Abstract:
:A combination of humoral immunodeficiency and isolated growth hormone deficiency was observed in a girl with Mulibrey nanism. The humoral immunodeficiency consisted of subnormal concentration of serum IgG, in particular IgG2 and IgG4, and low concentration of serum IgM. Serum IgA and IgD were elevated, IgE was absent. Antibody response in vivo was very low or absent and opsonization in vitro was defective. Total B-cell number was low. In addition, the serum kappa/lambda light chain ratios within the immunoglobulin classes G, A, and M were abnormal. The defective antibody response may be linked to the abnormal kappa/lambda light chain ratios. Endocrine functions were normal except for isolated growth hormone deficiency. Therapy with human growth hormone resulted in increased growth velocity but did not improve humoral immune functions.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Haraldsson A,van der Burgt CJ,Weemaes CM,Otten B,Bakkeren JA,Stoelinga GBdoi
10.1007/BF01955061subject
Has Abstractpub_date
1993-06-01 00:00:00pages
509-12issue
6eissn
0340-6199issn
1432-1076journal_volume
152pub_type
杂志文章abstract::Chronic lung disease of prematurity (CLD) remains a common cause of morbidity and mortality in preterm infants. Oxygen toxicity remains a major risk factor for the development of CLD and as a consequence the antioxidant status of CLD babies is a major focus of interest. In the present study, we determined whether asco...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310000709
更新日期:2001-03-01 00:00:00
abstract::Clinical and EEG family data of 140 cases with early childhood epilepsy with absences are presented. The aim of the study was to evaluate, whether the occurrence of generalized tonic clonic seizures (GTCS) as a presenting symptom might correlate with family data, i.e. whether there are indications of heterogeneity. On...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01956423
更新日期:1994-05-01 00:00:00
abstract::Osteoporosis-pseudoglioma syndrome (OPPG) is a rare autosomal-recessive disorder, characterized by severe osteoporosis and early-onset blindness. Loss of function mutations in the gene encoding low-density lipoprotein receptor-related protein 5 (LRP5) have been established as the genetic defect of the disease. We repo...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-3299-3
更新日期:2019-03-01 00:00:00
abstract:UNLABELLED:This study was conducted to determine if clinical features can predict the risk of intracranial injury (ICI) in pediatric closed head trauma. We enrolled 3,806 children under 16 years consecutively referred for acute closed head trauma to the paediatric emergency room of five Italian children's hospitals. Re...
journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
doi:10.1007/s00431-005-0019-6
更新日期:2006-03-01 00:00:00
abstract::A 4.5-year-old male patient is described with chorioretinopathy, minor facial anomalies, delayed closure of the fontanel, mental retardation, moderate hypotonia, epilepsy and hepatic fibrosis. Postural control, intentional vocalising and manual dexterity were superior to the performance of patients with classical Zell...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441774
更新日期:1985-11-01 00:00:00
abstract:UNLABELLED:There are a number of respiratory diseases affecting infants in which there is surfactant dysfunction or deficiency. Surfactant is inactivated by cholesterol, free fatty acids and bilirubin in meconium aspiration syndrome, by haemoglobin and red blood cell lipids in pulmonary haemorrhage and plasma proteins ...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00008398
更新日期:2000-09-01 00:00:00
abstract:UNLABELLED:The inherited ion channel disorders (channelopathies) are a group of disorders caused by mutations in genes encoding ion channels. Ion channel disorders can affect any tissue, but the majority affect skeletal muscle or the central nervous system. These disorders include skeletal muscle sodium channelopathies...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00014403
更新日期:2000-12-01 00:00:00
abstract::Although thyroid medications are frequently prescribed during pregnancy, paediatricians treating the respective neonates often have no information about the underlying maternal thyroid disease, and inconsistencies in postnatal diagnostics may result. We analysed a cohort of 1819 mothers admitted for delivery in 1 year...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-3232-9
更新日期:2018-11-01 00:00:00
abstract:BACKGROUND:Calcaneal apophysitis, or Sever's disease, is a traction apophysitis. It is a frequent cause of heel pain in children. Knowledge about the exact incidence of calcaneal apophysitis in the general population, however, is lacking. DESIGN:Cross-sectional study. METHODS:From 34 general practices, records of pat...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-013-2219-9
更新日期:2014-05-01 00:00:00
abstract:UNLABELLED:The fatty acid composition of human breast milk was determined longitudinally after term and preterm delivery by high resolution gas liquid chromatography. Milk samples were obtained at days 5, 10, 20 and 30 after term (n = 38) or preterm (n = 19) delivery. The saturated fatty acids C10:0 and C12:0 and the p...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050573
更新日期:1997-02-01 00:00:00
abstract::Urinary tract infection (UTI) is a common bacterial infection among infants and children. Predicting which children with upper UTI will develop long-term sequelae remains difficult. We aimed at evaluating the predictive value of urine concentrations of interleukin-6 (UIL-6) and interleukin-8 (UIL-8) in subsequent rena...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1914-2
更新日期:2013-06-01 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::Female patients with congenital adrenal hyperplasia have been frequently studied in order to determine the impact of prenatal androgen exposure on various aspects of psychological, psychosocial and psychosexual development. There is no published study to evaluate the impact of the genital malformation, genital operati...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02276713
更新日期:1995-09-01 00:00:00
abstract:UNLABELLED:The pathogenesis of sporadic hemiplegic migraine remains unclear, and perfusion-weighted magnetic resonance imaging (PWI) has been used for characterization of hemodynamic changes in migraine aura. We report a case of sporadic hemiplegic migraine in which magnetic resonance perfusion imaging showed left cere...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-015-2609-2
更新日期:2016-02-01 00:00:00
abstract::In pediatric diabetes, insulin pump therapy is associated with less acute complications but inpatient pump education may lead to more hospital days. We investigated the number of hospital days associated with pump vs. injection therapy between 2009 and 2018 in 48,756 patients with type 1 diabetes < 20 years of age fro...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-020-03883-2
更新日期:2021-02-01 00:00:00
abstract:UNLABELLED:Self-medication is a common event. To use drugs correctly, a basic knowledge about drugs is required. Poor data are available about adults' drug knowledge. Furthermore, adolescents' basic drug knowledge has not been investigated. This study was designed in order to explore adolescents' drug use and knowledge...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310000503
更新日期:2000-08-01 00:00:00
abstract:UNLABELLED:We describe a rare case of acute acalculous cholecystitis (AC) due to Salmonella group D infection in a previously healthy child who developed gall bladder empyema and bacteraemia. Salmonella group D was recovered from blood culture, as well as cultures of stool, bile and gall bladder wall samples. The patie...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-002-1051-4
更新日期:2002-11-01 00:00:00
abstract::Antithyroid drugs, considered the treatment of choice for hyperthyroidism during pregnancy, may have an adverse effect on intellectual development of the offspring. We examined the intellectual capacity of 31 subjects aged 4-23 years, born to women with Graves disease who received antithyroid drugs throughout pregnanc...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01957719
更新日期:1992-08-01 00:00:00
abstract::Rasmussen's syndrome (RS) is a rare acquired progressive inflammatory encephalopathy characterized by drug-resistant partial seizures and cognitive deterioration resulting from a gradual impairment and a subsequent atrophy of a single brain hemisphere. It was firstly described by Theodore Rasmussen in 1958. The origin...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-010-1148-0
更新日期:2010-08-01 00:00:00
abstract::Exchange transfusion using a peripheral artery and vein was carried out 18 times in 17 newborn infants. The arteries use to withdraw blood were the radial [13], the ulnar [3] an the posterior tibial [2]. Infusion of blood was carried out simultaneously through a peripheral vein. There was no mortality or morbidity dir...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00443258
更新日期:1981-11-01 00:00:00
abstract:UNLABELLED:Sjögren syndrome (SS) is a common disorder in adults and involves both glandular and extraglandular systems. We report here four cases of childhood SS complicated by chronic thyroiditis, interstitial nephritis or sweat gland inflammation. Additionally, in one of these cases, the central nervous system was in...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02282840
更新日期:1996-10-01 00:00:00
abstract::The traditional recommendation that Swiss children receive vitamin D during the first year of life was recently extended to the second and third year of life and during winter for older children. The aim of the study was to identify how Swiss pediatricians prescribe vitamin D. Between December 2016 and March 2017, 795...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-019-03400-0
更新日期:2019-07-01 00:00:00
abstract::A 16-year-old girl developed rapidly progressive glomerulonephritis and renal failure. The disease was associated with high titres of antiglomerular basement membrane antibodies in serum, linear deposits of immunoglobulin G and diffuse epithelial crescents on renal biopsy. Past history revealed heavy smoking and delib...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00716479
更新日期:1987-05-01 00:00:00
abstract::An adolescent girl with severe thrombotic thrombocytopenic purpura (TTP) remained in a critical condition after 3 weeks of combined treatment with antiplatelet drugs, plasma infusions and plasma exchange. The introduction of vincristine resulted in gradual improvement and eventual complete remission which lasted for 2...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01957008
更新日期:1994-07-01 00:00:00
abstract:UNLABELLED:Monitoring fat free mass (FFM), an indicator of nutritional status and a predictor of exercise performance in children, is particularly important in patients with cystic fibrosis (CF). We assessed validity of the skinfold method for measuring FFM, and its changes with exercise training, in children with CF. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051209
更新日期:1999-10-01 00:00:00
abstract:UNLABELLED:Infection is a well recognised cause of spontaneous early preterm labour. Preterm labour of infective aetiology is refractory to the use of tocolytic agents and affected women have a higher risk of subsequent chorio-amnionitis and neonatal infection. Antibiotics used prophylactically for the prevention of pr...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00014313
更新日期:1999-12-01 00:00:00
abstract::To evaluate the ambulatory management of ileo-colic intussusception in infants and children, a retrospective study over 3 years of 113 children treated for ileo-colic intussusception in a paediatric emergency department was undertaken with the aim of shortening the length of stay. A total of 113 children aged 10 days ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051184
更新日期:1999-09-01 00:00:00
abstract::We review the validity of balloon occlusion aortography (BOA) on the basis of our personal experience with 18 patients with congenital heart disease (mean weight 4.55 g, including 8 neonates). Four of the 18 patients underwent aortic arch angiography using balloon occlusion of the descending aorta. Pulmonary angiograp...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01955515
更新日期:1991-02-01 00:00:00
abstract:UNLABELLED:Successful therapy of vasodilatory shock in adults and children with arginine-vasopressin (AVP) has been reported previously. Data on the use of vasopressin in neonates is limited. This retrospective study reports the effects of AVP-treatment in neonates with catecholamine-resistant systemic vasodilatation a...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0400-0
更新日期:2007-12-01 00:00:00
abstract::Two siblings with increased levels of serum phenylalanine were detected by newborn screening. The older sibling deteriorated neurologically and mentally, despite early dietary control, and died at the age of 6 1/2 years. In the younger sibling phenylalanine hydroxylase activity in liver tissue was normal. Further inve...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442368
更新日期:1978-09-08 00:00:00