脑脊液肿瘤细胞阳性视网膜母细胞瘤6例治疗观察

观察全身应用卡铂、替尼泊苷和长春新碱（CTV）方案化疗加鞘内注射治疗脑脊液细胞学检查肿瘤细胞阳性的视网膜母细胞瘤（RB）患儿的疗效。方法　6例患儿资料来自首都医科大学附属北京同仁医院2006年11月至2008年7月临床确诊为RB且治疗前行脑脊液细胞学检查发现肿瘤细胞者,对其进行6～9次CTV方案化疗加8～10次鞘内注射治疗,同时结合局部治疗,随访12～20个月,观察疗效。结果　6例患儿均完成治疗,病情稳定,复查脑脊液细胞学检查均未发现肿瘤细胞,无复发及死亡。结论　全身化疗加鞘内注射是对RB颅内转移有效的治疗方法,由于观察时间较短,长期疗效尚须行观察。     

骨髓细胞学检查在视网膜母细胞瘤中的应用价值

目的探讨骨髓细胞学检查在视网膜母细胞瘤转移中的应用价值。方法选取2008年1月至2009年4月临床确诊为视网膜母细胞瘤的211例患儿,在治疗前常规进行骨髓穿刺,并行骨髓细胞学检查,观察早期有无骨髓转移。结果在211例患儿中210例为正常骨髓象,1例骨髓中发现有肿瘤细胞,但不能确定是视网膜母细胞瘤肿瘤细胞。结论视网膜母细胞瘤早期骨髓转移发生率极低,入院患者是否应当常规做骨髓细胞学检查值得商榷。但骨髓细胞学检查仍然是早期发现转移的重要手段之一,对于存在高风险转移因素的患儿仍应积极进行此项检查。     

核素骨扫描在视网膜母细胞瘤中的应用价值

目的 探讨全身核素骨扫描在视网膜母细胞瘤的转移及预后中的应用价值.方法 对2006年8月-2007年2月临床确诊为视网膜母细胞瘤(Rb)的36例患儿,在治疗前进行全身核索骨扫描,观察扫描结果与预后的关系.结果 36例患儿中死亡4例,其中3例为骨侵犯或转移,此3例中只有1例脑脊液中发现肿瘤细胞;随访时间10～18个月,甲均13.6个月.结论 视网膜母细胞瘤的骨转移发生率极低,核素骨扫描不建议作为其首选检查方法;针对晚期病例,核素骨扫描可以对转移和预后作出评估.     

视网膜母细胞瘤中枢神经系统转移的诊疗现状

视网膜母细胞瘤(RB)是儿童最常见的眼内恶性肿瘤,占儿童恶性肿瘤的4%左右。RB1基因突变是最早被发现与RB发生有关的肿瘤抑制基因,常表现为点突变及RB1部分缺失。随着认识的深入以及诊治方法的改善,远处转移期尤其是中枢神经系统(CNS)转移的RB就诊率逐年增高。目前,CNS转移性RB主要通过对临床表现、影像学特征、脑脊液(CSF)细胞学检查、组织病理学检查、基因检测等综合分析而最终诊断,其治疗是目前的难点。虽然近年来针对性地应用了全身静脉化疗、腰穿鞘注化疗、全脑全脊髓放射疗法等手段,结合针对原发病灶的手术及局部治疗手段等进行综合治疗,患儿生存率未见明显提高。因此,全面认识目前CNS转移性RB的诊断、综合治疗及国内外研究进展,对于研究RB发生CNS转移的原因及机制、预后影响因素及发掘更加有效的治疗手段等工作十分必要。     

视网膜母细胞瘤的诊断与治疗

视网膜母细胞瘤是婴幼儿最常见眼内恶性肿瘤,严重危害患儿视力、眼球及生命.现代治疗主要采取全身系统化疗结合局部治疗,在挽救患儿生命的同时尽可能保留眼球及有效视力.早期诊断、早期治疗可使视网膜母细胞瘤的生存率达到90％以上.自体外周血造血干细胞移植支持下的大剂量化疗、动脉介入化疗、眼球摘除、眶内容物剜除及外放疗可改善部分晚期患儿的预后.     

参芪扶正注射液在眼外期视网膜母细胞瘤中的应用

目的 总结参芪扶正注射液对眼外期视网膜母细胞瘤（RB）患儿化疗相关毒副作用的疗效.方法 回顾性分析2005年9月-2010年5月本科收治的眼外期患儿101例（男61例,女40例）,总结研究组（应用参芪扶正注射液＋化疗及放疗）以及对照组（单纯应用化疗及放疗）的临床资料.分析外周血WBC下降水平及恢复时间、生活质量评分、化疗相关毒副作用的统计学差异.结果 （1）造血恢复：研究组WBC恢复中位时间为（10.1±1.5）d,对照组为（13.4±2.3）d,差异有显著性（x2=4.56,P=0.032）;（2） KPS评分：研究组为90.4±2.45,对照组为80.2±2.36,差异有显著性（t=4.659,P=0.001）;（3）化疗相关毒副作用：研究组 级51例（83.6％）,Ⅱ级8例（13.1％）,Ⅲ级2例（3.4％）;对照组Ⅰ级为28例（70.0％）,Ⅱ级6例（15.0％）,Ⅲ级3例（7.5％）,Ⅳ级3例（7.5％）,差异有显著性（t=3.058,P=0.03）.结论 参芪扶正注射液可提高眼外期RB患儿化疗后生活质量,缩短骨髓恢复时间并减轻化疗相关毒副作用.参芪扶正注射液可应用于晚期RB患儿的辅助治疗.     

侵犯视神经的视网膜母细胞瘤患儿465例预后分析

目的 观察眼球摘除术后病理侵犯视神经的视网膜母细胞瘤(RB)患儿的治疗及预后情况.方法 回顾性分析北京同仁医院2006年1月至2013年12月临床确诊为单眼RB,眼球摘除后病理诊断侵犯视神经的465例患儿的病例资料,根据其病理侵犯程度分为4级:1级为肿瘤细胞侵犯视乳头;2级为侵犯筛板;3级为侵犯筛板之后,未到视神经断端;4级为侵犯视神经断端.1级和2级统称为侵犯筛板前视神经,视为病理学低风险因素;3级和4级统称为侵犯筛板后视神经,视为病理学高风险因素.其他病理学高风险因素包括侵犯大范围脉络膜、巩膜、前房、虹膜和睫状体.将患儿分为合并及不合并其他病理学高风险因素组.分别给予相应的治疗并观察其预后.随访时间6个月～7年,平均随访时间3年7个月.结果 465例患儿中男279例,女186例.右眼260例,左眼205例.从发现症状到就诊的时间为1d～2年,平均2.7个月.465例患儿中25例死亡,总生存率为94.6％.侵犯筛板前视神经的患儿有338例,2例复发死亡,生存率为99.4％ (336/338);侵犯筛板后视神经的患儿有127例,23例复发死亡,生存率为81.9％ (104/127);二者差异有统计学意义(x2=52.299,P=0.000).1～4级视神经侵犯患儿的病死率分别为0.4％、1.0％、8.7％、60.9％;465例患儿中379例不合并其他病理学高风险因素,8例死亡,病死率为2.1％;86例合并其他病理学高风险因素,17例死亡,病死率为19.8％;二者差异有统计学意义(x2=42.955,P=0.000).侵犯筛板前视神经的338例患儿中304例不合并其他高风险因素,复发死亡O例;34例合并其他高风险因素,2例复发死亡,病死率为5.9％;二者差异有统计学意义(P =0.010).侵犯筛板后视神经的127例患儿中76例不合并其他高风险因素,9例复发死亡,病死率11.8％;51例合并其他高风险因素,14例复发死亡,病死率27.5％;二者差异有统计学意义(x2 =5.014,P =0.025).病理学高风险因素Cox回归分析显示:侵犯巩膜、视神经和视神经断端是影响预后的危险因素.结论 侵犯视神经的RB患儿总体治疗效果较好,但是侵犯视神经断端的患儿复发率高,应当积极予以辅助化疗.     

42例双眼眼内期视网膜母细胞瘤疗效分析

目的观察双眼眼内期视网膜母细胞瘤(RB)患儿全身化学药物治疗联合局部治疗的临床效果。方法总结我院2009年12月-2011年2月收治的42例RB患儿的临床资料。其中男22例,女20例。系统化疗采用长春新碱+足叶乙甙+卡铂(VEC)方案治疗,同时应用激光、冷凝技术进行局部治疗,对患儿的保眼率、化疗安全性进行评估。结果(1)中位年龄13个月,双眼发病患儿发病年龄较同期单眼患儿小,但未见显著统计学差异(P=0.075)。(2)29例患儿以白瞳为主要表现,占69%。(3)疗效与预后:患儿初诊时Group A期眼3只(3.6%);Group B期眼9只(10.7%);Group C期眼10只(11.9%);Group D期眼40只(47.6%);Group E期眼22只(26.2%)。眼球摘除患儿21人,化疗前后D及E期眼摘率有显著差异(P=0.016)。本组患儿保眼率:E期11只(50%);D期25只(62.5%);C期9只(90%);B期9只(100%);A期3只(100%)。总保眼率67.8%。本组患儿共有5例死亡(12%),4例为肿瘤进展,1例为化疗后颅内出血死亡。(4)化疗毒副作用:仅3例出现一过性听力异常并在随后的复查中双耳听力阈值恢复正常。结论未发现患儿存在明显的、不可逆的听力损害,提示本方案的安全性。对于双眼RB患儿,局部治疗联合化疗总体治疗效果非常好,大大提高了RB患儿的生存率和保留眼球及有用视力的比率。     

CTV化疗方案治疗视网膜母细胞瘤的不良反应及其防治

目的 观察CTV化疗方案治疗视网膜母细胞瘤的相关不良反应及其防治措施.方法 选取2008年1月至2008年12月临床确诊为视网膜母细胞瘤并化疗的患儿,共154例.采用CTV化疗方案(卡铂28 mg/kg、替尼泊苷8 mg/kg、长春新碱0.05 mg/kg),观察患儿化疗时及化疗后的不良反应.结果 未摘除眼球的眼内期患儿化疗后肿瘤明显缩小.化疗时及化疗后无任何不良反应者3例,占1.9%;有不良反应按WHO化疗不良反应分级为Ⅰ度者48例,占31.2%;Ⅱ度64例,占41.6%;Ⅲ度28例,占18.2%,Ⅳ度11例,占7.1%.不良反应主要为血液系统和消化系统症状、脱发,少数病例出现发热、过敏和周围神经症状;除脱发外,其余不良反应均恢复.结论 CTV化疗方案治疗视网膜母细胞瘤安全、有效.     

771例儿童视网膜母细胞瘤发病的流行病学分析

目的 分析我院儿科收治的视网膜母细胞瘤(RB)的发病特点及流行病学特征。方法 回顾性分析2016年1月—2020年12月,在我院儿科病房接受诊治的771例RB患者的临床资料。结果 其中男436人,女335人,起病中位年龄为20.0(0-153)个月。单侧眼发病542人,双眼发病222人,三侧性RB患者7人;中位发病年龄分别为24.0个月、10.5个月及6.0个月,三组患儿的平均发病年龄对比有统计学差异(P=0.000)。17.6%(136/771)患者存在延误诊治的情况;眼内期(703/771)、眼外期(55/771)及转移期(13/771)患儿延误的中位时间分别为5.0个月,7.0个月,12.0个月的诊治延误,差异有统计学意义(P=0.005)。随着地域经济水平的下降,患者诊治延误的比例及晚期患者比例有升高的趋势。结论 RB患者5岁以下多见,单眼RB患者发病年龄高,延误诊治与晚期疾病相关。就诊患者围绕区域医疗中心放射分布,地域经济水平可能影响RB患者的就诊情况,进而影响预后。     

The Diagnostic and Predictive Value of Cerebrospinal Fluid Lactate in Children with Meningitis

ABSTRACT. One hundred and thirty-three children with suspected meningitis aged from 11 days to 16 years were investigated with routine cerebrospinal fluid (CSF) laboratory methods: microscopy of a Gram-stained smear, bacterial culture, determination of leukocytes, lactate, and the CSF/blood glucose ratio. On the basis of bacterial cultures and clinical course, the children were classified into three groups: bacterial meningitis (n=18), aseptic meningitis (n=28), and a control group (n=87). The main intention was to study the relation between current diagnostic methods and lactate. CSF lactate levels and cell counts, related significantly (p<0.01) better to the presence of bacterial meningitis than CSF/blood glucose ratios. Lactate levels exceed 2.4 mmol/l in all children with bacterial meningitis, but in none of the control group. Of 28 children with aseptic meningitis 3 had lactate in the range 2.5-2.7 mmol/l, while the others had values of 2.4 mmol/l or less. We consider CSF lactate to be the best predictor in the clinical decision to institute antibiotic treatment of children with suspected bacterial meningitis.     

Evaluation of Suspected Cerebrospinal Fluid Shunt Complications in Children

The development and utilization of CSF shunts have saved the lives of countless children who would have otherwise died without treatment, and the widespread use of these devices has made the management of children with shunts one of the most common problems encountered by pediatric neurosurgeons. When children with shunts seek medical attention, it is frequently a pediatrician or an emergency physician who will be the first to evaluate the patient. The complications associated with shunting can lead to significant morbidity and even mortality if not recognized and treated promptly; therefore, an understanding of the common presentations and causes of shunt failure is critical for first-line providers. Neurosurgical consultation should be considered in any child with a suspected shunt malfunction or infection. The prompt recognition and treatment of these disorders have the potential to reduce the risk of neurological injury and even death in these children.     

Cerebrospinal Fluid Glutamine in Intracranial Hemorrhage in the Newborn

ABSTRACT. Cerebrospinal fluid concentration of glutamine was measured in 43 premature and mature babies suffering from intracranial hemorrhage. Compared with a control group of 38 infants we found elevated levels in all infants with intraparenchymal and subarachnoid hemorrhages. Two out of ten children with subependymal hemorrhages and approximately half of the children with intraventricular hemorrhages also had elevated levels. Elevated levels of cerebrospinal fluid glutamine persisted for up to three weeks and maximum levels were reached two days to two weeks after birth. The analysis of cerebrospinal fluid glutamine concentration might be useful in the early diagnosis of intraparenchymal and subarachnoid hemorrhage in the newborn infant.     

脑脊液神经元特异性烯醇化酶对高胆红素血症新生儿远期神经发育的预测价值

目的了解高胆红素血症足月新生儿远期神经发育状况，探讨脑脊液神经元特异性烯醇化酶（CSF-NSE）水平对远期神经发育的预测价值。方法采用首都儿科研究所与心理研究所制定的0～4岁婴幼儿精神发育量表对足月高胆红素血症及健康新生儿各39例进行3、24个月的智力发育随访；39例高胆红素血症足月新生儿在黄疸极期检测CSF—NSE水平。结果随访3、24个月均发现高胆红素血症足月新生儿总发育商（DQ）低于对照组，差异有统计学意义（P=0，0．047）；单项DQ比较显示精细动作和社交行为均明显低于对照组（Pa=0）；3、24个月DQ与CSF—NSE水平存在显著负相关（r=-0．46P=0．04；r=-0．32P=0．047），与TSB水平无明显关系（r=-0．21P=0．20；r=-0．30P=0．06）。结论高胆红素血症可能对远期智力发育有影响，CSF—NSE可作为判断高胆红素血症新生儿远期神经发育的预测因子之一。     

Immunological Markers in the Cerebrospinal Fluid of HIV-1-Infected Children

ABSTRACT. Several immunological abnormalities were detected in the cerebrospinal fluid (CSF) of human immunodeficiency virus type 1 (HIV-1)-infected children. Intrathecal synthesis of immunoglobulins, free light chains (FLC), IL-1β, IL-6, and M-CSF were demonstrated both in asymptomatic children and children with subacute encephalopathy. Our findings further support the hypothesis that an immunopathological subclinical process within the central nervous system (CNS) may be an early manifestation of acquired immunodeficiency syndrome (AIDS). Cytokine detection in the CSF may represent a useful diagnostic tool in evaluating the outcome of HIV-1-infected patients.     

Gamma-Aminobutyric Acid Concentration in Lumbar Cerebrospinal Fluid from Patients with Febrile Convulsions and Controls

ABSTRACT. The cerebrospinal fluid (CSF) concentration of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA) was analysed in 41 children with febrile convulsions (FC), 41 febrile controls of similar age (control group 1), and 59 controls, who had no fever and/or were outside the age range for FC (control group 2). A significant correlation between CSF-GABA and age was demonstrated for controls (1 + 2) (r= 0.63, p < 0.00001), as well as for patients with FC (r= 0.42, p= 0.003). Patients with FC did not differ significantly from control group 1 in respect to CSF-GABA. Duration of FC was related to both CSF-GABA and age (GABA: r=−0.29, p < 0.05; age: r =−0.32, p < 0.05). For 56 controls (1 + 2) > 1 year of age, a significant negative correlation between CFC-GABA and body temperature was found (r=−0.34, p = 0.01). The low CSF-GABA in the FC-labile age group, the negative correlation of CSF-GABA to body temperature, and the negative correlation of the duration of FC to both CSF-GABA and age, all indicate that GABA could be of importance in the pathophysiology of FC.     

高胆红素血症新生儿脑脊液神经元特异性烯醇化酶和胆红素水平变化的意义

目的了解足月新生儿胆红素脑损伤时脑脊液（CSF）神经元特异性烯醇化酶（NSE）是否增高，探讨CSF中NSE水平（CSF-NSE）与NBNA评分、CSF中胆红素（CSF-BIL）及血清间接胆红素水平（s-UCB）等的相关性。方法随机选择日龄3～7d的足月新生儿黄 疸39例，根据NBNA评分和核黄疸痉挛期的诊断，分为NBNA评分正常组（17例）、NBNA低分组（15例）和核黄疸痉挛期组（7例）；测定CSF-NSE、CSF-BIL和s—UCB水平。结果3组CSF-NSE、CSF-BIL和s-UCB均有显著性差异（P均〈0．01）；两两比较CSF-NSE均有显著性差异（P均〈0．05）；NBNA评分正常组CSF-BIL与NBNA低分组差异无显著性意义（P=0．259）；NBNA评分正常组s-UCB与NBNA低分组差异无显著性意义（P=0．279）；CSF-NSE和NBNA评分、CSF-NSE、s-UCB相关（r=-0．879，0519，0．661P均〈0．01）。发生胆红素脑损伤时CSF-NSE平均值的95％可信区间〉14．93μg／L。结论测定CSF-NSE对新生儿高胆红索血症脑损伤诊断具有较重要价值；足月高胆红素血症新生儿以NBNA评分可推测CSF-NSE水平；NBNA低分时，可能存在胆红素脑损伤。     

Peripheral Facial Palsy in Children: A Cerebrospinal Fluid Study

Seven children and five adults with acute, peripheral facial palsy were investigated by clinical, blood and cerebrospinal fluid (CSF) examinations. Routine blood examinations and blood and CSF serological tests were normal. In all children studied the CSF showed an increased number of mononuclear white cells despite the absence of clinical signs of meningitis in all but one. Two of the twelve patients studied had normal CSF. Contrary to that in adults the outcome in children was excellent, all recovered totally within three months. The present study indicates that acute, peripheral facial palsy is a manifestation of a generalized disorder with subclinical pathology of the central nervous system, and that in children a lumbar puncture should be done, even if there are no signs of meningitis.     

Elevation of Cerebrospinal Fluid Sialic Acid Concentration in Children with Central Nervous System Leukemia

ABSTRACT. We studied sialic acid in the cerebrospinal fluid (CSF) of 52 children with leukemia and 51 children with non-leukemic diseases. The CSF sialic acid concentration in the children with central nervous system (CNS) leukemia was significantly higher than that in the children with acute lymphoblastic leukemia without CNS involvement, acute non-lymphocytic leukemia without CNS involvement, non-hemopoietic diseases, non-suppurative meningitis, epilepsy, and other neurologic diseases. Serial determinations revealed a rapid decline in the CSF sialic acid concentrations in the patients with CNS leukemia who responded well to the therapy and who were free from relapse of CNS leukemia. The simultaneously determined CSF β2 microglobulin concentration did not show any significant changes. These results suggest that the CSF sialic acid may be a good indicator of CNS leukemia.     

脑脊液细胞学检查在中枢神经系统白血病中的应用

目的探讨脑脊液(CSF)细胞学检查在中枢神经系统白血病(CNSL)中的诊断价值。方法采用细胞离心涂片机收集CSF细胞,经染色镜检进行CSF细胞学检查。结果在59例白血病患儿438次CSF细胞学检查中,发现病例和标本阳性率分别为15.3%(9/59)和8.7%(38/438)。结论CSF细胞学检查对CNSL的早期诊断和疗效观察及复发判断有重要价值。