心脏电生理变化早期检测蒽环类药物对血液/肿瘤病患儿的心脏毒性

目的 评价通过检测白血病、实体瘤患儿心脏电生理活动变化,早期发现蒽环类药物(ANT)对血液/肿瘤病患儿心脏毒性的可行性.方法 选择2003年8月-2009年3月在本院接受ANT治疗、疗程全部结束并达完全缓解的白血病、实体肿瘤患儿65例.男38例,女27例;年龄1～16(6±4)岁;发病年龄5个月～14岁[(4.0±4.5)岁].ANT疗程结束后随访时间为1～49(20.88±13.23)个月;ANT累积剂量为25～350(125.82±74.85) mg·m-2.分别检测患儿QTc间期及常规超声(2-DE),并与25例健康体检儿童(健康对照组)比较.患儿心率变异(HRV)与本院正常参考值比较.结果 患儿均无心功能不全临床表现.与健康对照组比较,常规2-DE指标无明显变化.QTc间期:65例患儿化疗前QTc均在正常范围,化疗后44例患儿QTc较化疗前延长,3例延长超过正常范围(＞440 ms).化疗后QTc较化疗前及健康对照组均明显延长.QTc延长与ANT剂量及随访时间无相关性.45例患儿与本院HRV正常参考值比较,24 h内全部正常窦性心动周期(N-N)的标准差、24 h全程相邻N-N间期之差的均方根值、24 h内相邻N-N间期差值＞50 ms个数占所有N-N间期个数的百分数等时域指标及极低频率、低频、高频等频域指标均明显下降(Pa＜0.05).ANT化疗后随访时间最长者及联合接受长春新碱剂量最大者HRV受累更明显.结论 ANT累积剂量25～350(125.82±74.85) mg·m-2,随访1～49(20.88±13.23)个月的患儿发生恶性心脏事件风险不大,但患儿心室复极化及HRV已受影响,属危险人群,需长期监测;相比常规2-DE指标,QTc、HRV可更敏感地反映亚临床心脏毒性;ANT化疗及联合大剂量VCR可加重化疗后心脏自主神经功能受损.     

小于3岁儿童弥漫性桥脑胶质瘤特点及临床疗效分析

目的 探讨小于3岁儿童弥漫性桥脑胶质瘤的影像学检查和病理学特点及治疗效果.方法 回顾性分析10例年龄小于3岁的儿童桥脑胶质瘤患儿临床表现、肿瘤部位、MRI影像学检查结果和肿瘤组织病理学特点,并以生存期为标准与3岁以上患儿疗效进行对比.结果 10 例确诊患儿的平均年龄为2.2岁(0.8～2.7岁).从出现症状到确诊的平均间间隔时间为2.5个月.所有患儿出现颅神经麻痹,其巾7例合并其他脑干的神经功能缺损症状.MRI检查显示所有患儿为桥脑肿瘤,占脑干肿瘤的50％以上.其巾MRI表现不典型的2例患儿已通过病理学检查证实其为桥脑肿瘤.所有患儿接受治疗,其中2例单纯放疗,6例放疗结合化疗,2例单纯化疗.4例患儿死于肿瘤进展,平均存活时间为0.7年(0.5～3.7年);余6例患儿平均生存时间为2.3年(0.9～8年).3岁以内患儿的3年整体存活率和肿瘤无进展率分别为45％±19％和69％±19％,均高于3岁以上患儿.结论 尽管使用相同的治疗方法,年龄小于3岁的脑干胶质瘤儿童较年长患儿预后好,推测可能与年幼患儿脑干胶质瘤病理学类型有关.     

噬血细胞综合征治疗后继发急性白血病2例报告并文献复习

目的探讨噬血细胞综合征(HLH)治疗后继发急性白血病的临床特征及预后。方法收集2例EB病毒相关HLH(EBV-HLH)患儿在接受化疗后继发急性白血病的临床资料,并复习相关文献。结果 2例患儿均为男性,分别为4岁、7岁,均接受HLH-2004方案化疗,依托泊苷(VP 16)的累积量分别为1 500 mg/m2及3 900 mg/m2;例1于首次化疗18个月后继发急性早幼粒细胞白血病(M3),予维甲酸加柔红霉素诱导化疗后达完全缓解(CR),此后规则化疗,随访30个月,持续CR;例2于首次化疗50个月后继发急性单核细胞白血病(M5),予化疗后达CR,但于诊断急性白血病15个月后复发,继予造血干细胞移植后再次达CR,此后随访1年,持续CR。2例患儿加文献报道13例HLH接受化疗后继发急性白血病患儿,共15例患儿,男10例、女5例,中位年龄2岁6个月(4个月～19岁),VP16的累积剂量中位数3 900 mg/m2(400～20 975 mg/m2),HLH与继发白血病的间隔中位时间24个月(6～72个月)。15例患儿中,5例M3,只接受化疗,均无病存活;10例其他类型急性白血病,3例接受化疗(1例死亡、2例不明),6例造血干细胞移植(3例存活、3例死亡);1例放弃治疗。结论 HLH治疗后继发的急性白血病多为急性髓系白血病,其中急性早幼粒细胞白血病接受维甲酸为基础的联合化疗,预后良好;而其他类型急性白血病预后差,造血干细胞移植或可改善预后。     

血液系统肿瘤化疗后卷发初步分析

目的报告6例急性血液系统肿瘤化疗后出现卷发，通过临床治疗观察初步分析其原因。方法2001年4月～2004年4月，对6例急性血液系统肿瘤化疗后出现卷发患儿进行家族调查及家谱分析，对患儿白血病类型、化疗药种类、接受化疗的时间进行总结。结果6例急性血液病肿瘤患儿均无家族遗传史，急性淋巴细胞白血病3例，急性非淋巴细胞白血病2例，霍奇金淋巴瘤1例。共同接受的化疗药物为大剂量环磷酰胺(CTX)，剂量为800mg／m^2。结论血液系统肿瘤化疗可以引起卷发，可能与大剂量CTX有关。     

《小儿急性白血病诊疗建议(修定草案)》的初步使用疗效观察

白血病是小儿常见恶性肿瘤，占儿科肿瘤发病的首位，严重危害儿童健康，化疗是目前治疗白血病的主要方法之一。近年来国内外对小儿白血病治疗已有了长足的进步。国内于1993年4月北海会议制定了统一的小儿白血病治疗方案「'。现将我院儿科血液组初步使用该方案治疗初发白血病疗效观察报告如下。材料与方法卫．病例来源全部病例64例均为我科血液组1996年4月～1997年10月收治的白血病患儿，其中急性淋巴细胞白血病（ALI。）50例，男36例，女14例，年龄2～13岁，平均年龄6．3岁，其中I。l型8例，IJ2型42例；高危ALI。16例，标危AI。L34例…     

急性淋巴细胞白血病患儿中可逆性脑病临床总结

目的探讨急性淋巴细胞白血病患儿化疗后可逆性脑病的临床和影像学特点。方法回顾分析2015年9月1日至2018年9月1日住院时发生脑病的急性淋巴细胞白血病患儿的临床资料。结果研究期间共收治新发急性淋巴细胞白血病582例,9例患儿发生10次可逆性脑病(1例患儿发生2次),其中男6例、女3例,脑病发生中位年龄6.55岁(3.9～12.5岁)。最常见的神经系统临床表现是抽搐,其次是肌无力和感觉异常。7例患儿曾接受培门冬治疗;5例患儿在脑病发生前有急性高血压病史;6例患儿在脑病发生时有低钠血症,部分有低纤维蛋白原血症。头颅磁共振成像检查均提示T1和T2信号异常,累及部位多见于顶枕叶。结论联合化疗、化疗药物鞘内注射和急性高血压是可逆性脑病发生的高危因素;监测血压、血钠、纤维蛋白原,以及头颅磁共振成像检查有助于早期发现可逆性脑病。     

31例急性白血病停药后生存质量的调查

经强烈化疗与正规方案的循环交替治疗儿童急性白血病 (ＡＬ) ,部分患儿可获得持续缓解和无病生存[1] 。现总结我院 1984～ 1992年收治的急性白血病 ,经连续化疗停药后3～ 10年以上的共 31例患儿的生存质量。报告如下。一般资料31例患儿 ,男 17例 ,女 14例。初治时年龄 16个月～ 10岁 (平均 4.5岁 ) ,随访年龄 :10～ 2 0岁 (平均 14.5岁 )。按ＦＡＢ急性白血病分类标准 :急性淋巴细胞白血病 (ＡＬＬ) 2 2例(ＳＲ 18例 ,ＨＲ 4例 ) ;急性非淋巴细胞白血病 (ＡＮＬＬ) 9例 (Ｍ11例 ,Ｍ2ｂ2例 ,Ｍ35例 ,Ｍ4 1例 )。疗程与停药时间ＡＬＬ及ＡＮＬ…     

急性淋巴细胞白血病患儿预防性头颅照射对生长发育的影响

急性淋巴细胞白血病(ALL)的疗效日趋提高,详尽研究本病及其治疗的远期疗效十分必要。本文对照研究急性淋巴细胞白血病患儿行预防性头颅照射与未行预防性头颅照射对生长发育的影响。材料与方法急性淋巴细胞白血病患儿39例,女20例,男19例,平均发病年龄3.8岁(1.1～7.2岁),根据预防中枢神经系统病变的方法不同分为两组:头颅照射组,28例,平均发病年龄3.9岁(1.1～7.2岁),头颅照射量为24Gy;非头颅照射组,11例,平均发病年龄3.6岁(1.1～5.9岁),给予氨甲喋呤静注(500mg/m~2)和甲酰四氢叶酸治疗。两组均接受过氨甲喋呤鞘内注射。其他治疗如诱导治疗期间用强的松龙、阿霉素,长春新碱,维持治疗期间用6-巯基嘌     

肿瘤

890275 急性粒细胞白血病持续完全缓解9年1例报告/徐淑珍∥实用儿科杂志。-1988,3(4)。-184 患儿男,10岁。1978年12月26日入院。确诊后采用COAP方案化疗:疗程第1天长春新硷1mg静注;第2～5天阿糖胞苷75mg每12小时静注1次,环磷酰胺120 mg/日静注;从化疗开始给予强的松45mg/日,连用5个月;     

急性白血病儿童医院感染71例

目的 了解儿童白血病发生医院感染的临床特点,探讨其防治措施.方法 回顾性分析133例急性白血病患儿发生医院感染的特点.总结医院感染与病程阶段、住院天数的关系及感染部位、感染率,并进行统计学处理.结果 医院感染率为53.4%(71/133例).其中急性淋巴细胞白血病与急性非淋巴细胞白血病例次感染率有显著性差异(P＜0.05);住院第1～3次组医院感染例次高于其他组(Pa＜0.05),住院天数为1～7 d组医院感染例次低于其他组(Pa＜0.05);复发与未复发组人均医院感染例次无显著性差异(P＜0.05);败血症以革兰阴性杆菌为主,药敏结果显示普遍敏感的药物为阿米卡星、哌拉西林/他佐巴坦.结论 急性白血病患儿医院感染发生率高,医院感染发生与白血病类型、病程阶段、住院天数有关;与预后无关,败血症以革兰阴性杆菌为主.     

Spontaneous growth in idiopathic short stature. European Study Group.

Documenting the spontaneous growth pattern of children with idiopathic short stature (ISS) should be helpful in evaluating the effects of growth promoting treatments. Growth curves for children with ISS were constructed, based on 229 untreated children (145 boys and 84 girls) from nine European countries. The children were subdivided according to target range and onset of puberty, and the growth of these subgroups was evaluated from standard deviation scores (SDS). At birth, children with ISS were already shorter than normal (means; boys -0.8 SDS, girls -1.3 SDS). Height slowly decreased from -1.7 SDS at the age of 2 years to -2.7 SDS at the age of 16 years in boys and 13 years in girls. Final height was -1.5 SDS in boys and -1.6 SDS in girls (mean (SD): boys 164.8 (6.1) cm, girls 152.7 (5.3) cm)), which was 5-6 cm below their target height. The onset of puberty was delayed (boys 13.8 (1.3) years, girls 12.9 (1.1) years). Subclassification resulted in similar growth curves. These specific growth data may be more suitable for evaluating the effects of growth promoting treatments than population based references.     

Spontaneous growth in idiopathic short stature. European Study Group

Documenting the spontaneous growth pattern of children with idiopathic short stature (ISS) should be helpful in evaluating the effects of growth promoting treatments. Growth curves for children with ISS were constructed, based on 229 untreated children (145 boys and 84 girls) from nine European countries. The children were subdivided according to target range and onset of puberty, and the growth of these subgroups was evaluated from standard deviation scores (SDS). At birth, children with ISS were already shorter than normal (means; boys -0.8 SDS, girls -1.3 SDS). Height slowly decreased from -1.7 SDS at the age of 2 years to -2.7 SDS at the age of 16 years in boys and 13 years in girls. Final height was -1.5 SDS in boys and -1.6 SDS in girls (mean (SD): boys 164.8 (6.1) cm, girls 152.7 (5.3) cm)), which was 5-6 cm below their target height. The onset of puberty was delayed (boys 13.8 (1.3) years, girls 12.9 (1.1) years). Subclassification resulted in similar growth curves. These specific growth data may be more suitable for evaluating the effects of growth promoting treatments than population based references.     

Iron Deficiency Anemia in Children Presenting with Lymphoreticular Malignancies and the Effect of Induction Therapy

There is scant information regarding iron deficiency in children with malignant disorders. Serum iron status of children with lymphoreticular malignancies (LRMs) at onset and at the end of induction therapy, compared to the normal population, was evaluated. Prospective cohort study conducted between July 2002 and March 2004. Previously untreated children recently diagnosed with LRM were studied. Age-matched controls were enrolled from follow-up and growth monitoring clinics. Hematological (complete blood counts and red cell indices) parameters and markers of iron status (serum iron, serum ferritin, total iron binding capacity) were estimated at presentation and at the end of remission induction therapy, that is, 5 weeks after initial evaluation. Bone marrow iron store were only assessed in cases. Thirty-five children (31 with acute lymphoid leukemia, 2 with acute myeloid leukemia, and 2 with non-Hodgkin lymphoma; 27 boys and 8 girls; 2 to 12 years of age) were evaluated in the study cohort. Anemia was documented in 80% of children with LRM. Iron deficiency was an important etiological factor. In the majority of cases therapy resulted in significant improvement towards normalization of deranged hematological parameters. This phenomenon could be attributed to enhanced quantity and quality of erythropoietic activity and red cell transfusions. The observation suggests that therapeutic iron supplements are not indicated in the majority of children on therapy for malignant disorders. Various hematological and body iron status parameters should be assessed on a case-by-case basis.     

Iron deficiency anemia in children presenting with lymphoreticular malignancies and the effect of induction therapy

There is scant information regarding iron deficiency in children with malignant disorders. Serum iron status of children with lymphoreticular malignancies (LRMs) at onset and at the end of induction therapy, compared to the normal population, was evaluated. Prospective cohort study conducted between July 2002 and March 2004. Previously untreated children recently diagnosed with LRM were studied. Age-matched controls were enrolled from follow-up and growth monitoring clinics. Hematological (complete blood counts and red cell indices) parameters and markers of iron status (serum iron, serum ferritin, total iron binding capacity) were estimated at presentation and at the end of remission induction therapy, that is, 5 weeks after initial evaluation. Bone marrow iron store were only assessed in cases. Thirty-five children (31 with acute lymphoid leukemia, 2 with acute myeloid leukemia, and 2 with non-Hodgkin lymphoma; 27 boys and 8 girls; 2 to 12 years of age) were evaluated in the study cohort. Anemia was documented in 80% of children with LRM. Iron deficiency was an important etiological factor. In the majority of cases therapy resulted in significant improvement towards normalization of deranged hematological parameters. This phenomenon could be attributed to enhanced quantity and quality of erythropoietic activity and red cell transfusions. The observation suggests that therapeutic iron supplements are not indicated in the majority of children on therapy for malignant disorders. Various hematological and body iron status parameters should be assessed on a case-by-case basis.     

When and how to combine growth hormone with a luteinizing hormone-releasing hormone analogue

The effect of combined treatment with growth hormone (GH) and a luteinizing hormone-releasing hormone (LHRH) analogue, or GH alone, on pubertal height gain was assessed in an uncontrolled study in 15 boys and 10 girls with GH deficiency (GHD). Seven boys and six girls were treated with GH alone (group 1), and eight boys and four girls were treated with a combination of GH and an LHRH analogue during puberty (group 2). Mean ages (+/- SD) at the start of GH treatment and at the onset of puberty were significantly lower in group 2 (8.0 +/- 3.3 years and 11.2 +/- 0.8 years, respectively, in boys, and 6.3 +/- 1.6 years and 10.8 +/- 0.7 years in girls) than in group 1 (12.8 +/- 1.9 years and 13.7 +/- 1.4 years in boys, and 11.2 +/- 1.0 years and 12.5 +/- 1.2 years in girls). Height at the onset of puberty was less in group 2 than in group 1, but the difference was significant only for the boys. Combination treatment was started at a mean age of 11.7 +/- 1.2 years in boys and 11.5 +/- 1.0 years in girls. The duration of the combination treatment was 5.1 +/- 1.5 years in boys and 2.3 +/- 0.7 years in girls. The duration of the period between the onset of puberty and the end of GH treatment was significantly longer in group 2 (6.8 +/- 1.2 years in boys and 5.5 +/- 1.0 years in girls) than in group 1 (4.3 +/- 1.6 years in boys and 3.6 +/- 1.4 years in girls). The pubertal height gain was also significantly greater in group 2 (36.7 +/- 6.5 cm in boys and 29.0 +/- 8.3 cm in girls) than in group 1 (21.9 +/- 4.1 cm in boys and 18.6 +/- 4.1 cm in girls). Final height was significantly greater in group 2 than in group 1 in boys. Although there was no significant difference in final height between groups in the girls, the change in height SDS from the start of GH treatment until final height was significantly greater in group 2 (2.7 +/- 1.6 in boys and 4.5 +/- 0.5 SD in girls) than in group 1 (1.0 +/- 0.8 in boys and 1.8 +/- 0.9 SD in girls), in both boys and girls. In conclusion, it appears that combination of an LHRH analogue and GH may increase the pubertal height gain and the final height of children with GHD. The improvement is attributed to the prolongation of the treatment period, permitting slow bone maturation, and to the maintenance of height velocity. This combination treatment appears to be more effective in boys than girls. To fully assess this therapeutic approach, prospective controlled studies are needed.     

Juvenile chronic myelocytic leukemia—report of 10 cases

Ten children (five boys and five girls) with juvenile chronic myelocytic leukemia were seen over a period of 12 years (1980–1991) at the All India Institute of Medical Sciences, New Delhi. With the exception of one who was aged 4.5 years, all children were below 4 years of age (mean age 20.4 months). The presenting features included fever, bleeding secondary to thrombocytopenia, marked hepatosplenomegaly, and skin rash. The striking hematological features were anemia, thrombocytopenia, peripheral blood monocytosis, and normoblastemia. There was no significant myeloid proliferation in the bone marrow aspirate (mean M:E = 5:1), while erythroid proliferation was prominent along with monocytosis (mean 11.2%). Fetal hemoglobin was raised in 8 of the 10 patients (mean 14.1%). Long-term survival was poor, with maximum survival being 18 months in one case. New modalities of management of this rare entity are discussed. © 1995 Wiley-Liss, Inc.     

INCIDENCE OF CHILDHOOD LEUKEMIA IN SWEDEN 1975-1980

ABSTRACT. During the six-year period 1975-1980, leukemia was diagnosed in 466 children in Sweden, giving an estimated incidence of 4.4/100000 children per year (0-15 years at diagnosis). The incidence of acute lymphoblastic leukemia (ALL) was 3.7, of acute nonlymphocytic leukemia (ANLL) 0.6 and of chronic myelocytic leukemia (CML) 0.1/100000 children per year. The over all incidence among boys was 4.5/100000 per year and among girls 4.2. The male:female ratio was 1.13. This ratio was 1.22 in ALL and 0.71 in ANLL. Fifty per cent of the children were below 5 years of age at diagnosis, with a pronounced peak between 2-3 years, which was explained by the ALL distribution. In children with acute leukemia 13% had WBC values of >100×10⁹l, 4% had CNS leukemia and 10% had a mediastinal mass at diagnosis. The geographical distribution of leukemia in Sweden was analysed in a search for clusters of cases.     

新型冠状病毒疫情下多中心517例血液肿瘤患儿的家庭管理

目的了解新型冠状病毒疫情期间血液肿瘤患儿的家庭管理方法。方法回顾性总结疫情期间在多中心儿童血液肿瘤科就诊的517例血液肿瘤患儿的临床资料以及家庭管理情况。结果517例血液肿瘤患儿中,男298例,女219例,中位年龄5岁4个月。急性淋巴细胞白血病260例(50.29%),急性髓系白血病36例(6.96%),重型再生障碍性贫血40例(7.74%),其他(包括淋巴瘤、神经母细胞瘤、肾母细胞瘤等实体瘤)181例(35.0%)。其中发热61例,咳嗽31例,肺部CT异常表现77例,门诊进行了新型冠状病毒核酸及抗体筛查的患儿结果均为阴性,患儿在日常居家、外出就诊途中及居家网络化管理中均采取了合理的管理方式。结论多中心儿童血液肿瘤患儿COVID-19感染情况较为乐观,合理的家庭管理让血液肿瘤患儿能够较好地避免院外感染COVID-19。     

Precocious and premature puberty associated with treatment of acute lymphoblastic leukaemia.

Early puberty in 28 children (23 girls, five boys) treated for acute lymphoblastic leukaemia (ALL) at a mean age of 4.0 years (range 1.4-7.8) is described. All but one had received prophylactic cranial irradiation (1800-2400 cGy) and three children had received additional cranial or craniospinal irradiation as treatment for relapse of their leukaemia. Mean age for the onset of puberty was 8.8 (SD 0.8) years in the girls and 9.3 (0.8) years in the boys; this is greater than two standard deviations from the mean for normal girls and boys. Five children (three girls, two boys) had precocious puberty. The onset of puberty occurred at greater than two standard deviations from the mean for normal girls and boys in 14(13%) girls and 4(3%) boys treated at less than eight years of age between 1970 and 1985. In a group of 55 girls treated for ALL who had survived in first remission for six years or more from diagnosis, there was a relation between young age at onset of treatment and early menarche. We suggest that premature activation of the hypothalamic-pituitary-gonadal axis occurs as a consequence of hypothalamic dysfunction due to cranial irradiation. Precocious and premature puberty in children treated for ALL may be an important factor in contributing to short stature.     

The incidence of diabetes mellitus in Swedish children 0-14 years of age. A prospective study 1977-1980

This is a prospective study of the incidence of insulin-dependent diabetes mellitus (IDDM) in children 0-14 years of age, including all newly diagnosed cases in the whole of Sweden from July 1, 1977 until June 30, 1980. All 45 Swedish departments of paediatrics participated. During the three-year-period studied, 1108 Swedish children, 0-14 years of age had their onset of diabetes. That means around 369 new diabetics yearly in the age groups studied. The mean yearly incidences in the years 1977-80 were 22.6, 22.8 and 22.6 per 100000 children, respectively. Mean prevalence on June 30, 1980 and 1.48 per 1000 children 0-14 years with a wide range of 0.71-2.65. The age distribution at onset showed a gradual increase and peak incidences at 11 years of age for the girls and 4 and 13 years of age for the boys. There was a consistently higher incidence for boys in the younger age groups during the three-year-period studied. Peak incidences of new cases were reached in January, March and July through October for the age groups 5-9 and 10-14 years of age. No such seasonal variation was seen for children 0-4 years of age. The cumulative incidence of IDDM at 14 years of age was 3.2 per 1000 for the boys and 2.9 per 1000 for the girls. The degree of ascertainment in this study was 93.4%.