Bearbeitung und Transplantation hämatopoetischer Stammzellen

Background

New graft manipulation procedures make it possible to obtain highly purified hematopoietic stem cells from healthy donors.

Patients and Methods

A total of 116 children with leukemias, lymphomas, and nonmalignant diseases received purified (97%) peripheral stem cells from matched unrelated donors or mismatched related (haploidentical) donors.

Results

Acute and chronic GVHD were vastly minimized and a high overall rate of engraftment was achieved. Event-free survival at 5 years was 46% after haploidentical transplantation for children with ALL in remission who otherwise lacked a matched donor. Moreover, excellent results were observed in patients with nonmalignant diseases.

Conclusions

Graft manipulation results in well-characterized transplants with minimal risk of GVHD and makes a donor available for each patient by recruiting the parents. Thus, alternative donors should be strongly considered in all patients who need a stem cell transplantation but lack an identical donor.     

Enteropathisches h?molytisch-ur?misches Syndrom

Hemolytic uremic syndrome (HUS) is a life-threatening condition characterized by acute renal failure, hemolytic anemia and thrombocytopenia. More than 80% of pediatric HUS is caused by infection with enterohemorrhagic Escherichia coli (EHEC). EHEC-associated HUS mainly affects children under six years of age. HUS patients should be treated in specialized clinics, which are able to provide dialysis. Importantly, sporadic cases of HUS can signalize outbreaks of EHEC infections. In Germany, a rare sorbitol-fermenting variant of EHEC?O157 caused several large HUS outbreaks with fatal cases. Physicians are required to notify every suspected, confirmed or fatal case of HUS to their local health department, thereby enabling public health authorities to intervene promptly. This article describes the epidemic aspects of EHEC-associated HUS and assists physicians and pediatricians in terms of timely diagnosis and notification.     

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??Objective To discuss the changes of ambulatory blood pressure monitoring??ABPM?? in children with vasovagal syncope ??VVS??.Methods A total of 72 children ??VVS group??32 males??40 females??mean age ??10.98±1.86??years?? were enrolled in this study??who came from Children Syncope Outpatient Department or Inpatient Department of the Second Xiangya Hospital of Central South University from Sep 2008 to Feb 2010.After confirmed diagnosis of VVS by positive head-up tilt test ??HUTT????each patient was given ABPM.Forty healthy children ??19 males??21 females??mean age 10.24 ± 2.01 years?? were as controls ??control group??.Parameters of ABPM in children were analyzed.SPSS 17.0 software was used for the statistical analysis of these data.Results ??1??Mean pressure??24-hour mean systolic pressure ??24hSBP????24-hour mean diastolic pressure ??24hDBP????daytime mean systolic pressure ??DSBP?? and nighttime mean systolic pressure ??NSBP?? in VVS group were higher than those of the control group ??P??0.05??.??2??Blood pressure pattern??the ratio of non-spoon pressure pattern was higher than that of spoon pressure pattern with VVS ??67%vs.18%??P ??0.05??.NDBP ??P??0.05?? and NSBP ??P ??0.05?? were decreased and the systolic pressure differences and diastolic pressure differences in the daytime and at nighttime were higher in spoon pressure pattern than those of non-spoon pressure pattern with VVS ??P??0.05??.??3??Diagnostic experimental evaluation??the specificity??sensitivity??diagnostic coincidence??positive predict value and negative predict value of non-spoon pattern of ambulatory blood pressure pattern to VVS was 82.5%??66.67%??72.32%??87.27% and 57.89%??respectively.The Youden index was 45.28%.Conclusion There is autonomic nerve adjustment imbalance in children with VVS during asymptomatic time periods??especially the sympathetic nerve.The non-spoon pattern rate of fluctuation curve of ambulatory blood pressure in VVS children is higher??which is of diagnosis value to VVS children.     

Hypospadie—häufigste Fehlbildung des Knaben

Zusammenfassung  Die Hypospadia penis zeichnet sich durch eine proximal dystope Harnröhrenmündung, eine ventrale Penisschaftdeviation und eine dorsale Präputiumschürze aus. Sie ist die häufigste angeborene Fehlbildung des Knaben mit einer Inzidenz zwischen 4,7 und 8 auf 1000 Lebendgeburten. Je nach Lage des Meatus urethrae wird die Hypospadie in eine anteriore (distale), eine mittlere und eine posteriore (proximale) Form unterteilt, mit zunehmend aufwändigerer operativer Rekonstruktion und höherer Komplikationsrate. Ziel der Korrektur sind ein orthotoper Neomeatus, ein gerader Penisschaft mit nach vorne gerichtetem Harnstrahl und eine normale Sexualfunktion. Die Korrektur ist heute sehr viel früher möglich und reicht weit in das erste Lebensjahr hinein. Prinzipiell unterscheidet man einzeitige und zweizeitige Korrekturtechniken, wobei Erstere favorisiert werden. Die Komplikationsrate ist je nach Schweregrad und Technik sehr unterschiedlich, Urethralfistel und Meatusstenose dominieren. Die soziokulturelle sowie die sexuelle Entwicklung dieser Knaben verlaufen nach erfolgter Korrektur ungestört.     

Akutes hämorrhagisches Ödem des Kleinkindes

Acute hemorrhagic edema of infancy (AHEI; synonyms Finkelstein disease, idiopathic Seidlmayer’s purpura), which occurs mainly in children under 2 years of age, was for a long time considered to be a rare form of Henoch–Schönlein purpura. Meanwhile, this form of leukocytoclastic vasculitis is a separate entity in the literature, with the classic triad consisting of fever, edema, and rosette-like or circular purpura. The disease is self-limiting; however, serious differential diagnoses such as Waterhouse–Fridrichsen syndrome should certainly be excluded. Blood count and coagulation parameters are not changed. Attention should be paid to rare but acute complications.     

Untersuchungen zum Inhibitor-Effekt in hämophilen Plasmen

Zusammenfassung Es wurden die Plasmen und Seren von insgesamt 21 Hämophilen (12 der Gruppe A und 9 der Gruppe B) auf einen Inhibitoreffekt des Hämophilieplasmas untersucht. Während nach Ätherbehandlung des Plasmas bei der Hämophilie A keine Steigerung der Plasmathrombokinaseaktivität gesehen wurde, war diese nach Behandlung von Hämophilie B-Plasma mit Äther stets nachweisbar. Die Steigerung der Aktivität lag außerhalb der Streubreite der Methode. Diskutiert wird, ob der Defekt bei der Hämophilie B auf eine ätherlösliche Hemmsubstanz zurückgeführt werden kann.     

Wie häufig werden hyaline Membranen überlebt?

Zusammenfassung Hyaline Membranen sind eine häufige Todesursache Frühgeborener innerhalb des Trihemeron. Bisher wurde noch nicht untersucht, ob und wie häufig sie überlebt werden.Bei 44 Frühgeborenen, die in der Zeit vom 3.–30. Lebenstag starben, fanden wir 9mal in Auflösung begriffene hyaline Membranen sowie Alveolarwandverquellungen unterschiedlicher Stärke und Ausdehnung. Die Restzustände disponieren nicht zu sekundären Lungenaffektionen.Beim Vergleich mit dem klinischen Bild läßt sich zeigen, daß auch ausgedehnte hyaline Membranen symptomlos bleiben können. Entscheidend für das klinische Manifestwerden und Überleben ist die Geschwindigkeit der Lungenentfaltung.     

新生儿前囟压24 h动态监测及临床意义

小儿颅内高压的早期由于未闭的前囟或颅缝起一定的缓冲作用，患儿可无明显临床症状。因此颅内压力监测对于颅高压早期的诊断有重要的临床意义。无创性前囟压力测定(anterior fontanel pressure，AFP)与腰穿测压有很好的相关性，可以很好地反映颅内压(intracranial pressure，ICP)的变化，对前囟未闭的患儿作AFP测定有助于诊断颅内压增高和监测治疗效果。     

出生24h内新生儿肺出血病因与死因分析

目的 探讨出生24h内新生儿肺出血的病因与死因,并推测其与胎儿肺出血的关系.方法 选择114例出生24h内即发生肺出血的新生儿,根据临床与病理资料进行分析.结果 114例患儿的肺部病理检查结果,点状肺出血6例,局灶性肺出血印例,弥漫性肺出血48例.28例于生后0～6h,71例于生后7～12h,15例于生后13～24h内发病.肺出血病因中缺氧因素110例,包括吸入性肺炎62例、呼吸窘迫综合征24例、青紫型复杂心脏畸形7例、肺膨胀不全7例、新生儿窒息7例、其他原因缺氧3例;感染因素4例,病因均为宫内感染所致败血症.肺出血患儿死亡因素中缺氧因素113例,包括肺出血74例、吸人性肺炎15例、呼吸窘迫综合征12例、青紫型复杂心脏畸形7例、肺膨胀不全2例、颅内出血2例、新生儿窒息1例;感染因素I例,为宫内感染所致败血症.82例有宫内窘迫史,在直接死于肺出血的74例中,3例有宫内窘迫史,且出生数分钟即死亡.结论 出生24 h内新生儿肺出血病因及死因均以缺氧因素为主,大多有宫内窘迫.其中3例弥漫性肺出血患儿于出生数分钟即死亡,从而推测出生24h内新生儿肺出血,有相当部分可能为胎儿肺出血的延续.     

Intervallrehabilitation mit häuslichem Training bei Kindern mit Zerebralparese

Background

The goal of rehabilitation of children with cerebral palsy (CP) is to reduce sequelae and enhance function. Physiotherapy is a rehabilitative strategy for the treatment of patients with CP, but with little supporting evidence for the different treatment concepts. The Cologne Concept “Auf die Beine” combines interval rehabilitation with short and intensive in-patient stays with 6-month home-based whole body vibration training. In this article, we present gross motor function results after 6 months of training and an additional 6 months of follow-up.

Methods

Gross motor function was assessed with the Gross Motor Function Measure (GMFM). A total of 356 patients were evaluated using the GMFM-66 total score after 6 and 12 months and the GMFM-88 goal dimensions after 6 months.

Results

The GMFM-66 total score improved by 2.9 points (mean 3.35; p?<?0.001) after 6 months and by 0.2 points (mean 0.63; p?=?0.033) after 6 months of follow-up. Goal dimension A (lying) improved by 2.0?% (mean 3.8; p?=?0.001), B (sitting) by 3.3?% (mean 5.9; p?<?0.001), C (crawling) by 4.8?% (mean 6.7; p?<?0.001), D (standing) by 5.1?% (mean 8.0; p?<?0.001), and E (walking) by 2.8?% (median 4.6; p?<?0.001).

Conclusion

Interval rehabilitation combined with home-based training has a significant positive effect on gross motor function in patients with CP. The results were also sustained after 6 months of follow-up.     

新生儿肺炎30例24 h食管酸碱度监测的意义

目的探讨肺炎新生儿导致胃肠功能减退引起胃食管反流的相关性。方法用24h动态pH记录仪监测30例肺炎新生儿的24h食管pH值,以30例健康新生儿作为对照,记录反流发生次数、反流超过5min次数、最长反流持续时间、食管pH<4总时间、反流指数及病理性反流发生次数,并作分析。结果肺炎新生儿组反流发生次数、反流超过5min次数、最长反流持续时间、食管pH<4总时间、反流指数分别是(21.8±9.2)次、(3.7±1.6)次、(7.9±5.0)min、(30.4±13.5)min、(3.6±1.8)%,均高于健康对照组[(16.5±7.3)次、(1.1±0.3)次、(5.4±1.8)min、(23.7±10.2)min、(2.9±0.9)%],均有显著差异(Pa<0.05);病理性反流发生率为33.3%,高于对照组10.0%,差异有显著意义(P<0.05)。结论肺炎新生儿胃肠功能较差,较一般新生儿更易发生胃食管反流。     

住院不足24h放弃治疗出院新生儿相关因素分析

目的 探讨我院住院不足24 h放弃治疗出院新生儿的相关因素,提高本地区新生儿救治技术和人口生存质量.方法 对2007年至2011年住院不足24h的379例新生儿的临床资料进行回顾性分析,对24 h内放弃治疗出院的122例新生儿及住院大于24 h的244例新生儿的相关因素进行单因素及多因素回归分析.结果 （1） 2007年至2011年住院不足24h新生儿共379例,放弃治疗组122例.放弃、转院、好转和未愈四组的胎龄和体重比较差异均有统计学意义（P＜0.01）.（2） 2007年至2011年住院不足24h放弃治疗出院新生儿呈逐年下降趋势（卡方趋势检验x2=6.115,P=0.013）,未愈组呈逐年上升趋势（P＜0.05）,转院组与好转组无逐年上升或下降趋势（P＞0.05）.（3） 24 h内放弃治疗组与住院大于24h组比较,在胎龄、出生体重、剖宫产、产时窒息、宫内窘迫、出生医院、家庭年收入＜2万元、父亲文化程度高中及以下、诊断早产儿、极低体重儿、低体重儿、呼吸窘迫综合征、脑损伤等方面差异有统计学意义（P＜0.05）.男性、产时高危因素、母亲疾病状态、母亲既往流产史差异无统计学意义（P＞0.05）.（4）剖宫产、宫内窘迫、出生于县级医院及以下级别医院、家庭年收入＜2万元、诊断为呼吸窘迫综合征,与24h内放弃治疗出院有相关性,且均为危险因素.其他因素与24h放弃治疗出院无相关性.结论 建立系统的产检制度,尽量避免早产,减少呼吸窘迫综合征的发生.增加新生儿科的硬件配置及医护人员的配置,提高专业技术水平,完善社会保障机制,减少住院不足24 h放弃治疗出院新生儿,提高本地区新生儿救治技术和人口生存质量.     

儿童体位性心动过速综合征24h动态血压变化研究

目的总结儿童体位性心动过速(POTS)24 h动态血压监测(ABPM)变化特点。方法回顾性分析2009年1月至2013年6月因不明原因晕厥先兆、晕厥症状在中南大学湘雅二医院儿童晕厥门诊就诊或住院,经直立倾斜试验(HUTT)诊断明确并同时行ABPM的70例POTS患儿,年龄4~14岁。对照组为同期来门诊进行体检的健康儿童,年龄4~14岁。根据血压昼夜节律将两组再分成"勺型"血压和"非勺型"血压两种情况,并进行比较。结果(1)POTS组昼夜平均血压均低于对照组(P0.05)。收缩压昼夜差值亦低于对照组(P0.05),而舒张压昼夜差值在两组差异无统计学意义(P0.05)。(2)血压昼夜节律:"非勺型"血压在POTS组高于对照组(72.9%vs.45.0%,P0.01)。POTS组日间平均收缩压、收缩压昼夜差值、舒张压昼夜差值在"勺型"血压亚组高于"非勺型"血压亚组(P0.05),而夜间平均收缩压、夜间平均舒张压在"勺型"血压亚组低于"非勺型"血压亚组(P0.05)。结论 POTS儿童平均血压与健康儿童无明显差异,但POTS儿童血压昼夜节律以"非勺型"血压多见。     

出生1～7 d早产儿12 h自由排尿观察

目的 探讨出生7 d内早产儿的自由排尿特点.方法 选取2010年3-5月在本院NICU住院的113例出生7 d内无疾病的单胎早产儿.男60例,女53例;胎龄32～36周[(34.0±1.9)周];体质量1.16～2.07 kg[(1.48±0.34) kg].据日龄分为7组(第1天组～第7天组).第1天组6例,第2天组11例,第3天组11例,第4天组18例,第5天组18例,第6天组26例,第7天组23例.每组均进行12 h(900-2100)自由排尿观察,记录排尿时间、每次排尿量, B超测量排尿后残余尿量,排尿时觉醒状态.结果 113例早产儿12 h共观察排尿745次.第1天组6例早产儿观察时段位于出生0.5～12.5 h,12 h内排尿1～4次[(2.8±1.2)次],其中5例出生4 h内无排尿.第3天组开始发现早产儿排尿前有手臂或大腿微动或排尿后啼哭现象.第2、3、4天组平均2.5 h排尿1次.第5、6、7天组平均不到2 h排尿1次.第1天组、第4天组、第7天组排尿次数、排尿量比较差异均有统计学意义(Pa<0.05).各组残余尿量比较差异均无统计学意义(Pa>0.05),膀胱排空率均低于30%.总体27.39%(204/745次)的排尿发生在清醒状态.间断排尿(10 min内排尿次数≥2)见于第2天组以后的早产儿,达70%(522/745次).结论 出生7 d内的早产儿中枢神经系统参与排尿不明显.每次排尿量及排尿次数变异较大,但有随日龄增加的趋势.     

Quoi de neuf dans les hémangiomes infantiles ?

Infantile hemangioma is the most common vascular tumor of childhood. This benign tumor appears during the first weeks of life, grows during the first months (endothelial proliferation) and then involutes slowly and spontaneously until resolution (possibly with sequelae) which is observed after several years. Although they are always benign tumors, infantile hemangiomas can cause complications. Regarding their location or size, infantile hemangiomas can be life or function-threatening. They are also painful when ulcerated and can cause permanent disfigurement or long-term adverse psychological consequences. Since 2008 and the serendipitous discovery of the efficacy of propranolol in the treatment of infantile hemangiomas, systemic propranolol therapy has been widely used, with a very interesting benefits/risks balance. It has progressively superseded general corticosteroid therapy. A Compassionate Use Program (CUP) for systemic propranolol therapy has been developed in France and represents nowadays the first-line use for the vast majority of French prescribers.