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OBJECTIVE: Various studies have shown that aberrant expression of circular RNAs (circRNAs) has a pivotal role in multifarious cancers. However, the role of circRNAs in hepatoblastoma (HB) is not clearly understood. In the present study, we attempted to explore the underlying mechanism of hsa_cric_0000594 in HB along with its clinical importance. PATIENTS AND METHODS: In our research, the expression pattern of hsa_circ_0000594 in HB tissues and matched normal liver tissues was determined by in situ hybridization and RT-qPCR. Proliferation, viability, migration, and apoptosis of HB cell lines were detected via Cell Counting Kit-8 (CCK-8), colony formation, transwell, and flow cytometry assays. The interaction of hsa_circ_0000594 with miR-217 was investigated by Dual-Luciferase reporter assay. RESULTS: Expression levels of hsa_circ_0000594 were significantly upregulated in HB tissues compared with those in paired normal liver tissues and showed a clear association with the subtype of HB. The knockdown of hsa_circ_0000594 inhibited the malignant phenotype of HB. Bioinformatics analysis suggests that sirtuin 1 (SIRT1) may serve as a target gene of miR-217. CONCLUSIONS: Mechanically, hsa_circ_0000594 was identified to have a critical role in HB development through the hsa_circ_0000594/mir-217/SIRT1 regulatory axis, which might become a novel diagnostic marker and potential therapeutic target in HB. [ABSTRACT FROM AUTHOR]

Ben Cao Tu Jing had 48 materia medica illustrations related to the regional names in Shandong province. It was found that 42 of them were plant medicinal materials, distributed in seven areas in Shandong province. This study examined these illustration of plant medicinal materials and found that 26 species of these illustration of plant medicinal materials were identified with three genera and 11 illustrations were unverified. Most materia medica illustrations relating to the regional names in Shandong province were found mainly in Yanzhou, Qizhou and Zizhou. This indicated that materia medica were widely used in these areas in the Northern Song Dynasty. The haustorium of Cuscuta Chinensis were depicted in the "Shanzhou Tu Si Zi" and the habitat of wild poriacocos was described. This showed that the illustrators of Ben Cao Tu Jing might have conducted fieldwork and reflected on the main principles about how to identify materia medica in Ben Cao Tu Jing Zou Chi . Inconsistencies were found between the illustrations and the expressions of plant medicinal materials in some illustrations, such as Gui Jiu, Ginsen and Shan Zhu Yu. This suggested that in the Northern Song Dynasty Stemmacantha Uniflora, Belamcanda chinensis might have been mixed up with Dysosma and Pinellia Pedatisecta might have been mixed up with Pinellia Ternata. This was in line with the compiling theories of Su Song that they could be recorded together when the illustrations and literature were inconsistent with each other.

Ben Cao Tu Jing had 38 materia medica illustrations related to the regional names in Zhejiang province. It was found that 30 of them were identified as being named after regional names in Zhejiang province, involving 20 families and 29 genera. One of the 38 materia medica illustrations was mineral medicine and seven of them were unverified. The twenty-five of these materia medica illustrations with regional names were found to be consistent with their names used today. Five of them came from similar names in Zhejiang province in the Northern Song Dynasty, another five of the 25 names were known with the names but not entity for the lack of full descriptions and drawings. It was found that the drawing and the text of "Ming Zhou Huang Yao" and "Qin Zhou Hong Yao" in Ben Cao Tu Jing were reversed. By comparing the materia medica illustrations related to regional names in Da Guan Ben Cao by Liu Jia and Zheng He Ben Cao by Zhang Huicun, the name and the illustration of "Jin Zhou Bei Mu" in Zheng He Ben Cao by Zhang Huicun was more believable than "Yue Zhou Bei Mu" in Da Guan Ben Cao by Liu Jia. "Tai Zhou Wu Yao" and "Zhe Ba Wei", as genuine medicinal materials in Zhejiang, can be traced back to the illustrations of "Tai Zhou Wu Yao", "Yue Zhou Bai Zhu", "Mu Zhou Mai Dong", "Wen Zhou Peng 'e Shu", "Yue Zhou Bei Mu" and "Tian Tai Wu Yao" in Ben Cao Tu Jing. These findings provided text and drawing literature of herbs in the Song Dynasty for the research of genuine medicinal materials and their characteristics in Zhejiang province. They also showed that the utilization and development of the resources of traditional Chinese medicine in Zhejiang in the Northern Song Dynasty mainly focused on the coastal areas.

A total of 568 paintings of herbaceous plants and woody plants as medicine were involved in Ben Cao Tu Jing , accounting for 60% of the total drug illustrations and 76% of the botanical medicine paintings. These medicinal paintings can be classified into four types: original plant paintings, medicinal material paintings, plant and medicinal material paintings, and plant and their habitat paintings. The original plant paintings can be specifically divided into five sections: the whole plant paintings with roots, partial aerial part paintings with roots, plant paintings with ground lines, rootless plant paintings, and broken branch paintings. Among them, the drawings of " the broken branch paintings " and " the whole plant paintings with roots " are consistent with the basic principles of collecting plant specimens. Additionally, "the plant and medicinal material paintings " and "the medicinal material paintings "reflect that they were drawn based on the market survey of medicinal materials or collected medicinal materials specimens. These medicine painting demonstrate rich characteristics of medicinal plants and medicinal materials and a high artistic quality with realistic details.These paintings in Ben Cao Tu Jing echoes Tu Jing , combine the art of painting with the cognition ofnatural science, as the rudiment of early paintings of plant science.

Objective: To investigate the clinical and pathological features of pediatric NTRK-rearranged tumors. Methods: Four NTRK-rearranged soft tissue tumors and one renal tumor at Shanghai Children's Medical Center, Shanghai Jiaotong University and Singapore KK Women's and Children's Hospital from January 2017 to September 2019 were identified. Pan-TRK immunohistochemistry, and the ALK and ETV6 gene break-apart fluorescence in situ hybridizations (FISH) were performed. NTRK gene rearrangement was detected using sequencing-based methods. Results: There were 3 males and 2 females in this study. The patients were between 3 months and 13 years of age. Histologically, the tumors were infiltrative spindle cell tumors with variable accompanying inflammatory cells. Immunohistochemistry showed positive reactivity for pan-TRK in all tumors, with nuclear staining for NTRK3 fusion, and cytoplasmic staining for NTRK1 fusion. The molecular testing revealed NTRK gene fusions (one each of TPM3-NTRK1, ETV6-NTRK3 and DCTN1-NTRK1, and two cases of LMNA-NTRK1). Two patients were receiving larotrectinib. The others were are well without disease, with follow-up durations of 9 to 29 months. Conclusions: NTRK-rearranged mesenchymal tumors from soft tissue sites and kidney are identified. A novel DCTN1-NTRK1 fusion is described. Pan-TRK immunohistochemistry is useful for diagnosis. NTRK-targeted therapy may be an option for unresectable, recurrent or metastatic cases.

Objective: To study the differences in the bone marrow histopathology between acquired aplastic anemia (AAA) in children and refractory cytopenia of childhood (RCC) to facilitate their diagnoses and differential diagnosis. Methods: The clinical data and bone marrow biopsies of the RCC and AAA cases diagnosed from January 2008 to December 2018 in Xinhua Hospital, Shanghai Jiaotong University School of Medicine and Shanghai Children's Medical Center affiliated to Shanghai Jiaotong University School of Medicine were analyzed. Results: A total of 71 AAA and 79 RCC cases were analyzed. There were 52 males and 19 females, age ranged 1.0-15.0 years (median, 8.9 years) in the AAA group, and 53 males and 26 females, age ranged 0.5-16.0 years (median, 5.0 years) in the RCC group. All the biopsy specimens of AAA patients had severe hypocellularity; the cellularity of 88.7% (63/71) specimens was under 5.0%, and 11.3%(8/71) was 5%-24%. None of the AAA specimens showed any dysplastic change. All the biopsy specimens of RCC patients had hypocellularity, including 94.9%(75/79) of the specimens with a cellularity of 5%-50%. All of the RCC specimens showed a patchy distribution of hematopoiesis. A dysplastic change of erythroid cells and micromegakaryocytes was found in 40.5% (32/79) and in 60.8% (48/79) of the RCC cases, respectively. Conclusions: The degree of hypocellularity, the distribution pattern of hematopoiesis, the cell composition and localization of erythroid cell clusters and the appearance of micromegaryocytes could help the diagnosis and differential diagnosis of AAA and RCC.

Objective: To describe our experiences in application of the 2019 revision of "CCCG-WT-2016" for the diagnosis of Wilms tumors. Methods: Ninety-one cases of Wilms tumor diagnosed at Shanghai Children's Medical Center from January 2015 to December 2018 were collected. All cases were reviewed by two senior pathologists, including one from China and the other from Singapore, according to the 2019 revision of "CCCG-WT-2016." Results: The specimens were obtained by core biopsy ( n= 21), primary nephrectomy ( n= 41), post-chemotherapy nephrectomy/resection ( n= 18), or biopsy/resection of metastatic/relapse/post-chemotherapy metastatic lesion(s) ( n= 11). The specimens of core biopsy and primary nephrectomy ( n= 62) all had favorable histology.Twelve post-chemotherapy nephrectomy cases were subdivided into three risk groups: low risk ( n= 0), intermediate risk ( n= 10) and high risk ( n= 2). Six post-chemotherapy resection cases were subdivided into 3 risk groups:low risk ( n= 0), intermediate risk ( n= 5) and high risk ( n= 1). The remaining 11 cases were comprised of metastatic, relapse, and post-chemotherapy metastatic lesions. The concordance rate of the two senior pathologists was 100%(91/91). Conclusions: The 2019 revision of "CCCG-WT-2016" is clearly written and easy to use. It can serve as the basis of accurate classification for clinical treatment.

Aims: To investigate the protocol efficacy and prognostic factors for paediatric hepatoblastoma in a multidisciplinary model in our centre., Materials and Methods: Consecutive hepatoblastoma patients (<18 years old) treated at Shanghai Children's Medical Center in China from August 2011 to October 2017 were analysed retrospectively for clinical features, chemotherapy courses, surgical treatment and outcomes., Results: One hundred and four cases of paediatric hepatoblastoma (64 males, 40 females; median age at diagnosis 1.64 years) had a median follow-up of 30.68 months (range 8.3-73.3 months). First complete remission was achieved in 95 cases, 85 of which achieved continuous complete remission. Another three cases were lost to follow-up after a median of 24.73 months in complete remission. Seven cases relapsed later, with two achieving a second complete remission and four deaths. Nine cases did not achieve complete remission and five of them died. In general, the 5-year overall survival rate and 5-year event-free survival (EFS) rate were 86.3 ± 5.0% and 81.8 ± 4.3%, respectively. Thirty-two cases were classified as standard risk and 72 as high risk with 5-year EFS of 96.8 ± 3.2% and 75.7 ± 5.7% (P = 0.029) and 5-year overall survival of 100% and 80.5 ± 7.0%, respectively. The mean platelet count (P = 0.0036), lactate dehydrogenase (P = 0.0443) and ferritin level (P = 0.0006) at diagnosis were much higher in the high-risk group than in the standard-risk group. Univariate analysis showed that patients <5 years of age (P = 0.018), with higher α-fetoprotein (AFP) level (>100 ng/ml, P = 0.008), without metastases at diagnosis (P = 0.001) and postoperative AFP recovery after no more than three chemocycles (P = 0.014) had better overall survival. In addition, the above factors, except metastases at diagnosis and risk group, were associated with prognosis in the multivariate analysis., Conclusions: The result of this protocol had similar overall survival and EFS rates compared with those in developed countries. Normal postoperative AFP levels after three chemocycles has prognostic value., (Copyright © 2019 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Being a common materia medica, the confusion of Jixueteng caused by the homonyms of different substances for Jixueteng is always very complicated. Fengqing Jixueteng Paste was first created in the Qing Dynasty. Fengqing Jixueteng was derived from the dried stems of a variety of species in Kadsura Juss. and Schisandra Michx. from Schisandracea; while Kunming Jixueteng, derived from the dried stem of Millettia dielsiana Harms ex Diels, was firstly recorded in the Zhi wu ming shi tu kao ( Textual Research on Reality and Titles of Plants ). The earliest records of Spatholobus suberectus Dunn as the original plant of Jixueteng was in Guang xi ben cao xuan bian ( Selected Compilation of Chinese Materia Medica in Guangxi ). It was not until 1997 that Spatholobus suberectus Dunn was recorded as an origin of orthodox product of Jixueteng in the Chinese Pharmacopoeia . At present, the origins of Jixueteng is very complicated as recorded in different provincial standards, including three species of three genus from Leguminosae.The varieties of species from Kadsura Juss. and Schisandra Michx. had long been used as Fengqing Jixueteng, but currently the most extensively used one is Spatholobus suberectus Dunn as Jixueteng . There are more differences in records of Jixueteng in history up to the present, hence, further investigation and research on Jixueteng is still necessary.