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Neonatal Marfan Syndrome: Improving the Bad Prognosis with a Strict Conservative Treatment with Carvedilol?

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Open Journal of Thoracic Surgery 2014年第2期4卷 44-47页

作者： reiner Buchhorn Tuende Kertess-Szlaninka Sonja Dippacher Martin Hulpke-Wette klinik für kinder-und Jugendmedizin Caritas Krankenhaus Bad MergentheimGermany Pediatric Practice GottingenGermany

We report about a successful heart failure therapy with carvedilol in two children with neonatal Marfan syndrome (nMfS). As shown in Case 1, double valve replacement in an infant with neonatal Marfan syndrome is feasible but its benefit on long term is uncertain. Excluding our patient, 3 infants with nMfS from the literature died early after cardiac surgery. Our second case is a unique patient who survives nMfS despite diaphragmatic herniae, dilated neonatal cisterna magna and severe atrioventricular valve insufficiencies. Treated with 0.7 mg/kg/day Carvedilol since his seventh month of life, he never developed severe heart failure. However despite his good health status at the age of 9 years, a progressive aortic root dilatation and left conornary aneurysm are still waiting for surgical repair.

关键词： Marfan Syndrome Neonatal Marfan Syndrom Heart failure Beta Blocker Carvedilol Valve Surgery

Pediatric Head Injury: The Incidence of Multiple Injuries

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Journal of Behavioral and Brain Science 2016年第6期6卷 254-267页

作者： Thomas Kapapa Melanie Kapapa Carsten Posovszky Joachim Gülke ralph König Dieter Woischneck Christian rainer Wirtz Andrej Pala Neurochirurgische klinik Universitatsklinikum Ulm Ulm Germany Sektion kinderchirurgie Universitatsklinikum Ulm Ulm Germany klinik für kinder-und Jugendmedizin Universitatsklinikum Ulm Ulm Germany klinik für Unfall- Hand-und Plastische und Wiederherstellungschirurgie Universitatsklinikum Ulm Ulm Germany Neurochirurgische klinik Klinikum Landshut Landshut Germany

Objectives: Concomitant injuries play an important role when it comes to clinical management of traumatic brain injury (TBI). We examined the incidence of concomitant injuries and their relevance with respect to hospitalization. Methods: Children aged between 0 - 18 years hospitalized for treatment of TBI (ICD 10;S06.0 - 9) during 2010-2011 were included. The data relating to concomitant injuries and the course of treatment were evaluated. Statistical analysis included multivariate regressions at a level of significance of p ≤ 0.05. results: 794 children were treated for head injury in our hospital. Head injury with other associated injuries had been sustained by 158 (19.9%) children. The face and the extremities were the areas of the body most often affected (p = 0.001). Boys represent the majority within the cohort of multiple injured children (p = 0.0001). The older the child, the higher the percentage of children with concomitant injuries (r = 0.27;p = 0.034). There was a significant correlation between the severity of the head injury and the occurrence of concomitant injuries (r = 0.19;p = 0.046). Children with concomitant injuries were found to suffer significantly more falls (N = 82;51.9%) than road traffic accidents (N = 68;43%) (p = 0.0001). A comparison of different variables revealed that age (7 to 10 years), severity of head injury (mild TBI), and trauma mechanism (fall) were most influential (KB = ?1.55;p = 0.023) for concomitant injuries. Children with concomitant injuries have a significant longer stay in hospital than those without: mean stay 2.5 to 4.5 days (p = 0.0001). Conclusion: Concomitant injuries are hints for more severe head injuries and children should be examined with special care.

关键词： Isolated Traumatic Brain Injury Concomitant Injury facial Injury Length of Hospitalization

Genotype-dependent activation or repression of HBV enhancer Ⅱ by transcription factor COUP-Tf1

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World Journal of Gastroenterology 2006年第37期12卷 6054-6058页

作者： Silke f fischer Katja Schmidt Nicola fiedler Dieter Glebe Christian Schüttler Jianguang Sun Wolfram H Gerlich reinald repp Stephan Schaefer Instiut für Medizinische Virologie Justus-Liebig-Universitaet Giessen Germany Zentrum für kinderheilkunde Justus-Liebig-Universitaet Giessen klinik für kinder-und Jugendmedizin Klinikum Fulda Germany Institut for Medizinische Mikrobiologie Immunologie und Hygiene Technische Universitaet München Germany Abteilung für Virologie Universitatt Rostock Germany

AIM: To study the expression of HBV enhancer Ⅱ by transcription factor COUP-Tf1. METHODS: In order to study the regulation of HBV variants in the vicinity of the NrrE we cloned luciferase constructs containing the HBV enhancer Ⅱ from variants and from HBV genotypes A and D and cotransfected them together with expression vectors for COUP-Tf1 into HepG2 cells. rESULTS: Our fi ndings show that enhancer Ⅱ of HBV genotype A is also repressed by COUP-Tf1. In contrast, two different enhancer Ⅱ constructs of HBV genotype D were activated by COUP-Tf1. The activation was independent of the NrrE because a natural variant with a deletion of nt 1763-1770 was still activated by COUP- Tf1. CONCLUSION: regulation of transcription of the HBV genome seems to differ among HBV genomes derived from different genotypes. These differences in transcriptional control among HBV genotypes may be the molecular basis for differences in the clinical course among HBV genotypes.

关键词： Hepatitis B virus Hepatitis B virus x protein COUP-Tf1 Genotype Enhancer Ⅱ

Electroencephalogram response in premature infants to different odors: a feasibility study

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World Journal of Pediatrics 2022年第12期18卷 845-848页

作者： Janine Gellrich Sandy Schlage Veronika Messer Valentin A.Schriever Abteilung Neuropädiatrie Medizinische fakultät Carl Gustav Carus Technische UniversitätDresdenGermany klinik und Poliklinik für kinder-und Jugendmedizin Medizinische Fakultät Carl Gustav CarusTechnische Universität DresdenFetscherstrasse 7401307DresdenGermany Center for Chronically Sick Children(Sozialpädiatrisches Zentrum SPZ)Charité-Universitätsmedizin BerlinBerlinGermany Department of Pediatric Neurology Charité-Universitätsmedizin BerlinBerlinGermany

The importance of olfaction for newborns,especially for mother-associated odors is well *** receptor neurons were found in preterm infants as early as 24–27 weeks of gestation[1].Therefore,it can be assumed that the olfactory sense is functioning in preterm infants[2],but only very little is known about olfactory processing at this *** studies dealing with the processing of olfactory stimuli have been conducted on mature ***,in 2001 a research group demonstrated,using near-infrared spectroscopy,that a change in blood oxygenation of premature infants could be detected in the orbitofrontal cortex after olfactory stimulation with disinfectant and detergent[3].Electroencephalogram(EEG)techniques have broadly been used to measure central odor processing in adults and children[4,5,6].

关键词： mother stimulation cortex

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一7周龄婴儿罕见的不全性麻痹

世界核心医学期刊文摘（儿科学分册） 2006年第9期2卷 48-49页

作者： Seger-fritz B. Bertram U. 俞晓梅 klinik fr kinder-und Jugendmedizin Klinikum Stadt HanauLeimenstrafle 2063450 Hanau

A 7-week-old infant was sent to hospital because of paresis of the facial nerve and the left arm. On examination the girl showed Hufnagel’s palsy and pseudoparalysis of the left arm. Because high inflammation parameters were suspicious for osteomyelitis,antibiotic therapy was started. X-rays,MrI and scintigraphy of the bones showed no pathological findings. With changing pseudoparalytic symptoms and no signs of involvement of other organs,serologic examination then showed positive results for syphilis. A control X-ray of the extremities 2 weeks later showed periosteal thickening. The infant recovered completely after treatment with penicillin. After an uneventful pregnancy it is difficult to diagnose connatal syphilis if the only symptom is pseudoparalysis and the radiographic findings are normal. In newborns with unusual symptoms,connatal syphilis should be considered in the differential diagnosis.

关键词：先天梅毒臂麻痹血清学炎症指标病理发现鉴别诊断

婴幼儿免疫接种前的抗麻疹、腮腺炎和风疹病毒的抗体滴度

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德国医学 1998年第2期15卷 97-98页

作者： J.Klinge K.Korn U.Heininger K.Stehr 贺永文 klinik mit Poliklinik für kinder und jugendliche Universitt Erlangen-NürnbergLoschgestr.15D-91054 ErlangenGermany 同济医科大学附属协和医院

联邦德国麻疹、腮腺炎和风疹(MMr)三联疫苗免疫的一项研究报道,接种过疫苗的30名9～12月龄孩子全部产生了足量的抗体。美国1975年的一项研究也指出,15月龄孩子经接种疫苗后也都产生了保护性抗体。故德国联邦卫生部免疫接种常务委员会推... 详细信息

联邦德国麻疹、腮腺炎和风疹(MMr)三联疫苗免疫的一项研究报道,接种过疫苗的30名9～12月龄孩子全部产生了足量的抗体。美国1975年的一项研究也指出,15月龄孩子经接种疫苗后也都产生了保护性抗体。故德国联邦卫生部免疫接种常务委员会推荐从15月龄起进行首次免疫接种。1980年在德国施行三联疫苗接种时。

关键词：麻疹腮腺炎风疹抗体滴度免疫接种

Detection of bladder trabeculation by voiding cystourethrography and sonography:observations in boys with posterior urethral valves

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World Journal of Pediatric Surgery 2021年第3期4卷 16-20页

作者： Jonas Thüminger Bernhard Haid Josef Oswald kinder-und Jugendchirurgie Landeskrankenhaus Salzburg-Universitatsklinikum der Paracelsus Medizinischen PrivatuniversitatSalzburgAustria Pediatric Urology Ordensklinikum Linz Barmherzige Schwestern HospitalLinzAustria

Background As trabeculated bladder wall is often referred to as a sign of chronically increased intravesical pressure,we investigated whether voiding cystourethrography(VCUG)or sonography reliably predicts bladder trabeculation on later *** A total of 76 consecutive patients(2012-2017)with cystoscopically confirmed posterior urethral valves(PUV)and pre-endoscopy VCUG were *** data were available for 68 of these *** findings were reassessed and compared with endoscopic findings using fisher’s exact test and Spearman’s rank *** VCUG showed a sensitivity of 83.3%and a specificity of 30%in predicting trabeculation on a later urethrocystoscopy,with no significant difference in determining mild or severe forms(p=0.51).Sonography proved a sensitivity of 27.6%and a specificity of 70%,with no correlation between sonographic signs and trabeculation on cystoscopy(r=0.1311).In addition,vesicoureteral reflux had no significant influence as a possible pressure pop-off mechanism on the development of trabeculation in our *** While VCUG predicts bladder trabeculation in children with PUV with limited reliability,sonography mostly fails to detect ***,such findings should only be used with utmost caution in relation to clinical decision-making.

关键词： bladder sonography specificity

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婴儿广泛性动脉钙化症:存活期延长的两兄妹

世界核心医学期刊文摘（儿科学分册） 2006年第8期2卷 38-39页

作者： Ciana G. Trappan A. Bembi B. f.rutsch 高蕊 klinik und Poliklinik für kinder-und Jugendmedizin Allgemeine KinderheilkundeUniversittsklinikum MünsterAlbert-Schweitzer-Strasse 3348149 MünsterGermany

In generalized arterial calcification of infancy(OMIM no.208000),calcificat ion of the media and proliferation of the intima lead to arterial *** affected patients present with untreatable arterial hypertension and die within the first months of *** disease has recently been linked to mutations in *** report two siblings with prolonged survival,both of whom carry the co mpound heterozygous ENPP1 mutations C.913C > A and c.1164+2T > *** both sibli ngs,spontaneous regression of arterial calcifications occurred,and antihyperte nsive treatment could be tapered off *** some patients,the natural co urse of GACI may be more favourable than previously assumed.

关键词：存活期延长动脉钙化钙化症兄妹婴儿抗高血压治疗动脉中膜 P1基因自然病程患儿

慢性关节炎患者的心理社会适应能力:患有幼年型特发性关节炎和反应性关节炎的儿童和青少年的行为特征

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世界核心医学期刊文摘（儿科学分册） 2006年第11期2卷 61-62页

作者： Müller-Godeffroy E. Lehmann H. Küster r.M. ThyenU 郭战宏 klinik für kinder-und Jugendmedizin Universittsklinikum Schleswig-Holstein Campus LübeckRatzeburger Allee 160 23538 Lübeck Germany

We sought to measure psychosocial adaptation in children and adolescents with different forms of chronic arthritis and to determine associated factors. Mothers of 68 children aged 8-17 years with juvenile idiopathic arthritis (JIA) or reactive arthritis reported behaviour problems (CBCL), children reported their actual stress level (SSK), functional disability in conducting activities of daily living (CHAQ) and health-related quality of life (HrQOL) including self-esteem (KINDL-r). Children and adolescents with JIA or reactive arthritis reported lower self-esteem compared to normative data. Almost 20%of the samples were reported to have serious behaviour problems,mostly social isolation and depression/anxiety. Children with lower HrQOL, more functional limitations and a higher stress level showed more behaviour problems. Self-esteem and behaviour problems may be relevant outcome measures in children and adolescents with chronic arthritis and useful to monitor psychosocial support in this population.

关键词：特发性关节炎慢性关节炎反应性关节炎社会适应能力幼年型 CBCL 自我评价生活质量功能障碍