Background: Neuroblastoma is the most common malignancy in children younger than seven years of age and is the most frequent extracranial solid tumor that occurs in childhood. While opsoclonus-myoclonus syndrome (OMS), a paraneoplastic neurologic syndrome, affects 2-3% of children with neuroblastoma, and the percentage of mediastinal localization of the tumor is 49%. The objective of this study was to identify and characterize features of the OMS syndrome and treatments of mediastinal and non-mediastinal neuroblastoma associated with OMS.Methods: A systematic review of the literature was performed using PubMed, Medline, Web of Science, Embase and Cochrane. The search has no limit on date with the last search done on Dec 31, 2020. There is no publication restrictions or study design filters applied in the search.Results: Fifty-five out of 242 papers were identified and met our study eligibility. There were 77 cases found (28 cases had Mediastinal neuroblastoma, and 49 cases had non-mediastinal neuroblastoma). Data from trials showed that cases with mediastinal neuroblastoma who seemed to have undergone less treatment for OMS [rate ratio (RR) 0.41 (95% CI: 0.22-0.76)] had resulted in decreasing persistent neurologic symptoms [RR 0.31 (95% CI: 0.10-0.96)].Conclusions: Children who have OMS and mediastinal neuroblastoma may be associated with more favorable clinical and biological characteristics and better outcomes than children who have OMS and nonmediastinal neuroblastoma, and they are more likely to present with a single neurological symptom at first.The OMS in mediastinal neuroblastoma might also be treated effectively through resection of the tumor followed by appropriate radiotherapy and chemotherapy, and no long-term treatments of OMS is indicated.
BackgroundCholedochal cysts (CC) are rare disorders characterized by congenital biliary dilatation of the intrahepatic or extrahepatic bile ducts and always relate to pancreaticobiliary maljunction. Robot-assisted surgery has been able to complete almost all pediatric endoscopic surgery nowadays. But evidence of the post-operative outcomes of robotic-assisted operation is limited, comparing with the laparoscopic operation and traditional open operation. The aim of this meta-analysis was to identify the advantages and deficiencies about robotic-assisted operation for CC.MethodsA meta-analysis of retrospective studies published in PUBMED, MEDLINE, Web of Science and China National Knowledge Infrastructure (CNKI). No date limit was used, with the last search on April 30, 2022. No publication restrictions or study design filters were applied.ResultsNine retrospective cohort studies with 1,395 patients [366 in the robotic-assisted operation group (RG), 532 in the laparoscopic operation group (LG) and 497 in the open operation group (OG)] were enrolled in our study. Subgroup analysis demonstrated the RG had significant longer operative time [standardized mean difference (SMD) = 1.59, 95% CI = (0.02, 3.16), P < 0.05], less blood loss [SMD = −1.52, 95% CI = (−2.71, −0.32), P < 0.05], shorter enteral feeding time [SMD = −0.83, 95% CI = (−1.22, −0.44), P < 0.001], shorter time to stay in the hospital [SMD = −0.81, 95% CI = (−1.23, −0.38), P < 0.001], fewer post-operative complications [Relative risk (RR) =1.09, 95% CI = (1.04, 1.13), P < 0.001] but higher expenses [SMD = 8.58, 95% CI = (5.27, 11.89), P < 0.001] than LG. While a significant older age [SMD = 0.46, 95% CI = (0.26, 0.66), P < 0.001], longer operative time [SMD = 3.96, 95% CI = (2.38, 5.55), P < 0.001] and shorter time to stay in the hospital [SMD = −0.93, 95% CI = (−1.62, −0.25), P < 0.05] than OG.ConclusionsLaparoscopic and robotic-assisted procedure are both safe and minimal invasive operational strategies. Robotic-assisted procedure may slowly surpass and has a trend to replace laparoscopy for its advantages. More experiences in robotic-assisted operation should be accumulated for the unexpected complexities, so as to be more stable in the younger age of children.
Background:Acute appendicitis (AA) is one of the most common abdominal emergencies in children. There has been an increasing interest in endoscopic retrograde appendicitis treatment (ERAT) in the past twenty years. We performed a meta-analysis of ERAT for AA in children through a survey and an extensive literature search that summarized the clinical features and therapeutic efficacy of ERAT. Methods: A systematic review and meta-analysis of retrospective studies published in PUBMED, MEDLINE, Cochrane, China National Knowledge Infrastructure (CNKI), WanFang and VIP Database. The search has a limit on date from Jan 1, 2012 to Jun 31, 2022, with the last search done on Oct 31, 2022. No publication restrictions or study design filters were applied. The registration number in PROSPERO was CRD42022377739. Results: Seven retrospective cohort studies with 423 patients were enrolled in our study. In our study, almost 57.6% (95% confidence interval (CI) 52.8%-62.4%) of children who underwent ERAT were male, the pooled rate of successful intubation was 99.5%(95%CI 98.2%-100.0%), the average time of an ERAT is approximately 49 minutes(95% CI 48.179,49.833), and efficiency of ERAT for acute appendicitis was 99.0% (95% CI 96.5%-100.0%), while, the pooled rate of recurrence was 4.2% (95% CI 2.2%-6.7%), the mean time of hospital stays was 4.319 (95% CI 4.254-4.385) days probably, and occurrence of postoperative complication was about 3.9% (95% CI 2.0%-6.2%). Conclusions: Our results testify the relevant morbidity, clinical characteristics and prognosis in the ERAT, in spite of an enormous heterogeneity among few primary studies. ERAT has high efficiency and low recurrence rate in children with acute uncomplicated appendicitis, and preserves the physiological function of the appendix and avoids sizeabledamage, so it can be a safe and effective treatment for appendicitis in children.
Background In contrast to all other known kinds of regulated cell death, cuproptosis is a unique copper-dependent cell death pathway that has a close connection to mitochondria and metabolism. The pathogenesis of Wilms tumor (WT), a common pediatric abdominal tumor, has yet to be fully defined. Besides, previous studies on lncRNAs related to Cuproptosis in WT and the connection between Wilms tumor, the microenvironment, and cuproptosis are still scarce.Results Our results show that Individuals with WT and high-risk group identified utilizing the signature were related to poorer survival outcomes. The study revealed that high-risk group had a worse prognosis (P < 0.001) in contrast with the group of low-risk. The risk score AUC for this signature was 0.818, which was higher than the risk score for age (0.524), sex (0.580), race (0.489), stage (0.673) and the modeling of ferroptosis-related lncRNAs (0.775). Individuals in the group of low-risk had an elevated TIDE scores ratio and were more sensitive to 13 drugs, including BI-2536, EX-527, IspinesibMesylate, KIN001-135, etc.Conclusions Our model can precisely predict the WT patients' prognosis and differentiate between low- and high-risk WT patients. It is hoped that this study will give a novel method for clinical prognosis and appropriate WT patients therapy.
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