Abstract:
:A girl, 13 months of age, presented with generalised granulomatous skin, hair and mucosal candidiasis. Her lymphocytes failed to respond in vitro to Candida antigen (CA); the intradermal test with CA was also negative. Serum immunoglobulins, complement components, granulocyte functions (phagocytic and fungicidal), T-cell subsets, mitogenic and allogenic lymphocyte stimulation, natural killer cell activity and immune interferon production were all found to be normal. No circulating immune complexes were detected. Ketoconazole, an antimycotic drug, 5 mg/kg twice daily for 1 month and 2.5 mg/kg twice daily for another month spectacularly cleared all lesions. Afterwards, 4-monthly injections with transfer factor (TF) were given. Intradermal reactivity to CA was observed after the second TF injection. The lymphocyte responsiveness to CA in vitro became strongly positive 3 months after the last TF injection. The level of CA precipitins in serum, which was very high (11 lines) before ketoconazole treatment, decreased to 4 lines. No serum inhibitor of lymphocyte proliferation to CA could be demonstrated in the patient's serum before or after treatment. This specific CA unresponsiveness was not due to an excess of OKT8 + (suppressor) cells; macrophage migration inhibiting factor (MIF) production was normal. The nonresponsiveness might be due to antigenic overload or to suppressor cell induction not demonstrable in the present studies. The child has remained free of lesions during 3 years of follow-up without any further treatment.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Corbeel L,Ceuppens JL,Van den Berghe G,Claeys H,Casteels-Van Daele Mdoi
10.1007/BF00442747subject
Has Abstractpub_date
1984-11-01 00:00:00pages
45-8issue
1eissn
0340-6199issn
1432-1076journal_volume
143pub_type
杂志文章abstract::Serum lysozyme activity was measured in samples from children with acute leukemia, malignant tumours, and in normal children. All children with acute lymphatic leukemia (ALL) had significantly reduced levels of lysozyme at diagnosis, and none of the children fell within the normal range. Children with ALL in complete ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00493542
更新日期:1978-04-20 00:00:00
abstract::The renal function of 12 patients with non vitamin B12 responsive methylmalonic acidaemia has been investigated. Eight patients had reduced glomerular filtration rates, but the plasma creatinine concentration was only raised in those with values of less than 40 ml/min per 1.73 m2 surface area. The reduction in glomeru...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00444131
更新日期:1989-01-01 00:00:00
abstract::Despite rapidly evolving knowledge about COVID 19 infection, routes of perinatal COVID 19 transmission and viral load in mother neonate dyad remain uncertain. Data were analysed to describe the clinicodemographic profile and viral load in neonates born to COVID 19 positive mothers. Of 2947 deliveries, 69 mothers were ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-020-03800-7
更新日期:2021-02-01 00:00:00
abstract::The Rubinstein-Taybi syndrome is a condition characterized by mental retardation, typical facial changes and broad thumbs and big toes. The cause is unknown; almost all cases are sporadic. We describe a mother and son with Rubinstein-Taybi syndrome. Literature search documented at least 413 cases with 558 sibs. An aff...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00595907
更新日期:1989-02-01 00:00:00
abstract:UNLABELLED:Gain-of-function mutations of the extracellular calcium (Ca(2+)e)-sensing receptor (CaR) have been identified in patients with familial and sporadic hypercalciuric hypocalcaemia. We describe a patient with sporadic severe hypercalciuric hypocalcaemia with undetectable or very low levels of serum parathyroid ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-003-1331-7
更新日期:2004-02-01 00:00:00
abstract::An autosomal dominant hereditary bone dysplasia was observed in three members of a Sardinian family living in Germany--the father and two sons. The guiding clinical symptom is thickening of the wrist proximal to the styloid process of the ulna. The radiologically recognizable changes--non-calcified cartilaginous islan...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441850
更新日期:1986-04-01 00:00:00
abstract:UNLABELLED:To monitor infant care practices associated with risks for sudden infant death, 400 Belgian families with infants less than 6 months old were questioned by 21 paediatricians during routine visits to local paediatric practices and well baby services (11 in urban centres, 10 in rural areas). Because of incompl...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310100783
更新日期:2001-08-01 00:00:00
abstract:UNLABELLED:The aims of this study were to analyze reproductive outcomes of women and men born very preterm (gestational age <32 weeks) or with a very low birth weight (<1500 g) in 1983 in the Netherlands and to compare their reproductive outcomes with the total population at a similar age of 28 years. Young adults who ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-014-2470-8
更新日期:2015-06-01 00:00:00
abstract:UNLABELLED:Neurological complications of immunizations are rare. We report the case of relapsing acute encephalitis in a boy after two subsequent diphtheria-tetanus-poliomyelitis vaccinations. First the clinical signs were those of acute disseminated encephalitis. During the second episode, the boy experienced optic ne...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02075768
更新日期:1996-02-01 00:00:00
abstract::We report here our experience in the long-term management of 28 patients with citrullinaemia, 13 patients with carbamoyl phosphate synthase deficiency and 15 patients with argininosuccinic aciduria. In addition, we report a national French survey of 119 patients with ornithine transcarbamylase (OTC) deficiency enzymat...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/pl00014323
更新日期:1999-12-01 00:00:00
abstract::Diagnostic tools for the management of acute osteomyelitis (OM) and septic arthritis (SA) have improved over the last decade. To investigate the influence and availability of magnetic resonance imaging (MRI) and nucleic acid testing (NAT), a retrospective cohort study was done. Patients admitted with acute OM or SA be...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-3157-3
更新日期:2018-07-01 00:00:00
abstract:UNLABELLED:Chronic lung disease (CLD) is an inflammatory disorder; in patients with other inflammatory disorders exhaled nitric oxide (NO) levels are elevated. The aim of this study was to test the hypothesis that prematurely born infants with CLD would have elevated exhaled NO levels compared to those without CLD and ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-004-1480-3
更新日期:2004-09-01 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::We updated our 2010 systematic review on the efficacy of probiotics in the treatment of constipation in children. The MEDLINE, EMBASE, and Cochrane Library databases; clinical trial registries; and reference lists of included studies were searched to February 2017 for randomized controlled trials (RCTs) performed in c...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-017-2972-2
更新日期:2017-09-01 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::Since traction-associated hypertension seems to be a relatively unknown phenomenon, a survey was done of its incidence in children treated with skeletal traction for fractures and orthopaedic diseases. The correlation with hypercalcaemia, a possible aetiological factor, was also explored. Blood pressure was recorded t...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01957765
更新日期:1992-07-01 00:00:00
abstract:INTRODUCTION:Hypothermia at birth is strongly associated with mortality and morbidity in pre-term infants. BACKGROUND:A local audit showed limited effectiveness of occlusive wrapping in preventing admission hypothermia in very pre-term infants. Self-heating acetate gel mattresses were introduced as a result to prevent...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-009-1113-y
更新日期:2010-07-01 00:00:00
abstract:UNLABELLED:We report on a 5-year-old girl with an unusually severe course of tick-borne encephalitis following passive immunization with specific hyperimmune globulin after a tick bite in an endemic area. Serial investigations of the CSF revealed intrathecal production of specific antibodies paralleled by increasing nu...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02002905
更新日期:1996-09-01 00:00:00
abstract::This study is a retrospective analysis of seven patients with hypoechogenic lesions in the liver and/or spleen due to Bartonella henselae, who were followed from 1998 through to 2005 by the Department of Pediatrics, Turin University. In addition to showing constitutional symptoms, four children had skin lesions sugges...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-007-0500-5
更新日期:2008-04-01 00:00:00
abstract:PURPOSE:The study investigated the television watching habits of children in hospital compared with those at home and the factors which influence them. METHODS:A random sample of 546 school aged children hospitalized in paediatric hospitals (2) in Athens (Greece) was studied. RESULTS:Children's television watching ti...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0166-4
更新日期:2006-11-01 00:00:00
abstract::Blunt cardiac injury may occur in patients after suffering nonpenetrating trauma of the chest. It encompasses a wide spectrum of cardiac injury with varied severity and clinical presentation. Electrocardiographic abnormalities are frequently encountered. This article presents a case of a child who presented with compl...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-007-0663-0
更新日期:2008-11-01 00:00:00
abstract:UNLABELLED:Children with a painful hip present a diagnostic challenge since clinical differentiation between septic arthritis, transient synovitis and Perthes disease may be difficult. Septic arthritis, a potentially life-threatening and debilitating medical emergency, requires early recognition for successful treatmen...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051243
更新日期:1999-11-01 00:00:00
abstract::Renal tubular dysgenesis is a critical disorder characterized by the Potter phenotype and severe hypotension in the early neonatal period. We herein report a 3-year-old female with renal tubular dysgenesis. Endocrinological studies showed a high plasma renin activity (over 49.2 ng/ml/h; normal range 2.0-15.2), high ac...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-008-0743-9
更新日期:2009-02-01 00:00:00
abstract::An asthmatic child is presented who developed a cushingoid appearance with evidence of adrenal suppression and growth impairment while on low dose inhaled topical steroids. When the inhaled steroids were replaced by inhaled sodium cromoglycate his adrenal function recovered while his appearance and growth returned to ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02093730
更新日期:1991-04-01 00:00:00
abstract:UNLABELLED:Congenital hydrocephalus is a serious condition that can arise from multiple causes. It comprises a diverse group of conditions which result in impaired circulation and absorption of cerebrospinal fluid. Congenital malformations of the central nervous system, infections, haemorrhage, trauma, teratogens and, ...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s004310050830
更新日期:1998-05-01 00:00:00
abstract::One hundred and twenty two cases of severe hyaline membrane disease are reported. 68 of them survived (57%). Adverse clinical, radiological and laboratory factors, and their effects on the early mortality rate, are analysed with particular reference to the referring centers, delay in admission, transport and the criti...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442059
更新日期:1978-03-13 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::A 22 years old female with the late-onset of argininosuccinic aciduria was successfully treated for 5 months with a mixture of essential amino acids and their keto-analogues. There was a marked change in plasma ammonia, plasma amino acids and argininosuccinic acid excretion. A long term anabolic response was reflected...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00445607
更新日期:1978-07-19 00:00:00
abstract::In 166 full term, small-for-gestational-age (FT-SGA), 53 preterm, appropriate-for-gestational-age (PT-AGA), 27 PT-SGA and 206 FT-AGA infants a neurological examination at the age of 6 years was carried out. Data were collected on behaviour and school achievement. Major and minor neurological abnormalities were more fr...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441967
更新日期:1988-06-01 00:00:00
abstract::We report here our findings in two Japanese siblings who experienced recurrent bacterial and viral infections since early infancy. Recent symptoms included diarrhoea, conjunctivitis, rashes, headache, sore throat, joint pain, vomiting and vertigo, all similar to those seen in toxic shock syndrome, except for shock. Th...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01956412
更新日期:1994-05-01 00:00:00