Abstract:
:Forty-five children (25 girls; 20 boys) with meningomyelocele (MMC) were assessed for growth, skeletal maturation and pubertal development. The spinal defects were operated on shortly after birth and all children required cerebral drainage for hydrocephalus. Standard deviation scores for height, sitting height, sub-ischiac leg length, head circumference, weight and bone age were compared with aged-matched data from a normal Swiss population. The children with MMC were shorter (height SDS boys -2.34 +/- 1.57; girls -2.01 +/- 1.57, mean +/- SD, P less than 0.0005), secondary to a decrease in trunk and lower limb length: Arm length was normal. The head circumference was increased, significantly in the girls (SDS + 1.49 +/- 1.21, P less than 0.005). Weight was decreased in both sexes, this being significant in the boys (SDS -1.11 +/- 1.23, P less than 0.005); however, comparison of weight with height suggested that children with MMC were relatively obese. Bone age was significantly advanced in both boys (SDS + 1.07 +/- 2.13, P less than 0.025) and girls (SDS + 1.36 +/- 1.77, P less than 0.0001). Secondary sex characteristics appeared early in both boys and girls and one girl and one boy presented with puberty advanced by several years. Cryptorchidism occurred in 25% of boys with MMC.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Greene SA,Frank M,Zachmann M,Prader Adoi
10.1007/BF00451900subject
Has Abstractpub_date
1985-07-01 00:00:00pages
146-8issue
2eissn
0340-6199issn
1432-1076journal_volume
144pub_type
杂志文章abstract:UNLABELLED:The severity and duration of immunosuppression caused by corticosteroids (CSs) usage have not been extensively studied. We aimed to investigate the effects of CSs on the various compartments of immune system in relation to timing of initiation and persistence of therapy. Pediatric patients with idiopathic ne...
journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章
doi:10.1007/s00431-016-2694-x
更新日期:2016-05-01 00:00:00
abstract::Adolescence obesity and depression are increasingly prevalent and are associated with various health complications. The aim of this study was to examine the association between weight status (normal weight, overweight, and obese) and depressive symptoms in adolescents and to explore the probable mediators, including o...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1753-1
更新日期:2012-08-01 00:00:00
abstract::Severe neonatal centronuclear myopathy is inherited as an X-linked condition characterized by primary asphyxia, extreme muscular hypotonia and absent spontaneous movements. We report seven cases from three families to point out the importance of diagnosis with regard to prognosis, outcome and genetic counselling. In h...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02072056
更新日期:1990-12-01 00:00:00
abstract::We have previously shown that breast-fed infants have a considerably larger thymus at 4 months than formula-fed infants. The aim of the present study was to investigate whether breast-feeding also influences the thymic size in late infancy. In a cohort of 50 infants, all being partially breast-fed when recruited at 8 ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051258
更新日期:1999-12-01 00:00:00
abstract:UNLABELLED:Sleep duration has been identified as risk factor for obesity already in children. Besides investigating the role of fat mass (FM), this study addressed the question whether endocrine mechanisms act as intermediates in the association between sleep duration and overweight/obesity. Within the framework of the...
journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
doi:10.1007/s00431-011-1670-8
更新日期:2012-07-01 00:00:00
abstract:UNLABELLED:A 9-year-old boy lost almost all his small bowel after an acute volvulus due to a congenital, but previously unsuspected malrotation. Survival using total parenteral nutrition is possible in these cases, but the medical burden is heavy. Small intestinal transplantation was performed for the first time in the...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-003-1294-8
更新日期:2003-11-01 00:00:00
abstract::Metabolic liver diseases (MLD) are an important group of disorders presenting with neonatal cholestasis (NC). The spectrum of liver involvement is wide and the presumptive diagnosis is traditionally based on clinical and laboratory findings. Recently, next-generation sequencing (NGS) panels have emerged as an appealin...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-019-03328-5
更新日期:2019-04-01 00:00:00
abstract:UNLABELLED:A newborn boy was referred to our hospital because of hemolytic anemia and severe hyperbilirubinemia. Extensive investigations aimed at determining the cause of hemolysis was initiated at the time of admission and 3 months after blood transfusion. Notably, no intrinsic erythrocyte abnormalities could be dete...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-014-2374-7
更新日期:2014-12-01 00:00:00
abstract:UNLABELLED:Five neonates who suffered from an unexpected long period of respiratory failure, muscular hypotonia, and drowsiness were observed in a retrospective study. Prior to this general depression, unusually high doses of diazepam were administered to all patients via intravenous bolus injection. Serum levels of di...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310100745
更新日期:2001-06-01 00:00:00
abstract::Twenty-six children with cerebral palsy were examined with respect to structural asymmetry of the mouth. In 19 children there were clear cut correlations between symmetry/asymmetry of voluntary function and the oral findings. Patients with symmetrical patterns of movements had symmetrical dentition, while in those wit...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441639
更新日期:1980-12-01 00:00:00
abstract:UNLABELLED:The aim of this study was to establish the correlations between the polymorphisms of the genes interleukin (IL)-6 572, 190, and 174 in obese children. We assessed 222 hospitalized children divided into two groups: group I (control) included 110 patients with normal nutritional status, and group II consisted ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-014-2315-5
更新日期:2014-10-01 00:00:00
abstract::We have investigated four patients from three unrelated families with typical clinical and biochemical features of "late-onset" multiple carboxylase deficiency. All patients suffered from biotinidase deficiency (plasma biotinidase activities 1.4%-3% of normal). Intestinal absorption of biotin, measured in three of the...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00491919
更新日期:1985-05-01 00:00:00
abstract:UNLABELLED:Intensive multimodality treatment has led to a remarkable improvement of prognosis in paediatric cancer patients, however, a great number of long-term survivors suffer from considerable tumour- or treatment-related late effects. Between January 1990 and December 1998, 223 consecutive survivors of childhood m...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/pl00008340
更新日期:2000-10-01 00:00:00
abstract::Prolonged emergency department (ED) length of stay (LOS) is used as a proxy for ED overcrowding and is associated with adverse outcomes of patients requiring therapy and reduced patient satisfaction. Our aim was to identify and quantify variables which affect ED-LOS. Patients admitted to the pediatric ED of a large re...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-017-2879-y
更新日期:2017-05-01 00:00:00
abstract::A female patient with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency developed normally until 13 months of age after which she showed a gradual developmental delay, followed by progressive dementia, and a decrease in head circumference growth culminating in the diagnosis of Rett syndrome at 3.5 years. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01954516
更新日期:1994-04-01 00:00:00
abstract::The conclusions of this mini-workshop are not substantially different from those previously published based on the USA experience [9]. This does not necessarily mean that there are no important differences between Europe and the USA, rather it reflects the paucity of data, especially in Europe. Moreover, European data...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00014362
更新日期:2000-09-01 00:00:00
abstract:UNLABELLED:Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterised by periodic attacks of fever and serositis. Recent genetic and epidemiological research have highlighted the importance of this disease. FMF is the most frequent periodic fever syndrome and is transmitted in an autosomal recessi...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-003-1223-x
更新日期:2003-07-01 00:00:00
abstract::This was an uncontrolled, open-label follow up study of a previous 12-month, randomized, double-blind, placebo-controlled trial performed to assess the long-term efficacy and safety of Recombinant Human Interferon Gamma (rIFN-gamma) in patients with chronic granulomatous disease (CGD). In two centres, 28 patients (24 ...
journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1007/BF01957365
更新日期:1995-04-01 00:00:00
abstract::It is not clear if children with high fever are at increased risk for serious bacterial infection (SBI). Our aim was to systematically review if children suffering from high fever are at high risk for SBI. Our data sources were Embase, Medline, and Pubmed; from their inception until the last week of March 2017. The st...
journal_title:European journal of pediatrics
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00431-018-3098-x
更新日期:2018-03-01 00:00:00
abstract:UNLABELLED:Aminoglycoside prescriptions were rarely evaluated in children care facilities. Because of risk of toxicity, these narrow spectrum antibiotics are commonly misused. In this study, we evaluate aminoglycoside prescription and assess the impact of an information campaign on modalities of prescription and monito...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-016-2691-0
更新日期:2016-05-01 00:00:00
abstract:INTRODUCTION:Multiple sulfatase deficiency is biochemically characterized by the accumulation of sulfated lipids and acid mucopolysaccharides. CASE REPORT:We report clinical, biochemical, and molecular findings in a female newborn affected with a severe form of multiple sulfatase deficiency (Mendelian Inheritance in M...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-008-0871-2
更新日期:2009-08-01 00:00:00
abstract::The phenylalanine hydroxylase (PAH) deficiency trait is heterogeneous with a continuum of metabolic phenotypes ranging from classical phenylketonuria (PKU) to mild hyperphenylalaninaemia (MHP). More than 200 mutations in the PAH gene are associated with PAH deficiency. From theoretical considerations or in vitro expre...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00014253
更新日期:1996-07-01 00:00:00
abstract::Tonsillectomy is one of the commonest ENT paediatric procedures. Recovery is best achieved at home, and cost-effectiveness of the day case pathway is significant in tonsillectomy. This project scrutinised the local practice regarding the effectiveness of day case pathway in paediatric tonsillectomy in a large regional...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-3220-0
更新日期:2018-11-01 00:00:00
abstract:UNLABELLED:The aim of the study was to determine the characteristic features and outcome of carcinoid or mucoepidermoid tumours in children. A retrospective analysis of all patients treated for a carcinoid or mucoepidermoid tumour in France between 1984 and 2001 was performed. There were 11 cases of carcinoid tumour an...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-005-1740-x
更新日期:2005-12-01 00:00:00
abstract::Perinatal transmission remains the main cause of HIV infection in the pediatric population. Treatment of HIV-infected children has become less of a problem in resource-rich countries with a remarkable decrease of perinatal infections, resulting in an effective prevention of mother-to-child transmission and antiretrovi...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-008-0914-8
更新日期:2009-04-01 00:00:00
abstract:UNLABELLED:Severe head injuries in children (under 15 years of age) have many features that differentiate them from head injuries in adults. In such cases, non-surgical treatment cannot always prevent fatal herniation. We report on seven cases of children with severe head injury, presenting with decorticate posturing a...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
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更新日期:2002-02-01 00:00:00
abstract:UNLABELLED:To evaluate the indications, underlying cardiac disorders, efficacy and complications involved with implantable cardioverter-defibrillators (ICDs) in paediatric patients in The Netherlands, the records of all patients aged 18 years or younger who underwent ICD placement were reviewed retrospectively. Between...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-005-1668-1
更新日期:2005-07-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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更新日期:1986-08-01 00:00:00
abstract:UNLABELLED:In a national prospective study of risk factors for mother-to-child transmission of human immunodeficiency virus (HIV), 316 children of HIV-positive mothers were followed up for at least 6 months. Infection status was determined in 254 of them and 46 were found to be infected giving a transmission rate of 18...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02074831
更新日期:1995-07-01 00:00:00
abstract::A 4-month-old male infant was seen because of an asymptomatic undescended left testis and a right sided abdominal mass. CT revealed a calcified retroperitoneal tumour. Histological examination of surgical specimens showed a mature primary teratoma of the contralateral undescended testis. While this is very rare, infan...
journal_title:European journal of pediatrics
pub_type: 杂志文章
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更新日期:1993-11-01 00:00:00