Background Systematic pain management of children is not enough in China, and there is no summary of pain in children with sarcoma. Methods Collect clinical data of newly diagnosed sarcoma patients admitted to the Medical Oncology Department of Beijing Children’s Hospital from October 2018 to December 2020. Pain assessment was completed by part-time palliative doctors. Children with pain received analgesic treatment and regular assessment. Results A total of 188 newly diagnosed sarcoma patients were included. 37 patients (19.7%) suffered from pain. 6 cases (16.2%) had mild pain, 17 (46.0%) moderate, and 14 (37.8%) severe. The daily life of 31 patients (83.8%) was affected by pain. 26 cases (70.3%) had bone invasion. The analgesic rate was 54.1% (20/37) before admission and 89.2% (33/37) after admission. 9 cases were treated with oral morphine regularly, all of whose pain relieved before chemotherapy, and the actual dose of morphine was 0.14±0.034mg/kgQ4H when the target was reached. No serious adverse reactions were observed. The total time of morphine application after chemotherapy was 5-9 days (median 6 days), and there was no withdrawal reaction during the process of reduction. Conclusion The pain in children with newly diagnosed sarcoma was mainly moderate to severe pain, and the incidence of pain in sarcoma originating from bone or invading bone was higher and the intensity was more severe. Patients who received standardized pain assessment and regular analgesic reached pain relief quickly, and no serious adverse reactions were observed within the recommended dosage.

Abstract：Structural variation (SV) is a fundamental genetic cause for cancer, with demonstrated correlation to disease progression and treatment response. Traditional sequencing method cannot provide full genomic landscape especially large-scale and complex structural variation. To overcome these limitations, we adopted a combined sequencing approaches, including optical mapping, single molecular sequencing and short reads shotgun sequencing, to evaluate the SV in thyroid cancer. Different numbers, length and types of structural variation, with genes affected by SV were scrutinized. Integrating these results could showed comprehensive scenario for thyroid cancer in a genomic view. We demonstrate that integrated approaches could provide a powerful tool for capturing a higher level of genomic SV, creating new interpretation of sequencing data of particular relevance to human cancer.

In this study, we reported a patient who presents with multicentric reticulohistiocytosis (MRH)-like clinical feature, but the histology is not compatible with it. Like our patient, a couple of patients described previously also had the dilemma that they could not be classified appropriately into any current histiocytosis entity. Considering the clinical and pathological features together, all these patients had a similar clinical profile: xanthomatous rash, erosive arthritis and histopathological findings showing xanthomized histiocytes. Therefore, we propose a new disease entity - xanthomatous erosive arthritis (XEA), to accommodate these patients that cannot be classified appropriately into the current system.

Background: Chronic Rhinosinusitis (CRS) is a common disease in children. In recent years, a new airway epithelial ionocyte has been discovered and is closely related to the expression of cystic fibrosis transmembrane conductance regulator (CFTR), which provides new ideas for the study of the mechanism of CRS. However, the carrying status of the CFTR mutant gene in the Chinese population is not clear. Objective: To study the frequency and mutations of CFTR and FOXI1 (ForkheadBoxI1) in Chinese children with CRS, and to analyze whether they are predisposed to CRS. Methods: A controlled case study was conducted from 2020 to 2021. The CFTR and FOXI1 genomes of 46 children with CRS and 23 children with no history of CRS from the Chinese mainland area were sequenced, and the relationship between mutation rate and the disease were analyzed. Results: 13 CFTR gene mutation sites’ carrying rate in the CRS group was higher than the control group, and 2 of them was significantly higher than that in the East Asian population database. The children who had a history of recurrent upper respiratory tract infection carried CFTR gene mutations associated with the pathogenesis. There was no difference in the carrying of FOXI1 mutation between the two groups. Conclusion: The incidence of CF mutation is higher in Chinese children with CRS who have no history of Cystic Fibrosis (CF). c.650A> G (p.E217G) and c.1950C> A (P. F 650 L) and may play a role in the development of CRS conditions in children in China.

Abstract Objective: To compare the comprehensive developments between children with large vestibular aqueduct syndrome (LVAS) and children without LVAS and investigate the risk factors for developments of LVAS children. Design: A retrospective propensity score matching analysis. Setting: Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing, China. Method: 70 children with LVAS (41 boys, median of 28.50 months, range 6-72 months, hearing threshold: 82.54±12.58 dB nHL) and 70 gender-, age-, and auditory- matched overall children were recruited as LVAS and non-LVAS group, respectively. The developments of children were assessed by Gesell development schedules. Then the overall development and sub-development results were compared between the two groups. Regression analyses were used to assess the association between potential risk factors and developments of LVAS subjects. Results: Compared with normal developmental metrics, LVAS children and non-LVAS children both had developmental delay (both P<.001), which occurred not only in verbal but also in non-verbal aspects (all P<.05) except gross motor in non-LVAS subjects. Whereas, the deaf children of two groups had the similar performance including all sub-developments (all P>.05). For the developments of LVAS children, the age of intervention was risk factor (B<0, P<.05) and the developments of children older than 12 months was worse than those of children younger than 12 months (P<.05). Conclusion: Children with LVAS had the equal developmental level in comparison with children with same hearing impairments. Age of diagnosis was the risk factor for developments of LVAS children and the obvious delay of developments stared from 12 months.