LI Miao, ZHU Shuai, ZHI Tian, XIE Yao, SUN Yanling, WANG Shumei, DU Shuxu, GONG Xiaojun, JIN Mei, GE Ming, GONG Jian, ZHAO Weihong, LIU Rong, HUANG Dongsheng, SUN Liming, WU Wanshui, MA Xiaoli
Background: Central nervous system(CNS) tumors in infancy are rare and very few reports are available in China.
Objective: To investigate the clinical features, pathological spectrum, survival outcome and prognostic factors in infants with CNS tumors.
Design: Retrospective cohort study.
Methods: The starting point of the cohort was from June 2011 to December 2019, and infants under the age of 12 months with the initial diagnosis of primary CNS tumors were enrolled and treated by a multidisciplinary model of surgery in combination with chemotherapy. The end of followup was March 31, 2020. Metastasis, tumor site, tumor grade, tumor diameter, degree of resection, and chemotherapy were used as prognostic factors for overall survival (OS) and eventfree survival (EFS).
Main outcome measures: Prognostic factors for OS and EFS.
Results: Among 52 patients included in this study, there were 36 males and 16 females. The median age of onset was 6.7 (range, 012) months, less than 6 months in 18 cases, including 2 cases of neonatal onset. Thirtythree cases were supratentorial, 17 cases were infratentorial, and 2 cases were in spinal cord. The tumor diameter was <5 cm in 26 cases, ≥ 5 cm in 19 cases , and unknown in 7 cases. A total of 44 cases (84.6%) underwent surgical resection of tumors with 39 cases (89%) under gross total/ near total resection, five cases under partially resection, and 1 case of noninvasive brain biopsy. At the time of diagnosis, 4 patients experienced metastasis (M+). Eight cases gave up due to economic reasons or high surgical risk and all died 0.9 (01.24) months after diagnosis. Twentyfour cases of lowgrade tumors (WHO grade I+Ⅱ) and 20 highgrade tumors (WHO grade Ⅲ+Ⅳ) were confirmed. There were 17 cases of choroid plexus tumor, 11 cases of glioma, 13 cases of embryonal tumors, 2 cases of atypical teratoma, and 1 case of craniopharyngioma. Twentyfour patients (46%) received chemotherapy after surgery among whom eleven cases relapsed or experienced progression including 10 cases of death, and 13 cases were in eventfree survival and including 1 case of survival with events. Among twenty cases (46%) who received no chemotherapy, 1 case died after relapse or progression, 19 cases were in eventfree survival. Multivariate COX regression analysis demonstrated that the hazardratio of OS and EFS of nongross total tumor resections was 5.7 （95% CI：1.408~23.115） and 5.1（95% CI：1.260~20.731）compared with gross total /near total tumor resections; 18.0（95%CI：2.222~146.5897）and 8.3（95%CI：1.687~40.530）were for high WHO grade tumors compared with lowgrade tumors; 4.2（95%CI：1.563~11.291）and 4.9（95%CI：1.996~12.216）were for infratentorial tumors compared with supratentorial cases.
Conclusion: Primary lowgrade tumors in infancy have good prognosis. Highgrade tumors such as atypical teratoma /rhabdoid tumors, embryonic tumors with multiple rosettes and pineoblastoma have poor prognosis. Nongross total /near total tumor resections, highgrade tumor and infratentorial tumor location are poor prognostic factors for OS and EFS.
HE Leiyan, FU Pan, WU Xia, WANG Chuanqing, YU Hui, XU Hongmei, JING Chunmei, DENG Jikui, WANG Hongmei, HUA Chunzhen, CHEN Yinghu, CHEN Xuejun, ZHANG Ting, ZHANG Hong, CHEN Yiping, YANG Jinhong, LIN Aiwei, WANG Shifu, CAO Qing, WANG Xing, DENG Huiling, CAO Sancheng, HAO Jianhua, GAO Wei, HUANG Yuanyuan
Background: Chinese ISPED (Infectious Disease Surveillance of Pediatrics) was established in 2015. The data of antibiotics resistance were collected from each member hospital and summarized every year to monitor the bacterial drug resistance among Chinese children.
Objective: This study aimed to investigate the antimicrobial resistance profiles of pathogens in Chinese children and guide the reasonable use of antibiotics.
Design: This is a crosssectional survey. Every member hospital was required to perform the standard procedure of bacterial culture, identification and antimicrobial susceptibility test and to report the isolated strains and drugresistant bacteria. All data were analyzed to reflect the current bacterial infection and drug resistance among children in mainland China.
Methods: Clinical isolates were collected from 11 tertiary children's hospitals in China from January 1st to December 31st in 2020. Antimicrobial susceptibility testing was carried out according to a unified protocol using KirbyBauer method or automated systems. Penicillin susceptibility of streptococcus pneumonia was detected by Etest. All of the antimicrobial susceptibility testing results were interpreted according to the criteria of Clinical and Laboratory Standards Institute(2020).
Main outcome measures: The distribution of bacteria strains and the changes of bacteria drug resistance in pediatrics, especially the multidrugresistant organisms(MDRO) among pediatric patients.
Results: A total of 42,786 isolates were collected, of which 38.5% was grampositive organisms and 61.5% was gramnegative organisms. Top five pathogens were Escherichia coli (16.4%), Straphylococcus aureus(12.6%), Streptococcus pneumonia (9.8%), Coagulase negative staphylococci (7.3%) and Klebsiella pneunoniae (7.0%). Top three pathogens in the neonatal group were Staphylococcus aureus (19.3%), Escherichia coli (18.6%) and Klebsiella pneumoniae (13.2%) and top three pathogens in the nonneonatal group were Escherichia coli (16.2%), Staphylococcus aureus (11.7%) and Streptococcus pneumoniae (11.2%). The proportion of respiratory specimens decreased from 53.9% in 2019 to 45.2% in 2020. Most MDROs presented highlevel drug resistance to various antibiotics. The prevalence of Carbapenen Resistant Enterobacteriaceae (CRE) , Pseudomonas aeruginosa (CRPA) , and Acinetobacter baumannii (CRAB) was 4.9%, 9.5% and 33.5% respectively. The prevalence of CRPA was higher in the neonatal group than that in the nonneonatal group (19.4% vs 9.2%) and CRAB was just the opposite (14.8% vs 37.1%). The detection rates of MethicillinResistant Staphylococcus aureus (MRSA) and Coagulase negative staphylococci (MRCNS) were 31.5% and 75.9% respectively.
Conclusion: Both the detection rates of MDROs and their antimicrobial resistance in children were decreased in 2020.
Background: Researches have demonstrated the correlation between geneotype and phenotype as well as decreased head circumference and intracranial volume in Rett Syndrome (RTT),but it is unclear how the phenotypes and neuroimaging parameters correlate.
Objective: To investigate the correlations between the clinical severity and developmental level, sleep status and gray and white matter parameters in typical RTT to guide thte diagnosis and treatment.
Design: Crosssectional correlation analysis.
Methods: Children with typical RTT underwent MECP2 gene testing were included. The clinical severity was evaluated based on the Rett Syndrome Severity Scale (RSSS). Griffiths Development ScalesChinese (GDSC) was used to assess the developmental status. Children Sleep Habit Questionnaire (CSHQ) was used to assess the sleep status. Magnetic resonance imaging (MRI) was applied to obtain the brain white and gray matter index. Multiple linear regression analysis was applied.
Main outcome measures: The correlations between RSSS, GDSC, CSHQ and neuroimaging parameters.
Results: A total of 22 children with typical RTT administered at Children's Hospital of Fudan University from July 2014 to June 2021 were recruited. Twentytwo children were all females and aged 3.1±0.8 years old. All had been detected with pathogenic variants in the MECP2 gene. RSSS score was 5.91±1.38, the GDSC total DQ was 17.89±7.93 and the CSHQ score was 54.05±5.52. Multiple linear regression analysis revealed that RSSS was significantly correlated with BDQ(r=-0.518， P=0.007)，DDQ(r=-0.4， P=0.032) and total DQ(r=-0.429， P=0.023) of GDSC as well as GMV(r=-0.571， P=0.003)， WMV(r=-0.514， P=0.007)， FA(r=-0.472， P=0.013)， MD(r=0. 519， P=0.007)， RD(r=0.528， P=0.006) and AD(r=0.491， P=0.01) for MRI parameters.
Conclusion: The RSSS score of typical RTT correlated with developmental level and gray and white matter parameters,and it has potential value in assessing the clinical severity in patients with RTT.
Background: Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is a rare but lifethreatening congenital heart disease which can lead to changes in left ventricular configuration and affect left ventricular function. Therefore, it's necessary to evaluate the left ventricular configuration of the patients with ALCAPA before and after the surgical operation.
Objective: To evaluate the changes of left ventricular configuration in patients with ALCAPA before and after the operation by echocardiographic spherical index (SI).
Design: Casecontrol study.
Methods: Consecutive simple infantile ALCAPA patients diagnosed by echocardiography were enrolled who underwent left coronary artery transplantation and received echocardiography 1 week before and after the operation. Children receiving echocardiography because of the physical examination were taken as the normal controls. The pre and postoperative ALCAPA patients and the normal control group were measured by SI and left ventricular ejection fraction (LVEF) derived by echocardiography to evaluate the left ventricular configuration and left ventricular systolic function.
Main outcome measures: Changes of left ventricular configuration before and after ALCAPA operation.
Results: From February 2006 to October 2021, a total of 15 patients with simple infantile ALCAPA aged 7.5±6.6 months were included. In the group of ALCAPA patients, the preoperative and postoperative left ventricular SId was 1.22±0.11 and 1.42±0.15, and the preoperative and postoperative left ventricular SIs was 1.35±0.17 and 1.54±0.17, respectively. In the control group with the average age of 7.4±6.6 months for 15 patients, the left ventricular SId and SIs was 1.49±0.15 and 1.55±0.15, respectively. In the group of ALCAPA patients, the preoperative and postoperative LVEF was (44.7±13.8)% and (63.9±13.8)%（P<0.01）. There was a correlation between left ventricular SId, SIs and LVEF before the operation (P＞0.01).
Conclusion: SI derived by echocardiography can be used to evaluate the changes of left ventricular configuration in patients with ALCAPA before and after the operation. In patients with ALCAPA, the left ventricular spheroidization was distinct before the operation, and the left ventricular remodeling and configuration were improved after operation.
