97例小儿急性淋巴细胞性白血病临床分析

本文对我院儿科６年来收治的９７例小儿急性淋巴细胞白血病（ＡＬＬ）进行临床总结和分析，初治５７例，复治４０例。初治组中４３例采用ＶＤＬＰ方案进行诱导缓解，完全缓解（ＣＲ）率为１００％，复发率为６．９％（３／４２），２例为骨髓复发，１例为睾丸白血病复发，无中枢神经系统复发。本采用ＶＤＬＰ方案诱导缓解的另１４例初治病人ＣＲ率为７５％。复治组中ＶＤＬＰ再诱导缓解的ＣＲ率为６９．２％。非ＶＤＬＰ丙诱导缓解的     

早期连续强烈化疗治疗急性淋巴细胞白血病33例

采用CODPL、CAT、HDMTX、EA、VPDL连续强烈化疗方案治疗小儿急性淋巴细胞白血病（ALL）33例，全部病例获得骨髓缓解，93.9％获完全缓解（CR），2例在诱导期死于败血症。缓解病例随访1年以上，1年、2年、3年CR率分别为93.5％、85.7％、833％，4例患儿分别在缓解后9个月、11个月、16个月、25个月复发。认为早期连续强烈化疗方案治疗儿童ALL奏效快、CR率高、CR期长，最严重的并发症仍是骨髓抑制导致严重感染。     

DAE方案诱导治疗急性髓性白血病

急性髓性白血病（AML）在儿童中发病率逐年增加，据北京儿童医院统计近十年来急性淋巴细胞白血病（ALL）与AML比例由5：1上升至3：1。多年来各家认为DA、HA和TA方案是治疗AML有效的方案，其缓解率在30％左右，一次DA诱导达CR者16．67％，2次DA方案约32．22％病例达CR，自1994年以来应用DAE方案诱导治疗AML，使一次DAE方案CR可达51．02％，2次达73．43％，提高了AML在短时间内达CR的疗效，为进一步化疗奠定了基础。同时对DA和DAE方案骨髓抑制状况作了比较，两组无明显差异。此方案对于无层流设施的一般医院治疗AML提供一项容易推广的措施。     

婴儿白血病16例分析

目的探讨婴儿急性白血病（IAL）的临床特征及预后。方法对我院近几年收治的16例IAL患儿临床资料进行回顾性分析。结果16例中6例接受治疗，ALL5例采用VDLP方案，4例达cR。ANL1例，用HA、DA、EA方案，已达CR11个月。结论IAL是儿童白血病中的特殊类型，其肿瘤负荷大，死亡率高。近年来随着强化疗的应用，能达到CR，但易复发。     

VP-VDLP方案治疗儿童高白细胞急淋白血病18例

外周血白细胞计数≥100×109／L的急性白血病称高白细胞急性白血病。此型白血病极易发生颅内出血及呼吸窘迫综合症，早期死亡率高，缓解率低。本文报导我院1989年1月至1996年6月采用VP-VDCP方案治疗儿童高白细胞急淋白血病18例，目的在于探讨降低早期死亡率，提高长期缓解率的方法。18例中男，14例，女4例，年龄6个月至13岁，确诊时外周血白细胞计数100～200×109／L8例、200～300×109／L6例，＞200×109／L4例。ALL113例、ALL24例、ALL31例。确诊后先给以小剂量VP方案（VCRlmg／m2、Prcl40mg／m2／天），辅以扩容、抗凝〈低分子右旋糖酐〉，碱化尿液（5％碳酸氢钠、别嘌呤醇）等综合治疗。外周白血细胞数第3天开始下降，一般3～5天，最迟10天降至50×109／L以下，此后再给以VDLP方案强化疗，同时鞘内注射MTX．Ara－cDXM每周1次。用此方案使早期死亡率由文献报导的60％降至16．7％；缓解率由20％提高到83．3％，其中2例完全缓解已达7年。然而此型白血病髓外浸润严重，中枢神经系统及睾丸白血病发生率高，极易导致复发，本文复发率33．3％。所以如何加强髓外白血病的防治，是降低复发率，提高长期缓解率的关键，也是急待解决的问题。     

DVPL方案治疗小儿急性淋巴细胞白血病20例分析

为了加强小儿急性白血病的早期强化，不断提高缓解率及长期无病生存率。我院对初治住院的20例急性淋巴细胞白血病（ALL）患儿，采用DVPL方案诱导缓解化疗，对其疗效及毒副反应进行观察分析。20例中完全缓解（CR）19例，初治缓解率95％，全部病例均有不同程度骨髓抑制，时间为用药后2一15天，中位数7．3天；平均WBC第37天，PLT第23．3天恢复至正常范围。本组无明显感染、出血，无重要器官损害，个别病人有轻度脱发及消化道症状，毒性反应为可逆性。维持治疗中无复发及出现髓外白血病等。DVPL方案对ALL选择性好，是比较强烈有效的组合，疗效高耐药株产生少，副作用少，缓解期长，为下一步化疗打下基础，取得满意效果。     

PCR技术在儿童急性淋巴细胞白血病微小残留病的检测及临床意义

本文运用PCR技术，以IgH和TCRγ基因重排作为标志，检测了40例急性淋巴细胞白血病（ALL）微小残留病（MRD），其中有7例初治未缓解、12例部分缓解（PR）、13例完缓解（CR）的ALL患儿MRD检测阳性，另有8例（1例PR、7例CR）检测MRD阴性。文中发现CR时间越长，MRD阳性率越低，反之，CR时间越短，MRD阳性率越高。提示MRD可作为白血病治疗效果及判断预后的一个重要指标，CR后，MRD检测阳性有复发的危险，长期的MRD检测阴性，可作为临床停药观察的一个重要指标。PCR方法准确，先进，适于广泛推广应用。     

肿瘤

980895儿童恶性肿瘤与mdrl基因/应明//江苏医药一1997,23(7)一486一487 980896 156例小儿白血病临床分析/咚莉贞…//广东医学一1996,17(7)一450一451 男104例,女52例。急淋(ALL)114例,急非淋(ANLL)37例,慢性粒细胞白血病中国医学文摘·儿科学1998年第17卷第2期1 19(CML)5例。骨穿确诊152例,骨髓活检确诊4例。ALL16例做细胞免疫分型,B细胞型13例,T细胞型3例。ALL用VP方案或VDLP、VDP方案诱导缓解,ANLL用COAP或HOAP方案诱导缓解。然后预防脑白、强化等。接受治疗54例。ALL47例,CR43例,CR后中断30例,3年CRS例,5年CR4例。A…     

儿童急性淋巴细胞性白血病诱导化疗修正方案的近期临床评估

目的比较上海交通大学医学院附属上海儿童医学中心1999年开始使用的儿童急性淋巴细胞性白血病（ALL）治疗方案（以下简称99方案）,和在99方案基础上减轻治疗强度并从2005年开始使用的修正方案（以下简称05方案）诱导治疗的疗效、安全性,以提高儿童ALL的疗效。方法纵向对比1999年1月1日至2006年3月1日初治的311例ALL患儿临床资料;以2005年5月1日为分界线分为99方案组和05方案组。99方案组243例,05方案组68例。对两组患儿起病时临床资料、治疗反应、缓解情况、治疗相关的感染情况等进行统计分析。结果两组病例起病时各项临床资料（性别、年龄、诊断时外周血白细胞计数）差异均无统计学意义（P均〉0．05）,两组的缓解率（91．8％弧95．6％,P=0．29）和缓解时微小残留病（MRD）监测结果（P=0．17）差异也无统计学意义,99方案组与05方案组达到缓解所需时间（34．18±4．96d vs．32．34±3．36d）、治疗相关的感染率（54．7％伽．23．5％）、重症感染的发生率（9．1％vs.0）和病死率（3．7％vs.0）差异均有统计学意义。结论05方案和99方案在儿童ALL的诱导缓解治疗上都能取得良好的疗效,而05方案在疗效确切的同时具有更高的安全性。     

MRD监测下88例儿童急性B淋巴细胞白血病长期疗效的分析

目的对88例儿童急性B淋巴细胞白血病（B—ALL）治疗的长期随访结果进行分析,探讨微小残留病（MRD）监测下儿童B—ALL的无事件生存率（EFS）。方法回顾性分析2005年1月．2008年5月接受儿童ALL诊疗建议（第三次修订草案）治疗的88例B．ALL患儿,应用流式细胞术（FCM）检测MRD,采用Kaplan—Meier方法评估患儿EFS,各临床危险度分组间患儿EFS差异用Logrank检验。结果88例患儿骨髓完全缓解（CR）率为97．7％,2年、3年、4年、5年EFS率分别为87．5％、86．4％、81．8％、77．2％,标危、中危、高危5年EFS率分别为86．2％、84．6％、63．1％。5例死亡,9例复发（10．5％）,复发中位时间为23（3—59）个月。结论采用儿童ALL诊疗建议（第三次修订草案）治疗CR率较高,在MRD监测下指导治疗,总体上B—ALL患儿的5年EFS提高。     

住院患儿发生心跳呼吸骤停复苏后存活率的预测因素分析

目的 探讨发生心跳呼吸骤停(CRA)住院儿童复苏后存活率的预测因素.方法 回顾性分析PICU发生CRA患儿的临床及心肺复苏(CPR)、复苏后资料,并进行单因素分析以及多因素非条件Logistic回归分析,探讨近期和远期存活率的预测因素.结果 2006年1月至2008年12月烟台毓璜顶医院PICU发生CRA并接受CPR的87例患儿中,43例恢复自主循环,复苏成功率为48.3%,24 h存活31例(35.6%);存活出院19例(21.8%).单因素分析结果显示:原发病、合并症以及发生骤停类型、气管插管、有效复苏时问、应用肾上腺素的剂量、复苏后24 h内体温、复苏后6 h血糖值、复苏后合并症均影响复苏后24 h存活率和出院存活率;Logistic回归分析示原发病、复苏时间为24 h存活率的预测因素;原发病、复苏时间、复苏后24 h体温为出院存活率的预测因素.结论 住院患儿发生CRA后近期、远期存活率均低,原发病及合并症、CPR质量以及复苏后管理均影响存活率,其中原发病、复苏时间为近期存活率预测因素,原发病、复苏时间、复苏后24 h体温为远期存活率的预测因素.     

Evaluation of the LSA2L2 protocol for treatment of childhood non-Hodgkin's lymphoma. A report from the Polish Children's Leukemia/Lymphoma Study Group

From 1979 to 1982, 97 previously untreated children with non-Hodgkin's lymphoma were treated with the LSA2L2 protocol proposed by Wollner. Staging was done according to the criteria proposed by Wollner and re-staged according to Murphy's criteria. Each patient, regardless of clinical stage and histologic group, was given the same chemotherapy. A total of 28 nonrandomized patients received either cranial irradiation or intermediate-dose intravenous methotrexate as CNS prophylaxis. The complete remission rate was 72.6%. The 3-year actuarial estimate of survival was 73% and the disease-free survival rate was 62% for all responders, and was influenced by stage and main clinical features present at the time of initial presentation. The overall survival rate at 3 years is 52%. Of 26 children who failed to achieve complete remission, 21 had presented with disseminated disease. Also, 20/67 patients who entered remission have suffered relapses: four in the bone marrow, seven in the CNS, and nine with local relapses. Only one of 28 children who received CNS prophylaxis developed CNS disease as the site of first relapse, whereas six of those who received only intrathecal chemotherapy did so. This study confirms the improved outlook in comparison with a historical group for children with non-Hodgkin's lymphoma by the use of an intensive multiple-drug regimen and CNS prophylaxis.     

术前化疗治疗神经母细胞瘤的疗效随访及其影响因素

目的探索术前化疗治疗神经母细胞瘤患儿的远期疗效及对其生存的影响因素。方法对１３例经术前化疗后手术的病例进行２～６年的疗效随访，分析存活组与死亡组的临床分期、化疗时间、香草杏仁酸（ＶＭＡ）、病理标本增殖细胞核抗原（ＰＣＮＡ）、ＤＮＡ指数的差异。结果２年生存率为６９％（６／１３），５年生存率为２３％（３／１３），６例（４６％）无瘤生存至今。存活组化疗后ＶＭＡ、ＰＣＮＡ、ＤＮＡ指数较死亡组明显下降（Ｐ均＜０．０５）。结论术前化疗可以提高晚期神经母细胞瘤远期疗效和生存率，化疗后肿瘤增殖活性的抑制程度与临床疗效密切相关。     

儿童胸膜肺母细胞瘤的误诊原因分析

目的 探讨儿童胸膜肺母细胞瘤的误诊原因.方法 将1979年1月至2007年1月临床病理证实的小儿胸膜肺母细胞瘤32例,根据病理切片、免疫组织化学按Dehner分型分为Ⅰ、Ⅱ、Ⅲ三型;根据不同分型的X线和CT特点,结合临床表现,分析初诊诊断,总结漏诊原因,并对其存活率进行分析.结果 32例患儿平均年龄(4.0±2.7)岁(3个月～10岁),男女比例为3:2.32例患儿最早出现的症状均为咳嗽,28例伴发热,最终有18例伴呼吸困难.首诊肺囊性病6例,脓气胸、胸膜炎5例,肺炎18例,纵隔肿瘤2例.2例尸检,3例活检,27例手术,术后均化疗.术后随访至2007年7月,平均随访时间(36.5±40.1)个月(1～144个月).13例存活,18例死亡,1例失访.2年存活率35.5%,5年存活率29.0%.从诊断到死亡的时间为1d～26个月.结论 由于小儿肺母细胞瘤临床表现与成人肺母细胞瘤有明显不同,因此是一种极易漏诊的少见恶性肿瘤.提高对该病的认识,早期诊断、完整切除、综合治疗是决定预后的关键.     

117例儿童脊髓性肌萎缩症自然病史分析

目的 对重庆及周边地区脊髓性肌萎缩症（spinal muscular atrophy,SMA）的自然病史进行分析,为开展SMA的综合管理、基因修饰治疗提供临床依据。 方法 回顾性分析117例SMA患儿的临床资料及生存现状。 结果 117例患儿中,1型SMA 62例（53.0%）、2型45例（38.5%）、3型10例（8.5%）,中位起病年龄分别为2、10、15月龄。1型SMA起病、就诊、确诊时间均早于2、3型SMA（P<0.05）,1型SMA就诊时间窗（起病年龄至就诊年龄）短于2、3型SMA（P<0.05）。肺炎为首发症状、抬头无力、哭声无力、进食费力多见于1型SMA（P<0.05）,2型SMA脊柱侧弯和下肢关节挛缩发生率高于1型（P<0.05）。117例（100%）SMA患儿均为SMN1基因纯合缺失,其中以7号外显子纯合缺失最常见（68.4%,80/117）。1型SMA的6年生存率仅为10%±5%,低于2、3型SMA（P<0.05）。起病年龄≤3月龄、肺炎为首发症状、抬头无力为1型SMA死亡的危险因素（P<0.05）。2型SMA运动能力可呈非线性倒退。 结论 各型SMA患儿临床表现、生存率均存在异质性,1型SMA生存率低,2型SMA运动能力可呈非线性倒退,临床上应早期识别及管理SMA。     

Expression of myeloid differentiation antigens in acute nonlymphocytic leukemia: increased concentration of CD33 antigen predicts poor outcome--a report from the Childrens Cancer Study Group.

Ninety-eight cryopreserved specimens of acute nonlymphocytic leukemia (ANLL) cells obtained at initial diagnosis of children enrolled on the Childrens Cancer Study Group 251 protocol (CCG 251) were examined by indirect immunofluorescence using four monoclonal antibodies to myeloid differentiation antigens. The relationship between the level of differentiation of ANLL cells as determined by their antigen phenotype and the clinical outcome of treatment, including complete remission (CR) rate, survival, and event-free survival, was evaluated. Most leukemic specimens were determined to express the CD33 antigen (L4F3), a 67-kD protein. Because the level of differentiation of normal myeloid cells is reflected by the concentration of the CD33 antigen expressed, samples were categorized as CD33-bright (immature) versus CD33-dull (mature). Patients with CD33-bright leukemic blasts had a marginally inferior CR rate to those with CD33-dull blasts (P = 0.08). With respect to survival and event-free survival, there was a significantly inferior outcome in the CD33-bright patients (P = 0.04 and P = 0.06, respectively). Reactions of ANLL with anti-CD15 antibody (1G10), anti-CD36 antibody (5F1), or anti-CD17 antibody (T5A7) did not predict clinical outcome. This study indicates that patients whose ANLL blasts displayed the CD33 antigen in an amount associated with immature myeloid cells experienced a worse outcome than patients with ANLL blasts that expressed a phenotype associated with more mature cells.     

Primary peritoneal drainage in necrotising enterocolitis: an 18-year experience

Primary peritoneal drainage (PPD) was initially introduced as a method for the pre-operative resuscitation of critically ill infants with complicated necrotising enterocolitis (NEC). Some have recommended it as definitive strategy for a select group of extremely low birth weight babies. The role of laparotomy in neonates who do not respond to initial PPD has also been challenged. With this background, we analysed our experience with the use of PPD in babies with NEC over an 18-year period. We retrospectively reviewed all patients with NEC who had PPD as their initial surgical management over an 18-year period. A total of 122 babies with NEC were treated surgically, of whom 42 had PPD as the initial procedure. There were 28 survivors (67%) in the PPD group, of whom 7 recovered without laparotomy. Twenty-nine infants (69%) had a good clinical response to PPD with 80% (23/29) survival, compared to a 27% survival (3/11) in those who did not respond to drainage. Six patients underwent rescue laparotomy after a poor response to PPD and three of these survived. Six of the 28 pts who underwent laparotomy had isolated intestinal perforation and their clinical characteristics were no different from those with typical NEC. PPD is a useful option in the management of complicated NEC. It is difficult to recognise with certainty those infants who will not require a subsequent laparotomy and therefore we do not support the concept of PPD solely as a definitive strategy. The response to PPD is a good prognostic indicator for ultimate survival. Despite a low salvage rate of 27% in non-responders compared to 80% in responders, there is a role for early laparotomy for those infants who do not respond to PPD.     

儿童初治肝母细胞瘤83例临床特征及预后分析

目的探讨肝母细胞瘤(HB)患儿的临床特征、生存情况及预后危险因素。方法回顾性分析2012年1月至2019年10月郑州大学第一附属医院儿童医院血液肿瘤科收治的83例初治HB患儿的临床资料, 记录患儿的性别、年龄、首发临床表现、治疗前病变范围(PRETEXT)分期、病理类型、病初甲胎蛋白(AFP)、治疗方法及治疗结果。2018年之前确诊的患儿采用"武汉方案"治疗, 2018年之后确诊的患儿采用"儿童肝母细胞瘤多学科诊疗专家共识(CCCG-HB-2016)"方案治疗。Kaplan-Meier生存分析法计算生存率, 单因素分析采用Log-Rank检验, 多因素预后分析采用Cox回归模型。结果 83例患儿中男51例、女32例;发病年龄25.2(9.0, 34.0)月龄, <3岁64例(77%)。最常见的首发临床表现为腹部包块(45例, 54%)。PRETEXT Ⅰ期8例, Ⅱ期43例, Ⅲ期20例, Ⅳ期12例。随访时间40(17, 63)个月, 全组HB患儿1年总体生存率(OS)和无事件生存率(EFS)分别为(84±4)%和(79±5)%, 5年OS和EFS分别为(78±5)%和(76±...     

Outcome of children treated for cancer in the republic of Namibia

The data of a survey undertaken to record all cases of childhood cancer in Namibia from 1983 to 1988 were analysed to estimate 5-year survival rates. The projected survival rate for 150 children with cancer was 37% with no difference between boys and girls. The calculated survival rates for most of the tumour groups were poor with the exception of Wilms' tumour which had a 5-year survival rate of 76%. The zero survival rate of children with malignant bone disease may have been due to inadequate treatment. Neuroblastoma and retinoblastoma presented with advanced disease which contributed to the poor survival rates of 13% and 46%, respectively. The overall survival rate for lymphoma of 53% and 39% for all leukaemias compares poorly with the rates obtained in industrialised countries. The relatively poor 25% survival rate in tumours of the central nervous system (CNS) may partly be due to the long delay between the initial diagnosis and the institution of appropriate treatment for raised intracranial pressure and for the tumour. Both cure and long-term follow-up are difficult to achieve in a developing country. Improved early diagnosis and appropriate treatment are necessary to improve survival rates. © 1996 Wiley-Liss, Inc.     

Expression of myeloid differentiation antigens in acute nonlymphocytic leukemia: Increased concentration of CD33 antigen predicts poor outcome—A report from the childrens cancer study group

Ninety-eight cryopreserved specimens of acute nonlymphocytic leukemia (ANLL) cells obtained at initial diagnosis of children enrolled on the Childrens Cancer Study Group 251 protocol (CCG 251) were examined by indirect immunofluorescence using four monoclonal antibodies to myeloid differentiation antigens. The relationship between the level of differentiation of ANLL cells as determined by their antigen phenotype and the clinical outcome of treatment, including complete remission (CR) rate, survival, and event-free survival, was evaluated. Most leukemic specimens were determined to express the CD33 antigen (L4F3), a 67-kD protein. Because the level of differentiation of normal myeloid cells is reflected by the concentration of the CD33 antigen expressed, samples were categorized as CD33-bright (immature) versus CD33-dull (mature). Patients with CD33-bright leukemic blasts had a marginally inferior CR rate to those with CD33-dull blasts (P = 0.08). With respect to survival and event-free survival, there was a significantly inferior outcome in the CD33-bright patients (P = 0.04 and P = 0.06, respectively). Reactions of ANLL with anti-CD15 antibody (1G10), anti-CD36 antibody (5F1), or anti-CD17 antibody (T5A7) did not predict clinical outcome. This study indicates that patients whose ANLL blasts displayed the CD33 antigen in an amount associated with immature myeloid cells experienced a worse outcome than patients with ANLL blasts that expressed a phenotype associated with more mature cells. © 1992 Wiley-Liss, Inc.