ARID1B/SUB1-activated lncRNA HOXA-AS2 drives the malignant behaviour of hepatoblastoma through regulation of HOXA3.

It has been becoming increasingly evident that long non-coding RNAs (lncRNAs) play important roles in various human cancers. However, the biological processes and clinical significance of most lncRNAs in hepatoblastoma (HB) remain unclear. In our previous study, genome-wide analysis with a lncRNA microarray found that lncRNA HOXA-AS2 was up-regulated in HB. Stable transfected cell lines with HOXA-AS2 knockdown or overexpression were constructed in HepG2 and Huh6 cells, respectively. Our data revealed knockdown of HOXA-AS2 increased cell apoptosis and inhibited cell proliferation, migration and invasion in HB. Up-regulation of HOXA-AS2 promoted HB malignant biological behaviours. Mechanistic investigations indicated that HOXA-AS2 was modulated by chromatin remodelling factor ARID1B and transcription co-activator SUB1, thereby protecting HOXA3 from degradation. Therefore, HOXA-AS2 positively regulates HOXA3, which might partly demonstrate the involvement of HOXA3 in HOXA-AS2-mediated HB carcinogenesis. In conclusion, HOXA-AS2 is significantly overexpressed in HB and the ARID1B/HOXA-AS2/HOXA3 axis plays a critical role in HB tumorigenesis and development. These results might provide a potential new target for HB diagnosis and therapy.

Clinical characteristics and prognosis of adrenocortical tumors in children.

PURPOSE: The purpose of this study was to review the clinical characteristics and prognosis of children with adrenocortical tumors (ACT). METHODS: We retrospectively reviewed the medical records of 28 patients with ACT at our hospital between March 2010 and March 2017. RESULTS: The main clinical presentations were sexual prematurity (n = 17) and Cushing's syndrome (n = 15). All patients without metastasis underwent complete resection by laparotomy (n = 19) or laparoscopic surgery (n = 9). Pathological diagnosis confirmed adrenocortical carcinomas (ACC, n = 12) and adrenocortical adenomas (ACA, n = 16). Dehydroepiandrosterone (939.4 ± 148.2 µg/dl vs 630.9 ± 376.3 µg/dl; p = 0.031) and testosterone (235.7 ± 89.1 ng/dl vs 164.6 ± 47.5 ng/dl; p = 0.012) were significantly increased in ACC compared with ACA. The ACC tumor volumes were larger than those in ACA (107.5 ± 69 vs 25.5 ± 23.1 cm3; average diameter 6 cm vs 4 cm p = 0.001) and the immunochemical expression of Ki-67 was higher in ACC than in ACA (30.2 ± 22.7 vs 9.9 ± 4.9 p = 0.013). The mean follow-up of patients with ACA was 40 ± 23 months without recurrence. Seven patients with ACC had postoperative distant metastases and five patients died within 2 years. Five patients with ACC survived with a median follow-up of 27 months. The 2-year overall survival was 44.6%. CONCLUSIONS: Patients with ACC had significantly larger tumor volumes than those with ACA. The discordantly elevated serum levels of sexual corticosteroid hormones and lactate dehydrogenase may predict the malignant nature of these tumors. The prognosis of patients with ACA was good, while those with ACC had high postoperative metastasis and mortality rates.

The Clinical Characters of Congenital Choledochal Cysts in Perinatal Patients: A Retrospective Analysis in a Single Institution.

OBJECT: We aimed to investigate the clinical characters of congenital choledochal cysts (CC) in perinatal patients and to determine the most suitable time for their management. STUDY DESIGN: We retrospectively analyzed all the data of patients with CC in our hospital between 2003 and 2013. Clinical characteristics during the perinatal, infancy, and childhood periods were examined. RESULTS: A total of 216 cases were analyzed, including 12, 39, and 165 patients in their perinatal, infancy, and childhood periods, respectively. The incidence of abdominal pain and jaundice in the perinatal group was lower than that in other groups (p < 0.0001; p = 0.001). The incidence of abnormal inflammatory markers and liver function in perinatal cases was lower than in the other cases (p = 0.012; p = 0.002). The receiver-operating characteristic curves for liver function indicated that the cut-off point for age at which liver damage was found was 120 days. Of the 12 perinatal cases, there were no significant differences, with regards to operative time and hospital stay, between open and laparoscopic surgical approaches (p = 0.164; p = 0.722). CONCLUSION: Abdominal pain, jaundice, inflammatory markers, and liver function in perinatal patients with CC are better than in the infancy and childhood groups. We recommend surgical treatment before 120 days of age.

Effective multidimensional approach for practical management of the emergency department in a COVID-19 designated children's hospital in east China during the Omicron pandemic: a cross-sectional study.

Background: Characterized by rapid transmission but lower severity, the new Omicron wave brought about an acute increase in local corona virus disease 2019 (COVID-19) cases in Shanghai, followed by stricter infection prevention and control strategies. Inevitably, more time was required for emergency consultation and treatment of children with critical illnesses. Therefore, a multidimensional approach was designed to streamline the emergency service and reduce the incidence of nosocomial infection of sever acute respiratory syndrome coronavirus 2 (SARS-CoV-2) during the Omicron wave for the emergency department (ED) at the Children's Hospital of Fudan University (CHFU). Methods: A multidimensional approach was implemented in the ED to help achieve a balance between the demand for emergency services and pandemic control, consisting of ED layout adjustment; electronic screening (E-screening) measures; standard management processes for patients, medical staff, and goods transfer; reliable disinfection measures; and a surveillance system for infection prevention and control. To evaluate the effect of the management strategy, the data on nosocomial infection cases and occupational exposure episodes among staff in the ED were collected. The demographic and clinical characteristics of level I/II children by the five-level pediatric triage tool and their mean duration of stay in the resuscitation room were collected. Results: There were 12,114 ED visitors from March 1 to May 31 in 2022, among which 53.24% were medical emergencies (6,449/12,114) and 46.76% were surgical emergencies (5,665/12,114). Twenty-nine patients were sent to the buffer zone, four of whom were transferred to the pediatric intensive care unit (PICU) because of the critical situation. Six patients tested positive for COVID-19 after entering ED, including three in the buffer zone and three in the ED clinic, causing a temporary closure of the ED for disinfection. There were no reports on medical care delays, unintended deaths, staff with COVID-19 infection, or occupational exposures to COVID-19. Conclusions: Our findings highlight the effectiveness of the multidimensional approach, which can simultaneously meet the emergency care needs of patients as well as pandemic prevention and control. However, the results were obtained against the proportional decrease in clinic visitors due to the Shanghai lockdown. Dynamic assessment and further optimization may be adopted to cope with the pre-pandemic visit volume.

Household transmission of SARS-CoV-2 during the Omicron wave in Shanghai, China: A case-ascertained study.

OBJECTIVES: We used a case-ascertained study to determine the features of household transmission of SARS-CoV-2 Omicron variant in Shanghai, China. METHODS: In April 2022, we carried out a household transmission study from 309 households of 335 SARS-CoV-2 pediatric cases referred to a designated tertiary Children's Hospital. The detailed information can be collected from the 297 households for estimating the transmission parameters. The 236 households were qualified for estimating the secondary infection attack rates (SARI ) and secondary clinical attack rates (SARC ) among adult household contacts, characterizing the transmission heterogeneities in infectivity and susceptibility, and assessing the vaccine effectiveness. RESULTS: We estimated the mean incubation period and serial interval of Omicron variant to be 4.6 ± 2.1 and 3.9 ± 3.7 days, respectively, with 57.2% of the transmission events occurring at the presymptomatic phase. The overall SARI and SARC among adult household contacts were 77.11% (95% confidence interval [CI]: 73.58%-80.63%) and 67.03% (63.09%-70.98%). We found higher household susceptibility in females. Infectivity was not significantly different between children and adults and symptomatic and asymptomatic cases. Two-dose and booster-dose of inactivated COVID-19 vaccination were 14.8% (5.8%-22.9%) and 18.9% (9.0%-27.7%) effective against Omicron infection and 21.5% (10.4%-31.2%) and 24.3% (12.3%-34.7%) effective against the symptomatic disease. CONCLUSIONS: We found high household transmission during the Omicron wave in Shanghai due to presymptomatic and asymptomatic transmission despite implementation of strict interventions, indicating the importance of early detection and timely isolation of SARS-CoV-2 infections. Marginal effectiveness of inactivated vaccines against Omicron infection poses a great challenge for outbreak containment.

The impact of a closed-loop electronic blood transfusion system on transfusion errors and staff time in a children's hospital.

OBJECTIVES: To assess the impact of a closed-loop electronic blood transfusion system on transfusion errors and staff time. MATERIALS AND METHODS: Before and after study in all wards of a children's hospital, involving patients and staff of all the wards. The changes were closed-loop electronic blood transfusion, barcode patient identification, electronic blood transfusion administration records and error pop-up warning. The main outcome measures were percentage of blood transfusion errors, time spent on transfusion tasks. RESULTS: Transfusion errors were identified in 3.87% of 2556 blood transfusion orders pre-intervention and 0.78% of 2577 orders afterwards (P<0.01). Phlebotomists, nurses, and physicians may make mistakes, including wrong blood type when apply for blood, wrong patient when blood draw or transfusion, wrong dose when apply for blood and the wrong tube label when blood draw or cross-matching, which are significantly reduced after change (1.09% vs 0.31%, 1.13% vs 0%, 0.31% vs 0%, 1.33% vs.0.78%, P<0.01). Time spent on blood apply was 5.3±1.2min, hand over blood bag at the transfusion department was 14.9±1.4min and blood transfusion was 15.8±2.4min. Time per transfusion round decreased to 2.6±1.0min, 6.3±1.6min and 9.3±2.2min respectively (P<0.01). CONCLUSIONS: A closed-loop electronic blood transfusion, barcode patient identification and error pop-up warning reduced transfusion errors, and increased confirmation of patient and blood types identity before transfusion. Time spent on blood transfusion tasks reduced.

Epidemiological and clinical features of SARS-CoV-2 infection in children during the outbreak of Omicron variant in Shanghai, March 7-31, 2022.

OBJECTIVES: This study aimed to understand the epidemiological and clinical characteristics of pediatric SARS-CoV-2 infection during the early stage of Omicron variant outbreak in Shanghai. METHODS: This study included local COVID-19 cases <18 years in Shanghai referred to the exclusively designated hospital from March 7 to March 31, 2022. Clinical data, epidemiological exposure, and COVID-19 vaccination status were collected. Relative risks (RRs) were calculated to assess the effect of vaccination on symptomatic infection and febrile disease. RESULTS: A total of 376 pediatric cases of COVID-19 (median age: 6.0 ± 4.2 years) were referred to the designated hospital, including 257 (68.4%) symptomatic cases and 119 (31.6%) asymptomatic cases. Of the 307 (81.6%) children ≥3 years eligible for COVID-19 vaccination, 110 (35.8%) received two doses of vaccines. The median interval between the completion of two-dose vaccination and infection was 3.5 (interquartile range [IQR]: 3, 4.5) months. Compared with no vaccination, two-dose COVID-19 vaccination reduced the risks of symptomatic infection and febrile disease by 35% (RR 0.65, 95% confidence interval [CI]: 0.53-0.79) and 33% (RR 0.64, 95% CI: 0.51-0.81) among confirmed cases. Eighty-four percent of symptomatic cases had fever (mean duration: 1.7 ± 1.0.8 days), 40.5% had cough, and 16.4% had transient leukopenia. Three hundred and seven (81.6%) had an epidemiological exposure in household (69.1%), school (21.8%), and residential area (8.8%). CONCLUSION: The surge of pediatric COVID-19 cases and multiple transmission model reflect wide dissemination of Omicron variant in the community. Asymptomatic infection is common among Omicron-infected children. COVID-19 vaccination can offer some protection against symptomatic infection and febrile disease.

Tumor Suppressive Role of MUC6 in Wilms Tumor via Autophagy-Dependent ß-Catenin Degradation.

Wilms tumor is the most common renal malignancy in children. Known gene mutations account for about 40% of all wilms tumor cases, but the full map of genetic mutations in wilms tumor is far from clear. Whole genome sequencing and RNA sequencing were performed in 5 pairs of wilms tumor tissues and adjacent normal tissues to figure out important genetic mutations. Gene knock-down, CRISPR-induced mutations were used to investigate their potential effects in cell lines and in-vivo xenografted model. Mutations in seven novel genes (MUC6, GOLGA6L2, GPRIN2, MDN1, MUC4, OR4L1 and PDE4DIP) occurred in more than one patient. The most prevalent mutation was found in MUC6, which had 7 somatic exonic variants in 4 patients. In addition, TaqMan assay and immunoblot confirmed that MUC6 expression was reduced in WT tissues when compared with control tissues. Moreover, the results of MUC6 knock-down assay and CRISPR-induced MUC6 mutations showed that MUC6 inhibited tumor aggression via autophagy-dependent ß-catenin degradation while its mutations attenuated tumor-suppressive effects of MUC6. Seven novel mutated genes (MUC6, GOLGA6L2, GPRIN2, MDN1, MUC4, OR4L1 and PDE4DIP) were found in WT, among which MUC6 was the most prevalent one. MUC6 acted as a tumor suppressive gene through autophagy dependent ß-catenin pathway.

Diagnosis and outcome of neutropenic enterocolitis: experience in a single tertiary pediatric surgical center in China.

BACKGROUND/PURPOSE: Neutropenic enterocolitis (NE) is clinically defined by the triad of neutropenia, abdominal pain and fever. This retrospective study is to review 24 cases of NE in a single Chinese tertiary center, to elucidate clinical feature, treatments and outcome for this dangerous gastrointestinal complication of neutropenia. PATIENTS AND METHODS: The medical records of pediatric patients who were diagnosed with neutropenic enterocolitis from 2000 to 2009 were reviewed. RESULTS: Of 24 cases, the ratio of male to female was 2:1, the mean age was 7.2 years. There were eight cases of acute lymphocytic leukemia, eight cases of acute non-lymphocytic leukemia, four cases of non-Hodgkin's lymphoma, one case of severe aplastic anemia, one case of neuroblastoma and two cases of simple neutropenia without underlying cause. The hematologic malignancy was significantly associated with the occurrence of NE (OR = 19.4). Seventeen cases developed NE during anticancer chemotherapy (chemo group), four cases presented with leukemia and one case presented with aplastic anemia before the initiation of chemotherapy and their presenting event leading to diagnosis. Two cases simply presented with NE without definitive reasons (no chemo group). All the patients had the typical clinical presentation, six cases had disseminated peritonitis, toxic shock, and assisted ventilations were necessary in three of these six cases. CT or ultrasound demonstrated bowel wall thickness, paucity of air in the cecum and/or right colon, pneumatosis or pneumoperitoneum. There was no difference in the nadir neutrophil count in patients, who received chemotherapy versus those who did not (P = 0.001), but the recovering time from NE in chemo group (9.3 ± 1.9) was shorter than non-chemo group (10.7 ± 5.3, P = 0.034). Sixteen (88.8%) cases have been successfully managed medically, using aggressive hemodynamic support, bowel rest, and broad-spectrum antibiotic therapy with surgical intervention reversed only for the more severe six cases (25%). Two cases died. CONCLUSION: NE is a life-threatening gastrointestinal complication of neutropenia. Physicians might remain vigilant and consider NE in any neutropenic patient rather than only in oncologic patients. It has typical clinical presentation and CT can provide clear delineation for diagnosis. Early recognition and progressed management have reduced mortality. Most children with NE may be treated without surgery with favorable outcome.

Hypertension in 172 Chinese Children: An 8-Year Retrospective Study.

BACKGROUND: We evaluated children with hypertension and compared those with essential and secondary (including renal and non-renal) hypertension. METHODS: This retrospective study analyzed data from hypertensive children (age, 0-18 years) referred for treatment between January 2008 and December 2015. Demographic factors, causative factors, and medical treatments were evaluated. Treatment failure was defined as a systolic or diastolic blood pressure ≥95th percentile for age, gender, and height on three separate occasions, despite treatment. All patients not meeting the failure criteria were considered to have controlled hypertension. The control rate was defined as the proportion of patients with controlled blood pressure. RESULTS: Among 172 consecutive patients, 28% had essential hypertension and 72% had secondary hypertension. As compared with children with secondary hypertension, those with essential hypertension had a higher frequency of family history of hypertension (P<0.001), a higher body mass index (BMI) (P=0.001), lower frequency of proteinuria (P=0.003), lower uric acid (P=0.04), and lower triglyceride (P=0.048). The medications used in the controlled group were similar to those used in the uncontrolled group. Angiotensin-converting enzyme inhibitors (ACEIs) were only used in nephrogenic patients, and a higher rate of ACEI use seemed to increase control rates. Control rates did not significantly differ by age, number of drugs, or cause of hypertension. CONCLUSIONS: As compared with children with secondary hypertension, those with essential hypertension were more likely to have a family history of hypertension and had a higher BMI, lower frequency of proteinuria, and lower uric acid and triglyceride concentrations. Treatment guidelines for essential and secondary hypertension should be established for children of all ages.

Point-of-care training program on COVID-19 infection prevention and control for pediatric healthcare workers: a multicenter, cross-sectional questionnaire survey in Shanghai, China.

BACKGROUND: To evaluate the effectiveness of training on knowledge and practice of infection prevention and control (IPC) among pediatric health care workers (HCW) in Shanghai, China, in the context of COVID-19 pandemic. METHODS: An online training program was designed by the Shanghai Pediatric Clinical Quality Control Center (SPQCC) during the early phase of COVID-19 pandemic on disease knowledge and practice of IPC. Training took place in the 81 partner hospitals affiliated with SPQCC. A multicenter, cross-sectional questionnaire survey was designed with a 25-item self-administered questionnaire to evaluate the knowledge gained from the training. Stratified-random sampling was used to select HCW according to three professionals (i.e., pediatricians, nurses and administrators) within each partner hospital. Awareness and knowledge of COVID-19 and its related infection control and practice were assessed by comparing survey results between different types of hospitals, professionals and professional ranks. A higher survey score meant that the respondent was more prepared and knowledgeable about COVID-19 and its infection control measures. RESULTS: Completed questionnaires were returned from 1,062 subjects (385 pediatricians, 410 nurses, and 267 administrators), giving a response rate of 96.5%. Overall, awareness of clinical information related to COVID-19, importance of personal hygiene and isolation policy was high among the respondents. No statistical difference of scores on knowledge of COVID-19, IPC and relevant practice between the tertiary and peripheral hospitals. Among all respondents, middle-ranked health care personnel were most knowledgeable and achieved the highest score. CONCLUSIONS: Majority of pediatric HCW showed good recognition and practice in infection protection and control measures. The online training was able to achieve its aim to enhance knowledge and awareness and could have contributed to the zero infection rate among HCW caring for confirmed COVID-19 cases in Shanghai.

Clinical features and risk factors for severe influenza in children: A study from multiple hospitals in Shanghai.

BACKGROUND: The incidence and mortality of influenza in children had risen, but data are limited on children with severe influenza virus infection in China. METHODS: We conducted a retrospective case-control study and collected the patients' clinical data. Clinical data including demography, clinical presentation, laboratory findings, radiologic findings, treatment and outcomes were collected. Children were clinically confirmed to have virus infection in Shanghai in three hospitals from June 2014 to June 2019. RESULTS: During the study, 36,047 children were enrolled. Among them, 118 met the criteria for severe flu. Clinical symptoms such as fever, cough, gastrointestinal symptoms, coma and epilepsy were higher in the severe group. Complications such as pneumorrhagia, heart failure, septic shock, acute renal failure and influenza-associated encephalitis were higher in the severe influenza group than the death group. The laboratory findings including decreased hemoglobin, high alanine aminotransferase, high urea nitrogen and high lactate levels were risk factors for death in children with influenza. CONCLUSION: Influenza-associated encephalopathy (IAE), acute respiratory distress syndrome (ARDS) were the common clinical manifestations and complications for the severe influenza, and delayed use of oseltamivir was found to be associated with fatality.

Improvement of the functioning and efficiency of a Code Blue system after training in a children's hospital in China.

BACKGROUND: Code Blue is a popular hospital emergency code that is used to alert the emergency response team to any medical emergency requiring critical care. By retrospectively studying Code Blue cases in a children's hospital, we looked for high-risk factors associated with survival and how to improve the effectiveness of Code Blue systems through training. METHODS: Data were collected on age, gender, department, diagnosis, time of Code Blue call activation, time between call and arrival of the Code Blue team, treatment details and outcome before and after the training process from January 2016 to December 2019. Chi-square test and logistic regression analysis were used to analyze the data. RESULTS: A total of 139 Code Blue cases from the period of January 2016 to December 2019 were retrospectively studied. The wards where Code Blues occurred most frequently were the infectious diseases ward (n=31, 22.3%), the hematology and oncology ward (n=30, 21.6%), and the cardiology ward (n=15, 10.8%). Age, inpatient status, time of arrival, the time of cardiopulmonary resuscitation (CPR), and the cause of shock were all risk factors for death. After the training, the arrival time and recovery time were significantly reduced (P<0.01). The proportion of patients who were transferred to the ICU had increased (P<0.05), and the proportion of deaths had decreased (P<0.01). The survival curve improved (P<0.05). CONCLUSIONS: It is very important to summarize the risk factors related to Code Blue. It is clear that the efficacy of the Code Blue events improved after training of the hospital staff in the Children's Hospital.

S119N Mutation of the E3 Ubiquitin Ligase SPOP Suppresses SLC7A1 Degradation to Regulate Hepatoblastoma Progression.

A previous study on hepatoblastoma revealed novel mutations and cancer genes in the Wnt pathway and ubiquitin ligase complex, including the tumor suppressor speckle-type BTB/POZ (SPOP). Moreover, the SPOP gene affected cell growth, and its S119N mutation was identified as a loss-of-function mutation in hepatoblastoma. This study aimed to explore more functions and the potential mechanism of SPOP and its S119N mutation. The in vitro effects of SPOP on cell proliferation, invasion, apoptosis, and in vivo tumor growth were investigated by western blot analysis, Cell Counting Kit-8, colony formation assay, flow cytometry, and xenograft animal experiments. The substrate of SPOP was discovered by a protein quantification assay and quantitative ubiquitination modification assay. The present study further proved that SPOP functioned as an anti-oncogene through the phosphatidylinositol 3-kinase/Akt signaling pathway to affect various malignant biological behaviors of hepatoblastoma both in vitro and in vivo. Furthermore, experimental results also suggested that solute carrier family 7 member 1 (SLC7A1) might be a substrate of SPOP and influence cell phenotype by regulating arginine metabolism. In conclusion, these findings demonstrated the function of SPOP and revealed a potential substrate related to hepatoblastoma tumorigenesis, which might thus provide a novel therapeutic target for hepatoblastoma.

A quickly, effectively screening process of novel corona virus disease 2019 (COVID-19) in children in Shanghai, China.

BACKGROUND: A recent cluster of pneumonia cases in China was caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We report the screening and diagnosis of corona virus disease 2019 (COVID-19) in our hospital. METHODS: Developed a procedure for the identification of children cases with COVID-19 in outpatient and emergency department of our hospital, then we observed how this process works. RESULTS: (I) There were 56 cases considered suspected cases, and 10 cases were confirmed as COVID-19. (II) Of the 10 confirmed COVID-19 cases admitted in our hospital, 5 were males and 5 were females, aged from 7 months to 11 years, the average age is 6.0±4.2 years, 6 cases were mild pneumonia, the others were upper respiratory tract infection. (III) We followed up 68 patients in isolation at home until symptoms disappeared. Non were missed in the patient's first visit. The sensitivity of this method is 100% and the specificity is 71.3%. CONCLUSIONS: Our screening process works well, and it is also necessary to establish a screening network in the hospital.

Clinical features, treatment, and outcomes of bilateral Wilms' tumor: A systematic review and meta-analysis.

BACKGROUND: Wilms' tumor(WT) is the most common malignant renal tumor of childhood. Despite the good prognosis of WT, bilateral Wilms' tumor (BWT) still has a poor outcome. We systematically reviewed the literature on BWT, aiming to define its clinical features, treatment, and outcomes. METHODS: PubMed, OVID EMbase, Web of Science, and Cochrane Library were systematically searched for studies published from 1980 to 2017. Case series and comparative studies reported clinical data of BWT patients were included. RESULTS: A total of 32 studies comprising 1457 patients were retained for primary outcome. Hemihypertrophy, cryptorchidism, and Beckwith-Wiedemann syndrome(BWS) are the most common congenital anomalies and syndrome. 86% of patients had favorable histology (FH). Patients with local stage I or II accounted for 64%, and 12.6% had metastasis at diagnosis. Bilateral nephron-sparing surgery (NSS) was achieved in 33.8%. Recurrence and renal failure occurred in 20% and 8%. The overall survival (OS) was 73%. In comparative studies, OS of patients undergoing bilateral NSS was similar to that of other operation types. CONCLUSION: Prognosis of BWT has been improved but is significantly poorer than WT. Bilateral NSS was recommended by most centers to preserve more renal volume. However, finding a balance between retaining renal function and avoiding recurrence remains a question. TYPE OF STUDY: Systematic review. LEVEL OF EVIDENCE: Level IV.

Aberrant RASSF5 gene transcribed region hypermethylation in pediatric hepatoblastomas.

BACKGROUND: Aberrant DNA methylation plays an important role in cancer and has been recognized to contribute to the activity of oncogenes and inactivity of tumor suppressor genes. RAS association domain family (RASSF) members have been shown to be epigenetically silenced by promoter methylation in cancers, including hepatoblastoma. METHODS: We assessed the methylation patterns in the gene of RASSF5 from hepatoblastoma tissue samples harvested from patients using high-throughput mass spectrometry on a matrix-assisted laser desorption/ionization time-of-flight mass array. RESULTS: Hypermethylation was found in the RASSF5 gene transcribed regionand was correlated with downregulation of RASSF5 RNA expression levels in the hepatoblastoma samples. CONCLUSIONS: The results indicate that aberrant methylation of RASSF5 may contribute to its downregulated mRNA expression in hepatoblastoma.

The surgical choice for unilateral thyroid carcinoma in pediatrics: Lobectomy or total thyroidectomy?

BACKGROUND: Total thyroidectomy is no doubt the standard procedure for patients with bilateral thyroid carcinoma in pediatrics. However, for lesions confined in unilateral thyroid gland, lobectomy or radical total thyroidectomy is still controversial in pediatrics. METHODS: Thirty-five cases of pediatric thyroid carcinoma which were confirmed by pathology were retrospectively analyzed in our hospital from 2005 to 2016. In 13 cases of unilateral lesions, 6 received lobectomy, while others underwent total thyroidectomy. Recurrence and complications were compared between 2 groups. Also, there were 20 cases of primary total thyroidectomy and 4 cases of supplementary resection of the residual gland. The short-term complications were compared. RESULTS: There were no differences between groups of lobectomy and total thyroidectomy in size, extrathyroid extension, stage, cervical lymph nodes metastases, and distant metastases. They were the same in incidence of postoperative hypocalcemia, calcium supplement, recurrent laryngeal nerve damage, and recurrence rate and mortality, except that the length of stay in hospital was shorter in lobectomy group. Patients in supplementary resection were more likely to present the symptoms of postoperative hypocalcemia and hypokalemia, and it was difficult to identify the parathyroid gland during the operation by comparing with patients of primary total thyroidectomy. CONCLUSIONS: Thyroid carcinoma in pediatrics is inclined to be multifocal, and even in a single focus, the volume is large. Total thyroidectomy does not bring more complications. In contrast, the morbidity increases in staged resection. Endocrine hormone supplement can satisfy the need for growth and development. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.

Circulating D-dimer level correlates with disease characteristics in hepatoblastoma patients.

OBJECTIVES: Hepatoblastoma (HB) is the most common pediatric liver malignancy, typically affecting children within the first 4 years of life. However, only a few validated blood biomarkers are used in clinical evaluation. The current study explored the clinical application and significance of D-dimer levels in patients with HB. METHOD: Forty-four patients with HB were included in this retrospective study. D-dimer and plasma fibrinogen levels were examined, and their correlation with other clinical features was analyzed. D-dimer and plasma fibrinogen levels were examined between HB and other benign hepatic tumors. RESULTS: D-dimer levels were significantly associated with high-risk HB features, such as advanced stage and high α-fetoprotein (AFP) levels. Higher D-dimer levels were observed in stage 4 patients compared with stage 1/2/3 patients (P = .028). Higher D-dimer levels were also observed in patients with higher AFP levels before chemotherapy compared with patients with lower AFP levels after chemotherapy (P< 0.001). In addition, higher D-dimer levels were observed in HB compared with other benign hepatic tumors such as hepatic hemangioma and hepatocellular adenoma (P = .012). No significant difference in D-dimer levels was found between sex (P = .503) and age (≥12 vs <12 months, P = .424). There was no significant difference in plasma fibrinogen levels between sex or age and high-risk HB features, such as advanced stage and high AFP levels. CONCLUSIONS: Elevated D-dimer levels could be a useful determinant biomarker for high-risk features in patients with HB. This finding also supports the clinical application of D-dimer in HB.