Microglia involved in restricted and repetitive behaviors / 中华行为医学与脑科学杂志

Restricted and repetitive behaviors is the characteristic clinical manifestation of many neuropsychiatric diseases, which seriously affects the work, study and daily communication of patients, and brings huge mental and economic burden to the family and society.Its causes are complex and its manifestations are diverse.Current studies have shown that microglia is closely related to the occurrence of restricted and repetitive behaviors, and the in-depth study of microglia has become a research hotspot to explore the mechanism of restricted and repetitive behaviors.In recent years, studies have found that restricted and repetitive behaviors of various neuropsychiatric diseases (such as frontotemporal dementia, obsessive-compulsive disorder, autism spectrum disorder) are related to microglia.However, reliable evidence for the exact mechanism of microglia involved in restricted and repetitive behaviors remains lacking.This article reviews the recent research progress of microglia involved in restricted and repetitive behaviors.It is expected to provide a new theoretical basis and therapeutic targeting cell for the development and treatment of neuropsychiatric diseases related to restricted and repetitive behaviors in the future.

Clinical characteristics and prognosis analysis of 217 infant patients with retinoblastoma / 中华实用儿科临床杂志

Objective:To investigate the clinical characteristics and prognosis of infants with retinoblastoma (RB).Methods:The clinical data of 217 (335 affected eyes) infantile RB patients admitted to the Department of Pediatrics, Beijing Tongren Hospital, Capital Medical University from July 1, 2009 to June 30, 2019 were collected for analyzing the clinical efficacy and prognosis after comprehensive treatment.Results:(1) Clinical characteristics: 217 infantile RB patients included 129 males and 88 females, with the ratio of male to female being 1.47∶1.00; the median age was 6.06 months; there was monocular disease in 99 cases, and binocular disease in 118 cases, with the incidence ratio of binocular to monocular disease being 1.19∶1.00; all the 5 cases with family history of RB had binocular disease; the first symptoms included white pupils and yellow-white reflections in pupils (183 cases, 84.3%), followed by strabismus (18 cases, 8.3%). There were 335 affected eyes, of which 304 counts were in the intraocular stage (90.7%), most commonly in stage D (146 counts, 43.6%); 26 counts (7.7%) in the extraocular stage, mainly invading the optic nerve and/or optic nerve stump; 5 counts (1.5%) in the metastasis stage.(2) Survival analysis: the medical follow-up continued to March 31, 2020, with a median follow-up time of 67 months.There were 2 cases with a loss of follow-up, 21 relapsed cases, and 19 death cases, with the overall survival rate being 91.2%.According to Kaplan-Meier survival analysis, the expected 5-year survival rate was 91.1%; the survival rate was 96.2% in the intraocular stage and 73.1% in the extraocular stage.All 5 cases died during the distant metastasis stage, and the difference was statistically significant ( χ2=7.492, P<0.001); there was also a statistical difference in the survival rate between the monocular disease (95.9%) and binocular disease (87.3%) ( χ2=4.335, P=0.023). (3) Eyeball removal and eye protection: the eye protection rate of 217 children was 68.9%, 100.0% in stage A, B and C, 80.1% in stage D and 35.1% in stage E, which showed significant differences ( χ2=6.573, P=0.004). There were 35 children who underwent eye extraction before chemotherapy and 67 cases after chemotherapy, among which the difference was statistically significant ( χ2=6.076, P=0.012) in eye removal rates before and after chemotherapy in stage D and E(6 and 11 cases before chemotherapy, 22 and 36 cases after chemotherapy). (4) Adverse reactions: according to the World Health Organization′s classification of adverse reactions to chemotherapy, there were 26 cases in grade 0 (12.0%), 98 cases in grade Ⅰ (45.1%), 59 cases in grade Ⅱ (27.2%), 23 cases in grade Ⅲ (10.6%), and 11 cases in grade Ⅳ (5.1%), mainly manifested as bone marrow suppression after chemotherapy (132 cases). No second tumors appeared, and only 4 cases had transient hearing abnormalities, which returned to the normal state in the subsequent review. Conclusions:Infantile RB has its own characteristics.Such factors as different eye types and different clinical stages can affect the prognosis of children.The survival rate of such children can be improved significantly and the rate of eyeball removal can be reduced after treatment.

Management of patients with retinoblastoma treated with vitrectomy due to misdiagnosis / 中华眼底病杂志

Objective:To explore the clinical treatment of retinoblastoma (RB) after being treated with vitrectomy (PPV) due to misdiagnosis.Methods:A retrospective case study. From July 2015 to July 2018, 5 cases and 5 eyes of RB children diagnosed by pathological examination at the Eye Center of Beijing Tongren Hospital were included in the study. Among them, there were 3 males with 3 eyes and 2 females with 2 eyes; all of them had monocular disease. The average age was 4.8±1.7 years old. At the first visit, the diagnosis was endophthalmitis in 2 eyes (40%, 2/5); vitreous hemorrhage in 3 eyes (60%, 3/5). All were treated with PPV. All children underwent slit lamp microscopy, orbital MRI and CT, and eye color Doppler ultrasound blood flow imaging. If there was no clear extraocular spread, the eyeball removal combined with artificial orbital implantation was performed; if there was clear extraocular spread, the modified orbital content enucleation operation was performed with part of the eyelid preserved. The average follow-up time after surgery was 34.6±7.9 months.Results:Among the 5 eyes, 2 eyes (40%, 2/5) underwent eyeball enucleation combined with stage I artificial orbital implantation, and 3 eyes (60%, 3/5) with modified orbital content enucleation. There were 2 eyes of endogenous type (40%, 2/5), 1 eye of diffuse infiltration type (20%, 1/5), and 2 eyes of mixed type (40%, 2/5). The orbit spread in 3 eyes, the tumor invaded the optic nerve in 1 eye, and regional lymph node metastasis in 2 eyes. All children received systemic chemical therapy (chemotherapy). During the follow-up period, there were no new metastatic diseases and no deaths.Conclusions:After RB misdiagnosis and PPV, surgical treatment should be performed as soon as possible. If there is no clear extraocular spread, eyeball removal or combined stage I orbital implantation should be performed. If there is clear extraocular spread, the orbital contents should be enucleated; Chemotherapy should be combined after surgery.

Etiology and drug sensitivity analysis of catheter-related bloodstream infection in children with solid tumors / 中华实用儿科临床杂志

Objective:To investigate the pathogen types and drug resistance of catheter-related bloodstream infection (CRBSI) in children with solid tumor after chemotherapy.Methods:The clinical characteristics, pathogenic bacteria composition and drug sensitivity test results of children hospitalized with chemotherapy and indwelling periphe-ral venous catheter (PICC) and central venous catheter (CVC) in the non-Intensive Care Unit (ICU) of the Pediatric Ward of Beijing Tongren Hospital, Capital Medical University from January 2014 to December 2015 were retrospectively summarized.Results:A total of 3 361 cases received chemotherapy, 3 300 cases received PICC and CVC, and the blood cultures of 64 cases were sent for test. Twenty-four cases had CRBSI, 4 of who were of fungal infection. The infection rate of CRBSI was 0.7% and the infection rate of fungi was 0.12%. A total of 14 pathogenic bacteria were isolated, including 3 strains of Gram-negative bacteria (21.4%), 9 strains of Gram-positive bacteria (64.3%), and 2 strains of fungi (14.3%). The main pathogenic bacteria detected positive in 24 cases (12 cases were drug-resistant) included the methicillin-resistant coagulase-negative staphylococci (3 cases), carbapenem-resistant Klebsiella pneumoniae (7 cases) and extended spectrum beta-lactamases(ESBLs)producing Escherichia coli (2 cases), and their detection rates were 12.5%, 29.2% and 8.3%, respectively.The sensitivity rate of coagulase-negative staphylococci to Vancomycin, Meropenem and Linezolid was 100%.Candida glabrata and candida 100% sensitive to Voriconazole, Amphotericin B and Flucytosine but not sensitive to Fluconazole and Itraconazole. Conclusions:Monitoring the occurrence and etiological changes of CRBSI in children with solid tumors is helpful to further strengthen effective prevention and control measures and provide early empirical antimicrobial therapy.

Diagnosis, treatment and prognosis of 10 children with advanced clear cell sarcoma of kidney / 中华实用儿科临床杂志

Objective:To investigate the clinical features, diagnosis, treatment and prognosis of advanced clear cell sarcoma of kidney(CCSK) in children.Methods:The clinical data of 10 children with advanced CCSK hospitalized in Department of Pediatrics of Beijing Tongren Hospital, Capital Medical University from January 2014 to December 2017 were collected, and their clinical features, diagnosis, treatment and prognosis were analyzed retrospectively.Results:(1) Clinical features of CCSK: ten cases of CCSK included 6 boys and 4 girls, with the median onset age of 32 months; 7 cases were left CCSK and 3 cases were right CCSK.There were 9 cases of stage Ⅲ and 1 case of stage Ⅳ at the time of initial diagnosis, when 4 cases were misdiagnosed as other renal tumors at the time of initial diagnosis(40%, 4/10 cases). Five patients with stage Ⅲ CCSK had recurrence and metastasis during treatment and follow-up, and the main distant metastasis sites were lung, bone, liver and brain.(2) Treatment and prognosis of CCSK: seven cases received surgery combined with radiotherapy and chemotherapy, and 3 cases whose parents gave up treatment adopted non-standardized treatment.The median follow-up time was 33.5 months.Seven patients survived and 3 cases died.The 3-year overall survival rate of all 10 patients was 65.6%.The 3-year overall survival rate of stage Ⅲ was 74.1%, and that of stage Ⅳ was 0.The prognosis of stage Ⅲ was significantly better than that of stage Ⅳ( χ2=9, P=0.003). Among the 5 recurrent cases, only 1 case achieved completely remission, 2 cases achieved partially remission, 1 case suffered from disease progression and 1 case died.The 3 cases without recurrence were given standardized treatment of surgery, che-motherapy and radiotherapy, and all were completely remitted. Conclusions:CCSK is easy to be misdiagnosed, and the risk of recurrence and distant metastasis is high in stage Ⅲ patients during treatment and follow-up.Stage Ⅲ patients who actively receive standard treatment including surgery, chemotherapy and radiotherapy have good prognosis, while the mortality of patients with relapse and distant metastasis is high.

Study on the clinical characteristics and prognosis for children high-risk group of head and neck rhabdomyosarcoma / 中华实用儿科临床杂志

Objective:To study the relationship between high-risk factors with the diagnosis, treatment and prognosis of children with high-risk head and neck rhabdomyosarcoma (RMS).Methods:Children with high-risk RMS primarily found in head and neck were selected as research objects according to the criteria of RMS risk degree formulated by Children Oncology Group (COG) and Children′s Rhabdomyosarcoma Cooperative Group, Children′s Hematological Oncology Group, China Anti-Cancer Association (CCCG-RMS), and combined treatment including puncture pathology/surgery, chemotherapy and radiotherapy were performed.The relationship with primary site, age, pathology type and metastasis and prognosis were analyzed.Results:Twenty-nine patients were collected as research object, 17 cases (58.6%) of male, and 12 cases (41.4%) of female.The primary site: orbit was 7 patients (24.1%), and para-meningeal was 22 patients (75.9%). Clinical stage: 2 cases were stage Ⅲ (6.9%), and 27 cases were stage Ⅳ (93.1%). Followed up to December 31, 2018, 14 patients were dead, 15 patients were survival, and 10 patients were event free survival.The overall survival rate was 48.3%(15/29 cases), and the event free survival rate was 34.5%(10/29 cases). According to Kaplan- Merier survival curve analysis, the average overall survival time was (76.0±12.0) months, 95% confidence interval (95% CI): (53.5-93.2)months.The event free survival time was (62.5±10.6) months, 95% CI: (47.0-83.9)months. Conclusions:The prognosis of high-risk head and neck RMS is poor.The center system metastasis is cause of death.Thus, the main task is how to close follow-up and prevention of central nervous system invasion for improving prognosis.

Prognostic analysis of 48 children with hepatoblastoma after pulmonary metastasectomy / 国际儿科学杂志

Objective:To summarize the prognostic characteristics of hepatoblastoma in children undergoing pulmonary metastasectomy.Methods:The clinical data of 48 hepatoblastoma patients with pulmonary metastases who underwent pulmonary metastases resection in our hospital from January 1 2009 to December 31, 2017 were collected, and prognosis of these patients was analyzed.Results:Forty eight cases were diagnosed as hepatoblastoma with pulmonary metastasis, including 30 cases with pulmonary metastasis alone and 18 cases accompany with other high risk factors affecting prognosis. All the 48 patients underwent pulmonary metastasectomy, in which 43 patients were given pulmonary metastases resection completely and 5 patients were given partial resection of metastases. Twenty-five cases recurred after the first pulmonary metastasis resection(25/48, 52.1%), and 2 cases progressed. With the median follow-up time of 45.5 months, survival time of 48 patients with pulmonary metastasectomy was(83.59±7.01)months, and the 3-year overall survival rate was 58%. Of the groups those were patients with pulmonary metastasis alone and patients with other high risk factors affecting prognosis, the survival time and the 3-year overall survival rate showed a significant statistical differences[(95.89±8.19)months vs.(58.95±9.40)months; 79.4% vs.50%; χ2=4.849, P= 0.028)]. The survival time and 3-year survival rate of the two groups between with recurrence or progression and the group without recurrence or progression after pulmonary metastases resection were showed a significant difference[(56.84±7.22)months vs.(112.48±7.08)months; 55.6% vs.90.5%; χ2=11.03, P= 0.001)]. Results of COX regression analysis showed that the main risk factor affecting prognosis was pulmonary metastasis accompany with other high-risk factors for hepatoblastoma patients. The death risk of patients with pulmonary metastasis accompany with other high-risk factors was 3.424 times than that of patients without other high-risk factors( RR=3.424, 95% CI: 1.264~9.275). Conclusion:The overall prognosis of patients with pulmonary metastases is not good, and the proportion of postoperative recurrence is very high. Pulmonary metastasis accompanied with other high risk factors is the main risk factor affecting the prognosis of patients with pulmonary metastasectomy.

Clinical analysis of central nervous system metastasis in children with malignant solid tumor / 中华神经科杂志

Objective:To improve the clinicians′ understanding of central nervous system (CNS) metastasis in children with solid tumors.Methods:The clinical data of 33 cases of CNS metastasis among 611 children with malignant solid tumors in the chest and abdomen from September 2005 to December 2016 in Beijing Tongren Hospital, Capital Medical University were retrospectively analyzed. The clinical characteristics, treatment and prognosis of metastasis were statistically analyzed according to different pathological types of malignant solid tumors in children with CNS metastasis.Results:Among the 611 children with malignant solid tumors in the chest and abdomen, 15 patients were neuroblastoma with CNS metastasis (5.9%, 15/264), 13 patients were hepatoblastoma with CNS metastasis (4.8%, 13/274), two cases were Wilms tumor with CNS metastasis (3.2%, 2/63), and three cases were clear cell sarcoma of the kidney with CNS metastasis (3/10). The onset age of the malignant solid tumors was 3-189 months, and the median age of onset was 26.5 months. The time from the diagnosis of primary malignant solid tumor to diagnosis of CNS metastasis was 0-100 months, and the median time of metastasis was 18 months. The neuroimaging features of CNS metastasis were as follows:30 cases with brain metastasis, three cases with meningeal metastasis, and five cases with spinal cord involvement. Cerebrospinal fluid cytology was positive in two cases (2/3). Of the 33 patients with CNS metastasis, five underwent intracranial tumor resection combined with radiotherapy and chemotherapy, two underwent surgery alone, three underwent radiotherapy alone, and 23 underwent chemotherapy alone. After a median follow-up of 36 months, the survival time of 33 malignant solid tumors with CNS metastasis was (16.96±3.19) months, and the overall survival rate of three years was only 14.7%. The survival time of the patients with intracranial tumor surgery combined with radiotherapy and chemotherapy, surgery alone, radiotherapy alone, and chemotherapy alone was (40.50±4.32), (3.00±0), (35.50±5.50) and (10.35±2.84) months, respectively; and the three-year overall survival rates were 50.0%, 0, 50.0% and 4.3%, respectively (χ 2=15.19, P=0.002). The prognosis of the patients with intracranial tumor resection combined with radiotherapy and chemotherapy was the best. Conclusions:The highest incidence of CNS metastasis in children with malignant solid tumors in the chest and abdomen was found in the clear cell sarcoma of the kidney. The prognosis of CNS metastasis was found to be poor. But the comprehensive therapy of intracranial tumor resection combined with radiotherapy and chemotherapy can prolong survival and improve prognosis.

Correlation between IN1 gene expression levels and clinical characteristics and prognosis of hepatoblastoma in childhood / 中华实用儿科临床杂志

Objective To study on the mRNA expression level of IN1 gene associated with the clinical efficacy and prognosis of hepatoblastoma(HB) in children and to elucidate the early warning value of IN1 gene for prognosis and chemotherapy sensitivity.Methods Forty HB patients were selected based on primary diagnosis and treated from January 2015 to May 2018 in Tongren Hospital Affiliated to Capital Medical University.The expression level of INI1 gene was detected by using fluorescence immuno-PCR.Clinical data of children with HB were collected,including staging,grouping,risk,efficacy and prognosis of chemotherapy.The relationship between the mRNA expression of IN1 gene and clinical data was analyzed by SPSS 21.0 software.Results (1) Clinical characters:the medium age of 40 HB patients was (30.50 ± 2.39) months,male 29 cases (72.5 ％),and female 11 cases (27.5 ％).There was 1 case (2.5％) of stage Ⅱ HB,13 cases (32.5％) of stage Ⅲ HB,and 26 cases (65.0％) of stage Ⅳ.The low-and intermediate-risk group had 12 cases (30.0％) of HB,and the high-risk group had 28 cases (70.0％) of HB.(2) Clinical efficacy and prognosis:by July 1st,2018,the medium following-up time was (12.2 ± 10.1) months,4 patients (10％) were treated by chemotherapy without surgery,and 36 patients (90％) were treated by surgery.All patients (100％) received chemotherapy.The average cycle of chemotherapy lasted (13.17 ± 0.02).Ten patients were dead and 30 patients survived till following-up.The overall survival (OS) rates were 75％ and the event-free survival rates were 50％ (20 patients with HB).(3) IN1 gene mRNA expression:The average PCR quantitative of IN1 gene mRNA of 40 HB patients (2-△CT) was 0.31 ± 0.70.The average mRNA quantitative of HB patients (2-△CT) with high-risk and low/intermediate-risk group was 0.23 ± 0.43 and 0.48 ± 1.13 (t =6.363,P =0.05).According to histology diagnosis,the average mRNA quantitative of small cell undifferentiated (SCU) type (12 cases)and other type (25 cases) was 0.09 ± 0.11 and 0.43 ± 0.86 (t =4.533,P =0.04).The average mRNA quantitative of patients with or without radical surgery was 0.04 ± 0.03 and 0.34 ± 0.74 (t =2.935,P =0.022).The data of children with poor chemotherapy sensitivity(19 cases)and those sensitive to chemotherapy (21 cases) were 0.03 ± 0.04 and 0.30 ± 0.82,and the difference was statistically significant (t =5.688,P =0.018).Conclusions Poor therapeutic effect results in low mRNA expression of IN1 gene in children of HB.The IN1 gene expression could be an early warning factor for treatment sensitivity and prognosis.

CIinicaI anaIysis of 12 rhabdomyosarcoma chiIdren with intracraniaI extension / 中华实用儿科临床杂志

Objective To kxplork thk clinical charactkristics,trkatmknt and prognosis of rhabdomeosarcoma (AMS)with intracranial kxtknsion in childrkn. Methods Thk clinical data of 12 casks of childrkn(5 malks and 7 fk-malks)with AMS and intracranial involvkmknt admittkd into thk Dkpartmknt of Pkdiatrics,Bkijing Tongrkn Hospital Lffiliatkd to Capital Mkdical Rnivkrsite from Dkckmbkr 2012 to Dkckmbkr 2017 wkrk analezkd rktrospkctivkle. Thkrk wkrk 5 malks and 7 fkmalks in 12 casks. Thk onskt agk rangkd from 1. 2 to 10. 2 ekars old,with a mkdian agk of 3. 4 ekars old. Thk clinical fkaturks,trkatmknt mkthods and prognosis wkrk summarizkd. ResuIts Thk primare sitks of 9 casks wkrk in thk hkad and nkcc(75﹪),2 casks wkrk in thk chkst and bacc(17﹪),and 1 cask was in thk pkrinkum (8﹪). Nink patiknts had obvious ckntral nkrvous sestkm involvkmknt semptoms,and cranial imaging findings wkrk studikd in all of thk 12 patiknts. Thk clinical pathological stagk and risc classification wkrk:6 casks of stagkⅣ(1 cask of ckntral aggrkssion);6 casks of stagk Ⅲ(4 casks of ckntral aggrkssion);10 casks wkrk in thk high-risc group(4 casks of stagk Ⅲ with ckntral violations),and 2 casks(stagkⅢ)in thk intkrmkdiatk risc group. Lll 12 casks rkckivkd sestkmic chkmothkrape,8 casks rkckivkd local or total cranial╱spink radiothkrape,9 casks undkrwknt primare rkskction, and 4 casks undkrwknt craniotome to rkmovk intracranial lksions. Rp to Januare 2018,4 casks of 12 childrkn survivkd (including 2 diskask-frkk patiknts)and 8 childrkn dikd. Thk ovkrall survival ratk was 33﹪(4╱12 casks)and thk diskask-frkk survival ratk was 17﹪(2╱12 casks). ConcIusions Thk prognosis of AMS with intracranial infiltration is vkre poor,and thk trkatmknt kffkct is not good. Complktk rkskction of karle lksions is thk cke to curk AMS. Aadiothkra-pe,individualizkd chkmothkrape and surgical rkmoval of intracranial lksions mae havk thk valuk in controlling diskask and prolonging survival timk.

Correlation between IN1 gene expression levels and clinical characteristics and prognosis of hepatoblastoma in childhood / 中华实用儿科临床杂志

Objective@#To study on the mRNA expression level of IN1 gene associated with the clinical efficacy and prognosis of hepatoblastoma(HB) in children and to elucidate the early warning value of IN1 gene for prognosis and chemotherapy sensitivity.@*Methods@#Forty HB patients were selected based on primary diagnosis and treated from January 2015 to May 2018 in Tongren Hospital Affiliated to Capital Medical University. The expression level of INI1 gene was detected by using fluorescence immuno-PCR. Clinical data of children with HB were collected, including staging, grouping, risk, efficacy and prognosis of chemotherapy. The relationship between the mRNA expression of IN1 gene and clinical data was analyzed by SPSS 21.0 software.@*Results@#(1)Clinical characters: the medium age of 40 HB patients was(30.50±2.39) months, male 29 cases (72.5%), and female 11 cases (27.5%). There was 1 case (2.5%) of stage Ⅱ HB, 13 cases (32.5%) of stage Ⅲ HB, and 26 cases (65.0%) of stage Ⅳ. The low-and intermediate- risk group had 12 cases (30.0%) of HB, and the high-risk group had 28 cases (70.0%) of HB.(2)Clinical efficacy and prognosis: by July 1st, 2018, the medium following-up time was (12.2±10.1) months, 4 patients (10%) were treated by chemotherapy without surgery, and 36 patients (90%) were treated by surgery .All patients (100%) received chemotherapy.The average cycle of chemotherapy lasted(13.17±0.02). Ten patients were dead and 30 patients survived till following-up.The overall survival (OS) rates were 75% and the event-free survival rates were 50% (20 patients with HB). (3) IN1 gene mRNA expression: The average PCR quantitative of IN1 gene mRNA of 40 HB patients(2-ΔCT)was 0.31±0.70.The average mRNA quantitative of HB patients (2-ΔCT)with high-risk and low/intermediate-risk group was 0.23±0.43 and 0.48±1.13(t=6.363, P=0.05). According to histology diagnosis, the average mRNA quantitative of small cell undifferentiated (SCU) type (12 cases)and other type (25 cases) was 0.09±0.11 and 0.43±0.86(t=4.533, P=0.04). The average mRNA quantitative of patients with or without radical surgery was 0.04±0.03 and 0.34±0.74 (t=2.935, P=0.022). The data of children with poor chemotherapy sensitivity(19 cases)and those sensitive to chemotherapy (21 cases) were 0.03±0.04 and 0.30±0.82, and the difference was statistically significant (t=5.688, P=0.018).@*Conclusions@#Poor therapeutic effect results in low mRNA expression of IN1 gene in children of HB.The IN1 gene expression could be an early warning factor for treatment sensitivity and prognosis.

Survival of head and neck rhabdomyosarcoma in children: a clinical analysis of 98 cases / 中华全科医师杂志

Objective To analysis the prognosis of head and neck rhabdomyosarcoma (RMS) in pediatrics. Methods The clinical data of 98 pediatric patients with head and neck RMS admitted from February 2005 to September 2017 were retrospectively analyzed. The clinical staging and risk grouping were performed according to international rhabdomyosarcoma study group criterion. The treatment included chemotherapy, radiotherapy and surgery accordingly. The association of prognosis with the disease characters, treatment strategy of 98 patients RMS was analyzed by Chi-square test and Kaplan-Merier analysis with SPSS 20.0 software. Results There were 53 boys (54.1％) and 45 girls (45.9％) with a median age of 70 months (2-161 months). There were 43 cases of orbit RMS(43.9％), 46 cases of paramenigneal RMS (46.9％)and 9 cases of non-paramenigneal and non-orbit RMS(9.2％). Twenty seven patients were classified as low risk (27.6％), 42 patients as medium risk (42.9％),and 29 patients as high risk (29.5％). The patients were followed up for a medium of 42 months (9-149 months), 37 cases were dead, 60 cases were survival, and 1 lost the follow-up. Kaplan-Merier survival analysis showed that the overall survival (OS) was 61.9％(60/97), and the average survival time was (92.3±6.9) months. Univariate analysis showed that the OS of patients with paramenigneal RMS was poorer than that of patients with orbit RMS, non-paramenigneal and non-orbit RMS. The OS of high risk RMS patients was poorer than that of intermediate and low risk patients (χ2=6.132, P=0.047). Conclusions The prognosis of head and neck RMS in children is generally good, but the prognosis of paramenigneal RMS and high-risk RMS is significantly declined. The primary site of paramenigneal and high risk are independent risk factors for the prognosis of head and neck RMS in children.

Role of HMGB1 and TNFAIP3 involvedin mesangial cell proliferation of lupus nephritis / 中国药理学通报

Aim To investigate the mechanism of high mobility group protein B1(HMGB1)and tumor necrosis factor α-induced protein-3(TNFAIP3)involved in cell proliferation in lupus nephritis(LN)patients and human mesangial cells(HMC).Methods Immunofluorescence and immunohistochemistry technique were employed to detect HMGB1,TNFAIP3 and IκBα expression levels in glomerular cells of type Ⅳ LN patients.BrdU incorporation technology was used to detect cell proliferation level in HMC after stimulated by recombinant HMGB1.TNFAIP3 and IκBα expression levels in HMC were detected after HMGB1 stimulation by Western blot.Results The expression levels of HMGB1 and TNFAIP3 were increased in LN patients,while IκBα was decreased.HMC proliferation levels increased significantly after HMGB1 stimulation.At the same time,30 minutes after HMGB1 stimulation,the expression level of TNFAIP3 was significantly increased(P<0.05),while IκBα decreased(P<0.05)and then p65 increased significantly(P<0.05),compared with control group.Conclusion HMGB1 and TNFAIP3 are probably involved in mesangial cell proliferation by activating of NF-κB signaling pathways in LN pathogenesis.

Pancreatoblastoma in children:9 case reports and literature review / 中华实用儿科临床杂志

Objective To analyze the clinical features,treatment and prognosis of the pancreatoblastoma (PB).Methods A total of 9 patients including 4 boys and 5 girls diagnosed as pancreatoblastoma in Beijing Tongren Hospital Affiliated to Capital Medical University between July 2008 and February 2016 were enrolled.The clinical features,therapy and prognosis were analyzed.Results (1) Clinical manifestations:6 cases had abdominal mass,1 case had jaundice,2 cases had abdominal pain at the time of diagnosis;5 cases were found in caput pancreatis,2 cases were found in corpora pancreatis and 2 cases were found in cauda pancreatis;4 cases had local tumor,3 cases had local invasion,and 2 cases had liver metastasis at diagnosis.(2) Enzymology and tumor marks:elevated alpha-fetoprotein (AFP) was found in 5 cases (48.5-52 971.0 μg/L),elevated neuron-specific enolase(NSE) was found in 5 cases(22.4-53.4 pg/L),elevated carbohydrate antigen 125 (CA-125) was found in 5 cases [(40.3-122.8) × 103 U/ L],elevated carbohydrate antigen 19-9 (CA19-9) was found in 2 cases [(65.4-362.5) × 103 U/ L],elevated lactate dehydrogenase was found in 2 cases(380 ～ 838 U/L) and elevated hemodiastase was found in 2 cases (105.5-122.8 U/L).(3) Treatment and prognosis:in 9 patients,the tumors were resected at first in 6 patients,and 5 of them remitted until now,1 of them relapsed after the comprehensive treatment for 9 months.Two of the patients underwent operation after the chemotherapy,in which 1 case received complete resection but without chemotherapy after the surgery,whom relapsed 1 year later;and the other one was resected partly.But after stopping the comprehensive treatment for 6 months,the disease relapsed.One patient could not receive operate because of huge tumor size,and the chemotherapy failed to control tumor progression;the patient died without operation.Three relapsed children were still in chemotherapy.The median follow-up period was 21 months (8-98 months).Conclusions AFP may be used as monitoring indicator for PB.Complete tumor resection is an important factor affecting the outcome.Adjuvant chemotherapy may reduce recurrence and prolong survival.

Clinical analysis of 20 cases of retinoblastoma without calcification on CT scan / 中华实用儿科临床杂志

Objective To analyze the clinicopathologic characteristics,treatment and prognosis of retinoblasto-ma without calcification.Methods The clinic and pathologic data of 20 cases of retinoblastoma without calcification were analyzed retrospectively.All the cases were selected from 710 cases of retinoblastoma archived in Beijing Tongren Hospital,Capital Medical University from April 2010 to April 2015.The inclusion criteria was no calcification on CT scan before treatment.The patients after enuclea-tion were diagnosed according to pathology;the patients without enu-cleation were diagnosed according to clinical manifestations,Retcam fundus,ophthalmic ultrasound and the reduction of tumor after chemotherapy.The clinical features,treatment and prognosis were summarized through statistical analysis of the 20 cases.The patients were followed -up through clinical reexamination and telephone calls.Results In this study,there were 14(70.0%)male and 6(30.0%)female patient′s;there were 19 cases(95.0%)less than 5 -year -old,14 cases (70.0%)less than 3 -year -old,1 case (5.0%)less than 1 -year -old.Eighteen cases (90.0%)were unilateral disease,and 2 cases (10.0%)were bilateral disease.According to international Intraocular Retinoblastoma Classification stage:4 cases (18.2%)in stage E ,17 cases (77.3%)in stage D ,1 case (4.5%)in stage C.Five cases (25.0%)were enucleated eyes,and pathology report was retinoblastoma.Nine cases (45.0%) were treated with pre -enucleation chemotherapy,and finally enucleated after poor tumor shrinkage or recurrence,and the pathology was retinoblastoma.Five cases (25.0%)without enucleation were stable.One case (5.0%)failed to follow -up owing to the patients irregular treatment.In 14 enucleation cases,histopathologic high risk factors could be found in 3 cases (21 .4%),and couldn′t be found in the other 11 cases (78.6%).In the cases that were not enuclea-ted,the tumor diminished in size or new calcification presented in the fundus examination,and in 12 cases,new calcifi-cation was pre-sented on follow -up CT scan.Follow -up failed in 1 case(5.0%),but follow -up was available for 19 cases,and among them 13 cases (68.4%)were disease -free survival and 6 cases (31 .6%)were alive with tumor. Conclusions Close attention should be paid to the untypical retinoblastoma with no calcification on CT scan through well -taken medical history and meticulous imaging examination,in order to give early diagnosis and therapy.

Clinical,pathological and radiological features of retinoblastoma with central nervous system metastasis / 中华实用儿科临床杂志

Objective To summarize the clinical,pathological and radiological features of retinoblastoma (RB)with central nervous system (CNS)metastasis.Methods Twenty -three patients were confirmed to have RB with CNS metastasis in Beijing Tongren Hospital from December 2005 to December 201 3,and their clinical data were retrospectively analyzed.Results (1 )The incidence of RB with CNS metastasis was 1 .83% (23 /1 260 cases),and the incidence of CNS metastasis was 7.64%(1 1 /1 44 cases)if RB with optic nerve involvement which was confirmed through pathology.At first visit 1 0 cases had a confirmed diagnosis of RB with CNS metastasis,while the other 1 3 cases had a diagnosis of RB in extraocular stages.(2)Eleven patients with RB in extraocular stages had the pathological evi-dence of optic nerve involvement which was pathologically confirmed,6 patients had optic nerve involvement with cho-roid involvement,2 patients had optic nerve involvement with nerve sheath involvement.(3)All the patients whose diagnosis of CNS metastasis had the radiological evidence of CNS metastases,and 1 case had autopsy and pathological evidence for extensive brain metastases.The main radiological feature of CNS metastasis was meninges metastasis.Thir-teen cases had the radiological features of meninges metastasis and 5 cases accompanied with spinal cord meninges me-tastasis.Then,the following radiological feature was that 7 cases had the mass of suprasellar pool.(4)Twelve cases with CNS metastasis showed the cerebrospinal fluid (CSF)RB cells positive,and the positive rate was 52.1 7%(1 2 /23 ca-ses).Conclusions (1 )The occurrence of RB with CNS metastasis is very low,but the risk of CNS metastasis in-creased in the patients with the pathological evidence of the optic nerve involvement.(2)The main site of CNS metasta-ses is meninges,followed by mass formation in the suprasellar pool.The main route for CNS metastases was along the optic nerve and /or nerve sheath directly invading and /or disseminating via the CSF.(3)The positive rate of CSF cy-tology is low,and it can be improved through repeated examination.Enhanced magnetic resonance imaging should be the most effective radiology examination for diagnosis and monitoring CNS metastasis.

Prognostic observation of 465 patients with retinoblastoma with optic nerve invasion / 中华儿科杂志

<p><b>OBJECTIVE</b>To observe the treatment and prognosis of optic nerve invasion in retinoblastoma (RB).</p><p><b>METHOD</b>The children who had been diagnosed with unilateral RB and had received enucleation from January 2006 to December 2013 in our hospital were recruited. Tumor extension into the optic nerve were disclosed. Optic nerve involvement was classified into four grades according to the degree of invasion. Grade I is superficial invasion of the optic nerve head only, grade II is involvement up to and including the lamina cribrosa, grade III is involvement beyond the lamina cribrosa, and grade IV is involvement up to and including the surgical margin. Grade I and II are called invasion of the optic nerve before the sieve plate. Grade III and IV are called invasion of the optic nerve after the sieve plate. Other high-risk factors included extensive invasions of the choroid, sclera, anterior chamber, iris, and ciliary body. They were divided into two groups according to whether the merger of other high histopathologic risk factors. Treatment was delivered accordingly, and the prognosis of different degrees of optic nerve invasion was observed. The subjects were followed up for 6 months to 7 years (average: 43 months).</p><p><b>RESULT</b>There were 465 subjects in this study, including 279 boys and 186 girls. The right eye was affected in 260 patients and the left eye in 205 patients. The average time from onset of symptoms to visit was 2.7 months (range 1 day-24 months). Twenty-five patients died, resulting in an overall survival rate of 94.6%. The mortality rate of patients with optic nerve involvement with grade I was 0.4%, grade II was 1.0%, grade III was 8.7% was and grade IV was 60.9%. Of the 338 with invasion of the optic nerve before the sieve plate, two died of recurrence, with a survival rates of 99.4% (336/338). Of the 127 patients who had invasion of the optic nerve after the sieve plate, twenty-three died of recurrence, with a survival rate of 81.9% (104/127) , the difference was statistically significant (χ² = 52.299, P = 0.000). A total of 379 patients did not have any other merged pathology high-risk factors, 8 died, the mortality rate was 2.1%. Of the 86 patients who had complicated with other high-risk factors, 17 died, the mortality rate was 19.8%, the difference was statistically significant (χ² = 42.955, P = 0.000). Of the 338 patients, 304 had invasion of the optic nerve before the sieve plate had not merged other pathology high-risk factors, none died, of the 34 patients who had complicated with other pathology high-risk factors, 2 had died, the mortality rate was 5.9%, the difference was statistically significant (P = 0.010). Of the 127 patients with invasion of the optic nerve after the sieve plate, 76 had not complicated with other pathological high-risk factors, 9 of whom had died, the mortality rate was 11.8%, 51 had complicated with other pathological high-risk factors, 14 of whom had died, the mortality rate was 27.5%, outcomes did significantly differ between the two subgroups (χ² = 5.014, P = 0.025). Cox multivariate analysis showed that invasion of the retrolaminar optic nerve, surgical margin of the optic nerve and sclera were influential factors of colorectal cancer.</p><p><b>CONCLUSION</b>Patients with optic nerve invasion have an excellent outcome with current therapy. But for those whose resection margin was invaded, which has a high incidence of recurrence, chemotherapy is recommended for patients with postlaminar optic nerve involvement.</p>

Clinical treatment and prognostic observation for different pathological infiltrations in 537 patients with unilateral retinoblastoma / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The use of post-enucleation adjuvant therapy to decrease the extraocular relapse rate is frequently considered, but there is much controversy about the indications for adjuvant therapy. The aim of this retrospective study was to observe the treatment and prognosis for different degrees of invasion of eye tissue in retinoblastoma (RB) and identify the indications for post-enucleation adjuvant therapy.</p><p><b>METHODS</b>We recruited 537 children who had been diagnosed with unilateral RB and had received enucleation from January 2006 to December 2012 in our hospital, and divided them into three groups according to their number of histopathologic risk factors: 0 factor, 1 factor, or ≥2 factors. Histopathologic high-risk factors included invasion of the optic nerve posterior to the ethmoid plate (including optic nerve stumps) and extensive invasions of the choroid, sclera, anterior chamber, iris, and ciliary body. Treatment was delivered accordingly, and the prognosis of different degrees of histopathologic invasion was observed. The subjects were followed up for 6 months to 7 years (average follow-up time: 35 months). Statistical analysis was analyzed using χ(2) test. P < 0.05 was considered significant.</p><p><b>RESULTS</b>Of the 537 RB patients who received enucleation, 25 died (overall survival: 95.3%). Of the 369 (68.7%) with no histopathologic risk factors, 1 died of recurrence, with a mortality rate of 0.3%, whereas of the 168 (31.3%) with histopathologic risk factors, 26 had recurrences and 24 died (mortality rate: 14.3%; P = 0.000). Of the 93 patients (17.3%), each of whom had a single risk factor, nine had recurrences, 16 died (8.6%). Of the 75 patients (14%) with two or more high-risk factors, 16 died (21.3%). These differences were statistically significant between the three (P = 0.000).</p><p><b>CONCLUSION</b>Chemotherapy is recommended for patients with histopathologic risk factors, especially those with two or more histopathologic risk factors.</p>

Clinical research on encircling constriction of superficial femoral vein with electrocoagulation in the varicose veins of lower extremity / 中国综合临床

Objective To observe the effect of the operation and application value of encircling constriction of superficial femoral vein with electrocoagulation in the varicose veins of lower extremity.Methods 271 cases were divided into the research group(n=166)with encircling constriction of superficial femoral vein with electro-coagulation and the control group(n=105)with traditional encircling constriction of superficial femoral vein with electrocoagulation.The degrees of the venous reflux,velocities of blood flow,CEAP classification and clinical scoring of the two groups were compared.Results The research group was better than the control group on decrease in de- grees of the venous reflux.increase in velocities of blood flow and decrease in clinical scores(P<0.01).Conclu- sions The encircling constriction of superficial femoral vein with electrocoagulation is the desirable method in the treatment of varicose veins of the lower extremity.