of neuroblastoma cells in vitro and in nude mice, suggesting that targeting β-catenin may be useful in clinical control of neuroblastoma.

Neuroblastomas in Eastern China: a retrospective series study of 275 cases in a regional center

Purpose Most studies on neuroblastoma (NB) have been conducted in Western countries or Japan. The objective of our study was to analyze clinical and pathological features, MYCN status, surgical methods, and prognosis in Chinese NB patients. Methods A retrospective, single-center case series study of 275 NBs was implemented. Clinical manifestations, pathological features, MYCN status, and surgical treatment were analyzed. Log-rank test and Cox hazards models were used to assess overall survivals (OSs). Results The cohort consisted of 105 females and 170 males, with an age range of five days to 15 years. MYCN amplification was detected in 21.5% of all cases. The median OS was 15.0 months for MYCN amplified group. The five-year OS rates were 70.8% and 18.3% for MYCN unamplified and amplified groups, respectively, and the comparison of Kaplan–Meier curves for these two groups showed statistical significance (P < .001 by log-rank test). Gross total resection (GTR, n = 111) and subtotal resection (STR, n = 58) were administered in 169 patients at stages 3 and 4 who received chemotherapy and the comparison of Kaplan–Meier curves for different groups in these patients had statistical significance (STR vs. GTR, P = .009; MYCN unamplified vs. amplified, P < .001 by log-rank test, respectively).The multivariate survival analyses showed statistical significance (STR vs. GTR, P = .047; MYCN unamplified vs. amplified, P = .001 by Cox regression model). Conclusions MYCN amplification is an independently adverse prognostic factor in Chinese NB patients at stages 3 and 4 and GTR is associated with improved OS compared with STR in these patients.

Ectopic nephrogenic rests in children: A series of 13 cases in a single institution

Ectopic nephrogenic rests (ENR) are extremely rare, and their diagnosis and treatment have not yet been standardized. Our study aimed to analyze the clinical and pathological features of ENR in children and explore the optimal methods for diagnosis and treatment.