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??Abstracts?? Neonatal alloimmune thrombocytopenia??NAIT??is a leading cause of neonatalthrombocytopenia andone of the main reasons for thedisease and full-term infantswith intracranial hemorrhage.This paperisfocusedon the NAIT pathogenesis??clinical features??auxiliary examination??diagnosis and intervention and treatment.     

Repeated antenatal intracranial haemorrhage: magnetic resonance imaging in a fetus with alloimmune thrombocytopenia

Magnetic resonance imaging was used to show repeated antenatal intracranial haemorrhage in a fetus with alloimmune thrombocytopenia.     

Follow up care of bereaved parents after treatment withdrawal from newborns

OBJECTIVE: To explore parents' experiences of bereavement care after withdrawal of newborn intensive care. DESIGN: Face to face interviews with 108 parents of 62 babies born over two calendar years in the East of Scotland. RESULTS: Only 22% were seen by six weeks after the death, 10% were not recalled before 6-11 months, and 8% were not seen at all in the first year. All except one couple saw the neonatologist who had cared for their baby. Only 33% remembered a neonatal nurse being present. Most (88%) were seen in the study hospital. Parents highlighted a number of specific needs. Appointments should be: (a) scheduled soon after the death of the baby and certainly within two months of the death irrespective of whether or not autopsy results are available; (b) with the named neonatologist; (c) in a setting away from the hospital if possible. Parents value: (a) efforts to find out how they are coping; (b) full frank information given sensitively to enable them to build up a cohesive picture of what happened and assess their future risks; (c) reassurance where possible, but half truths, false reassurances, and broken promises are unacceptable. CONCLUSIONS: Follow up care is a crucial part of the management of families from whose babies treatment has been withdrawn. Resources devoted to it should be re-examined to provide a service more in tune with parental need. In choosing the place, timing, and conduct of the meeting, staff should be sensitive to the expressed wishes of the parents themselves.     

Review of neonatal alloimmune thrombocytopenia

Neonatal alloimmune thrombocytopenia (NAIT), with an incidence of one in 1000 live births, is the most common cause of severe thrombocytopenia and intra-cerebral haemorrhage in term neonates. NAIT results from trans-placental passage of maternal antibodies against a paternally derived fetal platelet alloantigen. Clinical presentation varies from unexpected thrombocytopenia on a blood film in a well newborn to intracranial haemorrhage (ICH). In contrast to haemolytic disease of the newborn, NAIT can present in a first pregnancy, and subsequent pregnancies are usually more severely affected. The role of antenatal screening for maternal alloantibodies instead of fetal blood sampling to identify at-risk fetuses remains uncertain, but there is a trend towards less invasive maternally directed treatment for at-risk pregnancies. Neonatal management is aimed at preventing or limiting thrombocytopenic bleeding with transfusion of antigen-matched platelets.     

Follow up of survival and quality of life in children after intensive care

OBJECTIVE:To evaluate the health related quality of life of children after intensive care and to assess their long term survival. DESIGN: Prospective. Setting: Tertiary Care Hospital Intensive Care Unit (ICU). SUBJECT AND METHODS: All patients admitted to ICU were enrolled prospectively over a period of 1 year. Children with ICU stay of less than 24 hrs, infants, readmission to ICU were however excluded. Survival was determined at the time of ICU discharge and 1 year later. Health status assessment was done with the help of the multiattribute health status classification (MAHSC), which has 6 domains; sensation, mobility, emotion, cognition, self care and pain. Assessment was done at two points of time - within 48 hours of admission to the ICU and 1 year after discharge. RESULTS: 150 children (mean age 5.68+/- 3.6 years) with a mean duration of ICU stay (5.7 +/- 5.5 days) were included in the study. The cumulative ICU mortality was 12.9%. Fifty-five (36.7% had no overall health impairment (no affected domains) preceding the present illness. There was overall health impairment (?1 affected domain) preceding the present illness in 95 of the 150 patients (63.3%). In the domain specific health status mobility was affected in 74 (49%) followed by pain 61 (41.2%), self care 56 (38.8%), sensation 29 (20%), cognition 21 (14.8%) and emotion 14(9.5%). After 1 year, overall state of health had improved or was equal to the premorbid state in 87 (75%). In domain specific health, the proportion improving or remaining unchanged varied from 75% (emotional) to 80% (cognition), 85.3% (pain) and 88.7% (mobility). The overall state of health had worsened as compared to the premorbid state in 29 (25%) majority with neurological illnesses. CONCLUSION: Quality of life in three-quarters of the patients was preserved and one year survival was favorable. Worsening was noted primarily in-patients with neurological illnesses.     

High-dose intravenous gammaglobulin for neonatal alloimmune thrombocytopenia in twins

We report the successful treatment of neonatal alloimmune thrombocytopenia with repeated infusions of high-dose immunoglobulin G (400 mg/kg/d for 5 days) in twins. Platelet counts increased within 3 days from less than 20 x 10(9)/l to more than 70 x 10(9)/l. The first twin survived without neurological or other sequelae. The second twin had probably developed intracranial hemorrhage (ICH) in utero. This infant developed long-term neurological sequelae with blindness, cerebral palsy and infantile spasms. Implications of the therapeutic approach and prevention of severe complications in pregnancies with known risk for neonatal alloimmune thrombocytopenia are discussed.     

左乙拉西坦单药治疗小儿癫癎的疗效和安全性随访研究

目的：评价左乙拉西坦(LEV)单药治疗小儿癫癎的疗效和安全性。方法：对该院2007年3月至2008年3月LEV单药治疗的32例癫癎患儿进行开放性自身对照随访研究,起始量每日10 mg/kg,每1周增加上述剂量1次,3~4周增加至维持剂量每日20～60 mg/kg,平均剂量为每日35 mg/kg。结果：LEV 单药治疗32例,失访1例,其余随访均在3个月以上。 80.6%（25/31）患儿发作减少≥50%,70.9%（22/31）患儿无发作,16.1%（5/31）患儿因发作控制不满意或者加重而停药。不良反应包括情绪异常19.4%（6/31）,乏力6.5%（2/31）,嗜睡6.5%（2/31）,皮疹3.2%（1/31）。上述不良反应均为一过性,在1～4 周内自然消失或者减量后消失,未导致停药,未发现过敏以及血液、肝、肾功能异常等严重不良反应。结论：LEV用于癫癎患儿（包括<4岁的婴幼儿）的部分性发作及全身性发作的单药治疗,疗效肯定、安全性好,是一个很有希望的用于儿童单药治疗的广谱抗癫癎药。     

Clinical and diagnostic comparison of neonatal alloimmune thrombocytopenia to non-immune cases of thrombocytopenia

BACKGROUND: Affected patients with neonatal alloimmune thrombocytopenia (AIT) are often severely thrombocytopenic and, if so, may suffer an intracranial hemorrhage (ICH). This study was undertaken to compare the outcome of cases of AIT to cases of neonatal thrombocytopenia shown not to be AIT and to identify clinical features that would facilitate the diagnosis. PROCEDURE: Two hundred twenty two cases of neonatal thrombocytopenia for which serologic testing was obtained by the referring physician were accrued for this study from 11 testing laboratories. The relevant clinical information was pursued. RESULTS: The mean birth platelet count in 110 neonates with AIT was 26,000/mm(3) x 10(9)/L and the rate of ICH was 11% (not all neonates had head sonos). Three criteria distinguished cases of AIT from other causes of neonatal thrombocytopenia (n = 56): (1) severe thrombocytopenia <50,000/mm(3) x 10(9)/L; (2) ICH associated with 1 or more of: a 1-min Apgar score >5, birthweight >2,200 g, grade >1, antenatal occurrence, or signs of bleeding, that is, petechiae, ecchymoses; and (3) no additional, non-hemorrhagic neonatal medical problems. CONCLUSIONS: AIT is a unique type of neonatal thrombocytopenia with significant hemorrhagic consequences. Identification of AIT at the bedside should guide institution of appropriate treatment and lead to serologic testing for confirmation.     

Follow up of long-term survivors after childhood cancer in Germany

BACKGROUND: In recent years, the long-term survival of childhood cancer patients has increased considerably. While this is desirable, more patients with late effects are to be expected and studies thereof become increasingly important. We will need to be able to stay in touch with as many former patients as possible in order to make a systematic and comprehensive long-term follow-up possible. PATIENTS: Childhood cancer patients under 15 years of age at diagnosis resident in Germany and registered at the German Childhood Cancer Registry (GCCR). METHODS: The GCCR has established a 3-phase procedure for follow-up. We developed principles for the long-term follow up of these patients. They are based on the many years of experience at the GCCR and were developed based on the long-standing collaboration between the therapy optimization studies (TOS) in the Society for Paediatric Oncology and Haematology (GPOH) and the GCCR. RESULTS: Currently 8 012 adult survivors diagnosed before 2000 are under observation at the GCCR and could be contacted for studies. About half of the children diagnosed in the 1980ies still in follow-up have been under observation more than 14 years. When asked to personally extend the originally parental consent to data storage at the GCCR and the TOS at about 16 years of age, about 85 % of the patients agree. CONCLUSIONS: Establishing an open-end, systematic long-term follow-up will provide a unique and broad basis for paediatric oncology in Germany to perform representative studies regarding long-term survival after childhood cancer in Germany in the long run.     

Follow up study eight years after diagnosis of sexual abuse

AIM—To study the outcome over an eight year period of children determined by paediatricians in 1989 as definitely or probably sexually abused.METHOD—Information was obtained on 140 of 148 children diagnosed in 1989 when aged 7 or less. Sources were hospital medical records and school health records. School health records of a comparison group of 83 children were also examined.RESULTS—A variety of problematic characteristics were found significantly more often in the abused group than the comparison group. These included surname changes (30% v 2%), removal from home (25% v 1%), number of home addresses (2.8 v 1.4), and schools attended (3.4 v 2.2). Other significant findings included further abuse (35% v 0%), adverse behaviours (60% v 16%), educational problems (24% v 5%), chronic health problems (54% v 36%), and involvement of mental health services (32% v 1%).     

Follow up study eight years after diagnosis of sexual abuse.

AIM: To study the outcome over an eight year period of children determined by paediatricians in 1989 as definitely or probably sexually abused. METHOD: Information was obtained on 140 of 148 children diagnosed in 1989 when aged 7 or less. Sources were hospital medical records and school health records. School health records of a comparison group of 83 children were also examined. RESULTS: A variety of problematic characteristics were found significantly more often in the abused group than the comparison group. These included surname changes (30% v 2%), removal from home (25% v 1%), number of home addresses (2.8 v 1.4), and schools attended (3.4 v 2.2). Other significant findings included further abuse (35% v 0%), adverse behaviours (60% v 16%), educational problems (24% v 5%), chronic health problems (54% v 36%), and involvement of mental health services (32% v 1%).     

Intravenous human immunoglobulin treatment for neonatal alloimmune thrombocytopenia due to anti-HPA-5b with severe previous history]

Fetal and neonatal alloimmune thrombocytopenia due to mothers' anti-HPA-5b alloimmunization has generally a milder clinical presentation compared to anti-HPA-1a alloimmunization. Nevertheless, a case with infant's death probably due to intracranial haemorrhage has been reported. However, if platelet-specific alloimmunized mothers with prior fetal or neonate injury receive intravenous immunoglobulins during pregnancy, thrombocytopenia in heterozygous fetus and neonate may be prevented. Here are reported 2 cases of anti-HPA-5b fetal-maternal alloimmunization, one with prior fetal death, the other with prior severe fetal intracranial haemorrhage, which were successfully treated with intraveinous immunoglobulins alone during a second pregnancy with HPA-5b incompatibility.     

艾曲波帕治疗儿童造血干细胞移植后血小板减少症24例分析

目的评价艾曲波帕治疗儿童造血干细胞移植(HSCT)后血小板减少症的疗效与安全性。方法回顾性分析2018年8月1日至2019年4月1日于中山大学孙逸仙纪念医院儿科接受HSCT后发生血小板减少症并采用艾曲波帕治疗的24例患儿的临床资料,评估治疗有效率及不良反应,根据造血干细胞来源分为脐带血组和外周干细胞组,根据疾病类型分为恶性病组和非恶性病组,分析各组间临床疗效。组间比较采用秩和检验。结果 24例患儿中男15例、女9例,艾曲波帕用药时的年龄7.7(2.6~13.7)岁,接受艾曲波帕治疗的时间为移植后第27.5(8.0~125.0) d,使用后完全有效的时间为23.5(6.0~83.0) d,用药疗程为36.5(8.0~90.0) d,艾曲波帕总剂量为1 400(200~5 900)mg,完全有效率92%(22/24),未发生艾曲波帕相关不良反应。脐带血干细胞移植(16例)的患儿使用艾曲波帕的疗程及总剂量均明显低于外周干细胞移植组(8例)[24.5(8.0~81.0)比65.5(35.0~90.0)d,900.0(200.0~3 850.0)比2 862.5 (1 175.0~5 900.0) mg,Z=-3.004、-2.604,P=0.002、0.007],而达到完全有效的时间及停药2周后血小板计数、随访终点血小板计数的差异均无统计学意义(均P>0.05)。与恶性病患儿(12例)相比,非恶性病患儿(12例)用药后至获得完全有效的时间、用药疗程、总剂量及停药2周后血小板计数、随访终点血小板计数的差异均无统计学意义(均P>0.05)。结论艾曲波帕用于儿童HSCT后血小板减少症有一定疗效,安全性高,尤其在脐带血移植中可能更有优势。     

Necrotizing enterocolitis after intravenous immunoglobulin treatment for neonatal isoimmune thrombocytopenia

A male neonate of 38 weeks' gestation with isoimmune neonatal thrombocytopenia treated with high dose intravenous immunoglobulin (IVIG) developed necrotizing enterocolitis (NEC) at 3 days of age. The known maternal and neonatal risk factors for the development of this disease were excluded. The association between high dose IVIG and the appearance of thrombotic events might be another aetiological factor for occurrence of NEC in a newborn infant.     

Follow up of low birth weight infants

Conclusion Considerable reduction in mortality of low birth weight infants over the past two decades has been achieved in developed countries without an increase in the prevalence of major h andicaps in the survivors. In the 1980’s overall prognosis for infants above 800 gm birth weight remains good. Continued efforts for improved perinatal and neonatal care can be expected to further reduce the risk of premature birth. Worldwise effort for improved care of the pregnant women and the newborn can be expected to result in similar advances for infant survival and improved child health.