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Retinoblastoma: Socio-Demographic, Clinical and Therapeutic Aspects in the Hemato-Oncology Unit of the Department of Pediatrics of Donka National Hospital, Guinea
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作者 Aïssata Barry Mamadou M. Diop +7 位作者 Mamadou A. Doukoure Emmanuel Camara Saliou B. Diallo Narcisse V. Gateu Tadjom Fatoumata Barry Hasmiou Dia Boubacar Togo Mamadou P. Diallo 《Open Journal of Pediatrics》 2020年第3期561-569,共9页
<strong>Introduction:</strong><span style="font-family:""><span style="font-family:Verdana;"> Retinoblastoma is one of the pediatric malignancies and its treatment is s... <strong>Introduction:</strong><span style="font-family:""><span style="font-family:Verdana;"> Retinoblastoma is one of the pediatric malignancies and its treatment is still challenging. The objective of this study was to highlight the sociodemographic, clinical, and therapeutic aspects of retinoblastoma at the Donka Paediatric Haematology/Oncology unit in Donka university hospital, Guinea, and thereby highlight the conditions of this malignancy in this country. </span><b><span style="font-family:Verdana;">Methods:</span></b><span style="font-family:Verdana;"> This was a retrospective study over 3 years (January 1, 2017 to December 31, 2019). The study population was all retinoblastoma patients who were treated in this unit. Socio-demographic, clinical, and therapeutic data were analyzed. </span><b><span style="font-family:Verdana;">Results:</span></b><span style="font-family:Verdana;"> Out of 210 pediatric patients followed up for cancer in this unit during the period, retinoblastoma accounted for 46 (22%). The patient age at diagnosis was 35.17 months (range: 3 - 132 months). At admission, metastases were observed in 16 patients (35%): 14 with brain metastases and 2 with lymph node metastases. Preoperative chemotherapy was employed in 38 (83%), tumor resection was performed in 20 patients (43%), and postoperative chemotherapy was performed in 19 patients. As for the prognosis, remission in 9, death in 14, and treatment discontinuation in 15. Only one patient had health insurance (2%). </span><b><span style="font-family:Verdana;">Conclusion:</span></b><span style="font-family:Verdana;"> In this region of Guinea, retinoblastoma is the most common ocular tumor in children. Its diagnosis is easy: strabismus and leukocoria are the early signs of retinoblastoma. The prognosis depends on how early the diagnosis is made. Physicians, care-givers, and family members should be aware of the nature of this malignancy and social education/message as such is necessary.</span></span> 展开更多
关键词 RETINOBLASTOMA LEUKOCORIA Chemotherapy ENUCLEATION
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小鼠3种α1,2岩藻糖转移酶的底物特异性 被引量:1
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作者 林蓓 齐藤真木子 岩森正男 《中国医学科学院学报》 CAS CSCD 北大核心 2005年第6期761-766,共6页
目的对比小鼠3种GDP岩藻糖O!半乳糖苷"112-岩藻糖转移酶("112-FT)的底物特异性。方法利用RT-PCR方法克隆小鼠3种"112-FT基因编码区MFUT-Ⅰ、MFUT-Ⅱ、MFUT-Ⅲ,测序后分别将其插入表达载体pcDNA3.1的多克隆位点,构建表达... 目的对比小鼠3种GDP岩藻糖O!半乳糖苷"112-岩藻糖转移酶("112-FT)的底物特异性。方法利用RT-PCR方法克隆小鼠3种"112-FT基因编码区MFUT-Ⅰ、MFUT-Ⅱ、MFUT-Ⅲ,测序后分别将其插入表达载体pcDNA3.1的多克隆位点,构建表达载体pcDNA3.1-MFUT-Ⅰ、pcDNA3.1-MFUT-Ⅱ及pcDNA3.1-MFUT-Ⅲ;采用磷酸钙法将其转染于COS-7细胞进行表达,通过检测3种酶对不同种底物特性比较酶的特异性。结果小鼠基因MFUT-Ⅰ、MFUT-Ⅱ、MFUT-Ⅲ分别与人类H基因(77%)、Se基因(79%)和Sec1基因(75%)具有序列同源性。MFUT-Ⅰ和MFUT-Ⅱ基因转染后的COS-7细胞均具有"112-FT活性1而MFUT-Ⅲ基因转染的COS-7细胞无此活性。MFUT-Ⅱ同时对底物缺乏唾液酸的神经节苷脂(GA1)及单唾液酸四己糖神经节苷脂(GM1)存在活性1分别生成产物岩藻糖基化GA1(FGA1)及岩藻糖基化神经节苷脂GM1(FGM1)<而MFUT-Ⅰ仅对底物GA1存在活性。MFUT-Ⅱ对GA1的比活性约为MFUT-Ⅰ的80~90倍。MFUT-Ⅱ对GA1的比活性为GM1的10 ̄20倍。MFUT-Ⅱ不仅具有合成Ⅳ型H抗原FGA1及FGM1活性,同时具有对乳丁糖神经酰胺(Lc4Cer)及异乳丁糖神经酰胺(nLc4Cer)的活性,可合成Ⅰ型及Ⅱ型H抗原。结论MFUT-Ⅱ是小鼠的主要"112-FT,具有合成Ⅳ型H抗原FGA1及FGM1的功能;MFUT-I仅有合成Ⅳ型H抗原FGA1的功能;MFUT-Ⅲ无"112-FT活性。 展开更多
关键词 小鼠 α1 2-岩藻糖转移酶 基因 糖脂 底物特异性
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Scene-trait coping style of military rescuers in Wenchuan earthquake
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作者 Li-yi ZHANG Xin-yang SUN +7 位作者 Ling-ming KONG De-hua TU Liang ZHANG Guang-yao LI Zi-xiang SONG Chun-xia CHEN Gao-feng YAO Jie SHI 《中国应用生理学杂志》 CAS CSCD 2014年第6期538-543,共6页
Objective This study aimed to explore the scene-trait coping style of military rescuers in Wenchuan earthquake in an effort to provide scientific evidences for mental intervention program for Chinese military personne... Objective This study aimed to explore the scene-trait coping style of military rescuers in Wenchuan earthquake in an effort to provide scientific evidences for mental intervention program for Chinese military personnel.Methods By cluster sampling,a total of 151 military rescuers and 331 control servicemen were administered the military personnel scene-trait coping style scale(MPSTCSS).Results All active coping factor scores,and passive coping factor scores of affection,health and economy in the rescuer group were significantly higher than those in control group(P<0.05).The 21-above age subgroup,the3-year plus service subgroup,and the officer subgroup had significantly higher active coping factor scores on military tasks,military experience and personal development than those of the 21-below age subgroup,3-year minus service subgroup and the soldier subgroup,respectively(P<0.05 or P<0.01).Conclusion The earthquake relief servicemen can cope with stressful situations better than control group by taking active coping style.The officers,servicemen older than 21 years,and servicemen with more service duration than3 years could usually take active coping style. 展开更多
关键词 军事任务 地震救援 场景 汶川 风格 因子得分 整群抽样 救援人员
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在1例γ-干扰素受体1缺乏症患儿的播散性非结核性分枝杆菌感染
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作者 Tsolia M.N. Chapgier A. +1 位作者 Taprantzi P. 韩浩 《世界核心医学期刊文摘(儿科学分册)》 2006年第12期22-22,共1页
We describe the case of a 2-year-old boy with disseminated infection by a rapidly growing,poorly pathogenic mycobacterial species that belonged to the Mycobacterium fortuitum-Mycobacterium peregrinum complex. He had a... We describe the case of a 2-year-old boy with disseminated infection by a rapidly growing,poorly pathogenic mycobacterial species that belonged to the Mycobacterium fortuitum-Mycobacterium peregrinum complex. He had a severe course characterized by a poor response to treatment and recurrent lymph node abscess formation. Sequencing of the interferon-γreceptor 1 gene (IFNγR1) revealed that he was homozygous for a novel null mutation,453delT. Patients presenting with disseminated infections by rapidly growing environmental mycobacteria must be investigated for complete IFNγR1 deficiency. The spectrum of IFNγR1 genotypes associated with this immunological disorder is expanding. 展开更多
关键词 缺乏症 Γ-干扰素 播散性 分枝杆菌菌种 分枝杆菌感染 无效突变 纯合型 完全性 免疫异常 致病性
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特应性皮炎患儿SCORADs经验:主观症状与疾病的范围和程度不符
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作者 Hon K.-L.E. Leung T.F. +2 位作者 Wong Y. Fok T.F. 王琼 《世界核心医学期刊文摘(皮肤病学分册)》 2006年第10期57-57,共1页
Background:Atopic dermatitis(AD)is a distressing disease associated with pruritus and sleep disturbance.It is not known how well these symptoms correlate with the extent and intensity of eczematous involvement.We eval... Background:Atopic dermatitis(AD)is a distressing disease associated with pruritus and sleep disturbance.It is not known how well these symptoms correlate with the extent and intensity of eczematous involvement.We evaluated whether:(i)the level of sleep loss correlates with pruritus and(ii)the level of pruritus correlates with the extent or severity of AD in children according to the SCORing Atopic Dermatitis(SCORAD)index.Method:Patients with AD younger than 18 years old were recruited from the pediatric dermatology clinic of a university teaching hospital,and AD severity was evaluated by the SCORAD index.Results:One hundred and eighty-two Chinese children with AD(107 boys and 75 girls)[mean(SD)age of 9.6(4.2)years] were recruited.Their mean(SD)overall SCORAD was 30.1(19.2).Sleep loss was strongly correlated with pruritus(r=0.57,P < 0.001).However,the two subjective symptoms were only weakly correlated with the objective signs(extent and intensity)of AD.The correlations between pruritus and extent and intensity were 0.42(P < 0.001)and 0.38(P < 0.001),respectively,and the correlations between sleep loss and extent and intensity were 0.38(P < 0.001)and 0.34(P < 0.001),respectively.Conclusion:We speculate that the lack of a better correlation was either because pruritus and sleep loss as reported by parents were imprecise,or that mechanisms other than disease extent or severity are responsible for the pathogenesis of these subjective symptoms. 展开更多
关键词 特应性皮炎 SCORADs 瘙痒程度 湿疹化 严重度 大学教学医院 睡眠障碍 患儿父母 客观体征
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超声心动检查对疑有宫内感染早产儿循环状态的评估
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作者 Murase M. Ishida A. 刘莉(译) 《世界核心医学期刊文摘(儿科学分册)》 2006年第8期25-26,共2页
Objective:To assess early circulatory status in very low birthweight(VLBW)infants with suspected intrauterine infections.Patients:Thirteen VLBW infants who were diagnosed with prenatal infections because of raised ser... Objective:To assess early circulatory status in very low birthweight(VLBW)infants with suspected intrauterine infections.Patients:Thirteen VLBW infants who were diagnosed with prenatal infections because of raised serum IgM at birth(infectious group),and 39 infants matched for gestational age and birth weight(control group).Methods:Echocardiographic assessments were performed consecutively from birth to day 28 in all VLBW infants.Left ventricular output(LVO)and left ventricular stroke volume(LVSV)were measured using Doppler echocardiography.Pulsed Doppler assessment of pulmonary artery pressure(PAP)was performed using the corrected ratio of the pulmonary artery acceleration time to the right ventricular ejection time(AT/RVET(c)).Blood flow in the superior mesenteric artery(SAAA)was also evaluated by Doppler ultrasound.Results:Mean LVO and LVSV were both significantly higher in the infectious group than in the control group at 12 hours(LVO;188 v 154 ml/kg/min)and 72 hours(LVO;216 v 173 ml/kg/min)of life.Pulsed Doppler assessment of PAP showed that mean AT/RVET(c)values were significantly lower in the infectious group than in the control group at 48 hours,96 hours,day 14,and day 28.In the analysis of SMA flow velocities,both peak systolic velocities and time averaged velocities had decreased significantly in the infectious group compared with the control group at 24 hours,36 hours,96 hours,and day 28.Conclusions:VLBW infants with suspected prenatal infection showed a unique circulation status,namely high cardiac output,latency of high PAP,and low organ flow. 展开更多
关键词 超声心动图 宫内感染 循环状态 早产儿 极低出生体重儿 脉冲多普勒 每搏输出量 查对 肠系膜上动脉 VLBW
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血管性血友病患儿行颅缝早闭成型术的围手术期管理:不同寻常的关注目标
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作者 Isabelle Maquoi, MD Vincent Bonhomme, MD, PhD +6 位作者 Jacques Daniel Born,MD,PhD Marie-Frangoise Dresse, MD, PhD Elisabeth Ronge-Collard,MD Jean-Marc Minon, MD, PhD Pol Hans, MD, PhD 蒋金娣(译) 钱燕宁(校) 《麻醉与镇痛》 2010年第5期69-73,共5页
我们报告一例在手术前评估时诊断为重型I型血管性血友病并用VII因子浓缩剂和瑞斯托霉素辅因子治疗的患儿,成功行颅缝早闭成形术的管理经验。麻醉医师、神经外科医师、临床病理医师和小儿血液科医师的合作是成功的关键。
关键词 血管性血友病 颅缝早闭 围手术期管理 患儿 成型术 神经外科医师 麻醉医师 瑞斯托霉素
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