[Objective]To analyze the biomechanics of different positions of femoral head-and-neck nail in fixation of proximal femoral fracture.[Method]Stable and unstable fracture models were made from 24 fresh femur specimens,and fixed with r-nail and DHS.Head-and-neck nails were placed in different positions to test their mechanical properties of anti-compression,anti-bending,anti-shearing and anti-torsion,and compared with each other.[Result]Mechanical properties of the femoral strength,stiffness,stability and loading capacity were tested and compared.To the stable fracture group,head-and-neck nail should be placed in middle-lower and posterior 1/3 of femoral neck,which leads to the best loading capacity.To the unstable fracture group,it should be placed between femoral head and neck,which lead to strong obliquity mechanics and valuable for bone union.[Conclusion]Confirm of the best position of femoral head-and-neck nail could improve the effect of fixation and enhance the surgical treatment of proximal femoral fracture.

Objective To evaluate the outcomes of children with stage Ⅳ malignant extracranial germ cell tumors. Methods Twenty-five patients were enrolled in the retrospective analysis. Event-free survival (EFS) and overall survival (OS) rates were estimated by Kaplan-Meier method with SPSS 13.0. Results Of the 25 children, there were 13 males and 12 females. The mean age at diagnosis was 2 years old (ranged 1 to 11). Five patients receiving chemotherapy in another hospital before (n=1), or giving up treatment after confirmed diagnosis (n=1), or giving up effective treatment after received less than 2 cycles (n=3) were excluded from this analysis. Of the 20 patients, 90.0% (18/20) achieved complete remission and 5.0% (1/20) achieved partial remission after treatment. The 5-year EFS rate and 5-year OS rate were 70.0%±10.2% and 82.4%±9.2% respectively. There was no death occurred due to complications. Conclusions The effect of this treatment program is positive. The cumulative dose of the drugs is not high, compared with other schemes such as PEB, but there are more drugs involved. Whether these drugs may cause long-term adverse reactions needs further research.

Objective To improve understanding of the clinical manifestations, diagnosis and treatment of childhood Kasabach-Merritt phenomenon (KMP). Methods The clinical data of 13 patients admitted for KMP to XXX from January 2010 to January 2016 was retrospectively analyzed, with a review of relevant literature. Results The patients were 10 males and 3 females. The age of presentation varied from newborn to 5 months. 12 patients had cutaneous manifestations, like petechiae, ecchymosis, jaundice, skin masses, etc, 1 patient had pleural effusion. The location of lesions varied. The laboratory hallmark consists of profound thrombocytopenia and hypofibrinogenemia with elevated D-dimers. The median time from initial presentation to diagnosis was 60 days. After approaches like surgery, corticosteroids, propranolol, interferon, sirolimus, etc, 10 patients got remission while 3 patients died. 6 patients treated with sirolimushad complete response. Conclusions KMP is characterized with vascular tumor, severe thrombocytopenia and consumptive coagulopathy. Clinically, KMP often presents with early-onset and delay in diagnosis. Surgery is an effective approach for KMP. Sirolimus appears to be a promising treatment for KMP.

Objective: To investigate the long-term efficacy and prognostic factors of pediatric relapsed Wilms tumor (WT) after retreatment. Method: Sixteen children in Shanghai Children′s Medical Center with relapsed Wilms tumor were enrolled consecutively in this study between April 2006 and June 2016. All patients were diagnosed according to pathology, imaging and medical and surgical oncologist′s assistance. Relapse treatment included surgical excision, chemotherapy and selective radiation therapy. The clinical features, long-term outcomes and prognostic factors of patients were analyzed retrospectively.Survival data were analyzed by Kaplan-Meier.Log-Rank analysis was used for univariate analysis. Result: One case was excluded because of giving up the therapy even though no disease progress was identified. A total of 15 cases (5 males and 10 females) were included in this study. The median age at diagnosis was 3.8 years (range 0.5-9.1 years). The tumor staging at diagnosis included one case of stageⅠ, 7 cases of stageⅡand 7 cases of stage Ⅲ. Among cases of stage Ⅲ, 6 cases had radiation therapy history. The pathology of all patients′ recurrent tumor was favorable histology (FH). The median follow-up time was 34.6 months (range 12.5-132.7 months) until March 21, 2017. The time from initial diagnosis to relapse was 7.9 months (range 3.1-17.9 months). Four cases experienced local recurrence, 9 cases relapsed with metastases (6 cases in lungs, 2 in livers, 1 in mediastinum) and 2 cases relapsed in both local site and with metastases. Except to 2 cases received irregular retreatment, 13 cases received regimen I (doxorubicin, vincristine, epoposide and cyclophosphamide for 25 weeks) as relapsed chemotherapy. Five cases received autologous bone marrow transplantation (ABMT). Until the last follow-up, 8 cases achieved continuous complete remission (range 6.7-104.3 months), 3 cases had relapse again or progressing and 4 cases died. The estimated 5-year overall survival (OS) rate and event free survival (EFS) rate were (70±15)% and (52±15)%. According to whether received ABMT or not, the 5-year EFS rate were 51% and 53%. According to whether relapsed within 6 months after diagnosis or not, the 5-year EFS rate were 38% and 56% respectively. Conclusion The 5-year EFS rate of pediatric relapsed FH WT have reached above 50% by multi-disciplinary treatment in our experience and we encourage patients and doctors to receive retreatment.

Objective: To assess the clinical features and long-term outcomes of neuroblastoma (NB) in children less than 18 months of age, so as to provide evidence for further improvement of treatment. Method: Clinical data(sex, age, stage, risk group, treatment response, follow-up, etc.) of 155 NB patients under age of 18 months from June 2000 to December 2015 in Shanghai Children′s Medical Center were analyzed retrospectively. The clinical features were summarized and the long-term follow-up results were evaluated. The overall survival (OS) and event-free survival (EFS) were analyzed by using Kaplan-Meier method. Factors including age, stage, risk group, bone marrow and bone metastasis, N-MYC status and dehydrogenase(LDH) level were analyzed by Log-Rank test. Result: Totally 155 eligible patients (96 males, 59 females) were included. The median age of disease onset was 7 months (11 days to 18 months). There were 31 cases of stage 1, 19 cases of stage 2, 45 cases of stage 3, 38 cases of stage 4 and 21 cases of stage 4S. The median follow-up time was 36 months (range 4 to 189 months), the 3-year and 5-year EFS rate were 89.6% and 85.2% respectively and the 3-year and 5-year OS rate were 96.2% and 94.1%, respectively. A total of 15 recurrent or progressed cases were observed. The median time to first recurrence was 11 months (range 3 to 39 months), 6 cases eventually died. Second malignancy occurred in one patient. The patients who had relapsed disease within 12 months from initial diagnosis have much lower 3-year OS rate than those in whom the disease recurred 12 months later (25.7% vs. 83.3%, P=0.020). Although the number of chemotherapy courses in median-high risk group reduced from 8.6 courses to 7.5 courses after the revision in 2008, the survival rate showed no significant difference between before and after (5-year EFS 74.4% vs. 84.3%, 5-year OS 89.0% vs. 92.9%, both P>0.05). In patients with stage 1 and stage 2, the 3-year EFS of 34 cases with surgery alone and 16 cases accepted chemotherapy were both 100%. Age at diagnosis, stage, risk group, MYCN status, LDH level, bone marrow involvement and bone infiltration had significant impacts on prognosis(all P<0.05). Conclusion Satisfactory outcomes could be achieved in neuroblastoma in children aged within 18 months; the prognosis was better in children at age less than 12 months compared with 12-18 months. MYCN amplification, LDH more than 5 times upper limit of normal range, bone marrow and bone infiltration were associated with worse prognosis.Excellent survival rates could be achieved in children with stage 1 and 2 disease within 18 month′s old accepted surgery alone, chemotherapy or radiotherapy could be avoided in these patients so as to reduce long-term adverse reactions.

Objective:To investigate the safety of Rituximab combined with intensive chemotherapy in the treatment of aggressive mature B-cell lymphoma/leukemia in children.Methods:The clinical data of 77 patients with primary pediatric aggressive mature B-cell lymphoma/leukemia who were treated according to the Chinese Children Cancer Group(CCCG)-mature B-cell lymphoma(BNHL)-2015 protocol at Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiaotong University School from November 1, 2014 to July 31, 2018 were collected.A comparison was drawn on the adverse reactions and recovery of immune function indexes between patients in the Rituximab combined with intensive chemotherapy group (R4 group) and the chemotherapy alone group (R3 group).Results:Rituximab combined with AA was associated with a significantly lower platelet count [79.5%(35/44 cases) vs.54.5%(24/44 cases), χ2=6.223, P=0.011] and a higher incidence of infection [70.5%(31/44 cases) vs.36.4%(16/44 cases), χ2=10.275, P=0.001] compared with AA alone; Rituximab combined BB was associated with a higher incidence of mucositis and infection compared with BB alone [40.8%(20/49 cases) vs.29.3%(22/75 cases) and 85.7%(42/49 cases) vs. 72.0%(54/75 cases), respectively], but the differences were not statistically significant.A greater proportion of patients in the R4 group had a decrease in peripheral blood CD 19 positive cells (no statistically significant difference, P>0.05) and a greater proportion had a decrease in serum IgG ( P<0.05) compared to the R3 group, but there was no significant difference in treatment-related mortality between both groups.For patients in the R4 group, the average recovery time of IgG and IgM level was 13.1 months, and the longest recovery time was 31 months after the end of treatment. Conclusions:Rituximab combined with intensive chemotherapy is generally safe in the treatment of aggressive mature B-cell lymphoma/leukemia in children.However, it is often accompanied with prolonged immunoglo-bulin deficiency and the potential risk of secondary infection.Therefore, the strict control over the indications for its application is required, and the gamma globulin replacement therapy deserves to be investigated in the future.

Objective:To evaluate the diagnostic value of multiparametric cardiac magnetic resonance(CMR)or detecting the occurrence of acute rejection(AR)after heart transplantation(HT).Methods:From 2019 to 2021, 44 HT recipients are prospectively recruited from Guangdong Provincial People's Hospital.Another 51 healthy volunteers are recruited from a local community as healthy controls.CMR studies are performed for obtaining baseline parameters.According to the clinicopathological diagnostic criteria of AR by the consensus of International Society for Heart and Lung Transplantation, 81 CMR studies of 44 HT recipients are further divided into two groups of AR (18 cases)and non-AR(71 cases). CMR parameters includ global ventricular structure/function, T2, T1, extracellular volume(ECV)and late gadolinium enhancement(LGE). A combined model is established by binary Logistic regression and receiver operator characteristic curve(ROC)constructed.Results:The age range is(41.8±16.8)years in 44 HT recipients and(41.8±9.7)years in 51 healthy controls.T1 mapping indicated that myocardial global ECV of left ventricle is significantly higher in AR patients than non-AR controls(32.4%±6.0% vs 28.5%±2.4%; P<0.001 9). Global native T1 is higher in AR group than that in non-AR group(49.8±3.1 vs 47.5±2.8 ms, P=0.009)and the difference is statistically significant.The cutoff value of global ECV is 30.62% with a sensitivity of 61% and a specificity of 86% for detecting AR.And T2 mapping reveale that T2 value of global left ventricle is significantly higher in AR group than that in non-AR group(49.8±3.1 vs 47.5±2.8 ms, P=0.009). LGE extent is significantly higher in AR group than those in non-AR group( P=0.004). Through including global native T1 and ECV into a logistic regression model, multiparametric CMR can yield an area under curve(AUC)of 0.794.It hints at the potential of CMR for detecting AR. Conclusions:Multiparametric cardiac magnetic resonance offers an excellent predictive capacity for a noninvasive detection of AR.

Objective To explore the effect of Yizheng Recipe on pyroptosis of alveolar epithelium in rats with recurrent respiratory tract infection(RRTI)based on HMGB1/TLR4/NF-κB signal pathway.Methods SD rats were firstly grouped into normal group and model group.In the model group,the RRTI rat model of spleen-Qi deficiency and lung- Qi deficiency was induced by fatigue combined with diet disorder and shavings and tobacco leaf smoking.Then,the rats in the model group were grouped into:RRTI model group(gavaged with normal saline 0.5 mL·100 g-1·d-1)and Yupingfeng Powder control treatment group(gavaged with Yupingfeng Powder suspension 0.5 mL·100 g-1·d-1),Yizheng Recipe high,medium and low-dose treatment groups(gavaged with Yizheng Recipe 0.75,0.5,0.25 mL·100 g-1·d-1),the normal group(gavaged with normal saline 0.5 mL·100 g-1·d-1).The general activities of the rats were observed and the pulmonary function of the rats was detected by the AniRes2005 animal pulmonary function analysis system;the expression of inflammatory factors in rat serum was detected by ELISA;HE,PAS and TUNEL staining were applied to detect the pathological changes of lung tissue;Western blot was applied to detect cell pyroptosis and the expression of HMGB1/TLR4/NF-κB signaling pathway-related proteins.Results Compared with the normal group,the lung function of the RRTI model group was weakened;the inflammatory response increased,and the lung tissue damage,edema and apoptosis were obviously increased;meantime,the pyroptosis was aggravated,and the HMGB1/TLR4/NF-κB pathway was activated(P<0.05).Compared with the RRTI model group,the Yupingfeng Powder control treatment group and the Yizheng Recipe high-dose,medium-dose and low-dose treatment groups all had different degrees of remission in lung function;inflammatory response reduced,the lung tissue damage,edema and apoptosis reduced;meantime,the pyroptosis and HMGB1/TLR4/NF-κB pathway were inhibited(P<0.05).Yizheng Recipe high-dose treatment group and Yupingfeng Powder control treatment group had similar curative effect on RRTI model rats(P>0.05);and Yizheng Recipe was dose-dependent in treating RRTI model rats(P<0.05).Conclusion Yizheng Recipe may inhibit the pyroptosis of alveolar epithelial cells in RRTI model rats by reducing the inflammatory response,and then protecting the lung tissue from damage,this process may be related to the HMGB1/TLR4/NF-κB pathway.

Objective:To analyze the clinical characteristics, therapeutic modalities and prognosis of desmoplastic small round cell tumor (DSRCT) in children, and to summarize the international research progress.Methods:A total of 8 children with DSRCT admitted to Shanghai Children′s Medical Center, Shanghai Jiaotong University, School of Medicine, from January 1999 to August 2019 were retrospectively studied.The clinical characteristics, consultation process and follow-up results were summarized, and the Kaplan-Meier survival analysis method was used to calculate the survival rate.Results:Among these 8 cases, there were 6 male children and 2 female children.Seven cases originated in the abdomen and pelvis, and 1 case originated in the sacral region.All cases had infiltrate surrounding tissues or viscera, and 4 cases(50%) had extra-peritoneal metastasis, including distant lymph node metastasis, liver, lung and bone metastasis.All patients received chemotherapy, among which 3 patients received radiotherapy, and 2 patients received autologous hematopoietic stem cell transplantation.The medical follow-up was continued to February 15, 2020, with the median follow-up period being 59 months.Three cases died and 5 cases survived (2 cases in complete remission, 1 case in recurrent relapse, 2 cases in partial remission still under treatment). The median relapse time was 14.5 months, the 3-year relapse-free survival rate was (30.0±17.5)%, and 3-year overall survival was (51.4±20.4)%.Conclusions:Half of DSRCT had distant metastasis; the prognosis was poor despite the aggressive multimodality therapeutic approaches, such as chemotherapy, cytoreductive surgery, and whole abdominopelvic radiotherapy and stem cell transplantation.