Abstract:
:Haemostasis was studied in 8 tall girls during treatment with high doses of synthetic oestrogens for a period of 1 to 28 months. The girls received 0.5 mg ethinyloestradiol daily from day 1 to day 25 and 15 mg norethisteron daily from day 21 to day 25. All were without clinical signs of thromboembolism. Factors of the prothrobmin complex were significantly elevated in the tall girls compared to healthy controls of the same age. Fibrinogen was also significantly elevated. Factor VIII-related antigen was significantly higher in the tall girls and the ratio between factor VIII activity and factor VIII-related antigen was significantly decreased in the tall girls, as compared to the controls. Thrombin time was significantly shortened. Tests of primary haemostasis showed an increased tendency for thrombocytes to adhere to glass and to aggregate spontaneously and after stimulation with ADP or collagen. Whether these in vitro signs of hypercoagulability indicate an enhanced risk of throbmoembolic complications in tall girls treated with high doses of oestrogens remains to be established. However, these girls should be watched carefully for clinical signs of throbmoembolic complications during treatment. :8 tall girls were studied during treatment with high doses of synthetic estrogen for from 1-28 months to determine effects on hemostasis. Doses of .5 mg of ethinylestradiol were given daily from Day 1-25 and of 15 mg of norethisterone daily from Day 21-25 to the girls, all of whom had no clinical signs of thromboembolism. Blood coagulation factors were determined. Factors of prothrombin complex were elevated significantly in the tall girls compared with healthy controls of normal height of the same age. Factor VII-related antigen was significantly higher in tall girls, and the ratio of Factor VIII activity and Factor VII-related antigen was significantly decreased in tall girls compared with normal controls. Fibrinogen was also significantly elevated in tall girls. Thrombin time was shortened significantly. Primary hemostasis tests showed increased tendency for thrombocytes to adhere to glass and to aggregate spontaneously after stimulation with ADP or collagen. The relationship between increased risk of blood coagulation abnormalities and these in vitro findings remains to be established, but tall girls should be carefully observed for clinical signs of thromboembolic complications if under treatment with steroid preparations.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Muntean W,Borkenstein Mdoi
10.1007/BF00441480subject
Has Abstractpub_date
1980-09-01 00:00:00pages
245-8issue
3eissn
0340-6199issn
1432-1076journal_volume
134pub_type
杂志文章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::An 18-month-old child with partial DiGeorge syndrome developed aplastic anaemia during an acute adenovirus infection. Assessment of the child's immune system revealed T-cell subset abnormalities consistent with DiGeorge syndrome. A possible link between the underlying immune deficiency and the observed complication is...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442482
更新日期:1988-08-01 00:00:00
abstract:UNLABELLED:Congenital central hypoventilation syndrome (CCHS, Ondine's curse syndrome) is a rare respiratory disorder; less than 100 cases have been reported. Familiality of the disease has been discussed, but only few familial cases have been reported so far. In this report we describe the occurrence of CCHS in two ma...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02282890
更新日期:1996-11-01 00:00:00
abstract::In a German pediatric oncology unit, the attending physicians diagnosed 27 cases of Clostridium difficile-associated disease (CDI) from January 01, 2010 to October 31, 2013. This refers to a CDI incidence density of 2.0/1000 inpatient days. According to the hospital hygiene standard, symptomatic patients with CDI were...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-017-3070-1
更新日期:2018-03-01 00:00:00
abstract::The heuristic concept of "inborn errors of metabolism" was introduced more then 70 years ago and by analogy has prompted the more recent introduction of the term "inborn errors of immunity". It is now well recognized that many inborn errors of immunity can be considered inborn errors of metabolism. Typically, many for...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00491916
更新日期:1985-05-01 00:00:00
abstract::A 4-month-old male infant was seen because of an asymptomatic undescended left testis and a right sided abdominal mass. CT revealed a calcified retroperitoneal tumour. Histological examination of surgical specimens showed a mature primary teratoma of the contralateral undescended testis. While this is very rare, infan...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01957524
更新日期:1993-11-01 00:00:00
abstract::We evaluated, both in toilet-trained and not-toilet-trained children, the impact of cleaning the genital area with plain water on the false positive rate at urine dipstick, and evaluated which factors could be associated to falsely positive findings. We prospectively enrolled 612 patients consecutively attending our n...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-3215-x
更新日期:2018-10-01 00:00:00
abstract::Two young boys were referred for evaluation of metabolic myopathy because of elevated serum levels of creatine kinase, cramps and pigmenturia. Immunohistochemical studies of dystrophin in muscle biopsies showed reduced intensity of the stain with a patchy and discontinuous pattern in most fibers. In both patients dyst...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02073385
更新日期:1993-10-01 00:00:00
abstract:UNLABELLED:Essential fatty acid deficiency is well known in cystic fibrosis patients, but its pathogenesis remains unclear. It might be related to protein-energy malnutrition which is a common feature of cystic fibrosis or to some specific defects in fatty acid metabolism. To avoid the deleterious effects of protein-en...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050750
更新日期:1997-12-01 00:00:00
abstract:UNLABELLED:Concentrations of galactose (Gal) in plasma and galactose metabolites in red blood cells (RBC) were determined in 18 normal neonates and 249 others with hypergalactosaemia according to the Paigen method. Normal neonatal values for plasma Gal, RBC galactose-1-phosphate (Gal-1-P), RBC uridine diphosphate gluco...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/pl00008352
更新日期:2000-11-01 00:00:00
abstract::A case of painful bilateral hip effusions resolving spontaneously in the diuretic phase of steroid responsive nephrotic syndrome in a 6-year-old girl is reported. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02072499
更新日期:1993-02-01 00:00:00
abstract::The fatty acid status of an unselected group of 84 Congolese children was determined by estimating the fatty acid composition of the plasma phospholipid (PL) and cholesterolester (CE) fractions using capillary gas chromatography. In comparison with North American and European children a wide range of values and low me...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00445928
更新日期:1988-11-01 00:00:00
abstract:UNLABELLED:An 8-year-old boy with frequently recurring pancreatitis-like abdominal pain, Fredrickson type V dyslipidaemia, and significantly decreased post-heparin plasma lipoprotein lipase (LPL) activity is described. In order to exclude familial LPL deficiency, the complete LPL coding gene sequence was analysed revea...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01957148
更新日期:1996-08-01 00:00:00
abstract:UNLABELLED:Nemaline myopathy, one of the most benign myopathies in older children and adults, may be rarely associated with early death in the neonate. The authors report two cases, describing the ante- and postnatal clinical courses. Family history may help in establishing the diagnosis, but this was not the case in t...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01955190
更新日期:1996-06-01 00:00:00
abstract::Pediatric palliative care (PPC) focuses on children and adolescents with life-limiting diseases. It may be initiated at various points of the disease trajectory, if possible early enough to support living with the best possible quality of life despite a limited lifespan. From birth to adolescence, children with a broa...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-012-1710-z
更新日期:2013-02-01 00:00:00
abstract::Adolescents have specific healthcare needs distinct from adults or younger children secondary to anatomical, physiological and socio-behavioural differences. Healthcare providers have been slow to address this, leading the UK Department of Health (2011) to publish 'You're Welcome' quality criteria for services for you...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-016-2815-6
更新日期:2017-02-01 00:00:00
abstract::Complex regional pain syndrome (CRPS) is a condition of unknown etiology characterized by autonomic, sensory, and motor disturbance. CRPS usually follows an injury in the affected limb, which is often trivial in nature. CRPS involving the facial region is rare, and there have been no previous descriptions in children....
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-013-2159-4
更新日期:2014-03-01 00:00:00
abstract:UNLABELLED:Ischaemic stroke is a rare event in childhood. In approximately one-fourth of cases an underlying cardiac disease can be detected. We investigated the importance of genetic risk factors of venous thromboembolism in childhood or stroke in adulthood as risk factors for ischaemic stroke in children in a multice...
journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
doi:10.1007/pl00014336
更新日期:1999-12-01 00:00:00
abstract:UNLABELLED:Intravenous immunoglobulin (IVIG) is currently the standard treatment for Kawasaki disease (KD). Although IVIG therapy is generally well tolerated, several minor adverse reactions have been reported. We report a patient with KD treated with IVIG, who developed a cutaneous reaction in the convalescent phase (...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-012-1898-y
更新日期:2013-03-01 00:00:00
abstract::Though children with Perlman and Wiedemann-Beckwith syndromes have a number of features in common, the two conditions are probably separate entities. The distinction may not always be easy, however, partly because of the extreme rarity of Perlman syndrome, only nine cases of which have been reported so far. We report ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01954125
更新日期:1992-12-01 00:00:00
abstract::Plasma vitamin E levels were determined serially in preterm infants surviving respiratory distress syndrome (RDS) and in premature infants without RDS (control). Vitamin E intakes of the RDS and control infant group were not significantly different. The results of the study show that preterm infants surviving RDS have...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00446056
更新日期:1986-08-01 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::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::Achondroplasia, the most common form of chondrodysplasia, has been associated with mutations in the gene of the fibroblast growth factor receptor-3 (FGFR-3) on chromosome 4p. All 39 achondroplasia alleles studied so far carried point mutations which caused the same amino acid exchange, a substitution of glycine by arg...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01954274
更新日期:1995-03-01 00:00:00
abstract:UNLABELLED:Dent disease is an X-linked tubulopathy mainly caused by inactivating mutations of CLCN5. Features of Bartter syndrome such as hypokalemic metabolic alkalosis are rarely observed in patients with Dent disease. We report a Japanese male patient with Dent disease who also manifested features of Bartter syndrom...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-011-1578-3
更新日期:2012-02-01 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::Purine nucleoside phosphorylase (PNP) deficiency is a rare combined immunodeficiency disorder presenting with clinically recurrent infections, failure to thrive, various neurological disorders, malignancies, and autoimmune diseases. Here, we report two sisters with a fatal course of PNP deficiency due to delay in diag...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-009-1029-6
更新日期:2010-03-01 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::The etiology of asthma includes lifestyle factors. Breastfeeding and introduction of complementary foods have been suggested to affect asthma risk, but the scientific foundation is not solid. Children from the birth cohort All Babies In Southeast Sweden study were included (n = 9727). Breastfeeding duration and timing...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-03312-5
更新日期:2019-03-01 00:00:00
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
pub_type: 杂志文章
doi:10.1007/s00431-008-0819-6
更新日期:2009-06-01 00:00:00