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 complete disappearance of the intracerebral lesions. Plasma itraconazole levels were between 40 and 3440 ng/ml depending on concomitant medication. Toxicity was restricted to transient elevation of alkaline phosphatase and gamma glutamyl transferase. We conclude that further trials with itraconazole are justified in high risk patients in whom conventional therapy has failed.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Kloss S,Schuster A,Schroten H,Lamprecht J,Wahn Vdoi
10.1007/BF01958428keywords:
subject
Has Abstractpub_date
1991-05-01 00:00:00pages
483-5issue
7eissn
0340-6199issn
1432-1076journal_volume
150pub_type
杂志文章abstract::Mitochondrial disorders are a clinically and biochemically diverse group of disorders which may involve multiple organ systems. General anaesthesia (GA) poses a potential risk of decompensation in children with mitochondrial disorders, and there is little guidance for anaesthetists and other clinicians regarding the o...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-016-2813-8
更新日期:2017-01-01 00:00:00
abstract:UNLABELLED:The study aims were to compare two models (The Pediatric Risk of Mortality III (PRISM III) and Pediatric Logistic Organ Dysfunction (PELOD-2)) for prediction of mortality in a pediatric intensive care unit (PICU) and recalibrate PELOD-2 in a Portuguese population. To achieve the previous goal, a prospective ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-015-2533-5
更新日期:2015-10-01 00:00:00
abstract::We evaluated chronic kidney disease (CKD) (proteinuria or estimated glomerular filtration rate < 60 mL/min/1.73 m2) or hypertension prevalence in 110 children with juvenile idiopathic arthritis (JIA). CKD and hypertension were clustered under the umbrella term of "renal injury". Median age at the last visit was 14 yea...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-020-03792-4
更新日期:2021-02-01 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::A partial deletion of the short arm of chromosome 9 is reported in a female newborn and a 12.5 year-old male. The features expressed by both patients, and especially the peculiar type of the craniofacial dysmorphism, confirm the existence of a typical clinical syndrome associated with this partial autosomal monosomy. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441473
更新日期:1980-09-01 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::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:UNLABELLED:Blueberry muffin lesions are associated with prenatal infections, severe and chronic anemia and neoplastic infiltrative diseases. In the first two instances they represent postnatal re-expression of cutaneous haematopoiesis, in the latter they are cutaneous localizations of a neoplastic disease. Chronic pren...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050970
更新日期:1998-11-01 00:00:00
abstract:UNLABELLED:During the last decade, much concern has been expressed about online pro-eating disorder communities (e.g., pro-anorexia websites and blogs) which encourage their users to engage in disordered eating behavior. The aim of the current paper is to reemphasize the importance of pro-eating disorder communities in...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-015-2487-7
更新日期:2015-04-01 00:00:00
abstract:UNLABELLED:Delta1-pyrroline-5-carboxylate synthase (P5CS) catalyses the reduction of glutamate to Delta1-pyrroline-5-carboxylate, a critical step in the biosynthesis of proline, ornithine and arginine. Recently, we reported a newly recognised inborn error due to deficiency of P5CS in two sibs, one presenting at birth w...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-004-1545-3
更新日期:2005-01-01 00:00:00
abstract::Serum immunoglobulin levels and naturally occurring antibody titres against Streptococcus pneumoniae were measured in seven children aged 1-1.9 years with recurrent pneumococcal acute otitis media (AOM). Three of them had low IgG2 levels. Mean antibody levels of anti-pneumococcal IgG1 and anti-pneumococcal IgG2 were s...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01958979
更新日期:1994-03-01 00:00:00
abstract::We describe a female infant with the Schinzel-Giedion syndrome. Features present in 11 patients include coarse face, midface retraction, urogenital anomalies, poor skull vault mineralisation and variable anomalies of the long bones. Outcome is poor and mental retardation is the rule among survivors. Prenatal diagnosis...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01955902
更新日期:1993-05-01 00:00:00
abstract::Inflammatory bowel disease (IBD) is a cause of chronic intestinal inflammation in children. In a subset of patients affected by IBD, arthropathy may be the leading presenting sign. In the past years, remarkable advances in gastrointestinal endoscopy techniques have been achieved; recently, the development of capsule e...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-011-1505-7
更新日期:2011-10-01 00:00:00
abstract::Male pseudohermaphroditism (MPH) is defined as incomplete masculinization in patients with normal male karyotype (XY) and testicular histology. MPH encompasses a spectrum of female to male phenotypes and presents both diagnostic and technical challenges to the surgeon. Once gender is assigned, based on phenotype, adeq...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02125449
更新日期:1993-01-01 00:00:00
abstract::The results of 24 h oesophageal pH monitoring, performed in 129 infants aged 6-10 weeks, were compared to those in the same patients after shorter periods (3, 6, 9 and 12 h). In the investigated population there was no significant difference between the reflux index (percentage of time with a pH < 4.0) after 12 or 24 ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01959212
更新日期:1994-02-01 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::In 238 boys with cryptorchidism, between the ages of 4 and 48 months, luteinizing hormone releasing hormone (LHRH) was administered as nasal spray 1.2 mg/day for 4 weeks. The nonresponders received human chorionic gonadotropin (HCG) 500 I.U. i.m. three times a week for 3 weeks. With the combined treatment 37.8% of tes...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02125433
更新日期:1993-01-01 00:00:00
abstract::We report on cytogenetic studies of a malformed fetus, whose clinical symptoms indicated the diagnosis of triploidy. This was confirmed by chromosome analysis of peripheral lymphocytes of cord blood. Using the C-banding method it was possible to identify the origin of the extra haploid set: marker chromosomes indicate...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442640
更新日期:1976-09-01 00:00:00
abstract:UNLABELLED:Desmopressin (DDAVP) is frequently used in the treatment of primary isolated enuresis nocturna if other approaches have failed. We report a further case of hyponatraemia and cerebral convulsion due to water intoxication after intranasal DDAVP application by a 6 year-old boy with enuresis. CONCLUSION:Althoug...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02115626
更新日期:1996-01-01 00:00:00
abstract::Staphylococcus saprophyticus commonly causes urinary tract infection (UTI) in young women; in males it is found mainly in the elderly. In this study S. saprophyticus UTI occurred in 59 children (45 girls and 14 boys) below 16 years of age, of whom 20 were less than 13 years of age. The common presentation was dysuria ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02072520
更新日期:1993-01-01 00:00:00
abstract:UNLABELLED:Growth retardation is a consistent finding in Williams-Beuren syndrome. The cause of short stature in this syndrome is unknown. Endocrine studies have failed to reveal abnormalities in the growth hormone-insulin-like growth factor I axis. We report a boy with confirmed Williams-Beuren syndrome, who was found...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051118
更新日期:1999-06-01 00:00:00
abstract::Two siblings with molybdenum cofactor deficiency are presented. They showed clinical, biochemical and neuroradiological features very similar to those of the few previously described cases. Difficulties in diagnosis are emphasised. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01955243
更新日期:1993-08-01 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::The correlation between a releasable pituitary growth hormone (GH) pool and degree of growth failure was examined in 30 children with GH deficiency (group I) and 19 children with normal short stature (group II). Based on the responsiveness of GH to GH-releasing hormone (GHRH), group I, with low GH responses (below 7 n...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442468
更新日期:1988-08-01 00:00:00
abstract::Factor VIII procoagulant activity and factor VIII related antigen were examined in 20 full-term and preterm newborn infants during the first days of life. The control group involved 15 adults volunteers. Factor VIII activity was estimated by a one-stage test and factor VIII related antigen was determined by immunelect...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00445613
更新日期:1978-07-19 00:00:00
abstract::We describe the natural history of the RTSII phenotype in a 7-year-old boy who developed intrauterine and postnatal growth retardation, failure to thrive and persisting diarrhoea. The growth hormone stimulation test identified an isolated growth hormone deficiency. Since infancy, the patient manifested skin lesions ch...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-007-0447-6
更新日期:2008-02-01 00:00:00
abstract::Congenital nephrotic syndrome (CNS) is a heterogeneous group of diseases with different causes and prognoses. Two thirds of cases of NS in the first year of life are caused by mutations in four genes (NPHS1, NPHS2, WT1, and LAMB2). The mutation of WT1 gene can lead to Denys-Drash syndrome (DDS). We report on female mo...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-011-1497-3
更新日期:2012-01-01 00:00:00
abstract:UNLABELLED:Over the last decade, substantial progress has been made in the field of pertussis research. This includes better understanding of virulence mechanisms and their influence on the pathogenicity of Bordetella pertussis, increased awareness of the broad spectrum of disease and more insight into the host's immun...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00008429
更新日期:2001-04-01 00:00:00
abstract::The role of the type-2 T helper (Th2) cell-mediated immune response in the immunopathogenesis of atopic dermatitis (AD) is well documented. Whether polarized immunoresponse is confined to antigen-specific T cells or is distributed among all T cell subsets is still controversial. We investigated frequencies of interleu...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0319-5
更新日期:2007-08-01 00:00:00
abstract:UNLABELLED:Sublingual immunotherapy (SLIT) is the only therapeutic option for allergic rhinitis (AR) that modifies the immunological process to an allergen, rather than treating symptoms simply. However, its regulatory mechanisms are largely unknown. B-cell-activating factor of the TNF family (BAFF) plays very importan...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-014-2287-5
更新日期:2014-08-01 00:00:00