Abstract:
UNLABELLED:Endocrine dysfunction and damage of the epiphysial growth plates have been reported as late effects of antileukaemic treatment during childhood. It is a common opinion that cranial irradiation (CI) is the most important factor for blunted growth. Accordingly, recent therapeutic strategies in acute lymphoblastic leukaemia (ALL) avoid cranial irradiation. Here we analysed longitudinal data on growth and puberty of 54 children in first complete remission, who were treated with 18 Gy CI or not submitted to radiotherapy. Two chemotherapeutic protocols were compared which were similar during the induction period but differed in the intensity of maintenance therapy. In cranial irradiated patients both in males and females the pubertal growth spurt started at a mean age of 1.2 years (SD: 0.93 years) earlier than controls. Age at diagnosis and age at pubertal growth spurt were significantly correlated (r = 0.35, P = 0.017). Similarly, menarche occurred at a mean age (n = 22) of 12.1 years and was correlated with the age at start of therapy in girls who were treated with 18 Gy CI (r = 0.61, P = 0.01). Adult height was reached spontaneously in 30 patients treated during prepubertal age and in 10 treated shortly before or during puberty. In all prepubertal patients treated for 2-3 years with intensive maintenance therapy blunted growth resulted in a significant loss of -1.85 H-SDS (median, P = 0.0051) compared to height at diagnosis. However, if continuation treatment used only methotrexate and 6-mercaptopurine (i.e. BFM protocol) final height equalled projected adult height, despite 18 Gy CI. CONCLUSIONS:(1) multiagent chemotherapy is of major impact for growth and puberty; (2) 18 Gy cranial irradiation is below the critical dosage responsible for blunted growth; (3) loss in potential growth might be prevented by current CT strategies; (4) onset of puberty depends on age when antileukaemic therapy is applied.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Mohnike K,Dörffel W,Timme J,Kluba U,Aumann V,Vorwerk P,Mittler Udoi
10.1007/s004310050599subject
Has Abstractpub_date
1997-04-01 00:00:00pages
272-6issue
4eissn
0340-6199issn
1432-1076journal_volume
156pub_type
杂志文章abstract::The phenylalanine hydroxylase (PAH) deficiency trait is heterogeneous with a continuum of metabolic phenotypes ranging from classical phenylketonuria (PKU) to mild hyperphenylalaninaemia (MHP). More than 200 mutations in the PAH gene are associated with PAH deficiency. From theoretical considerations or in vitro expre...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00014253
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::Cardiac imaging has had significant influence on the science and practice of pediatric cardiology. Especially the development and improvements made in non-invasive imaging techniques, like echocardiography and cardiac magnetic resonance imaging (MRI), have been extremely important. Technical advancements in the field ...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-007-0544-6
更新日期:2008-01-01 00:00:00
abstract::A detailed autopsy study of three children with ornithine carbamoyl transferase (OCT) deficiency is presented. Although variable in extent, a basic pattern of neuropathological lesions is discernible. Case 1 shows gross cerebral atrophy, cases 2 and 3 milder lesions in the basal nuclei but also multiple cerebellar het...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00572763
更新日期:1984-02-01 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:Neutrophils are an essential component of the human host defence system against infection. Recombinant human granulocyte colony-stimulating factor induces neutrophilia and enhances effector functions of mature neutrophils. Since the biological effects of granulocyte colony-stimulating factor (G-CSF) are medi...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051129
更新日期:1999-06-01 00:00:00
abstract:UNLABELLED:Tracheal bronchus (TB) associated with VACTERL has not been reported previously. A 5-month-old girl with VACTERL association was ventilator-dependent following surgical closure of a patent ductus arteriosus (PDA). Chest radiographs showed persistent hyperinflation of the right upper lobe. Bronchoscopy showed...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-002-1109-3
更新日期:2003-03-01 00:00:00
abstract::We describe here an infant with a large, solitary, fluid-filled lung cyst and hyperinflation of adjacent lung tissue in the same lobe. The combination of a fluid-filled cyst and ectatic emphysema in the same lobe suggests bronchial collapse and airway obstruction as a contributory mechanism for this unusual roentgenog...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00447379
更新日期:1979-10-01 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
doi:10.1007/s00431-020-03847-6
更新日期:2020-10-19 00:00:00
abstract::Through a systematic review and meta-analyses, we aimed to determine predictors for place of death among children. We searched online databases for studies published between 2008 and 2019 comprising original quantitative data on predictors for place of death among children. Data regarding study design, population char...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-020-03689-2
更新日期:2020-08-01 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::To nine cystic fibrosis patients with chronic bronchopulmonary infection of severely damaged lungs invaded by Pseudomonas aeruginosa, eleven courses of prolonged tobramycin treatment (5 mg/kg/day) for four to 16 weeks were administered. Pulmonary symptoms improved and a better quality of life was achieved in all but o...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441362
更新日期:1979-04-03 00:00:00
abstract::The glycogen storage disorders (GSD)-I, -III, -VI and -VIII are associated with hypertriglyceridaemia or mixed hyperlipidaemia which poses the question whether these patients have an increased risk for atherosclerosis. The atherogenicity of triglycerides has remained controversial, while increased plasma cholesterol l...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02072094
更新日期:1993-01-01 00:00:00
abstract::Strategies for the treatment of cystathionine beta-synthase (CBS) deficiency include (1) increasing residual enzyme activity by giving pyridoxine in those patients with vitamin responsive variants, (2) reducing the load on the affected pathway with a low methionine diet and supplementing the diet with cysteine; and (3...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/pl00014308
更新日期:1998-04-01 00:00:00
abstract::A 10-month-old infant is described who suffered from extensive atopic dermatitis, failure to thrive, hypoalbuminaemia and oedema. Large amounts of sticky exudate were lost through the skin and were shown to be rich in albumin. As renal and intestinal loss of protein was excluded, the patient's condition was ascribed t...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00446072
更新日期:1986-08-01 00:00:00
abstract:UNLABELLED:Congenital cardiovascular defects, commonly affecting the aortic valve or the aortic arch (50% to 70%), are seen in ca. 17%-44% of patients with Ullrich-Turner syndrome (UTS). However, there are only 36 case reports worldwide on the coincidental finding of partial anomalous pulmonary vein connection (PAPVC) ...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-003-1384-7
更新日期:2004-03-01 00:00:00
abstract::Phenylketonuria's (PKU) treatment based on low-protein diet may affect other metabolic pathways, such as that of asymmetric dimethylarginine (ADMA). The aim of this study was to evaluate the reliability of ADMA as a biomarker of adequate metabolic control and possible nutritional risk in a long-term PKU patient popula...
journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
doi:10.1007/s00431-019-03365-0
更新日期:2019-06-01 00:00:00
abstract::Chest radiographs from children with community-acquired pneumonia (CAP) were categorized into three distinct presentations and each presentation was correlated to clinical and laboratory findings. Children < 59 months with CAP presenting to pediatric emergency rooms during two years were enrolled prospectively in eigh...
journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s00431-018-3165-3
更新日期:2018-07-01 00:00:00
abstract:UNLABELLED:Fifty-two cases of childhood brucellosis which occurred in north-western Greece during the 15-year period 1979-1993, are reviewed. It is believed that they represent very closely the total incidence of the disease in the region which has a population of 100,000 children aged 0-14 years old. Brucellosis-affec...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02115616
更新日期:1996-01-01 00:00:00
abstract:UNLABELLED:Most of what is known about the metabolically healthy obese phenomenon is derived from studies in the adult population and no standardized criteria to identify these individuals exist to date. The aim of this study was to determine if the preserved insulin sensitivity evaluated by homeostatic model assessmen...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1867-5
更新日期:2013-02-01 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::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::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::Hypoglycaemia in children can be a life-threatening situation that needs to be assessed rigorously in order to treat efficiently and avoid relapse that can be responsible for cerebral damage. The diagnosis of impairment in glucose homeostasis requires the knowledge of the mechanisms regulating blood glucose concentrat...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-007-0600-2
更新日期:2008-03-01 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::Biochemical markers such as plasma and urinary metabolite concentrations and in vitro enzyme activity are of limited prognostic value in the most common disorders of propionate metabolism, methylmalonic acidaemia (MMA) and propionic acidaemia (PA). In vivo propionate oxidation was compared with conventional prognostic...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02009661
更新日期:1990-03-01 00:00:00
abstract::Chylothorax is defined as an effusion of lymph in the pleural cavity. In the neonate both congenital and traumatic (iatrogenic) forms exist. Birth asphyxia and respiratory insufficiency are major symptoms of congenital chylothorax, requiring resuscitation and artificial ventilation. Antenatal diagnosis by ultrasound a...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02072505
更新日期:1993-01-01 00:00:00
abstract::The varying clinical manifestations of Lyme borreliosis, transmitted by Ixodes ricinus and caused by Borrelia burgdorferi, frequently pose diagnostic problems. Diagnostic strategies vary between early and late disease manifestations and usually include serological methods. Erythema migrans is pathognomonic and does no...
journal_title:European journal of pediatrics
pub_type: 杂志文章,实务指引
doi:10.1007/s00431-012-1779-4
更新日期:2012-11-01 00:00:00
abstract::Henoch-Schonlein purpura (HSP) is an inflammatory vasculitis involving the skin, joints, gastrointestinal (GI) tract, and kidneys. This is the first case report describing a 5-year-old girl with HSP presenting duodenal involvement which might be associated with superior mesenteric artery syndrome (SMAS). ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0254-5
更新日期:2007-05-01 00:00:00
abstract::The New Vaccinations of Infants in Practice online survey in seven countries evaluated vaccination-related attitudes and concerns of parents of infants and health care providers (HCPs) who provide pediatric medical care. The survey showed that HCPs and parents were open to adding new vaccines to the immunization sched...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1904-4
更新日期:2013-04-01 00:00:00