Importance As the most common subtype of pediatric rhabdomyosarcoma (RMS), the prognosis of embryonal RMS has rarely been investigated solely. Objective To perform a population‐based study to characterize the prognosis of embryonal RMS in children and adolescents. Methods Demographic and clinical features were retrospectively evaluated in selected patients with embryonal RMS registered in the Surveillance, Epidemiology, and End Results (SEER) program from 1988 to 2016. Survival curves were compared using the log‐rank test. A multivariate Cox proportional hazards model was developed to assess the impact of each factor on the overall survival. A nomogram was constructed based on the results of Cox regression model. Results A total of 464 patients were included in the analysis, among which 64.6% were male and 70.2% were white patients. About 38.6% and 26.3% of the patients were at 1–4 years and 5–9 years, respectively. Cox analysis showed that patients at age group 5–9 years had the lowest risk of mortality (hazard ratio [ HR ], 0.277; 95% confidential interval [ CI ], 0.123–0.620), compared with patients diagnosed at less than 1‐year‐old, and age group 1–4 years had the second‐best prognosis. Patients having distant tumors had significantly higher mortality risk ( HR , 4.842; 95% CI , 2.804–8.362) than the patients with localized tumor. Compared with receiving no surgery or radiotherapy, receiving any combination of surgery and radiotherapy would lower the risk of mortality significantly (for surgery without radiotherapy: HR , 0.418; for radiotherapy without surgery: HR , 0.405; and for surgery plus radiotherapy: HR , 0.410). Interpretation Age, stage at diagnosis, and treatment received were found to be the most important predictors of the overall survival of pediatric embryonal RMS.
Background: Laparoscopic pyeloplasty (LP) has been widely accepted as an effective method to treat ureteropelvic junction obstruction (UPJO) in pediatric patients. Limited studies focused on the relationship between risk factors and negative outcomes. Our study aimed to seek independent risk factors for negative outcomes and construct a prognostic nomogram to assist clinical decision-making and improve outcomes.Methods: A total of 535 patients with UPJO treated with primary LP between January 2016 and December 2020 were retrospectively reviewed. Negative outcomes were defined as restenosis requiring reoperation and grade III and IV complications based on the Clavien-Dindo grading system. Univariate and multivariate logistic regression analyses were used to select risk factors for negative outcomes after LP and developed the prediction model. The model was internally validated by the parametric bootstrapping method.Results: Among the 535 patients, 33 (6.2%) developed negative outcomes. Ten patients developed ureteropelvic junction (UPJ) restenosis and underwent secondary surgery. UPJ leakage (two cases), difficulty of urinating after the removal of the Double-J (DJ) stent (two cases), and ileus (two cases) were the most common grade IIIa complications, while distal ureteral stricture (five cases), hernia formation (three cases), and delayed wound healing around the fistula (two cases) were the most common grade IIIb complications.After univariate and multivariate logistic analyses, the patient's weight, preoperative anteroposterior pelvic diameter (APD), and difficulty of DJ stent insertion were independent risk factors for negative outcomes, and they were used to fit the prediction model. The Brier score was 0.048. The model was relatively wellcalibrated. The area under the receiver operating characteristic curve was 0.831 (95% CI: 0.756-0.906). Decision curve analysis illustrated good clinical utility.Conclusions: Primary LP is a safe and effective method for pediatric patients with UPJO. The patient's weight, preoperative APD, and difficulty of DJ stent insertion were independent risk factors for negative outcomes after LP. We established and validated a predictive model for negative outcomes after LP. With the help of this model, clinicians can make better decisions and improve patient outcomes.
Neuroblastoma (NB) is a rare type of cancer but frequently occurred in children. However, it is still unclear whether circular RNAs (circRNAs) play key roles in NB tumorigenesis or progression. In this study, we identified 39,022 circRNAs across the 39 neuroblastoma and 2 normal cell lines. With the gene and circRNA expression data, we classified the NB cell lines, identified and characterized the functional circRNAs in the 3 NB classes. Specifically, 29 circRNAs were found to be dysregulated in the NB classes. Notably, 7 circRNAs located within MYCN-amplified regions were upregulated in cell lines with the high activities of MYC targets and MYCN amplification, and were highly correlated with expression of their parental gene, NBAS. Subsequently, we constructed ceRNA networks for the functional circRNAs. Specifically, hsa_circ_0005379 was identified as a critical regulator in the ceRNA networks because of targeting 13 genes, which formed a complex competing endogenous RNA (ceRNA) network. Moreover, hsa_circ_0002343, which was connected with few genes, might regulate the PI3K/Akt/mTOR signaling via RAC1. Furthermore, 3 genes, including NOTCH2, SERPINH1, and LAMC1, involved in epithelial mesenchymal transition (EMT) were observed to connect with hsa_circ_0001361, suggesting that this circRNA was closely associated with EMT. Consequently, 7 genes, such as DAD1, PPIA, NOTCH2, PGK1, BUB1, EIF2S1, and TCF7L2, were found to be closely associated with both event-free survival (EFS) and overall survival (OS). In conclusion, the present study identified functional circRNAs and predicted their functionality in neuroblastoma cell lines, which not only improved the understanding of circRNAs in neuroblastoma, but also provided the evidences for the related researchers.
Importance Morbidity and mortality of children are important indicators of the performance of the public health system in any country. In China, the children's disease spectrum has gradually changed in recent years. However, the gender‐ and age‐specific disease spectrum for hospitalized children under 15 years old is still unclear. Objective To explore the gender‐ and age‐based distribution of diseases in hospitalized children under 15 years in China. Methods Medical records home page data for 2016 to 2018 were collected from 18 tertiary children's hospitals in China. The gender‐ and age‐specific disease spectrum was analyzed, using the tenth revision of the International Statistical Classification of Diseases and Related Health Problems (ICD‐10). Results The most common diseases were those of the respiratory system (25.7% of all 2 232 142 hospitalized children). The top three diseases for boys were diseases of the respiratory system (25.6%), diseases of the digestive system (11.4%) and certain conditions originating in the perinatal period (8.6%). The top three diseases for girls were diseases of the respiratory system (25.9%), certain conditions originating in the perinatal period (10.1%), and factors influencing health status and contact with health services (9.4%). The most common diseases for children under 1 year old were certain conditions originating in the perinatal period (38.1%). For all other age groups, the most common conditions were respiratory diseases (33.8% for those aged 1–3 years, 25.2% for those aged 4–6 years, and 12.2% for those aged 7–14 years). Interpretation This study analyzed the medical records home pages of 18 children's hospitals to provide the first overview of the disease spectrum and its gender‐ and age‐specific distribution among children in China.
BackgroundNo prior reports have focused on spinal cord injury (SCI) characteristics or inflammation after destruction of the blood–spinal cord barrier by syringomyelia. This study aimed to determine the differences in syringomyelia-related central SCI between craniocervical junction (CCJ) syringomyelia and post-traumatic syringomyelia (PTS) before and after decompression.MethodsIn all, 106 CCJ, 26 CCJ revision and 15 PTS patients (mean history of symptoms, 71.5 ± 94.3, 88.9 ± 85.5, and 32.3 ± 48.9 months) between 2015 and 2019 were included. The symptom course was analyzed with the American Spinal Injury Association ASIA and Klekamp–Samii scoring systems, and neurological changes were analyzed by the Kaplan–Meier statistics. The mean follow-up was 20.7 ± 6.2, 21.7 ± 8.8, and 34.8 ± 19.4 months.ResultsThe interval after injury was longer in the PTS group, but the natural history of syringomyelia was shorter (p = 0.0004 and 0.0173, respectively). The initial symptom was usually paraesthesia (p = 0.258), and the other main symptoms were hypoesthesia (p = 0.006) and abnormal muscle strength (p = 0.004), gait (p < 0.0001), and urination (p < 0.0001). SCI associated with PTS was more severe than that associated with the CCJ (p = 0.003). The cavities in the PTS group were primarily located at the thoracolumbar level, while those in the CCJ group were located at the cervical-thoracic segment at the CCJ. The syrinx/cord ratio of the PTS group was more than 75% (p = 0.009), and the intradural adhesions tended to be more severe (p < 0.0001). However, there were no significant differences in long-term clinical efficacy or peripheral blood inflammation markers (PBIMs) except for the red blood cell (RBC) count (p = 0.042).ConclusionPTS tends to progress faster than CCJ-related syringomyelia. Except for the RBC count, PBIMs showed no value in distinguishing the two forms of syringomyelia. The predictive value of the neutrophil-to-lymphocyte ratio for syringomyelia-related inflammation was negative except in the acute phase.
Background Congenital TORCH (Toxoplasma gondii (T. gondii), rubella virus (RV), cytomegalovirus (CMV), and herpes simplex virus (HSV)) infections are associated with a variety of adverse prenatal and neonatal events, including miscarriage, malformations and developmental abnormalities, and they remain an issue that cannot be neglected in China. However, the current research focuses more on the general screening of TORCH in women of childbearing age, and the medical information of children hospitalized due to congenital and perinatal TORCH infections has not been described in detail. This study summarized and analyzed the epidemiological characteristics, clinical manifestations, length of stay (LOS), and the disease burden of hospitalized children diagnosed with congenital TORCH infections in 27 children’s hospitals in China. Methodology Based on the face sheet of discharge medical records (FSMRs) of hospitalized children in 27 tertiary children’s hospitals collected in the Futang Research Center of Pediatric Development and aggregated into FUTang Update medical REcords (FUTURE), we summarized and analyzed the epidemiological characteristics, clinical manifestations, LOS, the disease burden (in US dollars, USD) and potential risk factors for hospitalized children diagnosed with congenital toxoplasmosis, congenital rubella syndrome, congenital cytomegalovirus infection, and congenital HSV in 27 children’s hospitals in China from 2015 to 2020. Results One hundred seventy-three patients aged 0–<1 year were hospitalized for congenital TORCH infections. Among infections with TORCH, hospitalization with congenital toxoplasmosis was the least common, with only five cases were reported (2.89%), while the LOS was the highest. The proportion of patients with congenital rubella syndrome (CRS) was 15.61%, and 86% of children hospitalized with CRS had cardiovascular malformations, and the economic burden was the highest. Congenital CMV infection cases accounted for the largest proportion (76.30%). Overall, 5.20% of patients were infected with HSV, and the expense of hospitalization for congenital HSV infection was relatively low. Conclusion In the present study, the hospitalization proportion due to congenital TORCH infection was extremely low (17.56 per 100,000 neonates), indicating that China’s congenital TORCH infection prevention and control policies remain effective. The lowest proportion of patients was hospitalized with congenital toxoplasmosis, while the LOS was the longest. The economic burden of CRS was heavy, and infants are recommended be vaccinated against RV in a timely manner. Congenital CMV infections accounted for the largest proportion of patients, suggesting that the disease burden of congenital CMV infection cannot be ignored, and the prevention of congenital CMV infection during pregnancy is still an important issue that needs to pay attention. The expense of hospitalization for congenital HSV infection was relatively low, while the disease burden increases significantly when patients develop complications. These data illustrate the importance of improving screening for congenital TORCH infections in the early diagnosis and treatment of neonatal patients.
Introduction There was a lack of attention to the screw placement techniques for surgical treatment of scoliosis in children and adolescents. This meta-analysis aims to compare the accuracy and safety of pedicle screw placement between 3D-printed navigation template technique and free-hand technique during corrective surgery for scoliosis in children and adolescents. Methods A comprehensive search was conducted for relevant articles up to December 2021 in databases including PubMed, Embase, MEDLINE, Cochrane, and Web of Science. The systematic meta-analysis compared the efficacy of pedicle screw placement between the two techniques, including accuracy of pedicle screw placement, complication rate, operation time, blood loss, mean place time per screw, and mean times for fluoroscopy. Results The seven studies involved 229 patients altogether. A total of 2805 pedicle screws were placed by two methods. Our results revealed that the 3D-printed guide template technique was more accurate than the free-hand technique in pedicle screw placement (OR=2.96; 95% CI, 2.24~3.91, P˂ 0.000) with a lower complication rate (OR=0.21; 95% CI, 0.06~0.78, P=0.02). The operation time (MD= -34.37, 95% CI, -67.47~-1.28, P=0.04) and mean place time per screw (MD= -3.11, 95% CI, -6.13~-0.09, P=0.04) and mean times for fluoroscopy (MD= -6.60, 95% CI, -8.66~-4.55, P<0.000) significantly decreased among patients in the 3D-printed navigation template group compared with those in the free-hand technique group. In addition, the two techniques had no significant statistical difference in blood loss. Conclusions Compared with the traditional free-hand technique, the 3D-printed guide template is a promising technique with higher accuracy and safety in screw placement for the surgical treatment of scoliosis in children and adolescents and is worth popularizing and validating by more prospective clinical studies.
AIM:To include new relevant findings in a systematic review to provide the first comparison of foramen magnum decompression with duraplasty (PFDD) and without duraplasty (PFD) in adult Chiari malformation Type I (CM-I). MATERIAL and METHODS:We retrospectively searched Web of Science, PubMed, Embase and ClinicalTrials.gov to summarize all relevant published papers on adults. A systemic review was adopted to evaluate clinical or radiological improvement, surgical complications, and reoperation rates between the PFD and PFDD groups.RESULTS: Nine papers containing information on 497 adult participants met the criteria. PFDD was related to a lower revision rate (RR=2.96, 95% CI: 1.34-6.51, p=0.007) but a higher complication rate (RR=0.35, 95% CI: 0.22-0.55, p<0.00001). No significant difference was noted between PFD and PFDD in terms of overall symptom improvement (RR=0.93, 95% CI: 0.84-1.03, p=0.17) or syringomyelia reduction (RR=0.84, 95% CI: 0.63-1.12, p=0.24). No significant difference in symptom improvement was observed between patients with syringomyelia (RR=0.86, 95% CI: 0.69-1.08, p=0.20) and patients without syringomyelia (RR=0.94, 95% CI: 0.68-1. 30, p=0.73).CONCLUSION: This systematic review of observational studies reveals that PFDD may provide lower revision rates but pose a higher risk than PFD in the management of CM-I in adults. However, PFD is similar to PFDD in clinical and radiological improvements.
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