Objective To analyze the clinical characteristics and prognosis of 4 rare types of non -Hodgkin lymphoma(NHL)in children,and to discuss the progress in treatment.Methods Clinical data of 1 4 patients with rare types of NHL at Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine between January 2004 and December 201 4 were retrospectively analyzed,and their clinical features,treatment and prognosis were dis-cussed.Results Fourteen cases were reported including 6 subcutaneous panniculitis -like T -cell lymphoma (SPTCL),3 hydroa vacciniforme -like cutaneous lymphoma(HVLL),2 pediatric follicular lymphoma(PFL)and 3 ex-tranodal NK/T -cell lymphoma,and nasal type(ENKTL).Ten patients (71 .4%)primarily presented with skin lesions and underwent a long course of illness before they were finally diagnosed (the median was 1 0 months),71 .4%(1 0 /1 4 cases)of them associated with fever and 50.0%(7 /1 4 cases)with liver and spleen enlargement,and no evidence of central nervous system (CNS)and bone marrow (BM)involvement was observed,while 28.6% patients (4 /1 4 cases) had more than two lines′abnormalities in peripheral blood examination.Since there were no standard treatment guide-lines,most patients received CHOP (Cyclophosphamide +Adriamycin +Vincristine +Prednisone)and /or mature B -cell NHL -like therapy,and 50.0%(7 /1 4 cases)of them received interferon therapy in addition,while 1 patient re-ceived allogeneic hematopoietic stem cell transplantation after recurrence.The complete remission was achieved in 71 .4%(10 /14 cases)of all the patients.Two of them died,3 lost follow -up,and 1 relapsed.The 3 -year overall survi-val and event free survival rates were 0.84 and 0.57,respectively,after a median follow -up of 26 months (range 12 -64 months).Conclusions Pediatric rare types of NHL show atypical clinical manifestation,low incidence of CNS /BMinfil-tration and long course.It is hard to make pathological diagnosis and differentiation.It is also inappropriate to apply the commonly used staging system to these rare types of NHL.No standard treatment has been found by now.SPTCL,HVLL and PFL have relatively good outcomes when treated with mature B -cell NHL -type therapy plus interferon therapy.

Objective: To explore the statistical differences of the vocational exhaustion positive rate among various occupational stress factors, occupational stress reaction, personality characteristics and relieving factors, to lay a foundation for reducing occupational stress of the medical staffs. Methods: 656 medical staffs in Yangzhong and Chifeng hospitals were included to conduct the evaluation of the occupational stress and job burnout, analyze the differences among the various factors by comparing them. Results: Various levels of vocational exhaustion positive rate among different occupational stress factors, occupational stress reaction, personality characteristics and relieving factors show statistical difference (P<0.05) . Among occupational stress factors, the vocational exhaustion positive rate of high level groups of conflict in groups was the highest (73.4%) , among occupational stress reaction, the vocational exhaustion positive rate of high level groups of body complain was the highest (80.4%) , among personality characteristics, the vocational exhaustion positive rate of low level groups of self-esteem was the highest (74.4%) , among relieving factors, the vocational exhaustion positive rate of high level groups of family support was the highest (76.4%) . Conclusion The vocational exhaustion rate of medical staffs show very high in the profession, comprehensive factors should be considered to create harmonious working atmosphere and to reduce vocational exhaustion positive rate of the medical staffs, to reduce the positive rate of vocational exhanstion in medical staffs.

Objective: To explore the relationship between occupational stress factors and psychological stress reaction among petrochemical workers, detecting the effects caused by the occupational stress and putting forward solutions to reduce the occupational stress degree. Methods: 392 petrochemical workers in Oil refining enterprise were included to conduct the evaluation of the occupational stress, correlation analysis and ridge regression were used to analyze the relationship between occupational stress and five aspects of psychological stress reaction and to detect the influencing factors. Results: Mission control, decision control, environmental control, resources control, promotion, and participation in decision making were positively correlated with job satisfaction (P<0.01) , technology utilization degree, work order tonality, job prospects were negatively correlated with job satisfaction (P<0.05) ; mission control, resource control, promotion, participation in decision-making were positively correlated with mental health (P<0.01) , work order tonality, job prospects were negatively correlated with mental health (P<0.05) ; load change risk, work, work order tonality, job prospects were positively correlated with depression (P<0.05) resource control, promotion opportunities were negatively correlated with depression (P<0.05) ; quantitative risk load, work, work order tonality, job prospects were positively correlated with anxiety (P<0.05) , the task control, promotion, and participation in decision-making were negatively correlated with anxiety (P<0.05) ; work, load change, work order tonality, risk scoring for job prospects were positively correlated with body complaint (P<0.01) , mission control, decision control, resource control were negatively correlated with body complaint (P<0.05) . Conclusion Different stress factors make different effects in different psychological stress reaction, the main predictors and predictive power of each psychological stress reaction were not the same.

Objective: To explore the differences of occupational stress and its factors between petroleum refining workers and support staffs, and to propose more targeted measures to promote the health of petroleum refining and petrochemical workers. Methods: A questionnaire survey was conducted among 353 petroleum refining workers and 189 employees in the logistics department using the occupational stress inventory revised edition (OSI-R) scale between June 13, 2017 and July 27, 2017. Results: Among the occupational factors, there were significant differences in the job requirements, work risk, job monotony, participation in the decision-making scores among the male workers between labor group and logistics group (P<0.01) . And for female workers in the chemical group and logistics group there were also differences in the value of work control, job requirements, work monotonous, work prospects, improvement of opportunity (P<0.05) . In the aspect of personality traits, the score of work psychological control in chemical group was higher than that of the logistics group, while the organization loyalty score was lower than that of the logistics group. Be-sides, there were significant differences between chemical group and logistics group in the score of A behavior, self-esteem and anxiety traits among female group (P<0.05) . In terms of mitigating factors, there was a significant difference in social support score between chemical group and logistics group among female workers (P<0.01) . Comparison of the two groups of psychological stress showed that the mental health and anxiety status of the chemical group and the logistics group showed a difference (P<0.05) , and female workers in the chemical group job satisfaction score lower than the logistics group, but the body Complaints score higher than the logistics group. Conclusion The psychological stress response of petroleum refining workers is obviously more serious than the staff of logistics department, and there were significant differences between the two groups on occupational stress factors.

Pulmonary inflammatory myofibroblastic tumor (PIMT) is a rare lung tumor that is poorly understood by clinicians. Based on the data of patients with PIMT in our center, a comprehensive search and a brief summary analysis of previous reports of the disease were carried out in this article. PIMTs were more likely to be present in male patients and in the right lung, and their clinical and radiographic findings were heterogeneous. Surgery is the most important treatment method, and complete resection is of great significance to the prognosis of patients. Targeted therapy represented by crizotinib may be helpful for patients with positive ALK mutations.

Objective:To study the clinicopathological features, immunophenotype, molecular genetic characteristics and prognosis of renal cell carcinoma associated with TFEB gene rearrangement (TFEBr-RCC).Methods:Eight cases of TFEBr-RCC diagnosed at the West China Hospital of Sichuan University from 2014 to 2022 were collected for clinicopathological, immunohistochemical, fluorescence in situ hybridization and RNA sequencing analyses, with review of literature.Results:Six patients were male and two were female. The patient ages ranged from 25 to 50 years (mean: 34 years, median: 32 years). The tumors were present in the right kidney (3 cases) or the left kidney (5 cases). The maximum diameters of the tumors ranged from 4.0 cm to 18.5 cm, with an average of 8.5 cm. Histologically, majority of the cases (5/8) showed typical biphasic "pseudorosette" structure, while the remaining three cases demonstrated atypical morphology that was similar to epithelioid angiomyolipoma or clear cell renal cell carcinoma. Immunohistochemical study showed positivity of TFEB (8/8), PAX8 (8/8), MART-1 (7/7), and HMB45 (5/6). Interestingly, PD-L1 was variably expressed in all five tested cases. Staining for TFE3 in all cases was negative. TFEB translocation was verified in all 8 cases using TFEB fluorescence in situ hybridization. RNA sequencing showed MALAT1-TFEB gene fusion in 4 of the 5 tested cases (two of which showing novel MALAT1-TFEB fusion sites), and one case with a novel ACTB-TFEB gene fusion. Patient follow-ups ranged from 5 to 96 months (average 47 months). All patients were alive without recurrence or metastasis.Conclusions:TFEBr-RCC tends to occur in young adults and has a good prognosis. Histologically, most of the cases show characteristic biphasic structure, and some cases show epithelioid angiomyolipoma-like or clear cell RCC-like morphology. Immunohistochemical reactivity to TFEB, melanocytic markers and PD-L1 is characteristic. MALAT1-TFEB gene fusion is the most common molecular change, with variable fusion sites.

Objective To analyze the clinical characteristics and prognosis of rare soft tissue sarcomas.Method Clinical data of 51 patients with rare soft tissue sarcomas including fibrosarcoma,synovial sarcoma,extrarenal rhabdoid tumor,alveolar soft part sarcoma,desmoplastic small round cell tumor and undifferentiated sarcoma in children and adolescents,diagnosed at Shanghai Children's Medical Center from June 1998 to December 2013,were retrospectively analyzed.All types were treated with the same strategy and chemotherapy regimens.Their clinical features,treatment and prognosis were discussed.Result Seventeen patients with fibrosarcoma,10 with synovial sarcoma,9 with extrarenal rhabdoid tumor,6 with alveolar soft part sarcoma,3 with desmoplastic small round cell tumor and 6 with undifferentiated sarcoma were included.The mean age at initial diagnosis was 5 years(range from 1 month to 13.5 years).The most common primary site of tumors was limbs,followed by the thoracic and abdominal cavity,accounting for 41％ and 24％ respectively.Twelve cases presented distant tissue or organ involvement in which bone metastases occupied the first place.Seven cases(accounting for 14％)were at stage Ⅰ,13 cases were at stage Ⅱ (accounting for 25％),19 cases were at stage Ⅲ (accounting for 37％) and 12 cases were at stage Ⅳ (accounting for 24％).The median follow-up period was 36 months(range from 1 month to 123 months).Forty-four patients achieved complete remission and 3 patients achieved partial remission after initial treatment,the overall response rate was 92％.Subsequent follow-up showed 29 patients remained relapse-free while 13 patients had relapsed disease.Overall survival and event-free survival at 2 years were 88％ and 57％.Postoperative surgical staging was the main prognostic factors.Patients with stage Ⅲ + Ⅳ had poorer results than those with Ⅰ + Ⅱ (x2 =4.909,P =0.027).Conclusion These 6 types of soft tissue sarcomas are rare in children and adolescents.The tumor can occur anywhere in the body but commonly presents in the extremities.Complete resection of tumor remains the most important modality of treatment and is directly related to prognosis.Neoadjuvant chemotherapy helps improve the resection rate of some unresectable tumors at diagnosis.Radiation therapy is primarily adopted for focal tumor control.

In order to improve the salt tolerance of banana NHX genes, we cloned a MaNHX5 gene from Musa acuminata L. AAA group and predicted the key salt-tolerant amino acid sites and mutant protein structure changes of MaNHX5 by using bioinformatics tools. The 276-position serine (S) of MaNHX5 protein was successfully mutated to aspartic acid (D) by site-directed mutagenesis, and the AXT3 salt-sensitive mutant yeast was used for a functional complementation test. The results showed that after the mutated MaNHX5 gene was transferred to AXT3 salt-sensitive mutant yeast, the salt tolerance of the mutant yeast was significantly improved under 200 mmol/L NaCl treatment. It is hypothesized that Ser276 of MaNHX5 protein plays an important role in the transport of Na⁺ across the tonoplast.
Amino Acids/metabolism* ; Gene Expression Regulation, Plant ; Musa/metabolism* ; Plant Proteins/metabolism* ; Plants, Genetically Modified ; Saccharomyces cerevisiae/metabolism*

ObjectiveTo explore the underlying mechanism of Bushen Huatan prescription in alleviating postmenopausal osteoporosis （PMOP） by maintaining the balance of osteogenesis and adipogenic differentiation in ovariectomized rats with osteoporosis. MethodSeventy-five 6-month-old non-pregnant female SD rats were randomly divided into sham-operation group， model group， atorvastatin group， liviol group， and Bushen Huatan prescription group. Bilateral ovaries were removed in the four groups except the sham-operation group， while only the same mass of adipose tissue around the ovaries was removed in the sham-operation group. On the 5^th week after surgery， drugs were consecutively administrated for 8 weeks. Rats in the Bushen Huatan prescription group received 9.4 mg·kg^-1 of the prescription， rats in the atorvastatin group received 0.92 mg·kg^-1 of atorvastatin， rats in the Liviol group received 0.23 mg·kg^-1 of liviol， and rats in the model group and the sham-operation group received saline once a day. Micro-computed tomography （Micro CT） was used to detect bone mineral density （BMD） of rat tibia in each group. Hematoxylin-eosin （HE） staining was used to detect the relative area of rat bone marrow adipose tissue （BMAT） in each group. Real-time fluorescence quantitative polymerase chain reaction （Real-time PCR） and Western blot were used to detect the relative expression levels of Runt-related transcription factor 2 （Runx2）， peroxisome proliferator-activated receptor （PPARγ）， leptin （LPN）， and leptin receptor （OBR） in bone tissues. ResultAs compared with the sham operation group， the BMD of rats in the model group decreased （P<0.05）， while the relative area of BMAT increased （P<0.05）. In addition， the expression levels of LPN， OBR， and Runx2 decreased in the model group （P<0.05）， while the level of PPARγ increased （P<0.05）. As compared with the model group， the BMD of rats in the atorvastatin group， the Livial group， and the Bushen Huatan prescription group increased （P<0.05）， and the relative area of BMAT decreased （P<0.05）. The expression levels of LPN， OBR， and Runx2 in these groups increased （P<0.05）， while the expression level of PPARγ decreased （P<0.05）. ConclusionBushen Huatan prescription plays the anti-osteoporosis role in the rat model of PMOP through up-regulating LPN and OBR in bone tissues and maintaining the balance of osteogenesis and adipogenic differentiation， thereby reducing postmenopausal bone loss and playing a role in the prevention and treatment of PMOP.

Thymic neuroendocrine tumors (TNETs) are a series of rare diseases with aggressive biology and poor prognosis. Clinical manifestations of TNETs are atypical, and ectopic secretion of adrenocorticotropic hormone can be found in some cases, resulting in associated endocrine symptoms. Due to the low morbidity and strong heterogeneity, it’s difficult to diagnose, treat and obtain new treatment regimen. Early complete surgical resection is an effective treatment. For advanced cancer, clinical trials of new drugs are expected to improve the survival of patients.