Abstract:
:Vascularization of the arms has been studied by impedance plethysmography (rheography) in eight children with Poland syndrome, a common malformation characterized by unilateral hand anomaly and ipsilateral aplasia of the inferior head of the pectoralis major muscle. A marked decrease of the velocity of the systolic increase in the arterial volume (Velm) was shown in the affected arms, and the difference between the two arms was significantly higher in the patients than in a control group (P less than 0.02). These results, which are also observed in stenotic atherosclerosis, support the hypothesis of hypoplasia of the ipsilateral subclavian artery as the origin of the malformation, although a local anomaly in arterial-wall viscosity cannot be ruled out. The opacification of the aortic arch in another patient confirmed this hypothesis. Aortography in further patients will be necessary to substantiate our results. However, this technique is not without danger and should only be used during the first months of life when a vascular operation can be envisaged.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Bouvet JP,Leveque D,Bernetieres F,Gros JJdoi
10.1007/BF00496922subject
Has Abstractpub_date
1978-05-22 00:00:00pages
17-26issue
1eissn
0340-6199issn
1432-1076journal_volume
128pub_type
杂志文章abstract::Although they account for a small proportion of burns in paediatrics, injuries from chemicals can be just as devastating as other mechanisms of burn injury. At least 25,000 chemicals exist which can cause burns: in children, they are often caused by household chemicals via accidental exposure. The mechanism by which c...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-020-03905-z
更新日期:2021-01-06 00:00:00
abstract:UNLABELLED:The present case report describes a patient with Klippel-Feil anomaly (KFA) and oligodontia, carrying a de novo pericentric inversion of chromosome 2 (p12q34). KFA is characterised by congenital vertebral fusion of the cervical spine and a wide spectrum of associated anomalies. It therefore constitutes a het...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-003-1262-3
更新日期:2003-09-01 00:00:00
abstract:UNLABELLED:An 8-year-old boy with frequently recurring pancreatitis-like abdominal pain, Fredrickson type V dyslipidaemia, and significantly decreased post-heparin plasma lipoprotein lipase (LPL) activity is described. In order to exclude familial LPL deficiency, the complete LPL coding gene sequence was analysed revea...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01957148
更新日期:1996-08-01 00:00:00
abstract:UNLABELLED:Lymphocytic hypophysitis is a rare disorder predominantly affecting females during the antepartum or postpartum period. It is characterized by destruction and lymphocytic infiltration of the pituitary gland, probably by an autoimmune process, leading to a pituitary mass lesion and/or various degrees of hypop...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s004310050690
更新日期:1997-09-01 00:00:00
abstract::We evaluated the diagnostic significance of single versus repeated human chorionic gonadotropin (hCG) stimulation of testicular steroidogenesis in 25 boys (10 prepubertal group A; 15 early pubertal, group B) with suspected hypogonadism. All subjects received a single injection of hCG (5000 U/1.7 m2) and 1 month later,...
journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章
doi:10.1007/BF01957499
更新日期:1995-11-01 00:00:00
abstract::Retrospective analysis was performed at an affiliated university children's hospital with consecutive patients receiving a venoarterial extracorporeal membrane oxygenation (VA-ECMO) for refractory cardiogenic shock from July 2007 to May 2018. Fifty-six patients underwent VA-ECMO for refractory cardiogenic shock with t...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-019-03352-5
更新日期:2019-06-01 00:00:00
abstract::Most drugs that are currently prescribed in pediatrics have not been tested in children. Pediatric drug studies are stimulated in the USA by the pediatric exclusivity provision under the Food and Drug Administration Modernization Act (FDAMA) that grants patent extensions when pediatric labeling is provided. We investi...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0381-z
更新日期:2007-08-01 00:00:00
abstract::By the description of two cases of osteoarticular infections due to Kingella kingae in two young children we wish to draw the attention of clinicians to invasive infections due to this micro-organism. Since its biological characterization in 1976, K. kingae has been increasingly reported as a human pathogen. Most comm...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02072618
更新日期:1991-07-01 00:00:00
abstract:UNLABELLED:Extremely immature newborns develop a self-limiting normal anion gap metabolic acidosis in early life. This study examined the natural history of this acidosis in a population of infants of gestation less than 26 weeks in the first 14 days of life. The acidosis was maximal on day 4 with a mean base deficit o...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-014-2364-9
更新日期:2015-01-01 00:00:00
abstract:UNLABELLED:To examine the value of surfactant protein D and KL-6 as markers for the diagnosis and the severity of interstitial pneumonia caused by measles infection, surfactant protein D, KL-6 and lactic acid dehydrogenase were measured serially in three patients with measles complicated by interstitial pneumonia as co...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310100763
更新日期:2001-07-01 00:00:00
abstract::Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder and the most disabling condition of heterotopic (extraskeletal) ossification in humans. Extraskeletal bone formation associated with inflammation preceding the osseous conversion usually begins in the first decade, predominantly in the head, neck, ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-010-1232-5
更新日期:2010-11-01 00:00:00
abstract:INTRODUCTION:Population modelling using mixed-effects models provides a means to study variability in drug responses among individuals representative of those for whom the drug will be used clinically. DISCUSSION:The advantages of these models in paediatric studies are that they can be used to analyse sparse data, sam...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-006-0188-y
更新日期:2006-11-01 00:00:00
abstract::Patients with childhood-onset systemic lupus erythematosus (cSLE) are at risk of becoming short adults. To evaluate the growth patterns and risk factors of short final height, a retrospective study was conducted in 97 patients (87 females, 90%) with cSLE who grew from the time of diagnosis and reached their final heig...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-020-03910-2
更新日期:2021-01-03 00:00:00
abstract::Triple A syndrome (TAS) or Allgrove syndrome (OMIM #231550) is a rare autosomal recessive disorder characterised by adrenocorticotropic hormone-resistant adrenal insufficiency, alacrima, achalasia, and neurological and dermatological abnormalities. Mutations in the AAAS gene on chromosome 12q13 encoding the nuclear po...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-017-3068-8
更新日期:2018-03-01 00:00:00
abstract::Children with symptomatic hypoglycemia and asymptomatic hypoglycemia-prone children were shown to differ in the degree of ketosis after a 20 h fast. In the latter children the close negative correlation between ketone body levels and glucose levels yielded a regression line against which the former children's data cou...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442403
更新日期:1980-06-01 00:00:00
abstract:UNLABELLED:In acute-phase Kawasaki disease, neutrophils cause injury to the coronary arterial endothelium through the production of elastase. Previous research has demonstrated the modulation of neutrophil function and kinetics, such as development and maturation, by granulocyte colony-stimulating factor (G-CSF). To ex...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-002-1018-5
更新日期:2002-10-01 00:00:00
abstract::Pediatric palliative care (PPC) focuses on children and adolescents with life-limiting diseases. It may be initiated at various points of the disease trajectory, if possible early enough to support living with the best possible quality of life despite a limited lifespan. From birth to adolescence, children with a broa...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-012-1710-z
更新日期:2013-02-01 00:00:00
abstract::It is known that the 5%-10% of adults infected with hepatitis B virus (HBV) develop a chronic infection and that HBV infection acquired at birth by an hepatitis B surface antigen (HBsAg)/hepatitis B "e" antigen (HBeAg)-positive mother almost invariably leads to chronic infection. Little information is, however, availa...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01954381
更新日期:1992-03-01 00:00:00
abstract:UNLABELLED:Improved surgical care during the last decades, together with advances in medical management, led to a remarkable increase in survival of patients with congenital heart disease (CHD). However, aging of the CHD population brings new challenges, and loss of follow-up of adolescents and adults with CHD is a maj...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-015-2557-x
更新日期:2015-07-01 00:00:00
abstract::Iliac vessel disruption following blunt trauma is an unusual presentation, particularly in the absence of an orthopaedic injury. We present the unique case of a 14-year-old boy who sustained a blunt bicycle handlebar impalement that resulted in complete transection of the external iliac artery and laceration of the ex...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1767-8
更新日期:2012-12-01 00:00:00
abstract::We hypothesized that because 45,X/46,XY (X/XY) children share a cell line with Turner syndrome (TS), they also share co-morbidities described in TS. In addition, the presence of the Y chromosome in brain and in other body tissues would influence their function. On the basis of our findings, we aimed to establish optim...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-011-1600-9
更新日期:2012-03-01 00:00:00
abstract::The development of visual acuity and stereopsis was studied in 321 boys and 340 girls aged between 5 and 10 years. Visual acuity was assessed by the E test and a modified version of the STYCAR test, stereo acuity by the Lang-Stereo test [17]. Both vision tests showed an increase in the median visual acuity between the...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02429046
更新日期:1986-12-01 00:00:00
abstract::Through a systematic review and meta-analyses, we aimed to determine predictors for place of death among children. We searched online databases for studies published between 2008 and 2019 comprising original quantitative data on predictors for place of death among children. Data regarding study design, population char...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-020-03689-2
更新日期:2020-08-01 00:00:00
abstract::Three infants with severe combined immunodeficiency and adenosine deaminase (ADA) deficiency were treated by T-cell depleted bone marrow transplantation (BMT), using human leukocyte antigen (HLA)-haploidentical parents as donors. In the first patient, two initial transplants failed to engraft and no change of the immu...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01995857
更新日期:1989-11-01 00:00:00
abstract:UNLABELLED:This study assessed health-related quality of life (HRQoL) reported by overweight adolescents and compared to their parent-proxy perception of HRQoL, according to adolescent gender, adolescent age, and parent gender. Patients and a total of 179 adolescents aged 10 to 18 years with excess weight were evaluate...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-013-2050-3
更新日期:2013-10-01 00:00:00
abstract::Since the first description by Catel and Manzke of hyperphalangy and clinodactyly of the index finger associated with Robin malformation sequence, seven further cases have been published. In two families more than one case occurred. Another family is presented with possibly two affected boys showing variable features ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442455
更新日期:1984-08-01 00:00:00
abstract:UNLABELLED:Inflammation of the gastric and duodenal mucosa is the end result of an imbalance between mucosal defensive and aggressive factors. The degree of inflammation and imbalance between defensive and aggressive factors can then result in varying degrees of gastritis and/or frank mucosal ulceration. Gastritis and ...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s004310051142
更新日期:1999-07-01 00:00:00
abstract::A male patient with fucosidosis exhibited the following characteristics: 1. Early onset and rapid progression of neurological symptoms. 2.Skin changes compatible with angiokeratoma corporis diffusum. 3. Complete or nearly complete deficiency of alpha-fucosidase. 4. Survival to adult age (20 years). The deficiency of a...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441644
更新日期:1980-12-01 00:00:00
abstract::The incidence of intussusception in infants varies around the world. The epidemiology of intussusception in France has never been prospectively studied. We performed a prospective observational study with systematic inclusion of all infants aged <1 year with suspected intussusception admitted to the emergency departme...
journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
doi:10.1007/s00431-016-2838-z
更新日期:2017-03-01 00:00:00
abstract::Total serum LDH activity and isoenzyme distribution were studied in children with neuroblastoma at the time of hospital admission. The total LDH was determined in 26 cases, and 20 (77%) of them showed elevation of its activity. On the other hand, in 9 of these 26 cases, the isoenzyme distribution was determined along ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442638
更新日期:1976-09-01 00:00:00