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Background: Evidence regarding the characteristics and prognosis of neuroblastoma (NBL) in China is limited. We aimed to investigate the characteristics and prognosis of intermediate- or high-risk NBL in children in China. Methods: We included 147 patients with intermediate- or high-risk NBL evaluated from January 2006 to March 2015. The patients were aged 1 month to 15.5 years, 66% of them were boys, and 117 (79.6%) were diagnosed with high-risk NBL. Results: After a median follow-up of 32.5 months, 80 (45.6%) patients survived, with a median survival time of 48 months (95% confidence interval [CI]: 36.41–59.59). High-risk patients (hazard ratio [HR]: 12.467; 95% CI: 11.029–12.951), partial response (PR) (HR: 1.200; 95% CI: 1.475–2.509) or progression disease (PD) (HR: 1.924; 95% CI: 1.623–3.012) after induction chemotherapy, and intracranial metastasis (HR: 3.057; 95% CI: 0.941–4.892) were independent risk factors for survival (p < 0.05) and postrelapse survival (p < 0.05). NBL relapse, male sex, and PR or PD after induction chemotherapy were risk factors for event-free survival (p < 0.05). Conclusions: In addition to previously established independent risk factors, such as age, risk group, and relapse, efficacy of induction chemotherapy and intracranial metastasis play significant roles in the prognosis of NBL. [ABSTRACT FROM AUTHOR]

Background: The aim of the present study was to summarize the clinical characteristics of in children aged under 3 years and analyze the related factors affecting the prognosis.Methods: The clinical data of 316 children aged under 3 years (192 males and 124 females) who were admitted to Beijing Tongren Hospital with a pathological diagnosis of HB between May 2005 and May 2019 were analyzed retrospectively. The factors influencing the therapeutic effects on and survival of HB in children with HB were analyzed.Results: (1) The median age of the enrolled patients was 1.45 years. The most common initial symptom was an abdominal mass (69.0%). The average serum alpha-fetoprotein (AFP) level and platelet count at the initial visit were (97,406.5 ± 5022.8) ng/mL and (418 ± 206) × 109/L, respectively. The epithelial type was the main pathological type (51.9%). According to the PRETEXT preoperative typing system, the most common stage was stage III (57.0%), whereas according to the postoperative Evans staging system, the most common stage was stage IV (41.8%). At the initial visit, 62 cases (19.6%) had vascular invasion, 52 cases (16.5%) had extrahepatic tumor extension, and 20 cases (6.3%) had tumor rupture. Distant metastasis occurred in 132 cases, and the most common metastatic site was the lung (80.3%). The incidence in East China was relatively high (35.4%). (2) The children were followed up until May 2020 (the median follow-up duration was 62 months). It was found that 194 patients had complete remission and 62 had partial remission. The Kaplan-Meier survival analysis showed that the overall survival was 95.3, 88.2, and 79.8% at 1 year, 3 years, and 5 years, respectively, and the event-free survival was 91.1, 83.2, and 75.1%, respectively. The Cox regression analysis showed that AFP level, platelet count, PRETEXT IV, vascular invasion, and distant metastasis at the initial visit were independent risk factors for the prognosis of children with HB (p < 0.05 in all).Conclusion: The prognosis of HB was correlated with the AFP level, platelet count, PRETEXT staging, vascular invasion, and distant metastasis at initial diagnosis. [ABSTRACT FROM AUTHOR]

Background: This study explores and analyzes the clinical characteristics and prognostic factors of hepatoblastoma (HB) in children under 6 years old and establishes a new risk-stratification system for individualized therapy.Methods: The clinical data of 382 pediatric patients under 6 years old (231 males and 151 females) who had been diagnosed with HB by pathology between May 2005 and May 2019 were collected. By analyzing the risk factors influencing the survival rate of patients with HB, a new risk-stratification system was established, and it was compared with previous risk-stratification systems by a receiver operating characteristic (ROC) curve.Results: (1) According to a Kaplan-Meier survival analysis, the one-year, three-year, and five-year overall survival (OS) was 93.7, 84.0, and 73.9%, respectively, and the event-free survival (EFS) was 90.5, 79.2, and 67.5%, respectively. (2) The independent risk factors influencing prognosis in pediatric patients with HB were alpha-fetoprotein (AFP) < 100 ng/ml or > 1000 ng/ml (HR = 3.341, P = 0.005); platelet count > 400 × 109/L (pooled hazard ratio [HR] = 2.123, P = 0.026); PRETEXT stage IV (HR = 4.026, P = 0.001); vascular involvement (HR = 2.178, P = 0.019); distant metastasis (HR = 2.634, P = 0.010);and multifocality (HR = 2.215, P = 0.012). (3) A new risk-stratification system was established and divided into three groups: low risk, moderate risk, and high risk. There were statistical differences among the three groups (P = 0.002). Compared with the previous risk-staging systems, there was no significant difference in the survival rate. Although the effect in the guiding therapy was the same, the area under the curve for the ROC curve was 0.835 (95% CI: 0.784-0.885) for the new stratification system.Conclusion: This new risk-stratification system had a better predictive value for the prognosis of pediatric patients with HB than other stratification systems. [ABSTRACT FROM AUTHOR]

Background: To analyze the clinical characteristics, treatment and prognosis of children with unilateral retinoblastoma (RB) and intracranial segment of retrobulbar optic nerve invasion.Methods: A total of 14 children with unilateral RB and intracranial segment of retrobulbar optic nerve invasion were enrolled in this retrospective study from January 2009 to December 2018. Clinical characteristics, treatment and prognosis were collected and analyzed. Survival curves were calculated by Kaplan-Meier method.Results: Of 14 cases, there were 7 male and 7 female, ranging in age from 22.85 to 121.97 months (median, 41.03 months). Seventy-one percent of patients came from first-tier cities in China and effected in the left eye. Magnetic resonance imaging (MRI) results indicated that all patients presented with thickened and enhanced optic nerve and intracranial segment of optic nerve invasion. Nine patients received comprehensive therapeutic regimen (chemotherapy, eye enucleation, radiotherapy and intrathecal therapy). The patients were followed up to December 2019, with a median follow-up of 20.6 months. The median disease specific survival was 48.99 ± 8.62 months, and the overall survival (OS) rate was 64.3%. Radiotherapy and comprehensive therapeutic regimen had significant impact on survival time (all p < 0.05).Conclusions: The overall prognosis of unilateral RB patients with intracranial segment of retrobulbar optic nerve invasion was poor. Chemotherapy and surgical treatment were necessary, but more attention should be paid to radiotherapy and intrathecal therapy for improving prognosis. [ABSTRACT FROM AUTHOR]

Thoracic spinal stenosis is a relatively common disorder causing paraplegia in the population of China. Until nowadays, the clinical management of thoracic spinal stenosis is still demanding and challenging with lots of questions remaining to be answered. A clinical guideline for the treatment of symptomatic thoracic spinal stenosis has been created by reaching the consensus of Chinese specialists using the best available evidence as a tool to aid practitioners involved with the care of this disease. In this guideline, many fundamental questions about thoracic spinal stenosis which were controversial have been explained clearly, including the definition of thoracic spinal stenosis, the standard procedure for diagnosing symptomatic thoracic spinal stenosis, indications for surgery, and so on. According to the consensus on the definition of thoracic spinal stenosis, the soft herniation of thoracic discs has been excluded from the pathological factors causing thoracic spinal stenosis. The procedure for diagnosing thoracic spinal stenosis has been quite mature, while the principles for selecting operative procedures remain to be improved. This guideline will be updated on a timely schedule and adhering to its recommendations should not be mandatory because it does not have the force of law. [ABSTRACT FROM AUTHOR]

The Eastern Staging System, which was specially developed for patients undergoing surgical resection for hepatocellular carcinoma (HCC), has been proposed for more than ten years. To prospectively validate the predictive accuracy of the Eastern staging on long-term survival after HCC resection. Patients who underwent hepatectomy for HCC from 2011 to 2020 at 10 Chinese hospitals were identified from a prospectively collected database. The survival predictive accuracy was evaluated and compared between the Eastern Staging with six other staging systems, including the JIS, BCLC, Okuda, CLIP, 8th AJCC TNM, and HKLC staging. Among 2365 patients, the 1-, 3-, and 5-year overall survival rates were 84.2%, 64.5%, and 52.6%, respectively. Among these seven staging systems, the Eastern staging was associated with the best monotonicity of gradients (linear trend χ2: 408.5) and homogeneity (likelihood ratio χ2: 447.3), and the highest discriminatory ability (the areas under curves for 1-, 3-, and 5-year mortality: 0.776, 0.787, and 0.768, respectively). In addition, the Eastern staging was the most informative staging system in predicting survival (Akaike information criterion: 2982.33). Using a large multicenter prospectively collected database, the Eastern Staging was found to show the best predictive accuracy on long-term overall survival in patients with resectable HCC than the other 6 commonly-used staging systems. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: Hepatocellular carcinoma (HCC) is common among adolescents and young adults (AYAs) in areas with endemic hepatitis B virus infection. We sought to characterize clinical features and long-term outcomes among AYAs versus older adults (OAs) who underwent HCC resection., Methods: From a Chinese multicenter database, patients were categorized as AYA (aged 13-39 years) versus OA (aged ≥40 years). Patient clinical features, perioperative outcomes, overall survival (OS) and time-to-recurrence (TTR) were compared. Multivariable Cox-regression analyses were performed to identify the impact of age on OS and TTR., Results: Among 1952 patients, 354(22.2%) were AYAs. AYAs were less likely to have cirrhosis yet were likely to have advanced tumor pathological characteristics than OAs. Postoperative morbidity and mortality were comparable. Compared with OAs, AYAs had a comparable OS but a decreased TTR. Multivariable analyses identified that young age (<40 years) was independently associated with poorer TTR., Conclusions: Compared with OAs, AYAs had a higher incidence of recurrence following liver resection among patients with HCC, suggesting that enhanced surveillance for postoperative recurrence may be required among AYAs., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: To determine whether the matrix metalloproteinase 9 gene (MMP9) polymorphism is associated with the onset or progression of adolescent idiopathic scoliosis (AIS) in Chinese Han female., Methods: Three single nucleotide polymorphisms (SNPs) (rs17576, rs2250889, rs1805088) were genotyped through TaqMan-based real-time PCR assay in 190 AIS patients and 190 controls, all of whom were females from Chinese Han population with matched age. Analyses performed included Hardy Weinberg equilibrium test, Pearson chi-square test, Logistic regression analysis, linkage disequilibrium analysis and haplotype analysis. The mean maximum Cobb angles with different genotypes in case-only dataset were also compared., Results: All 3 SNPs have reached Hardy-Weinberg equilibrium in the controls. Genotype and allele frequencies of all SNPs were found similar between cases and controls by Pearson chi-square test and Logistic regression. Genotype-phenotype analysis showed that patients with CC genotype in rs2250889 featured larger maximum Cobb angles., Conclusion: MMP9 may not be a predisposition gene of AIS in Han female. However, homozygous mutation in rs2250889 can render scoliosis more severe, implying that MMP9 defect may result in deterioration of AIS.

Objective: To explore the polymorphisms of vitamin D receptor (VDR) gene Tru I polymorphisms and the influence of this variation on Bsm I polymorphisms in Han nationality., Methods: Venous blood samples from 80 healthy individuals of Han nationality were collected and genomic DNA was extracted, VDR Bsm I and Tru I were tested by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) to analyze the polymorphisms of VDR gene; After using another primers to test VDR Bsm I in the same samples, the consistence of each method was assessed., Results: The frequencies of the VDR Tru I genotype in the groups were: TT 68.7%, Tt 26.3%, tt 5.0%; VDR Bsm I were: BB 6.2%, Bb 52.5%, bb 41.3%; Both polymorphisms were under Hardy-Weinberg equilibrium. After using another pair of primer, the frequencies of Bsm I genotype were BB 20.0%, Bb 26.2%, bb 53.8%, 22 genotype Bb changed to genotype BB or genotype bb in comparison with the result of first detection., Conclusion: The VDR Tru I polymorphism is found in the Han nationality, the distribution of this site's polymorphism is different from that of other nationalities. The presence of Tru I variation can result in some allele of Bsm I genotype drop-out in some study.

Objective: To sum up the experience in liver transplantation in a period of ten years at a single center., Methods: We retrospectively reviewed the clinical records of 120 patients receiving liver transplantation from April 1993 to October 2002. The patients' clinical characteristics, surgical techniques, complications and survival were compared in the phases of 1993-1997 (phase I), 1999 (phase II), and 2000-2002 (phase III)., Results: Malignant liver diseases were major indications for liver transplantation in phase I (100%) and II (53.3%), but decreased markedly in percentage in phase III (34.0%). When compared with recipients in phase I and II, the survival of recipients with benign liver diseases in phase III was significantly improved with the 3-month, 6-month and 1-year survival rates of 85.7%, 84.5% and 83.1%, respectively. For patients with malignant liver diseases, the 3-month, 6-month and 1-year survival rates were 87.4%, 81.1% and 46.0%, respectively. The reinfection rate of hepatitis B virus was 24% 12 months after transplantation. With technical refinements, the incidence of postransplantation vascular complications has significantly decreased from 29.4% in phase I and II to 4.9% in phase III. Biliary complications remained one of the major obstacles to long-term survival. No veno-venous bypass was applied in phase III, providing a promising outcome., Conclusion: Strict selection of potential recipients, technical refinement, appropriate management of vascular and biliary complications, and prophylaxis of recurrences of hepatitis B and malignant liver diseases are important to obtain long-term survival of patients receiving liver transplantation in China.