Abstract:
UNLABELLED:Portal vein thrombosis is a rare but potentially lethal complication in children requiring splenectomy. We report on a 15-year-old boy with a dehydrated hereditary stomatocytosis, who underwent splenectomy and presented a postoperative partial portal vein thrombosis. With prompt heparin therapy, neither propagation of the thrombus nor further cavernous transformation in the following occurred 6 years. CONCLUSION:Recent data suggest that hereditary stomatocytosis carries a high risk of thrombotic complications, especially after splenectomy. This procedure, the benefit of which is limited in this condition, should therefore be strongly avoided.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Perel Y,Dhermy D,Carrere A,Chateil JF,Bondonny JM,Micheau M,Barbier Rdoi
10.1007/s004310051165keywords:
subject
Has Abstractpub_date
1999-08-01 00:00:00pages
628-30issue
8eissn
0340-6199issn
1432-1076journal_volume
158pub_type
杂志文章abstract::In Prader-Willi syndrome (PWS) hypothalamic dysfunction is the cause of hormonal disturbances, such as growth hormone deficiency (GHD), hypogonadism, and delayed or incomplete puberty. Only a few cases of central precocious puberty (CPP) have been reported. We describe an 8.8-year-old PWS boy, with microdeletion of ch...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-008-0679-0
更新日期:2008-12-01 00:00:00
abstract::A five-year-old-girl with a history of recurrent hypoglycemia presented with acidosis, intractable vomiting, and abdominal tenderness; the diagnosis of acute pancreatitis was made by abdominal ultrasonography and supportive biochemical studies. Urinary organic acid analysis revealed metabolites suggestive of HMG-CoA l...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00540255
更新日期:1984-09-01 00:00:00
abstract::The peak flow velocities in the middle cerebral artery were continuously recorded by transcranial Doppler sonography in 29 children. Arterial blood pressure and heart rates were measured every minute. The values observed during orthostasis and physical exercise were compared to baseline values obtained in the supine p...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01958769
更新日期:1991-08-01 00:00:00
abstract::Primary hyperparathyroidism is a rare endocrine disease in children and young adults. The early detection and treatment of primary hyperparathyroidism led to a marked decrease in classical bone and renal manifestations of the disease. Osteitis fibrosa cystica and brown tumors have become extremely rare clinical entiti...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-007-0414-2
更新日期:2008-01-01 00:00:00
abstract::A patient with a cerebro-hepato-renal syndrome was investigated. The visceral manifestations were those of the Zellweger syndrome (ZS); however, the child exhibited muscular hypertonia and survived into the 2nd year of life. Ultramicroscopically, hepatocytes were lacking peroxisomes, but, contrary to findings in one p...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441934
更新日期:1977-03-18 00:00:00
abstract::The Kabuki (Niikawa-Kuroki) syndrome was reported in 1981 by Niikawa et al. and Kuroki et al. in a total of ten unrelated Japanese children with a characteristic array of multiple congenital anomalies and mental retardation. The syndrome is characterized by a distinct face, mild to moderate mental retardation, postnat...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01983409
更新日期:1994-06-01 00:00:00
abstract::Analysis of both the human and rat galactose-1-phosphate uridyltransferase (GALT) genes reveal 5' regulatory consensus sequences suggestive of a housekeeping gene. This is in accord with the finding of GALT activity in all tissues. However, the complications seen in galactosemia, in particular ovarian dysfunction and ...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02143799
更新日期:1995-01-01 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::Phenobarbital has been shown to offer effective prophylaxis against childhood febrile convulsions. However, a high percentage of children do not tolerate phenobarbital, mainly due to behavioral changes. Valproate, due to its low toxicity, appears to be an attractive alternative to phenobarbital treatment. Ninety child...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441308
更新日期:1981-10-01 00:00:00
abstract::We updated our 2010 systematic review on the efficacy of probiotics in the treatment of constipation in children. The MEDLINE, EMBASE, and Cochrane Library databases; clinical trial registries; and reference lists of included studies were searched to February 2017 for randomized controlled trials (RCTs) performed in c...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-017-2972-2
更新日期:2017-09-01 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::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:UNLABELLED:Coronary artery disease (CAD) is a major cause of death in patients with insulin-dependent diabetes mellitus. Qualitative changes in low density lipoprotein (LDL) and high density lipoprotein (HDL) are thought to be important for evaluating the risk for CAD. In the present study, we evaluated LDL particle si...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050810
更新日期:1998-04-01 00:00:00
abstract::Premature newborns are particularly vulnerable to iatrogenic hypothyroidism due to iodine exposure, usually through skin absorption of iodine-containing disinfectants or intravenous administration of iodinated contrast agents. We report here a case of severe iatrogenic hypothyroidism with goiter and cholestasis, disco...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-008-0782-2
更新日期:2009-04-01 00:00:00
abstract:UNLABELLED:The phenotypes of chromosomal 22q11.2 microdeletion are quite variable among individuals and hypoparathyroidism (HP) constitutes a definite portion of the clinical spectrum. For the correct diagnosis and pertinent follow up of the HP children due to del22q11.2, we tried to delineate the clinical characterist...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050762
更新日期:1998-01-01 00:00:00
abstract:UNLABELLED:A prospective study was undertaken throughout Wales over the period from 1 January 1995 to 30 March 1997, to determine the incidence of childhood inflammatory bowel disease (IBD). Thirty-eight (24 male) new cases of IBD were reported. Twenty (16 male) of the reported cases had Crohn's disease, 11 (5 male) ha...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050066
更新日期:2000-04-01 00:00:00
abstract::Malignant thymoma was found in an 8-year-old Japanese boy with growth hormone (GH) deficiency who had received GH therapy for 3 years and 5 months. There may be a possible relationship between the occurrence of malignant thymoma and GH therapy. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02073374
更新日期:1993-10-01 00:00:00
abstract::Seven male Japanese children with chronic granulomatous disease were given sulfamethoxazole-trimethoprim (SMX-TMP) for recurrent pyogenic infections, most of which had proved difficult to control with other antibiotics. With continous treatment the children remained free of infections severe enough to necessitate hosp...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00493540
更新日期:1978-04-20 00:00:00
abstract::Estimation of the total number of infants with DS born each year in Belgium allows evaluation of changes in the proportion of children with DS in whom a karyotype has been established. This proportion steadily increased to 95% of the children with DS born in 1978. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442300
更新日期:1985-03-01 00:00:00
abstract::Continuous negative extrathoracic pressure (CNEP) has been recently reintroduced as therapy for respiratory failure. To determine its effects on cardiac output a pilot study was performed in ten patients aged 2 months-3 years (median 4 months). All had chronic respiratory failure (seven with bronchopulmonary dysplasia...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01954088
更新日期:1993-07-01 00:00:00
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
doi:10.1007/s00431-019-03526-1
更新日期:2020-02-01 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::Serial ultrasound examinations were performed through the anterior fontanelle to detect and follow subependymal pseudocysts (SP) in 19 neonates without severe malformations. A high-resolution real-time sector scanner was employed. In 8 cases the SP involved both lateral ventricles, in 11 cases they were unilateral and...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00595915
更新日期:1989-02-01 00:00:00
abstract:UNLABELLED:In this review, a simplified scheme for classification of cortical malformations is introduced and illustrated based on the work of Barkovich et al. [8]. Detailed MRI studies identify cortical malformations as a major cause of epilepsy in children. Two aspects that are becoming increasingly important for the...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s004310000452
更新日期:2000-08-01 00:00:00
abstract:UNLABELLED:Anatomical face mask with an air cushion rim might be placed accidentally in a false orientation on the newborn's face or filled with various amounts of air during neonatal resuscitation. Both false orientation as well as variable filling may reduce a tight seal and therefore hamper effective positive pressu...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-013-2122-4
更新日期:2014-01-01 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::Patients with Turner syndrome (n = 33) were subjected to a sequential oestrogen/gestagen replacement therapy (21 days 2 mg oestradiol valerate plus 0.5 mg norgestrel for 10 days) for 1-4 years. In individuals aged 12-17 years, growth velocities before treatment ranged between 2.9 and 1.9 cm/year and increased to 6.8-3...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00439400
更新日期:1986-09-01 00:00:00
abstract::This study is a retrospective analysis of seven patients with hypoechogenic lesions in the liver and/or spleen due to Bartonella henselae, who were followed from 1998 through to 2005 by the Department of Pediatrics, Turin University. In addition to showing constitutional symptoms, four children had skin lesions sugges...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-007-0500-5
更新日期:2008-04-01 00:00:00
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::So far, a recognizable pattern of clinical symptoms for child sexual abuse (CSA), especially in young male children, is lacking. To improve early recognition of CSA, we reviewed physical complaints, physical examination, and tests on sexually transmitted infections (STIs) in confirmed victims (predominantly preschool ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-017-2996-7
更新日期:2017-10-01 00:00:00