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1.
Zhonghua Xue Ye Xue Za Zhi ; 41(1): 16-22, 2020 Jan 14.
Artigo em Chinês | MEDLINE | ID: mdl-32023749

RESUMO

Objective: To probe the prognostic value of consolidation chemotherapy in non-favorable acute myeloid leukemia (AML) patients who were candidates for allogeneic hematopoietic stem cell transplantation (allo-HSCT) with first complete remission (CR(1)) and negative minimal residual disease (MRD(-)) . Methods: A retrospective analysis was conducted on 155 patients with non-favorable AML who received allo-HSCT in CR(1)/MRD(-) from January 2010 to March 2019. The survival data were compared between patients who received and those not received pre-transplant consolidation chemotherapy. Results: A total of 102 patients received pre-transplant consolidation chemotherapy (consolidation group) , and 53 cases directly proceeded to allo-HSCT when CR(1)/MRD(-) was achieved (nonconsolidation group) . The median ages were 39 (18-56) years old and 38 (19-67) years old, respectively. Five-year post-transplant overall survival [ (59.3±7.5) % vs (62.2±6.9) %, P=0.919] and relapse-free survival [ (53.0±8.9) % vs (61.6±7.0) %, P=0.936] were not significantly different between the two groups (consolidation vs nonconsolidation) . There was a weak relationship between consolidation therapy and cumulative incidence of relapse [consolidation: (21.9±5.4) % vs nonconsolidation: (18.3±6.0) %, P=0.942], as well as non-relapse mortality [consolidation: (22.4±4.3) % vs nonconsolidation: (28.4±6.5) %,P=0.464]. Multivariate analysis indicated that pre-transplant consolidation and the consolidation courses (< 2 vs ≥2 courses) did not have an impact on allo-HSCT outcomes. Conclusion: Allo-HSCT for candidate patients without further consolidation when CR(1)/MRD(-) was attained was feasible.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Leucemia Mieloide Aguda , Adolescente , Adulto , Idoso , Humanos , Leucemia Mieloide Aguda/terapia , Pessoa de Meia-Idade , Neoplasia Residual , Prognóstico , Estudos Retrospectivos , Transplante Homólogo , Adulto Jovem
3.
Zhonghua Er Ke Za Zhi ; 58(1): 13-18, 2020 Jan 02.
Artigo em Chinês | MEDLINE | ID: mdl-31905470

RESUMO

Objective: To evaluate the endothelial function in obstructive sleep apnea syndrome(OSAS) children and to identify related factors of endothelial dysfunction. Methods: This was a cross-sectional study. Children with habitual snoring (snoring ≥3 nights per week) admitted to the ward of otolaryngology, head and neck surgery, Beijing Children's Hospital were recruited to this study between 1(st) June 2015 and 1(st) March 2016. All children aged 3 to 11 years and of them 245 were boys and 110 were girls. All subjects underwent an overnight polysomnography (PSG), as well as endothelial function testing. All subjects were grouped into primary snoring (PS) and OSAS group according to the obstructive apnea hypopnea index (OAHI). T test or Wilcoxon test were used to compare the differences in PSG results between the two groups, and univariate and multivariate correlation analyses were used to explore the relevant factors affecting the endothelial function. Results: A total of 355 subjects were enrolled and 248 had OSAS, and 107 had PS. There were no significant differences in age, gender and body mass index (BMI) Z-score between the two groups (all P>0.05). OSAS group had higher OAHI, oxgen desaturation index and respiratory related arousal index (5.2 (2.2, 13.2) vs. 0.4 (0.1, 0.7), 4.1 (2.0, 13.1) vs. 0.5 (0.1, 1.0), 2.5 (1.0, 4.8) vs. 0.4 (0.1, 0.9), Z=-14.957, -11.790, -10.378, all P<0.01), and lower minimum oxygen saturation and reactive hyperemia index (RHI) than those of PS (0.89 (0.85, 0.92) vs. 0.94 (0.91, 0.95), 1.2±0.2 vs. 1.1±0.1, Z=-9.337, t=5.354, P<0.01). Univariate regression analysis showed that RHI was linearly correlated with age (parameter estimate=0.017, P<0.01), gender (parameter estimate=0.065, P<0.01), OAHI (parameter estimate=-0.023, P<0.01), oxygen desaturation index (parameter estimate=-0.019, P<0.01), respiratory related arousal index (parameter estimate=-0.031, P<0.01), and oxygen saturation nadir (parameter estimate=0.067, P=0.045). The relationship between BMI Z-score and RHI was quadratic. Multivariate regression analysis showed that age (parameter estimate=0.015, P<0.01), BMI Z-score (parameter estimate=0.040, P<0.01), BMI Z-score quadratic form (parameter estimate=-0.010, P<0.01), respiratory related arousal index (parameter estimate=-0.020, P<0.01) were independently correlated with RHI. Conclusions: Children with OSAS have significant endothelial dysfunction compared with PS. Frequent arousals due to obstructive respiratory events during sleep may be a candidate risk factor for endothelial dysfunction in children with OSAS.


Assuntos
Endotélio Vascular/fisiopatologia , Apneia Obstrutiva do Sono/fisiopatologia , Ronco/fisiopatologia , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Masculino , Polissonografia , Sono
4.
Zhonghua Bing Li Xue Za Zhi ; 48(12): 979-981, 2019 12 08.
Artigo em Chinês | MEDLINE | ID: mdl-31818077
5.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi ; 54(12): 902-906, 2019 Dec 07.
Artigo em Chinês | MEDLINE | ID: mdl-31887815

RESUMO

Objective: To investigate the snoring status and related family factors of children from 3 to 14 years old in Beijing. Methods: From May to July, 2015, data of children from 3 to 14 years old were obtained from a status survey from 7 districts(Xicheng, Chaoyang, Changping, Shunyi, Fangshan, Huairou and Mentougou) in Beijing. A total of 11 420 children from 25 primary and middle schools were randomly selected. Pediatric Sleep Questionnaire (PSQ) and a self-administered questionnaire were carried out for the adopted children. Self-administered questionnaire included the snoring related family factors. Logistic regression was used to estimate the odd ratio(OR) with 95% confidence intervals for variables. Results: A total of 9 198 children meet the inclusion criteria and are analyzed in the study, of whom 901 (9.80%) were found with snoring behavior. The incidence of boys is higher than girls. Obese children take higher risk of snoring. Compared with younger children (≤6 years old), older children (≥12 years old) have a significantly lower risk of snoring (OR=0.464, 95%CI 0.368-0.585). There is no statistical association between full-term infants, infant feeding pattern, parental cigarette smoking and child snoring.The children with family history of snoring have a significantly higher risk of snoring occurrence. The educational background of mother is statistically related to children snoring (OR=1.241, 95%CI 1.058-1.457). Conclusions: The incidence of children snoring in Beijing is 9.80%, male gender, obesity, and young age are all risk factors for children snoring. There is a significantly statistical relationship between snoring and related family factors, such as family snoring history and education experience.


Assuntos
Sono , Ronco , Adolescente , Pequim , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Masculino , Prevalência , Fatores de Risco , Ronco/epidemiologia , Inquéritos e Questionários
6.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi ; 54(12): 954-958, 2019 Dec 07.
Artigo em Chinês | MEDLINE | ID: mdl-31887827

RESUMO

For a long time, the diagnosis and treatment of pediatric thyroid nodules and cancer (PTNC) are mainly referred to adults. In recent years, it has been found that there are great differences between PTNC and TNC in adults. In 2015, the American Thyroid Association released the first Management Guidelines for Children with Thyroid Nodules and Differentiated Thyroid Cancer. In the guidelines, the characteristics of PTNC were described, and clear management strategies were provided. In this paper, we mainly interpret the surgical part of the guidelines and also review the associated research progress in recent years.


Assuntos
Adenocarcinoma , Neoplasias da Glândula Tireoide , Nódulo da Glândula Tireoide , Adenocarcinoma/terapia , Criança , Humanos , Guias de Prática Clínica como Assunto , Neoplasias da Glândula Tireoide/terapia , Nódulo da Glândula Tireoide/terapia , Estados Unidos
8.
Eur Rev Med Pharmacol Sci ; 23(24): 10740-10750, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31858541

RESUMO

OBJECTIVE: Increasing evidence demonstrated that long non-coding RNA (lncRNA) played a vital role in human tumorigenesis and progression, including colorectal cancer (CRC). However, the underlying mechanisms are still largely unknown. The aim of this study was to investigate the function of lncRNA LINC00324 on the development of CRC and explore the possible mechanisms. MATERIALS AND METHODS: The expression level of LINC00324 and miR-214-3p was measured by quantitative real time polymerase chain reaction (qRT-PCR) in CRC cells. The effects on cell proliferation, migration and invasion were assessed by MTT and transwell assays, respectively. In addition, the protein levels of cyclin D1, p21, p27 and three MMPs were detected by Western blot analysis. The target of LINC00324 was predicted by online software and confirmed by luciferase reporter assay. RESULTS: We first detected the expression of LIN00324 was increased while miR-214-3p was decreased in CRC cells. Knockdown of LIN00324 suppressed proliferation, migration and invasion in SW620 and HCT15 cells. Moreover, overexpression of miR-214-3p also inhibited CRC cell proliferation, migration and invasion. Then, we identified miR-214-3p as directly target of LINC00324 and the expression of miR-214-3p was downregulated by LINC00324. In addition, inhibiting miR-214-3p reversed the effects of LINC00324 on CRC cell proliferation, migration and invasion. CONCLUSIONS: Our results proved that LINC00324 regulated CRC cell proliferation, migration and invasion by sponging miR-214-3p, suggesting that it might be a potential therapeutic target for CRC therapy.

9.
Zhonghua Xue Ye Xue Za Zhi ; 40(11): 932-938, 2019 Nov 14.
Artigo em Chinês | MEDLINE | ID: mdl-31856443

RESUMO

Objective: To explore the clinical and prognostic values of TP53 gene mutation in patients with acute myeloid leukemia (AML) . Methods: A retrospective analysis of 265 newly diagnosed AML patients with next-generation sequencing (NGS) data in the Hematology Department of Changhai Hospital from January 2010 to January 2019 was performed. Mutation analysis was carried out by targeted sequencing technology including 200 hematological malignancy related genes. The association of TP53 mutation with clinical features was analyzed. Results: Alterations in TP53 were found in 20 (7.5%) patients, including 17 case (6.4%) of missense mutations, 2 cases (0.7%) of frame-shift deletion mutations and 1 case (0.4%) of splicing sites mutation. A total of 23 kinds of TP53 mutations were detected, most of them (16, 69.6%) were located in the DNA binding domain of exon 5-8, 4 in the DNA binding domain of exon 3-4, 2 in exon 10 and 1 in splice site, respectively. The median age of patients with TP53 alterations was higher than those without [52 (26-72) years old vs 45 (14-75) years old, P= 0.008]. The frequency of complex karyotypes was higher in patients with TP53 alterations than those without [45.0% (9/20) vs 6.1% (15/245) , P<0.001]. Median overall survival (OS) of patients with TP53 alterations was shorter than those without[14.1 (95%CI 6.78-21.42) months vs 31.4 (95%CI 13.20-49.59) months, P=0.029]. The OS of patients treated with "Decitabine + CAG" was superior than that of patients treated with "3 + 7" regimen [30.0 (95%CI 27.35-38.84) months vs 12.5 (95%CI 5.80-19.19) months, P=0.018]. Multivariate analysis indicated that TP53, DNMT3A and USH2A alterations, WBC ≥ 12.45×10(9)/L had negative impacts on OS. Conclusion: The frequency of TP53 mutation was 7.5% in our cohort. Most mutations were located in the DNA binding domain. TP53 alterations were strongly associated with older age, complex karyotype and shorter OS. Decitabine-based induction chemotherapy and hematopoietic stem cell transplantation may improve OS, more cases and/or multicenter randomized studies are needed for further confirmation.


Assuntos
Leucemia Mieloide Aguda , Proteína Supressora de Tumor p53/genética , Adolescente , Adulto , Idoso , Análise Mutacional de DNA , Humanos , Leucemia Mieloide Aguda/genética , Pessoa de Meia-Idade , Mutação , Prognóstico , Estudos Retrospectivos , Adulto Jovem
10.
Zhonghua Yi Xue Za Zhi ; 99(43): 3428-3431, 2019 Nov 19.
Artigo em Chinês | MEDLINE | ID: mdl-31752473

RESUMO

Objective: To evaluate the value of Gd-BOPTA enhanced MRI in the staging of liver fibrosis. Method: Fifty male SD rats (6-week-old, 180-220 g) were divided into the modeling group (n=42) and the control group (n=8). The model of liver fibrosis in the modeling group was established by carbon tetrachloride (animal license No. SYXK (Su) 2017-0043). From week 2 to week 10, rats in the modeling group (n=4) and control group (n=1) were randomly selected to scan 1 h(RER1), 2 h(RER2) and 3 h(RER3) after injection of Gd-BOPTA, respectively, to measure the relative enhancement rate (RER) of liver parenchyma. The shape of intrahepatic bile duct and the degree of enhancement at each time point were observed. Results: Forty-two rats (34 rats in the modeling group and 8 rats in the control group) completed the experiment. RER1, RER2 and RER3 of the control group were 1.44±0.37, 1.22±0.37 and 0.84±0.28 respectively. RER1, RER2 and RER3 of the modeling group were respectively: S1 (n=6): 1.49±0.48, 1.29±0.39, 0.91±0.38;S2 (n=9): 1.48±0.44, 1.34±0.37, 1.04±0.40;S3 (n=11): 1.49±0.43, 1.37±0.39, 1.21±0.30; S4 (n=8): 1.49±0.44, 1.40±0.37, 1.24±0.40. There was no significant difference in RER1 and RER2 values between the control group and the liver fibrosis group (F=0.022, P=0.999; F=0.301, P=0.875). There were significant differences between the control group and RER3 values of hepatic fibrosis stage S3 and S4 (t=2.249, P=0.031; t=2.274, P=0.029), there was no significant difference between the remaining groups (all P>0.05).In the control group, the intrahepatic bile duct was obviously strengthened within 1 hour after enhancement, and walked naturally. The intrahepatic bile duct was slightly enhanced 1h after the enhancement of S3-S4 stage of hepatic fibrosis, and the intrahepatic bile duct was significantly enhanced 2-3 hours later, with distorted alignment. Conclusion: Delayed 3 hours liver parenchymal RER and intrahepatic bile duct distortion delay enhancement after Gd-BOPTA enhancement contribute to the S3-S4 diagnosis of liver fibrosis.


Assuntos
Cirrose Hepática , Animais , Meios de Contraste , Gadolínio DTPA , Imagem por Ressonância Magnética , Masculino , Meglumina/análogos & derivados , Compostos Organometálicos , Ratos , Ratos Sprague-Dawley
12.
Zhonghua Er Ke Za Zhi ; 57(10): 767-773, 2019 Oct 02.
Artigo em Chinês | MEDLINE | ID: mdl-31594063

RESUMO

Objective: To summarize the clinical characteristics, treatment response and prognostic factors of rhabdomyosarcoma (RMS) in children. Methods: The clinical characteristics such as age at diagnosis, primary tumor site, tumor size, pathological type, clinical stage, and risk grouping of 213 RMS patients (140 males and 73 females) treated in Hematology Oncology Center of Beijing Children's Hospital, Capital Medical University, from May 2006 to June 2018 were analyzed retrospectively. The clinical characteristics, overall survival (OS), event free survival (EFS) and prognostic factors of children treated with the Beijing Children's Hospital-Rhabdomyosarcoma (BCH-RMS) regimen were analyzed. Survival data were analyzed by Kaplan-Meier survival analysis, and single factor analysis was performed by Log-Rank test. Results: The diagnostic age of 213 cases was 48.0 months (ranged 3.0-187.5 months), of which 136 cases (63.8%) were younger than 10 years old. The head and neck region was the most common primary site of tumor (30%, 64 cases), followed by the genitourinary tract (26.8%, 57 cases). Among pathological subtypes, embryonal RMS accounted for 71.4% (152 cases), while alveolar RMS and anaplastic RMS accounted for only 26.8% (57 cases) and 1.9% (4 cases), respectively. According to the Intergroup Rhabdomyosarcoma Study Group (IRS), IRS-Ⅲ and Ⅳ accounted for 85.0% (181 cases) of all RMS patients. In all patients, 9.4% (20 cases) patients were divided in to low-risk group, 52.1% (111 cases) patients in to intermediate -risk group, 25.8% (55 cases) patients in to high-risk group, and 12.7% (27 cases) patients in to the central nervous system invasion group, respectively. All patients with RMS received chemotherapy. The cycles of chemotherapy were 13.5 (ranged 5.0-18.0) for patients without event occurrence, while 14.2 (ranged 3.0-30.0) for patients with event occurrence. Among the 213 patients, 200 patients had surgical operation, of whom 103 patients underwent surgery before chemotherapy and 97 patients at the end of chemotherapy, 21 patients had secondary surgical resection. Radiotherapy was performed in 114 patients. The follow-up time was 23.0 months (ranged 0.5-151.0 months) . There were 98 patients with relapsed or progressed disease and 67 patients with death. The median time to progression was 10 months, of which 67 (68.4%) relapse occurred within 1 year and no recurrence occurred after follow-up for more than 5 years. The 3-year EFS and 5-year EFS were (52±4) % and (48±4) %, while the 3-year OS and 5-year OS were (65±4) % and (64±4) % by survival analysis. The 5-year OS of the low-risk, intermediate-risk, the high-risk were 100%, (74±5) %, (48±8) %, and the 2-year OS of the central nervous system invasion group was (36±11) % (χ(2)=33.52, P<0.01). The 5-year EFS of the low-risk, intermediate-risk, the high-risk were (93±6) %, (51±5) %, (36±7) % and the 2-year EFS of the central nervous system invasion group was (31±10) % (χ(2)=24.73, P<0.01) . Survival factor analysis suggested that the OS of children was correlated with age(χ(2)=4.16, P=0.038), tumor TNM stage (χ(2)=22.02, P=0.001), IRS group (χ(2)=4.49, P<0.01) and the risk group (χ(2)=33.52, P<0.01). Conclusions: This study showed that the median age of newly diagnosed RMS patients was 4 years. The head and neck and the genitourinary tract were the most common primary origin of RMS. The OS was low in single-center RMS children. The median time to recurrence was 10 months, and recurrence was rare 3 years later.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias de Cabeça e Pescoço/diagnóstico , Neoplasias de Cabeça e Pescoço/terapia , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/terapia , Neoplasias Urogenitais/diagnóstico , Neoplasias Urogenitais/terapia , Adolescente , Criança , Pré-Escolar , Terapia Combinada , Intervalo Livre de Doença , Feminino , Neoplasias de Cabeça e Pescoço/mortalidade , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Masculino , Recidiva Local de Neoplasia/terapia , Prognóstico , Estudos Retrospectivos , Rabdomiossarcoma/mortalidade , Rabdomiossarcoma/patologia , Rabdomiossarcoma Alveolar , Rabdomiossarcoma Embrionário , Análise de Sobrevida , Neoplasias Urogenitais/mortalidade , Neoplasias Urogenitais/patologia
13.
Artigo em Chinês | MEDLINE | ID: mdl-31623049

RESUMO

Objective:To investigate the clinical characteristics, imaging features and treatment of neurogenic tumor in chilehood and to improve the experience in diagnosis and treatment for the disease. Method:The twenty-nine inpatients of histopathologically proven neurogenic tumor from January 2015 to December 2018 were retrospectively analyzed. The pathological types, clinical characteristics, imaging findings, and management were analyzed. Result: There were five cases of schwannoma aged from 9 years to 14 years, five cases of neurofibroma aged from 9 months to 9 years, and nineteen cases of neuroblastic tumor aged from 3 months to 5 years in our series. The chief manifestations were soft tissue masses, snore, and Honer syndrome. As the tumors had different components pathologically, on scans they presented as masses with heterogeneous density. Schwannoma showed as oval masses with clear margins, with the characteristic of"tail sign". The imaging findings of neurofibromas showed unclear boundaries and plexiform neurofibromas showed multiple clumps with visible separation. The ultrasonography of neuroblastic tumor was characterized by hypoechoic, heterogeneous and spotty calcification. All the cases underwent surgical resections. In 5 cases of schwannoma, it was confirmed that the tumors originated from the vagus nerve during the operation, which could be completely removed without postoperative complications. Two cases of neurofibromas were completely resected, 2 cases were only partially resected, and 1 case which located at the entrance of the esophagus was resected under supportive laryngoscopic for three times. Thirteen of 19 children with neuroblastogenic tumors underwent resection in our department after chemotherapy, and 2 patients with postoperative recurrence underwent another operation. Conclusion:Neuroblastoma is the most common neurogenic tumor in the head and neck of children. Most neurogenic tumors have their corresponding characteristics on imaging and should be differentiated. Schwannoma has clear boundary and intact capsule, so it is easier to complete resection. The neurofibroma is commonly difficult to remove with safety margin because of its unclear boundaries. Neuroblastoma should be treated according to the lesion range and stage. Most of the lesions are difficult to remove completely due to the wide range, peripheral blood vessels and important nerves. So preoperative chemotherapy is generally required.


Assuntos
Neoplasias de Cabeça e Pescoço , Neurilemoma , Neurofibroma , Criança , Humanos , Lactente , Recidiva Local de Neoplasia , Estudos Retrospectivos
14.
Zhonghua Xue Ye Xue Za Zhi ; 40(8): 625-632, 2019 Aug 14.
Artigo em Chinês | MEDLINE | ID: mdl-31495127

RESUMO

Objective: To compare the difference of efficacy between traditional Hyper-CVAD/MA regimen and the adolescents inspired chemotherapy regimen, CH ALL-01, in treatment of adult Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph(+) ALL) . Methods: In this study we retrospectively analyzed 158 Ph(+) ALL patients receiving Hyper-CVAD/MA regimen (n=63) or CHALL-01 regimen (n=95) in our center and Changzheng hospital from January 2007 to December 2017, excluding patients with chronic myeloid leukemia in blast crisis. Tyrosine kinase inhibitor (TKI) was administered during induction and consolidation chemotherapy. Patients who underwent hematopoietic stem cell transplantation received TKI as maintenance therapy. Results: Of them, 91.1% (144/158) patients achieved complete remission (CR) after 1-2 courses of induction. CR rate was 90.5% (57/63) for patients in Hyper-CVAD/MA group and 91.6% (87/95) for patients in CHALL-01 group. There was no difference in CR rates between the two groups (χ(2)=0.057, P=0.811) . The last follow-up was June 2018. A cohort of 134 CR patients could be used for further analysis, among them, 53 patients received Hyper-CVAD/MA regimen and other 81 patients received CHALL-01 regimen. The molecular remission rates were significantly higher in CHALL-01 group (complete molecular response: 44.4%vs 22.6%; major molecular response: 9.9% vs 18.9%) (χ(2)=7.216, P=0.027) . For the patients in Hyper-CVAD/MA group, the 4-year overall survival (OS) was 44.81% (95%CI: 30.80%-57.86%) and the 4-year disease free survival (DFS) was 37.95% (95%CI: 24.87%-50.93%) . For patients received CHALL-01 regimen, the 4-year OS was 55.63% (95%CI: 39.07%-69.36%) (P=0.037) and 4 year DFS was 49.06% (95%CI: 34.24%-62.29%) (P=0.015) , while there was no significant difference in 4 year cumulative incidence of relapse (CIR) (P=0.328) or cumulative incidence of nonrelapse mortality (CI-NRM) (P=0.138) . The rate of pulmonary infection was lower in patients received CHALL-01 regimen compared with patients received Hyper-CVAD regimen (43.4% vs 67.9%, χ(2)=7.908, P=0.005) . Conclusions: Outcome with CHALL-01 regimen appeared better than that with the Hyper-CVAD/MA regimen in Ph(+) ALL, which has lower incidence of pulmonary infection, higher molecular remission rate and better OS and DFS.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras , Adulto , Ciclofosfamida , Dexametasona , Doxorrubicina , Humanos , Pessoa de Meia-Idade , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Estudos Retrospectivos , Vincristina
15.
J Pediatr Urol ; 15(5): 556.e1-556.e7, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31377020

RESUMO

INTRODUCTION: Pediatric Ewing sarcoma (ES)/primitive neuro-ectodermal tumor (PNET) occurring in the genitourinary system has been rarely reported. And the result of long-term follow-up is still a matter of debate. OBJECTIVE: The aim of the study was to identify the characteristics, therapeutic strategies, and long-term outcomes of pediatric ES/PNET in the genitourinary system. PATIENTS AND METHODS: All children with genitourinary system PNET from a single center were retrospectively reviewed. The American Joint Committee on Cancer (AJCC) staging system was used to evaluate tumor grade. RESULTS: Four patients were included. Three were boys, and 1 was a girl. The locations of the lesions were the penis in 1 patient, ureter in 1 patient, and kidney in 2 patients. Two patients were of AJCC stage IVB, and the other 2 patients were of stage IIA. In the follow-up ranging from 2.5 to 8.0 years, 3 patients had survival, and 1 patient died. The patient with penis PNET survived during the 8 years of follow-up. One patient with renal PNET had tumor thromboses in the renal vein, inferior vena cava, and right atrium, which was the first definite report in children (case 3). DISCUSSION: Primitive neuro-ectodermal tumor as a highly malignant subgroup of blue round cell tumor is extremely rare in the genitourinary system, especially in children. The current case series represents the first report of penis PNET in children with the longest (8 years) follow-up and first definite report of pediatric renal PNET with vena cava and atrium tumor thrombus. In contrast to the previous literature, the patient with ureteral PNET in this study was much younger, who was the youngest child to be reported in the literature thus far. Although the key prognostic factor of the outcomes is detectable metastases at diagnosis, the patient with penis PNET and bone and lung metastasis in this series still survived. It was hypothesized from the data of present cases that young age was a protective factor, which was consistent with the previous literature. Aggressive therapy is not trivial for patients with multiple recurrences who can also be a long-term survivor. The survival outcomes of these high-stage patients were favorable with combination treatment. As the patient with penis PNET in this series had bone metastasis at his 7.5 years after definite diagnosis, five years of follow-up was not enough. The follow-up period should be extended, even to a lifetime follow-up. CONCLUSIONS: Children with PNET have a better prognosis than adults. Aggressive combination treatment should be performed to improve prognosis and the survival rate. It is better to monitor the changes of the disease by extending the follow-up period.

16.
Artigo em Chinês | MEDLINE | ID: mdl-31262105

RESUMO

Objective: To investigate the sleep quality of children in Beijing and to analyze the related factors. Methods: The data were collected from the survey of 3-14 years old children in 7 urban districts of Beijing in 2015. By using multi-stage stratified cluster random sampling method, 26 kindergartens and primary and secondary schools in 7 districts and counties, including Xicheng, Chaoyang, Changping, Shunyi, Fangshan, Huairou and Mentougou, were randomly selected, with a total of 11 420 children. Children's sleep status was investigated with Children's Sleep Questionnaire (PSQ), and the proportion of children with sleep quality problems when the PSQ score was greater than 7. Various sleep related factors were investigated with self-made questionnaire. A multilevel model was used to analyze the relationship between PSQ score and related factors. Results: The average PSQ score of the children was 3.60±2.69. The proportion of children with sleep quality problems was 8.87%(816/9 198). Multilevel model analysis showed that the younger the children, the higher the PSQ score (<6 years old vs. 6~12 years old vs. >12 years old: 3.94±2.58 vs. 3.58±2.66 vs. 3.30±2.84, F=33.015, P<0.001); male PSQ score higher than female (3.89±2.75 vs. 3.30±2.60, t=10.560, P<0.001); and snoring, obesity, father/mother snoring, playing games before bed, surfing the internet, eating and other factors were statistically related to PSQ. Conclusions: Sleep quality of children in Beijing should not be neglected, especially preschool children with high PSQ scores. Parents should pay attention to children's snoring problems and try to reduce some pre-sleep behaviors that may affect sleep quality.


Assuntos
Sono/fisiologia , Adolescente , Pequim , Criança , Pré-Escolar , Inquéritos Epidemiológicos , Humanos , Distribuição Aleatória , Ronco/fisiopatologia , Inquéritos e Questionários
17.
Artigo em Chinês | MEDLINE | ID: mdl-31262116

RESUMO

More than seventy percent of lymphatic malformations (LMs) occur in head and neck region. The management of head and neck LMs is challenging,especially for diffuse and infiltrative cases.It can cause deformity and death in severe patients. Surgical resection is the main method to treat LMs in the past and sclerotherapy has also greatly improved the treatment out come of LMs over the past 20 years. However, it is still hardly to achieve satisfactory prognosis for the patients with extensively infiltrative lesions, regardless of surgical resection or sclerotherapy. In the past five years, some scholars have made a preliminary exploration of new treatment methods, such as oral medicine, to diversify the treatment options of head and neck LMs. This article will review the general situation, biological properties, clinical characteristics, diagnostic methods, and current treatment strategies for LMs as well as trends in management of LMs.


Assuntos
Anormalidades Linfáticas/diagnóstico , Anormalidades Linfáticas/terapia , Cabeça , Humanos , Pescoço , Escleroterapia , Resultado do Tratamento
18.
J Pediatr Urol ; 15(5): 554.e1-554.e8, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31301975

RESUMO

INTRODUCTION: Urogenital tract foreign bodies (FBs) have been rarely reported in children, and the management is still challenging. OBJECTIVE: The aim of this study is to review a 10-year experience with urogenital tract FBs in a single center. PATIENTS AND METHODS: The authors reviewed the records of children suspected with urogenital tract FBs and first admitted to the hospital, including demographic characteristics, presenting symptoms, methods of diagnosis, and management. The authors compared the surgery strategies in different locations of FBs and age, and the locations of FBs in different age groups. RESULTS: Two hundred and thirty-nine cases were reviewed, and 188 were confirmed to retain urogenital tract FBs (150 girls and 38 boys). The number of the patients increased progressively in the last 10 years and mainly concentrated in spring and summer in the last 4 years. The peak ages were 3-5 years old and 9-13 years old. General anesthesia surgeries were performed on 20 patients (Fig. 1). Vagina FBs were more likely to require day surgery, whereas bladder FBs required surgery in hospital. Patients younger than 6 years were more likely to be girls with vagina FBs, and patients older than 11 years were more likely to be boys with bladder FBs. DISCUSSION: Urogenital tract FBs in children is a great challenge. As the vagina is shorter and wider than the urethra, girls with vagina FBs are usually treated by day surgery and adolescent boys of urethra FBs are treated by hospital surgery. Misdiagnosis may occur when patients conceal FBs insert history, have severe urinary tract infections, or have previous surgery history. Ultrasonography helps to reduce misdiagnosis. FBs should be taken into consideration when patients have new symptoms after hypospadias repair, and postoperative changes of hypospadias repair, such as urinary calculi, have been excluded. Appropriate surgery techniques, based on the size, nature, and location of FBs, should be performed for complete removal of FBs with minimal complications to reduce secondary injury. Sharp FBs could be migrated among the digestive system, urogenital system, and deep pelvic. If the procedure is difficult, patients with a stable needle can be conservatively managed with close follow-up. Nevertheless, symptomatic patients should be treated actively. CONCLUSION: The awareness of potential severity of pediatric urogenital tract FBs should be raised. Appropriate toys and timely sex education help prevent children from urogenital tract FBs insertion. Selecting appropriate techniques for particular situations is the best way to reduce secondary injury, especially for cases with migrated FBs (needles), magnetic FBs, and postoperative FBs.

20.
Insect Mol Biol ; 28(6): 837-849, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31106480

RESUMO

The intensely studied white gene is widely used as a genetic marker in Drosophila melanogaster. Here, we cloned and characterized the white gene in an important pest of the fruit industry, Bactrocera dorsalis, to understand its functional role in pigmentation. We obtained BdWhite knockout strains, based on the wild-type strain, using the CRISPR/Cas9 genome editing system, and found that mutants lost pigmentation in the compound eye and their black head spots. We then examined differences in the expression levels of genes associated with melanin pigmentation between mutants and the wild-type strain using quantitative reverse transcription PCR. We found that transcription levels of the Bd-yellow1 were lower in the head of mutants than in the wild-type strain, and there were no significant differences in expression of the other six genes between mutants and the wild type. Since yellow is critical for melanin biosynthesis (Heinze et al., Scientific Reports. 2017;7:4582), the lower levels of expression of Bd-yellow1 in mutants led to reduced dark pigmentation in head spots. Our results provide the first evidence, to our knowledge, that white may play a functional role in cuticle pigmentation by affecting the expression of yellow.


Assuntos
Sistemas CRISPR-Cas , Olho Composto de Artrópodes/fisiologia , Proteínas de Insetos/genética , Pigmentação/genética , Tephritidae/fisiologia , Sequência de Aminoácidos , Animais , Sequência de Bases , Feminino , Técnicas de Inativação de Genes , Proteínas de Insetos/química , Proteínas de Insetos/metabolismo , Masculino , Filogenia , Alinhamento de Sequência , Tephritidae/genética
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