Abstract:
:Following a description of the genetic aspects of the human histocompatibility antigens system HLA and its principle typing methods, this paper reviews the relationship between HLA antigens, transplantation immunology and certain diseases. In particular, the role of the lymphocyte-defined antigens of the HLA-D system is emphasized on the basis of a special typing method, the PLT test, used in our laboratory. Aside from its necessity in bone marrow and kidney transplantation, HLA typing can be used as an additional diagnostic or prognostic tool for certain diseases. Among the pediatric age group, this includes rheumatic fever and other rheumatic diseases, insulin-dependent juvenile diabetes mellitus, some forms of Addison's disease, thyrotoxicosis, myasthenia gravis, celiac disease, and some complement deficiency disorders. Close linkage of the HLA system with steroid 21-hydroxylase deficiency has made it possible to diagnose this form of congenital adrenal hyperplasia in utero. This approach is illustrated in a large family at risk for this disorder.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Passarge E,Valentine-Thon Edoi
10.1007/BF00441576subject
Has Abstractpub_date
1980-03-01 00:00:00pages
93-100issue
2eissn
0340-6199issn
1432-1076journal_volume
133pub_type
杂志文章,评审abstract::The name of the co-author Wendy J. Ungar was inadvertently omitted on the original published article. Her name and affiliation have now been added to the author list. ...
journal_title:European journal of pediatrics
pub_type: 已发布勘误
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abstract::Clinical, morphological, ultrastructural and immunological studies were performed in a case of congenital self-healing non-Langerhans cell histiocytosis. The patient showed several aspects that have not been published before: a large nodule in the vulvar region, vesiculobullous elements and pneumonia (asymptomatic). T...
journal_title:European journal of pediatrics
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abstract::Our prior studies of the molecular epidemiology of group A streptococcus (GAS) pharyngitis indicated that the most common emm types associated with pediatric pharyngitis in North America were 12, 1, 28, and 4. We previously reported that the proportions of pediatric pharyngitis due to emm types 12 and 4 decreased with...
journal_title:European journal of pediatrics
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更新日期:2009-06-01 00:00:00
abstract::The primary objective of this study was to evaluate whether pediatric respiratory rate-oxygenation index (p-ROXI) and variation in p-ROXI (p-ROXV) can serve as objective markers in children with high-flow nasal cannula (HFNC) failure. In this prospective, single-center observational study, all patients who received HF...
journal_title:European journal of pediatrics
pub_type: 杂志文章
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更新日期:2020-10-19 00:00:00
abstract::Soon after birth a 36-week-gestational age, appropriate for dates, newborn infant developed hyperinsulinaemic hypoglycaemia which responded to diazoxide and glucose infusion. While normoglycaemic, he became increasingly ill with high lactate, pyruvate, and alanine levels. Provocation tests suggested a defect in lactat...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00496802
更新日期:1983-12-01 00:00:00
abstract::Given the worldwide increase prevalence of overweight, obesity, and nonalcoholic fatty liver disease (NAFLD), the objective of this study was to evaluate whether the triglycerides and glucose (TyG) index is associated with hepatic steatosis in children with overweight or obesity. Apparently healthy children aged 5‑17 ...
journal_title:European journal of pediatrics
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abstract::A South African family with autosomal dominant split hand, split foot (SHSF) malformation has been studied. The pedigree was unusual in that three unaffected siblings with normal parents had each produced affected offspring. New mutation, germinal mosaicism and autosomal recessive inheritance are unlikely. Possible ex...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442225
更新日期:1988-02-01 00:00:00
abstract::Infantile hepatic hemangioma is the most common benign liver tumor during infancy. Prompt diagnosis and timely institution of therapy are of utmost importance. Magnetic resonance imaging (MRI) plays a key role in the correct diagnosis and monitoring of treatment. We report on a 15-week-old girl with multifocal infanti...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-011-1671-7
更新日期:2012-07-01 00:00:00
abstract:UNLABELLED:A 6-year-old male with partial ornithine transcarbamylase (OTC) deficiency had acute and rapidly progressive symmetrical swelling of the head of the caudate nuclei and putamina. Clinical presentation was ataxia and dysarthria progressing to seizures and coma; these symptoms gradually resolved with supportive...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-002-1135-1
更新日期:2003-04-01 00:00:00
abstract:UNLABELLED:Persistent müllerian duct syndrome is a relatively rare inherited defect of sexual differentiation characterised by failure of regression of the müllerian ducts in males. In affected individuals, uterus and tubes are present because of defects of synthesis or action of anti-müllerian hormone (AMH), normally ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310100840
更新日期:2001-11-01 00:00:00
abstract:UNLABELLED:Convincing evidence of moderate hyperhomocysteinaemia as a risk factor for vascular disease has accumulated within the last decade being independent of conventional risk factors and equally strong as hypercholesterolaemia and smoking. A synergistic interaction with hypertension and smoking in their joint art...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00014292
更新日期:1998-04-01 00:00:00
abstract::To determine the relationship between total serum bilirubin (TSB) during the first 2 days of life and subsequent neonatal morbidity in very low birth weight (VLBW, less than 1500 g) infants. We performed a prospective study of 582 VLBW infants born between July 1, 2005 and December 31, 2009. TSB was measured in umbili...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-011-1634-z
更新日期:2012-04-01 00:00:00
abstract:UNLABELLED:Hypotension is a commonly diagnosed and treated complication of extremely low gestational age newborns (ELGAN), but enormous variation in diagnosis, management and clinical practice has been documented. We sought to evaluate practice regarding the management of hypotension in ELGANs and developed a web-based...
journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
doi:10.1007/s00431-013-2251-9
更新日期:2014-06-01 00:00:00
abstract::A case of subacute meningitis caused by Brucella mellitensis is described. The meningitis was refractory to an antibiotic regime. Diagnosis was established by raised antibody titres to Brucella in serum and cerebrospinal fluid (CSF) and positive bone marrow and blood cultures. While treatment with tetracycline trimeth...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00445917
更新日期:1988-11-01 00:00:00
abstract::Portal hypertension was observed in a 17-year-old girl with urticaria pigmentosa since 2 months of age. Liver biopsies showed portal and sinusoidal infiltration with mast cells although spleen biopsies showed only fibrosis. CONCLUSION. Portal hypertension is a complication of systemic mastocytosis that can lead to dea...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01959789
更新日期:1995-10-01 00:00:00
abstract::West syndrome or infantile spasms is one of the most frequent epileptic syndromes in the first year of life. The clinical symptoms of infantile spasms are very different than any other type of seizure because of both the absence of paroxysmal motor phenomena (i.e., as in a convulsion) and the lack of significant durat...
journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
doi:10.1007/s00431-012-1813-6
更新日期:2012-11-01 00:00:00
abstract::A Japanese patient presented with lymphedema, severe Varicella zoster, and Salmonella infection, recurrent respiratory infections, panniculitis, monocytopenia, B- and NK-cell lymphopenia, and myelodysplasia. The phenotype was a mixture of the monocytopenia and mycobacterial infection (MonoMAC) and Emberger syndromes. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1715-7
更新日期:2012-08-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-013-2088-2
更新日期:2014-12-01 00:00:00
abstract::In our clinic the decision on whether to continue with dietary treatment of phenylketonuria or not is left to each adolescent and adult patient after the advantages and disadvantages, as discussed in this paper, of continuing diet have been presented to them. As a result 61 of 132 patients have stopped diet or decline...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/pl00014261
更新日期:1996-07-01 00:00:00
abstract::Early identification of infants being at high risk to become obese at their later childhood or adolescence can be of vital importance in any obesity prevention initiative. The aim of the present study was to examine the utility and applicability of the "Childhood Obesity Risk Evaluation (CORE)" index as a screening to...
journal_title:European journal of pediatrics
pub_type: 杂志文章
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abstract:UNLABELLED:Patients diagnosed with intracranial teratoma are at risk for developing a recurrent malignant germ cell tumor. We describe a 14-year-old boy initially diagnosed with a mature teratoma in the pineal region that recurred as a metastatic beta-human chorionic gonadotropin (βHCG)-secreting germ cell tumor 3 year...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-014-2282-x
更新日期:2014-08-01 00:00:00
abstract::This study aimed to estimate the number of infants who died of unrecognized congenital adrenal hyperplasia (CAH) in Austria and the Czech Republic within the past 13 years, before the introduction of adequate neonatal screening. The study was based on retrospective analysis of neonatal screening cards of 242 infants w...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0154-8
更新日期:2007-01-01 00:00:00
abstract::Health-related quality of life (HRQOL) is an important indicator of the burden of illness in moderate-to-severe plaque psoriasis. This study evaluated self-reported generic HRQOL among pediatric patients with moderate-to-severe plaque psoriasis based on pooled baseline clinical trial data and compared them to four com...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-011-1587-2
更新日期:2012-03-01 00:00:00
abstract:OVERVIEW:In newborns with symptomatic congenital cytomegalovirus (CMV) infection, neuroimaging is the best available predictor of neurodevelopmental outcome. Cerebrospinal fluid (CSF) findings in congenital CMV infection have seldom been described. Neonates with central nervous system infections present high CSF Beta(2...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0160-x
更新日期:2006-09-01 00:00:00
abstract:UNLABELLED:An isolated eosinophilic granuloma involving the posterior elements of a lumbar vertebra is reported in a 3-year-old boy presenting with progressive limp. Radiological investigations revealed osteolysis of the L5 right pedicle. MRI showed a well-defined homogeneous mass with nonspecific signal intensity. An ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051097
更新日期:1999-05-01 00:00:00
abstract::We report the 9-year follow-up of a patient suffering from N-acetylglutamate synthetase deficiency, an urea cycle disorder leading to severe neonatal hyperammonaemia. Hitherto two patients from two families with this inborn error of metabolism had been observed. Our management consisted mainly of a protein-restricted ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01955939
更新日期:1991-03-01 00:00:00
abstract:UNLABELLED:In a 2 year period seven patients who presented with stridor, without respiratory compromise, and three patients without obstructive symptoms were prospectively selected, and underwent MRI. In eight patients with a vascular ring and a pulmonary sling, MRI delineated the vascular abnormality and normal great ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050651
更新日期:1997-07-01 00:00:00
abstract:UNLABELLED:A postal survey of 31 European centres was conducted to document current practices regarding screening and management of hepatitis C virus (HCV)-infected pregnant women and their children. Antenatal HCV prevalence was low. Universal antenatal screening programmes were in place in ten centres, selective scree...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051219
更新日期:1999-10-01 00:00:00
abstract::Cough and wheezing are the predominant symptoms of acute bronchitis. Hitherto, the evaluation of respiratory symptoms was limited to subjective methods such as questionnaires. The main objective of this study was to objectively determine the time course of cough and wheezing in children with acute bronchitis. The impa...
journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s00431-019-03426-4
更新日期:2019-09-01 00:00:00
abstract::The long-term consequences of transient neonatal hypoglycemia are sparsely studied. We performed a follow-up of a cohort of neonates with blood glucose recordings < 1.7 mmol/L (< 30 mg/dL), treated with > 2.5 mmol/L (> 45 mg/dL), compared with healthy siblings. Exclusion criteria were gestational age < 35 weeks, sever...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-020-03729-x
更新日期:2020-12-01 00:00:00