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 death. Treatment with interferon alpha might be effective.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Fonga-Djimi HS,Gottrand F,Bonnevalle M,Farriaux JPdoi
10.1007/BF01959789subject
Has Abstractpub_date
1995-10-01 00:00:00pages
819-21issue
10eissn
0340-6199issn
1432-1076journal_volume
154pub_type
杂志文章abstract::To access outcome following hypoxic ischemic encephalopathy (HIE), survivors without cerebral palsy were invited for formal developmental assessment. Children aged ≥ 42 months were assessed using the NEPSY-2, Movement Assessment Battery for Children 2 (Movement ABC-2), Behavior Rating Inventory of Executive Function, ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-017-3028-3
更新日期:2018-01-01 00:00:00
abstract:INTRODUCTION:Reduced concentrations of glucose-6-phospate dehydrogenase (G6PD) render erythrocytes susceptible to hemolysis under conditions of oxidative stress. In favism, the ingestion of fava beans induces an oxidative stress to erythrocytes, leading to acute hemolysis. DISCUSSION:The simultaneous occurrence of met...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-009-0952-x
更新日期:2009-07-01 00:00:00
abstract::Education is necessary to improve child physical abuse detection and management. A few studies have described national child abuse training programs, but none has measured changes in knowledge among participants. A collaboration of child abuse experts from the USA, an academic pediatric department, and a non-governmen...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-020-03625-4
更新日期:2020-09-01 00:00:00
abstract:UNLABELLED:Intensive multimodality treatment has led to a remarkable improvement of prognosis in paediatric cancer patients, however, a great number of long-term survivors suffer from considerable tumour- or treatment-related late effects. Between January 1990 and December 1998, 223 consecutive survivors of childhood m...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/pl00008340
更新日期:2000-10-01 00:00:00
abstract::Severe neonatal centronuclear myopathy is inherited as an X-linked condition characterized by primary asphyxia, extreme muscular hypotonia and absent spontaneous movements. We report seven cases from three families to point out the importance of diagnosis with regard to prognosis, outcome and genetic counselling. In h...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02072056
更新日期:1990-12-01 00:00:00
abstract::Medical clowns have an important role in helping patients cope with their pain and distress. This is especially true in the pediatric population. However, their activity in a disaster area is unheard of. Following the Nepal earthquake in 2015, the Israeli field hospital set up in Kathmandu, Nepal was joined by five vo...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-017-3018-5
更新日期:2018-02-01 00:00:00
abstract::Growth hormone (GH) secretion was determined by evaluating ultradian GH profiles for 12 h and GH responses to insulin stimulated hypoglycaemia (ITT) in 28 stunted boys with beta-thalassaemia major aged 15.2-17.4 years, who presented with pubertal failure (FP). Healthy non thalassaemia prepubertal boys (n = 10) aged 7....
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01953984
更新日期:1993-09-01 00:00:00
abstract::An 8-month-old boy and a 7-month-old girl presented with an acute, Coombs-positive auto-immune haemolytic anaemia and severe hepatitis. The clinical manifestations were pallor, jaundice and hepatomegaly. The liver histology revealed diffuse giant cell transformation and extensive necrosis with central-portal bridging....
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01955929
更新日期:1991-03-01 00:00:00
abstract:UNLABELLED:Primary spontaneous pneumothorax (PSP) is not uncommon, and its recurrence is often a challenging clinical problem. Surgical management and predisposing factors for the recurrence of PSP, however, have not yet been well elucidated in adolescent patients. The major aim of this study was to investigate factors...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-014-2352-0
更新日期:2014-11-01 00:00:00
abstract:UNLABELLED:Compliance by residents in pediatrics to pediatric resuscitation guidelines is low. In many French faculties, a 1-h traditional lecture is still used to educate medical students about pediatric cardiopulmonary arrest (CPA). We developed an innovative pedagogic course combining a 23-min video and 3-h simulati...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-016-2702-1
更新日期:2016-06-01 00:00:00
abstract:BACKGROUND:We aimed to determine the status of and factors associated with adolescent health care delivery and training in Europe on behalf of the European Paediatric Association-UNEPSA. MATERIALS AND METHODS:A questionnaire was mailed to the presidents of 48 national paediatric societies in Europe. For statistical an...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-008-0759-1
更新日期:2009-04-01 00:00:00
abstract:UNLABELLED:Transient hypothyroxinaemia with normal thyroid stimulating hormone (TSH) levels is a well-known condition in preterm neonates and is generally assumed to be a harmless epiphenomenon of prematurity. This assumption is, however, based on studies that included very few neonates with a gestational age (GA) belo...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01953940
更新日期:1996-03-01 00:00:00
abstract::Two patients with chronic granulomatous disease who had previously been intolerant to trimethoprim-sulfamethoxazole because of various adverse reactions completed a desensitisation protocol with a favourable clinical outcome. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-002-0944-6
更新日期:2002-06-01 00:00:00
abstract::An adapted cow's milk formula with or without supplemental taurine (480 mumol/l) was fed for 16 weeks to 20 low-birth-weight infants. In the 2nd and 16th weeks of life, respectively, the following parameters were determined: growth, sonography of heart and brain, ECG, EEG, neurological development and the taurine conc...
journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章
doi:10.1007/BF00442699
更新日期:1988-04-01 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:UNLABELLED:Familial Mediterranean fever (FMF) is characterised by recurrent fever and serositis. The most important complication of the disease is amyloidosis. Cheap and non-invasive methods would be important in predicting or establishing the early diagnosis of amyloidosis. For this purpose, we studied the role of uri...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-003-1173-3
更新日期:2003-05-01 00:00:00
abstract::We report two children with hemolytic anemia during the course of hepatitis A infection. On admission, the patients had high blood urea nitrogen, creatinine, and uric acid levels, as well as anemia, leucocytosis, and direct and indirect hyperbilirubinemia. Both patients had a glucose-6-phosphate dehydrogenase deficien...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-008-0694-1
更新日期:2008-12-01 00:00:00
abstract::Positional plagiocephaly (PP) denotes flattening of the skull that occurs frequently in healthy infants. Aim of this study was to estimate the prevalence of positional plagiocephaly and to identify the risk factors in a cohort of healthy infants in order to help prevention of PP. In a prospective design, all healthy f...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-3212-0
更新日期:2018-10-01 00:00:00
abstract::Shwachman-Diamond syndrome (SDS) is an autosomal recessive disorder characterised by skeletal dysplasia, exocrine pancreatic insufficiency and bone marrow failure. Various other conditions, such as hepatopathy and failure to thrive have been associated with SDS. A retrospective study was conducted to describe mutation...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1908-0
更新日期:2013-05-01 00:00:00
abstract:UNLABELLED:The treatment of blunt splenic injuries (BSI) has undergone a significant shift away from an operative approach to a conservative treatment regimen in the last decades. Data concerning long-term follow-up of children sustaining BSI are largely confined to telephone surveys. Children treated with BSI over a 3...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-013-2022-7
更新日期:2013-09-01 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:UNLABELLED:The severity and duration of immunosuppression caused by corticosteroids (CSs) usage have not been extensively studied. We aimed to investigate the effects of CSs on the various compartments of immune system in relation to timing of initiation and persistence of therapy. Pediatric patients with idiopathic ne...
journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章
doi:10.1007/s00431-016-2694-x
更新日期:2016-05-01 00:00:00
abstract:UNLABELLED:A total of 22 Japanese patients with hypophosphatasia were included in a study analysing the relationship between mutations in the tissue-nonspecific alkaline phosphatase (TNSALP) gene and the severity of the phenotype in Japanese patients with hypophosphatasia. The enzymatic activity of some of the identifi...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-004-1612-9
更新日期:2005-05-01 00:00:00
abstract::Familial hemophagocytic lymphohistiocytosis (FHL) is probably a genetically transmitted disease affecting infants and very young children. Cardinal symptoms are fever, hepatosplenomegaly, and pancytopenia. Frequently meningeal involvement is seen, manifested by neurologic symptoms and a lymphohistiocytic pleocytosis w...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF00443367
更新日期:1983-06-01 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:UNLABELLED:We describe the case of a boy with steroid sensitive nephrotic syndrome and left pulmonary artery thrombo-embolism. clinical presentation initially suggested sepsis and respiratory signs were minor. Treatment with tissue plasminogen activator infused into the pulmonary artery was successful. CONCLUSION:Pulm...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050684
更新日期:1997-08-01 00:00:00
abstract:UNLABELLED:Gaucher disease is an inherited pan-ethnic disorder that commonly begins in childhood and is caused by deficient activity of the lysosomal enzyme glucocerebrosidase. Two major phenotypes are recognized: non-neuropathic (type 1) and neuropathic (types 2 and 3). Symptomatic children are severely affected and m...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-012-1771-z
更新日期:2013-04-01 00:00:00
abstract::Twenty patients with rheumatoid arthritis or Still's disease associated with growth failure were treated with human growth hormone, 7.5 to 17 U/m2 body surface per week. Five patients did not respond with better growth. In the remainder the mean growth rate increased from 1.9 cm/year (range: 0 to 3.3) to 6.2 cm/year (...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441894
更新日期:1979-01-18 00:00:00
abstract::We aimed to determine whether precocious adrenarche (PA) has a different impact on screening tests for metabolic issues and pubertal timing in boys and girls born appropriate for gestational age (AGA). Puberty and initial metabolic screening results of 47 girls and 23 boys with PA born AGA followed up from our outpati...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1796-3
更新日期:2012-11-01 00:00:00
abstract::We report a female newborn with focal dermal hypoplasia (Goltz-Gorlin Syndrome) and marked asymmetric malformations on the right side of the body. Diaphragmatic hernia on the same side, which has not been reported in this syndrome, led to perinatal complications. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00538945
更新日期:1979-06-28 00:00:00