Abstract:
:Twenty patients with enzymatically proven glycogen storage disease type III (GSD III) aged 3-30 years underwent cardiological evaluation. Seventeen showed subclinical evidence of cardiac involvement in form of ventricular hypertrophy on ECG. Of 16 patients in whom an ECG examination was performed, 13 had abnormal echocardiographic features. Only 2 patients had cardiomegaly on X-ray. The cardiac findings in 1 of the patients, a 25-year-old female with clinically evident cardiomyopathy are described in detail. In view of our findings, patients with established GSD III, should not only be investigated regarding their muscular involvement, but should also undergo a detailed evaluation of their cardiac status.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Moses SW,Wanderman KL,Myroz A,Frydman Mdoi
10.1007/BF00443106subject
Has Abstractpub_date
1989-08-01 00:00:00pages
764-6issue
8eissn
0340-6199issn
1432-1076journal_volume
148pub_type
杂志文章abstract::The aim of this study is to evaluate and compare mothers' and grandparents' feeding behaviours, the differences between the behaviours, the relationship between children's feeding problems and grandparents' feeding behaviours, as well as the relationship between grandparents' feeding behaviour and children's body mass...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-3244-5
更新日期:2018-12-01 00:00:00
abstract::In five L-thyroxine-substituted hypothyroid children with partial epilepsy serum total thyroxine (T4) and free T4 (FT4) significantly (P less than 0.01) decreased following 2 months of carbamazepine (CBZ) administration (20 mg/kg per BW per day) from mean (+/- SD) values of 12.7 +/- 1.1 micrograms/dl and 15.5 +/- 1.8 ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441860
更新日期:1986-04-01 00:00:00
abstract::We aimed to describe the experiences of children and adolescents with congenital heart disease (CHD). Electronic databases were searched until August 2016. Qualitative studies of children's perspectives on CHD were included. Data was extracted using thematic synthesis. From 44 studies from 12 countries involving 995 c...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-017-3081-y
更新日期:2018-03-01 00:00:00
abstract:UNLABELLED:Increased plasma tumour necrosis factor alpha (TNF) concentration correlates with mortality in sepsis. We suggested that pentoxifylline (PTXF), which is known to inhibit TNF production, may improve survival and attenuate clinical symptoms of sepsis in neonates. Plasma TNF levels were evaluated in 29 newborn ...
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/BF01955273
更新日期:1996-05-01 00:00:00
abstract::Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) are among the most common malignancies of childhood. (18)F-fluorodeoxyglucose (FDG) positron emission tomography (PET) can be employed for accurate staging, treatment planning, and response assessment in pediatric patients with lymphomas, taking advantage of the inc...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-013-1993-8
更新日期:2013-06-01 00:00:00
abstract:INTRODUCTION:IgE-dependent activation of mast cells and basophils through the high-affinity IgE receptor (Fc(epsilon)R1) is involved in the pathogenesis of allergen-induced immune responsiveness in atopic disease including bronchial asthma. MATERIALS AND METHODS:We genotyped 650 children for allelic determinants at tw...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-009-0960-x
更新日期:2009-12-01 00:00:00
abstract::A mother carrying a fetus affected with 21-hydroxylase deficiency received prenatal treatment with dexamethasone (0.5 mg, tid, p.o.) started from the very beginning of the 8th week of gestation. Prenatal diagnosis had to rely on amniocentesis with karyotyping and steroid hormone determination, because HLA and DNA data...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02190657
更新日期:1994-08-01 00:00:00
abstract:UNLABELLED:The aim of this study was to assess the physical performance in long-term survivors of acute leukaemia in childhood and to evaluate the effects of anthracycline therapy. Electrocardiography, echocardiography and spiroergometry were carried out on 56 patients aged 9-28 years, of whom 44 patients had been trea...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050854
更新日期:1998-06-01 00:00:00
abstract::A 7-year-old girl was admitted with a severe abdominal pain. Abdominal ultrasound and CT revealed a large splenic infarction, leading to the diagnosis of chronic myeloid leukemia. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1675-y
更新日期:2012-07-01 00:00:00
abstract:UNLABELLED:The objective of this study was to evaluate health-related quality of life (HRQoL) and behavioral functioning in pediatric patients with hereditary spherocytosis (HS). A cross-sectional study was conducted in 132 Dutch children and adolescents with HS and aged 8-18 years of whom 48 underwent splenectomy prio...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-014-2299-1
更新日期:2014-09-01 00:00:00
abstract::Direct measurements of parathyroid activity are available in only small numbers of children with vitamin D deficiency rickets (VDR). Therefore serum immunoreactive parathyroid hormone (iPTH) and the urinary cyclic adenosine-3',5'-monophosphate excretion (UcAMP) were measured together with other important indices of ca...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00443214
更新日期:1984-01-01 00:00:00
abstract::Forty-five children (25 girls; 20 boys) with meningomyelocele (MMC) were assessed for growth, skeletal maturation and pubertal development. The spinal defects were operated on shortly after birth and all children required cerebral drainage for hydrocephalus. Standard deviation scores for height, sitting height, sub-is...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00451900
更新日期:1985-07-01 00:00:00
abstract::Despite multiple recommendations, intramuscular epinephrine is poorly prescribed in emergency department receiving pediatric anaphylaxis. To evaluate the role of severity symptoms on this use, we included all admissions for a diagnosis linked to possible allergy in the two pediatric emergency departments of our instit...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-3246-3
更新日期:2019-01-01 00:00:00
abstract::Within a 2-year period, from March 1974 until May 1976 10 patients with anorexia nervosa were seen at the children's university hospitals of München (2 patients) and Giessen (8 patients). The patients were 10-16 years old, with one exception all girls, and had the typical cachexia, hypothermia, bradycardia, gray, hair...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00452416
更新日期:1976-12-09 00:00:00
abstract::Type I collagen is the major component of bone matrix; circulating carboxyterminal propeptide of type I procollagen (P-I-CP) levels reflect type I collagen synthesis in tissues and may be an useful index to investigate bone metabolism. We measured P-I-CP by a new radioimmunoassay in 300 healthy children and adolescent...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01959087
更新日期:1992-10-01 00:00:00
abstract:UNLABELLED:This prospective clinical case-control study describes the effect of an inpatient multicomponent treatment programme for obese children and adolescents on their weight and psychological well being. We studied 38 patients and 38 controls on the waiting list, matched for age and gender, referred because of obe...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-003-1155-5
更新日期:2003-06-01 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:UNLABELLED:Successful therapy of vasodilatory shock in adults and children with arginine-vasopressin (AVP) has been reported previously. Data on the use of vasopressin in neonates is limited. This retrospective study reports the effects of AVP-treatment in neonates with catecholamine-resistant systemic vasodilatation a...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0400-0
更新日期:2007-12-01 00:00:00
abstract::We examined the long-term outcome in 111 children who had convulsions during shigellosis and were followed for 3-18 years after the incident. No deaths or persistent motor deficits occurred as sequellae. Poor coordination of fine hand movements were noted in 3.3% of the 92 children who had no pre-existing neurological...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02106298
更新日期:1990-01-01 00:00:00
abstract::When boys affected with steroid sulfatase deficiency are delivered, the lack of the enzyme in the placenta may cause birth complications. In postnatal life this gene defect gives rise to X-linked recessive ichthyosis. In a series of 25 patients birth complications were reported in 9 cases. Of these boys, 4 displayed b...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00661898
更新日期:1983-03-01 00:00:00
abstract:UNLABELLED:Two patients are described with acute collapse caused by pericardial effusion following central venous catheterisation. Echocardiography during resuscitation allowed prompt identification and management. CONCLUSION:Pericardial effusion should be considered in any baby experiencing acute deterioration with a...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310100828
更新日期:2001-12-01 00:00:00
abstract:UNLABELLED:In very low birth weight (VLBW) infants, colloid infusion is associated with impaired perinatal lung function and increased oxygen dependency duration. The aim of this study was to determine whether perinatal colloid infusion was associated with abnormal neurodevelopmental outcome. All perinatal fluid input ...
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/s00431-002-0950-8
更新日期:2002-06-01 00:00:00
abstract::Adolescence obesity and depression are increasingly prevalent and are associated with various health complications. The aim of this study was to examine the association between weight status (normal weight, overweight, and obese) and depressive symptoms in adolescents and to explore the probable mediators, including o...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1753-1
更新日期:2012-08-01 00:00:00
abstract::Triple A syndrome (TAS) or Allgrove syndrome (OMIM #231550) is a rare autosomal recessive disorder characterised by adrenocorticotropic hormone-resistant adrenal insufficiency, alacrima, achalasia, and neurological and dermatological abnormalities. Mutations in the AAAS gene on chromosome 12q13 encoding the nuclear po...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-017-3068-8
更新日期:2018-03-01 00:00:00
abstract:UNLABELLED:Click-evoked oto-acoustic emissions (CEOAE) are acoustic responses produced by the inner ear, reflecting functional auditory integrity. We studied both the success rate of the CEOAE method and the CEOAE presence in preterm infants during their stay at the Neonatal Intensive Care Unit (NICU), by analysis of t...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050986
更新日期:1998-12-01 00:00:00
abstract:UNLABELLED:Atypical hemolytic uremic syndrome (HUS) refers to the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury in the absence of Shiga toxin-producing Escherichia coli exposure or Streptococcus pneumoniae infection. Currently, approximately 50 % of the atypical cases have demons...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1763-z
更新日期:2012-10-01 00:00:00
abstract::The risk factors for recurrent apparent life-threatening event (ALTE) are unclear although the risk of recurrent ALTE is an important consideration for the management of ALTE patients. This study aimed to identify the risk factors for recurrent ALTE. We conducted a secondary analysis of the data from a single center r...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-017-2855-6
更新日期:2017-04-01 00:00:00
abstract::Retraction Note to: Eur J Pediatr (1994) 153:668-671 DOI 10.1007/BF02190689. This article, "Adenosine triphosphate treatment for supraventricular tachycardia in infants", published in the European Journal of Pediatrics (1994) 153/9: 668-671, Springer-Verlag 1994, has been retracted at the request of the Publisher as i...
journal_title:European journal of pediatrics
pub_type: 撤回出版物
doi:10.1007/s00431-015-2640-3
更新日期:2015-11-01 00:00:00
abstract::This systematic review and meta-analysis assessed the effects of early fortification (EF) versus late fortification (LF) of breast milk (BM) on growth of preterm infants. Randomized and quasi-randomized controlled trials (RCTs) dealing with the effects of EF versus LF on growth parameters, incidence of adverse events,...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-020-03677-6
更新日期:2020-07-01 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