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 male, 4 female) with a mean age of 16 years (range 3-37) entered the open-label phase. The patients were treated for a mean of 880 days (range 97-1375 days). Visits were scheduled every 180 days and patients completed one to six visits. rIFN-gamma was administered subcutaneously three times weekly at a dose of 0.05 mg per m2. During the open-label phase of the study 12 patients experienced a serious infection requiring hospitalization within 880 days. The median infection-free time was 993 days. No obvious increase of infections over time was seen. Phagocyte superoxide anion production and phagocyte staphylococcal killing were not influenced by therapy. Seven patients were withdrawn from the study, one because of an adverse reaction, three on their own wish and the other three because they changed to another trial. No patient died during the study. Conclusion. Treatment of patients with CGD with intracellular active antibiotics and additional interferon gamma as infection prophylaxis is safe and justified.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Weening RS,Leitz GJ,Seger RAdoi
10.1007/BF01957365subject
Has Abstractpub_date
1995-04-01 00:00:00pages
295-8issue
4eissn
0340-6199issn
1432-1076journal_volume
154pub_type
临床试验,杂志文章,随机对照试验abstract:UNLABELLED:A formerly premature, exclusively breast-fed infant with severe zinc deficiency syndrome is presented. He showed the characteristic erosive skin changes, including alopecia, as seen in acrodermatitis enteropathica. In addition, he manifested a failure to thrive and irritability. The diagnosis was confirmed b...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01972977
更新日期:1995-01-01 00:00:00
abstract::In order to determine the hepatotoxicity of rifampicin in children treated for tuberculosis, a survey was performed of 18 children receiving this medicine in combination with isoniazid. Fifteen of the 18 children (83%) showed a rise in ASAT values and 11 (61%) in ALAT values exceeding 29U/L. Seven children with maxima...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441477
更新日期:1980-09-01 00:00:00
abstract::An 11-year-old boy with chronic granulomatous disease caused by cytochrome b deficiency developed right upper lung lobe aspergillosis. Intracerebral lesions developed on maximum doses of flucytosine and amphotericin B. Treatment with 16 mg/kg oral itraconazole was followed by a dramatic clinical improvement and almost...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01958428
更新日期:1991-05-01 00:00:00
abstract::The aim of this study was to assess the influence of active and passive maternal smoking on placenta total oxidant/antioxidant status in term infants. The levels of cord blood total antioxidant capacity (TAC), total oxidant status (TOS), and oxidative stress index (OSI) were measured in samples of fetal placental tiss...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-010-1338-9
更新日期:2011-05-01 00:00:00
abstract::An infant girl three weeks of age with the leading symptom of skin haemorrhages is presented. On further investigation, the signs of severe hepatic damage with hypofibrinogenaemia and prothrombin complex impairment, and renal tubular dysfunction were disclosed. All these pathological symptoms, which were reversed on f...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00465567
更新日期:1977-12-30 00:00:00
abstract:UNLABELLED:Group A streptococcal (GAS) infection is the most common cause of bacterial pharyngitis and has an important role in the pathogenesis of post-infective phenomena including rheumatic fever and glomerulonephritis. Mortality from GAS is uncommon, particularly in the paediatric population. Toxic shock syndrome r...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-002-1032-7
更新日期:2002-09-01 00:00:00
abstract::Five patients, 11 months to 3 4/12 years old with severe normochromic, normocytis anemia and reticulocytopenia are reported. At the height of erythropoietic arrest when erythroid precursor cells were completely absent, undifferentiated stem cells (transitional cells), accumulated in the bone marrow. They disappeared a...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00497680
更新日期:1976-08-16 00:00:00
abstract:UNLABELLED:Dynamic processes are of great interest in the study of lipid and fatty acid metabolism. Their in vivo investigation is now possible with the use of stable isotope tracers and the available sensitive analytical technology. We present some examples demonstrating the assessment of lipid oxidation as well as mo...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00014276
更新日期:1997-08-01 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::We have evaluated 46 patients with Turner syndrome by clinical examination, M-mode and two-dimensional echocardiography, dynamic exercise testing and 24 h Holter monitoring. Twelve patients (26.1%) had mitral valve prolapse and 7 patients (15.2%) had isolated non stenotic bicuspid aortic valve. Aortic root dilation wa...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441551
更新日期:1989-04-01 00:00:00
abstract:UNLABELLED:Establishing enteral feeding in the very low birth weight infant as soon as possible after birth has been shown to promote growth and reduce the need for intravenous lines. Human milk can be administered either as a continuous infusion or as intermittent boluses. The aim of this study was to investigate the ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310100814
更新日期:2001-10-01 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::Abnormalities of chromosome 14 are encountered infrequently in clinical cytogenetics and only few studies of paracentric inversion have been published. This paper reports the first case of hypospadias associated with paracentric inversion of chromosome 14. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441748
更新日期:1986-02-01 00:00:00
abstract::Presentation of cystic fibrosis (CF) with an acrodermatitis enteropathica-like skin rash, anemia, and hypoproteinemia without pulmonary disease is rarely reported before. We describe an 11-month-old boy with rash and edema as the presenting signs of cystic fibrosis. The interesting additional finding in our patient wa...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-011-1447-0
更新日期:2011-10-01 00:00:00
abstract:UNLABELLED:The primary objective of this review was to assess the diagnostic accuracy of voiding urosonography (VUS) in detecting reflux (VUR). As a secondary objective, the reported technical suggestions and diagnostic mistakes were shown to improve the examination protocol and provide the most accurate results. Using...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-002-0954-4
更新日期:2002-07-01 00:00:00
abstract:UNLABELLED:Two years after an accident resulting in either a mild head injury or a fractured bone, two groups of 22 children each, aged 4-14 years, were examined for the existence of any neurobehavioural symptoms by means of a standardized questionnaire filled out by their caretakers. Selection of the children was base...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051061
更新日期:1999-03-01 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::Congenital syphilis is now rare in Australia, particularly in suburban areas. The disease is both preventable and treatable, however, missed or late diagnosis can lead to catastrophic effects. We report an infant who developed congenital syphilis after multiple opportunities for preventing this condition were missed. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-005-0050-7
更新日期:2006-05-01 00:00:00
abstract::Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory cytokine-related disorder associated with overproduction of interleukin (IL)-6, IL-1β, and IL-18. Macrophage activation syndrome (MAS) is a critical and lethal complication of sJIA. Therefore, prompt induction of remission in the active phase of sJIA is ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-013-2093-5
更新日期:2014-12-01 00:00:00
abstract::All 270 patients presenting with Henoch-Schönlein Purpura over a 13-year period from a total childhood population of 155,000 were studied. This is an incidence of 13.5/100,000 children per year. Fifty-five (20%) were found to have initial evidence of renal involvement, and were re-examined at a mean of 8.3 years later...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442205
更新日期:1988-02-01 00:00:00
abstract::Failure to thrive, feeding difficulties, variable forms of infantile epilepsy or psychomotor developmental delay and hypotonia were the most frequent clinical disease presentations in eight children with combined oxidative phosphorylation enzyme complex deficiencies carrying mutations in the polymerase gamma (POLG1) g...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0234-9
更新日期:2007-03-01 00:00:00
abstract:LARYNGOSCOPE BURNS IN NEONATAL INTUBATION:Following burns during neonatal intubation, we mounted an in vitro study of laryngoscopes to determine the temperatures reached during clinical use. The temperature of 10 different bulb laryngoscopes heads and two fibre optic heads were measured with a thermocouple, once opened...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-019-03381-0
更新日期:2019-07-01 00:00:00
abstract::IgG subclass levels were determined in 448 normal children from 6 months to 18 years of age and in 141 healthy adults by radial immunodiffusion using monoclonal antibodies. Age-normal percentile values were calculated for each year of age up to 18 years for IgG1, IgG2, IgG3 and in adults for all four subclasses. The b...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01958271
更新日期:1989-12-01 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::Of all newborn infants, 5% require some degree of basic life support at birth. Newborn resuscitation therefore is one of the most frequent procedures carried out in medicine. It is therefore important that the routines in use are evidence based. Newborn resuscitation can be divided into ten steps: (1) initial stabilis...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00014284
更新日期:1998-01-01 00:00:00
abstract::Vitamin K prophylaxis in infancy aims to prevent life-threatening vitamin K deficiency bleeding (VKDB). The Dutch prophylactic oral daily regimen was increased sixfold from 25 to 150 μg because of a high failure rate. To evaluate the efficacy of this new regimen, incidences of intracranial VKDB under both regimens wer...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-019-03391-y
更新日期:2019-07-01 00:00:00
abstract:UNLABELLED:The aim was to assess the incidence of complications related to skin-to-skin contact (SSC) in newborns with an umbilical venous catheter (UVC). We carried out a prospective follow-up study of all UVCs in a level 3 unit where SSC is systematic. A total of 333 babies were included (mean gestational age of 31.3...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-015-2665-7
更新日期:2016-04-01 00:00:00
abstract::We report a 28-day-old female infant with pertussis presenting as severe acute bronchiolitis with cyanosis. On admission, the patient's symptoms were similar to those of acute bronchiolitis. However, occasional apneic episodes with cyanosis and peripheral lymphocytosis suggested neonatal pertussis and prompted us to e...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-008-0744-8
更新日期:2009-03-01 00:00:00
abstract:UNLABELLED:Of the many complications associated with glycogen storage disease type I, hepatic tumours cause great concern because of their malignant potential and the current difficulties in monitoring them. Hepatic adenomas occur in 22%-75% of affected adults, according to the population studied, and from those report...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-002-1002-0
更新日期:2002-10-01 00:00:00
abstract:UNLABELLED:Bone mineralization of healthy preterm infants fed human milk were compared with that of similar fed preterm formula. Bone mineralization was studied by dual energy X-ray absorptiometry in 43 preterm infants divided into two groups; 21 preterm infants were fed with maternal breast milk and 22 preterm infants...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050864
更新日期:1998-06-01 00:00:00