1.Clinical Characteristics and Prognostic Analysis of Extracranial Malignant Rhabdoid Tumor in Children
Shihan ZHANG ; Wen ZHAO ; Mei JIN ; Hongjun FAN ; Xisi WANG ; Libing FU ; Tong YU ; Yan SU
JOURNAL OF RARE DISEASES 2026;5(1):34-42
To investigate the clinical characteristics and prognosis of extracranial malignant rhabdoid tumor (eMRT) in children, and to provide a reference for the clinical treatment of this disease. A retrospective analysis was performed on the clinical data of children with newly diagnosed eMRT who were admitted and treated in the Department of Pediatric Oncology, Beijing Children's Hospital Affiliated to Capital Medical University, from March 2009 to December 2024. The clinical characteristics were summarized, and survival analysis and prognostic risk factor analysis were conducted. A total of 43 children with eMRT were included in this study, the median age at diagnosis of all patients was 20 months (range: 2-138 months). Among them, 24 cases were malignant renal rhabdoid tumors and 19 cases were extracranial, extrarenal rhabdoid tumors. Of the 43 children, 23 cases (53.5%) were complicated with distant metastasis. Twenty-nine (67.4%) underwent primary tumor resection. Among the children, 24 (55.8%) underwent gross total resection (GTR), 5 (11.6%) partial resection, and 14 (32.6%) biopsy only. Their 3-year overall survival (OS) rates were 40.8%, 35.3%, and 33.3%, respectively ( Children with eMRT have an overall poor prognosis. A diagnostic age < 12 months is an independent risk factor for higher mortality in these children. Further large-scale, long-term follow-up studies are needed to explore the prognostic factors of this disease.
2.Elucidating the renal injury effects of uranium exposure based on a human kidney organoid model
Ailin TAN ; Yu HUANG ; Jian CHU ; Shaoyu ZHANG ; Zhixia HAN ; Libing YU
Chinese Journal of Radiological Health 2025;34(6):871-879
Objective Uranium compounds are critical materials in the nuclear industry, and their mining, processing, and use pose occupational exposure risks. Epidemiological studies have shown that uranium exposure can impair health, with acute uranium poisoning primarily causing structural and functional damage to the kidneys. Currently, the molecular mechanisms underlying uranium-induced kidney injury remain unclear, and traditional biological models used in toxicological research are inadequate for simulating the human microenvironment. This study employed a human kidney organoid model system to elucidate the nephrotoxic mechanisms of uranyl ions, providing a scientific basis for the prevention and control of uranium poisoning. Methods Kidney organoids were constructed using the human embryonic stem cell line H1 and exposed to solutions with different uranyl ion concentrations. Morphological observation, ATP detection, reactive oxygen species detection, apoptosis assay, and untargeted metabolomics analysis were performed. Mechanisms of toxicity were further explored through KEGG pathway enrichment analysis. Results Uranium exposure led to structural damage in the organoids, accompanied by a dose-dependent decrease in ATP levels, accumulation of reactive oxygen species, and increased apoptosis rate. After 24-hour exposure to 300 μmol/L uranium, significantly disturbed differential metabolites and five core metabolic pathways were identified. Conclusion Uranium induces oxidative stress, mitochondrial dysfunction (ATP reduction), and metabolic disorder (disruption of phospholipid/amino acid metabolism), which synergistically cause DNA damage, apoptosis, and/or necrosis. These findings provide new insights into the mechanisms of uranium-induced kidney injury and support the development of prevention and control strategies.
3.Analysis of clinical features and prognosis in pediatric malignant solid tumors of head and neck in single-center
Peiyi YANG ; Chao DUAN ; Shengcai WANG ; Mei JIN ; Dawei ZHANG ; Libing FU ; Tong YU ; Zhikai LIU ; Xiaoli MA ; Xin NI ; Yan SU
Journal of Capital Medical University 2025;46(3):545-552
Objective To summarize the clinical features and prognosis of children suffered from malignant solid tumors of head and neck.Methods The clinical data of children with primary malignant solid tumors located in the head and neck was retrospectively analyzed from January 2007 to December 2021 in the Department of Oncology,Beijing Children's Hospital,Capital Medical University,and the clinical features,prognostic factors were summarized.Results A total of 234 children with malignant solid tumors of head and neck were included,with a male to female ratio of 1∶0.7,aged from 3 months to 17 years and 6 months(median age 51 months).173 cases(73.9%)were treated with local painless masses.Other symptoms included snoring and facial paralysis.The proportion of rhabdomyosarcoma(RMS)was the highest(145 cases,62.0%),followed by neuroblastoma(NB)(25 cases,10.7%),Ewing sarcoma(19 cases,8.1%),etc.A total of 47 cases(20.1%)had distant metastasis.The patients received surgery,chemotherapy and radiotherapy under the mode of multidisciplinary treatment(MDT).The 3-year and 5-year overall survival(OS)were 80.8%and 75.8%,respectively,and the 3-year and 5-year progression free survival(PFS)were 64.0%and 58.9%,respectively.Tumor survivors had abnormal appearance or facial motor function(49 cases,41.2%),developmental problems or abnormal tooth loss(18 cases,15.1%),and other long-term complications that may be related to the tumor or treatment.Conclusion There are various pathologic types of pediatric head and neck malignant solid tumors,RMS and NB are the most common.Local painless mass was the most common complaint.Distant metastasis is an independent risk factor for the prognosis of head and neck malignant solid tumors.Under the MDT model,the prognosis of malignant solid tumors of the head and neck in our center was generally good.In the treatment of the tumors,the side effects and sequelae should be controlled as small as possible under the premise of long-term survival.
4.Analysis of clinical features and prognosis in pediatric malignant solid tumors of head and neck in single-center
Peiyi YANG ; Chao DUAN ; Shengcai WANG ; Mei JIN ; Dawei ZHANG ; Libing FU ; Tong YU ; Zhikai LIU ; Xiaoli MA ; Xin NI ; Yan SU
Journal of Capital Medical University 2025;46(3):545-552
Objective To summarize the clinical features and prognosis of children suffered from malignant solid tumors of head and neck.Methods The clinical data of children with primary malignant solid tumors located in the head and neck was retrospectively analyzed from January 2007 to December 2021 in the Department of Oncology,Beijing Children's Hospital,Capital Medical University,and the clinical features,prognostic factors were summarized.Results A total of 234 children with malignant solid tumors of head and neck were included,with a male to female ratio of 1∶0.7,aged from 3 months to 17 years and 6 months(median age 51 months).173 cases(73.9%)were treated with local painless masses.Other symptoms included snoring and facial paralysis.The proportion of rhabdomyosarcoma(RMS)was the highest(145 cases,62.0%),followed by neuroblastoma(NB)(25 cases,10.7%),Ewing sarcoma(19 cases,8.1%),etc.A total of 47 cases(20.1%)had distant metastasis.The patients received surgery,chemotherapy and radiotherapy under the mode of multidisciplinary treatment(MDT).The 3-year and 5-year overall survival(OS)were 80.8%and 75.8%,respectively,and the 3-year and 5-year progression free survival(PFS)were 64.0%and 58.9%,respectively.Tumor survivors had abnormal appearance or facial motor function(49 cases,41.2%),developmental problems or abnormal tooth loss(18 cases,15.1%),and other long-term complications that may be related to the tumor or treatment.Conclusion There are various pathologic types of pediatric head and neck malignant solid tumors,RMS and NB are the most common.Local painless mass was the most common complaint.Distant metastasis is an independent risk factor for the prognosis of head and neck malignant solid tumors.Under the MDT model,the prognosis of malignant solid tumors of the head and neck in our center was generally good.In the treatment of the tumors,the side effects and sequelae should be controlled as small as possible under the premise of long-term survival.
5.Investigation on the use of ulinastatin in critically ill children
Zizhen ZHANG ; Qin YU ; Xingqiang DONG ; Libing ZHOU ; Saihu HUANG ; Shuiyan WU ; Zhenjiang BAI
Chinese Pediatric Emergency Medicine 2025;32(8):597-600
Objective:To investigate the current use of ulinastatin in the treatment of critically ill children by pediatricians in China.Methods:A anonymous questionnaire survey was conducted among 147 pediatric critical care physicians from 36 hospitals across 16 provinces,autonomous regions,and municipalities in China.The survey content consists of three parts: respondents' basic information, the application status of ulinastatin, and the clinical indicators referenced for evaluating the use of ulinastatin. Descriptive statistical analysis was performed on the collected data.Results:Among the 147 respondents,99.32%(146/147) were from tertiary hospitals;72.11%(106/147) worked in specialized ICUs,and 4.08%(6/147)in emergency medicine departments.A total of 68.03%(100/147) of the physicians reported using ulinastatin in clinical practice.The main diseases for which ulinastatin was used were pancreatitis(26.40%),sepsis and septic shock(23.76%),capillary leak syndrome(21.78%),acute respiratory distress syndrome(8.91%),and disseminated intravascular coagulation(6.27%).A total of 90.00% of physicians combined ulinastatin with other medications,including glucocorticoids(26.82%),albumin(23.51%),plasma(17.22%),and immunoglobulins(13.58%). Clinical indicators referenced during ulinastatin use included elevated interleukin(IL)-6(76.87%),tumor necrosis factor-α(44.22%),IL-8(31.97%),IL-1(19.73%),IL-18(10.20%),blood lactate(59.18%),decreased serum albumin levels(70.07%),increased pleural or peritoneal effusion(67.35%),skin and mucosal edema(65.31%),and elevated thrombomodulin among the four coagulation parameters(58.50%).Conclusion:Ulinastatin is mainly used for the treatment of critical illnesses such as pancreatitis and sepsis.Most physicians combine ulinastatin with other drugs,such as glucocorticoids and albumin.Clinical indicators commonly referenced when using ulinastatin include elevated IL-6,increased lactate,and increased pleural effusion,which suggest a high inflammatory state and endothelial damage.
6.Investigation on the use of ulinastatin in critically ill children
Zizhen ZHANG ; Qin YU ; Xingqiang DONG ; Libing ZHOU ; Saihu HUANG ; Shuiyan WU ; Zhenjiang BAI
Chinese Pediatric Emergency Medicine 2025;32(8):597-600
Objective:To investigate the current use of ulinastatin in the treatment of critically ill children by pediatricians in China.Methods:A anonymous questionnaire survey was conducted among 147 pediatric critical care physicians from 36 hospitals across 16 provinces,autonomous regions,and municipalities in China.The survey content consists of three parts: respondents' basic information, the application status of ulinastatin, and the clinical indicators referenced for evaluating the use of ulinastatin. Descriptive statistical analysis was performed on the collected data.Results:Among the 147 respondents,99.32%(146/147) were from tertiary hospitals;72.11%(106/147) worked in specialized ICUs,and 4.08%(6/147)in emergency medicine departments.A total of 68.03%(100/147) of the physicians reported using ulinastatin in clinical practice.The main diseases for which ulinastatin was used were pancreatitis(26.40%),sepsis and septic shock(23.76%),capillary leak syndrome(21.78%),acute respiratory distress syndrome(8.91%),and disseminated intravascular coagulation(6.27%).A total of 90.00% of physicians combined ulinastatin with other medications,including glucocorticoids(26.82%),albumin(23.51%),plasma(17.22%),and immunoglobulins(13.58%). Clinical indicators referenced during ulinastatin use included elevated interleukin(IL)-6(76.87%),tumor necrosis factor-α(44.22%),IL-8(31.97%),IL-1(19.73%),IL-18(10.20%),blood lactate(59.18%),decreased serum albumin levels(70.07%),increased pleural or peritoneal effusion(67.35%),skin and mucosal edema(65.31%),and elevated thrombomodulin among the four coagulation parameters(58.50%).Conclusion:Ulinastatin is mainly used for the treatment of critical illnesses such as pancreatitis and sepsis.Most physicians combine ulinastatin with other drugs,such as glucocorticoids and albumin.Clinical indicators commonly referenced when using ulinastatin include elevated IL-6,increased lactate,and increased pleural effusion,which suggest a high inflammatory state and endothelial damage.
7.Repair effect of resveratrol combined with Schwann cell-like cells on sciatic nerve injury in rats
Xing LIU ; Ling ZHENG ; Yu LIU ; Liming YI ; Libing RAO ; Xiaojun JU
Chinese Journal of Neuroanatomy 2024;40(4):485-491
Objective:To explore the repair effect of resveratrol combined with Schwann cell-like cells(SCLCs)dif-ferentiated from adipose-derived stem cells(ADSCs)on sciatic nerve injury in rats.Methods:ADSCs were primarily cultured and induced to differentiate into SCLCs.Cell morphology was observed by scanning electron microscopy.West-ern Blot method was used to detect the expressions of S100 calcium-binding protein β(S100β),p75 neurotrophin receptor(p75NTR),and glial fibrillary acidic protein(GFAP).Rats were randomly divided into Control group(Con-trol),Schwann cell-like cell group(SCLCs),resveratrol group(Res),and resveratrol+Schwann cell-like cell group(Res+SCLCs).Eight weeks after the successful establishment of the sciatic nerve injury model,the sciatic nerve func-tion index(SFI)of each group was detected by footprint experiment;the mechanical withdrawal threshold(MWT)was measured by von Frey filament stimulation needle;The wet weight ratio(WR)of the tibialis anterior muscle was deter-mined by weighing method;Western Blot and RT-qPCR methods were used to detect the expressions of neurotrophin-3(NT-3),nerve growth factor(NGF),insulin-like growth factor-1(IGF-1),and brain-derived neurotrophic factor(BDNF)at the injury site.Results:After 8 days of induction of ADSCs,the cells had elongated poles and increased extracellular components;S100β,p75NTR,and GFAP proteins were highly expressed.After treatment with SCLCs,Res,and Res+SCLCs,the SFI and WR of the treatment groups were significantly better than those of the Control group(P<0.05);the MWT of rats in the Res+SCLCs group and SCLCs group was reduced(P<0.05).Western Blot re-sults showed that the expressions of NT-3,IGF-1,NGF,and BDNF proteins in rats in the Res+SCLCs group were higher than those in other groups(P<0.05);The expressions of NT-3,NGF,and BDNF proteins in rats in the SCLCs group were higher than those in the Control group(P<0.05);the expressions of NT-3 and NGF proteins in rats in the Res group were higher than those in the Control group(P<0.05).RT-qPCR results showed that the expressions of NT-3,IGF-1,NGF,and BDNF mRNA in rats in the Res+SCLCs group were highly expressed;the expressions of NT-3,IGF-1,NGF,and BDNF mRNA in rats in the SCLCs group were higher than those in the Control group(P<0.05);The expressions of IGF-1 and NGF mRNA in rats in the Res group were higher than those in the Control group(P<0.05).Conclusion:Res combined with SCLCs differentiated from ADSCs has a good repair effect on sciatic nerve inju-ry in rats.
8.Expression and clinical significance of microRNA-451 in children with microcytic anemia and chronic infectious anemia
Jinmei XU ; Libing ZHANG ; Duonan YU
Journal of Clinical Medicine in Practice 2024;28(3):51-57
Objective To analyze the expression of microRNA-451 (miR-451) in children with microcytic anemia and chronic infectious anemia, and to explore the correlation between miR-451 and erythrocyte-related parameters and its diagnostic value for anemia in children. Methods The expression levels of miR-451 in serum were detected in children with microcytic anemia (
9.Effect of Huaier granule on apoptosis of sorafenib resistant hepatocellular carcinoma cells
Libing WANG ; Jingkun YU ; Fengzhi QU ; Daming CHENG ; Xiaogang LIU
Journal of Clinical Medicine in Practice 2024;28(5):44-52
Objective To investigate the inhibitory effect and molecular mechanism of Huaier granule on the growth of sorafenib resistant hepatocellular carcinoma (HCC) cells. Methods The gradient concentration of Huaier granule was used to treat HCC cells, and the effect of Huaier granule on the proliferation, migration and invasion of sorafenib resistant HCC cells was analyzed. Bioinformatics methods were used to analyze the possible interaction between microRNA-31-5p (miR-31-5p) and sprouty-related proteins with an EVH1 domain 1 (SPRED1). The expression levels of miR-31-5p and SPRED1 in HCC cells were detected by real time fluorescence quantitative polymerase chain reaction (qRT-PCR); cell viability, proliferation, migration, invasion and apoptosis were detected by CCK-8, colony formation, scratch healing, Transwell and flow cytometry; RNA immunoprecipitation (RIP) assay and dual luciferase assay were used to verify the binding relationship between miR-31-5p and SPRED1. Results Huaier granule could significantly inhibit the proliferation, migration and invasion of sorafenib resistant HCC cells, and induce apoptosis. Bioinformatics analysis showed that miR-31-5p was highly expressed in HCC, and Huaier granule was able to down-regulate the expression of miR-31-5p, inhibit the proliferation and metastasis of sorafenib resistant HCC cells, and induce apoptosis; miR-31-5p showed a targeted inhibition effect on the expression of SPRED1. SPRED1 was down-regulated in HCC, and overexpression of SPRED1 was able to reverse the promoting effect of overexpression of miR-31-5p on proliferation and metastasis of sorafenib resistant HCC. Conclusion Huaier granule can inhibit sorafenib resistant HCC metastasis through the miR-31-5p/SPRED1 axis, indicating that Huaier granule has the potential to be used as a novel drug for HCC treatment.
10.Clinical application of positioning grid combined with CTA assisted design of anterolateral thigh perforator flap in reconstruction of soft tissue defects around foot and ankle
Zhiwu CHEN ; Guanghao LIN ; Enxing YU ; Linhai CHEN ; Qinghua SONG ; Peng WEI ; Libing CAI
Chinese Journal of Microsurgery 2023;46(6):625-630
Objective:To explore the feasibility and clinical effectiveness of preoperative positioning grid combined with CTA assisted design of anterolateral thigh perforator flap (ALTPF) in reconstruction of soft tissue defects around foot and ankle.Methods:From May 2018 to December 2021, a total of 18 cases with soft tissue defects around foot and ankle were admitted to the Department of Plastic and Reconstructive Surgery, First Affiliated Hospital of Ningbo University. The patients were 11 males and 7 females, aged from 32 to 78 years old, with an average age of 57.5 years old. Among them, 10 had chronic ulcer wounds, 6 had traumatic wounds, and 2 had postoperative wounds after malignant tumor resection. The sizes of soft tissue defect ranged from 6.0 cm×8.0 cm to 9.0 cm×13.0 cm, and the flap sizes ranged from 8.0 cm×10.0 cm to 11.0 cm×15.0 cm. Preoperative positioning grid combined with CTA three-dimensional reconstruction were used to mark the exit point of the perforator vessels in digital format, in order to restore the course of vessels and calculate the length of the vascular pedicles. ALTPFs were accurately designed based on the digitally reconstructed images, and then the ALTPFs were used to repair the soft tissue defects around the ankle. The flap donor sites were directly closed in stage Ⅰ. After the operation, all the patients were included in scheduled follow-ups at the outpatient department to observe the appearance of the recipient flaps and donor sites. The functional evaluation of the affected feet were assessed according to the American Orthopaedic Foot and Ankle Society (AOFAS) ankle-hindfoot score.Results:All 18 flaps survived, and the wounds in both the recipient and donor sites healed in stageⅠ. Postoperative follow-up for the 18 patients were 5 to 36 months, with an average of 13 months. At the last follow-up, the appearances of the flaps were good without swelling, the donor sites had good recovery, and there was no obvious scar hypertrophy. The function evaluation of the affected feet were found at excellent in 10 cases, good in 6 cases, and fair in 2 cases.Conclusion:Preoperative position grid combined with CTA three-dimensional reconstruction can digitally mark the exit points and running courses of the perforator vessels. It is an effective method for accurate position of perforator vessels before surgery. It can effectively reduce the operation time, lower the surgical risks, and achieve a high survival rate of the flap, thus holding considerable clinical value.


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