Abstract：
UNLABELLED:A retrospective analysis was performed of the records of 133 patients with extrahepatic biliary atresia (EHBA) who had undergone a Kasai portoenterostomy. The patients were divided into a non-transplantation group who survived but did not receive liver transplantation after the procedure and a failure group of those who died or received liver transplantation. A score was calculated that assessed nine factors, including laboratory values and complications. The data were assessed at the time complications occurred. The scores were analysed by a trend analysis to see if serial scores predicted the evolution of liver disease. A receiver operating characteristic (ROC) curve was plotted to assess the optimal cut-point for the scoring system. There were 98 patients in the non-transplantation group and 35 in the failure group. The latter group had significantly higher post-operative bilirubin (9.3+/-7.2 mg/dl versus 3.5+/-3.1 mg/dl), ALT (136+/-89 U/l versus 92+/-88 U/l), prothrombin time, and incidence of cirrhosis, ascites, oesophageal varices, portal hypertension, cholangitis and sepsis than the non-transplantation group (P<0.05). A score of > or =8 had a high sensitivity (96.9%) and specificity (89.5%) for predicting the need for liver transplant. CONCLUSION:Based on easily available clinical information, our scoring system can predict which patients with biliary atresia who have already undergone a Kasai procedure should be considered for liver transplantation.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Jiang CB,Lee HC,Yeung CY,Sheu JC,Chang PY,Wang NL,Yeh CYdoi
10.1007/s00431-003-1268-xkeywords:
subject
Has Abstractpub_date
2003-09-01 00:00:00pages
603-6issue
9eissn
0340-6199issn
1432-1076journal_volume
162pub_type
杂志文章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：:A 5-year-old male child presented with progressively increasing asymmetrical overgrowth of limbs, hyperpigmentation on the right half of the body, and mental retardation since 9 months of age. ...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-010-1346-9
更新日期：2011-06-01 00:00:00
abstract：:A 10-year-old boy presented with priapism of 10 h duration which after unsuccessful conservative measures, was relieved by a saphenocorporeal shunt. A 4-year history of intermittent vague aching of fingers and toes accompanied by low-grade fever was reported. Fabry disease was confirmed by the lack alpha-galactosidase...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF01959404
更新日期：1990-04-01 00:00:00
abstract：UNLABELLED:A 14-year-old boy developed acute quadriplegia, associated with sensory impairment and bowel and urinary dysfunction. MRI of the cervical cord showed diffuse increased signal intensity on T2-weighted images with gadolinium-diethylenetriamine penta-acetic acid enhancement. Based on the clinical presentation a...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF02072107
更新日期：1995-05-01 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：UNLABELLED:The purpose of this study was to assess how much of the variance in intellectual outcome at 2 and 6 years of age could be attributed to treatment variables in children with congenital hypothyroidism, and which of the parameters used for monitoring treatment predicted later development. Forty-five children, e...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF01955261
更新日期：1996-05-01 00:00:00
abstract：:Neonatal diabetes, which may be transient or permanent, is rare. Most patients are full-term but small- for-date infants. Typical symptoms of diabetes mellitus occur within the first 4 weeks of life, requiring insulin therapy and very strict blood glucose monitoring. Subsequent growth and psychomotor development are u...
journal_title：European journal of pediatrics
pub_type： 杂志文章,评审
doi：10.1007/BF01958635
更新日期：1995-12-01 00:00:00
abstract：:The clinical presentation of combined pituitary hormone deficiency (CPHD) is variable. Some patients present with hypoglycemia during the neonatal period or during the first few years of life. Others present later in childhood with growth failure. We report on 7 patients with very late diagnosed severe hypopituitarism...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-019-03489-3
更新日期：2020-01-01 00:00:00
abstract：:In 238 boys with cryptorchidism, between the ages of 4 and 48 months, luteinizing hormone releasing hormone (LHRH) was administered as nasal spray 1.2 mg/day for 4 weeks. The nonresponders received human chorionic gonadotropin (HCG) 500 I.U. i.m. three times a week for 3 weeks. With the combined treatment 37.8% of tes...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF02125433
更新日期：1993-01-01 00:00:00
abstract：:We studied the effects of an alginate compound (Gaviscon) on the frequency and the duration of gastroesophageal reflux (GOR) episodes in children. Twenty infants and children with characteristic symptoms of GOR were divided at random into two groups which were given either Gaviscon (ten patients, mean age: 21 months) ...
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/BF02343223
更新日期：1987-03-01 00:00:00
abstract：:We report the 9-year follow-up of a patient suffering from N-acetylglutamate synthetase deficiency, an urea cycle disorder leading to severe neonatal hyperammonaemia. Hitherto two patients from two families with this inborn error of metabolism had been observed. Our management consisted mainly of a protein-restricted ...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF01955939
更新日期：1991-03-01 00:00:00
abstract：:The total height gain was studied in 19 adult male subjects with non-tumorous hypopituitarism after they had been treated with human growth hormone (hGH) for more than 3 years and achieved final adult height. In patients with a history of breech delivery or severe perinatal asphyxia, a high multiple correlation was fo...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF00442688
更新日期：1988-04-01 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：:Cortisol and growth hormone (GH) secretion (spontaneous variations at night and the release induced by insulin hypoglycaemia) were investigated in 69 children and adolescents. Statistical analysis of approximately 600 pairs of cortisol and GH values in this study demonstrated that physiological fluctuations of cortiso...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF00496996
更新日期：1978-06-20 00:00:00
abstract：UNLABELLED:Breast milk samples from three groups of Brazilian women were evaluated for their inhibitory effect on enteropathogenic Escherichia coli (EPEC) adhesion to HEp-2 cells: G1, mothers delivering preterm babies of appropriate birth weight (n = 12); G2, mothers delivering term babies of low birth weight (n = 11);...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s004310050646
更新日期：1997-06-01 00:00:00
abstract：:Congenital high airway obstruction syndrome (CHAOS) is a rare prenatal diagnosis consisting of a typical fetal triad of large hyperechogenic lungs, flattened or inverted diaphragms and ascites. Most cases are sporadic with unknown incidence. Before attempts of fetoscopic fetal salvage or ex utero intrapartum treatment...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-006-0134-z
更新日期：2006-10-01 00:00:00
abstract：:The laryngo-tracheo-oesophageal cleft is marked by a missing anatomical separation of the oesophagus and the larynx. The cleft can be restricted to the dorsal part of the larynx (type I), extend to the upper area of the trachea (type II) or involve the whole of the trachea (type III). In reviewing our three cases and ...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF00661903
更新日期：1983-03-01 00:00:00
abstract：UNLABELLED:In 135 women and 85 men who initially presented for tall stature, the outcome in treated (56 women and 33 men; cases) and untreated (controls) was investigated. At the time of height prediction, cases were significantly taller (P < or = 0.03) than the controls, they had higher target heights (P < 0.001) and ...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s004310050740
更新日期：1997-12-01 00:00:00
abstract：UNLABELLED:Although bacterial colonisation of bronchi may occur from early childhood onwards, infections extending beyond the lungs are uncommon in patients with cystic fibrosis. A 12-year-old boy with cystic fibrosis, receiving oral corticosteroids for 3 weeks because of allergic bronchopulmonary aspergillosis, experi...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/pl00008408
更新日期：2000-09-01 00:00:00
abstract：UNLABELLED:A formerly premature, exclusively breast-fed infant with severe zinc deficiency syndrome is presented. He showed the characteristic erosive skin changes, including alopecia, as seen in acrodermatitis enteropathica. In addition, he manifested a failure to thrive and irritability. The diagnosis was confirmed b...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF01972977
更新日期：1995-01-01 00:00:00
abstract：UNLABELLED:Schimke immuno-osseous dysplasia is a multisystem disorder consisting of spondylo-epiphysial dysplasia, progressive renal insufficiency due to focal segmental glomerulosclerosis, and immunodeficiency. Cerebrovascular complications have only been described in five patients. Here we report a patient with promi...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s004310050714
更新日期：1997-10-01 00:00:00
abstract：UNLABELLED:Extremely immature newborns develop a self-limiting normal anion gap metabolic acidosis in early life. This study examined the natural history of this acidosis in a population of infants of gestation less than 26 weeks in the first 14 days of life. The acidosis was maximal on day 4 with a mean base deficit o...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-014-2364-9
更新日期：2015-01-01 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：:Effective management of pain remains an important indicator of the quality of care provided to neonates. Since the review of McIntosh in this journal over a decade ago, an extensive number of papers on assessment, prevention, and treatment of pain have been reported. In addition, preclinical insights into neurodevelop...
journal_title：European journal of pediatrics
pub_type： 杂志文章,评审
doi：10.1007/s00431-009-0932-1
更新日期：2009-07-01 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 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
abstract：:In 41 preterm neonates with a gestational age (GA) varying from 27 to 36 weeks, glomerular filtration rate (GFR) was measured by means of the continuous inulin infusion technique. The reliability of the technique was confirmed. During postnatal development GFR was found to increase in two ways: firstly, an increase wi...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF00441807
更新日期：1988-10-01 00:00:00
abstract：UNLABELLED:The fatty acid composition of human breast milk was determined longitudinally after term and preterm delivery by high resolution gas liquid chromatography. Milk samples were obtained at days 5, 10, 20 and 30 after term (n = 38) or preterm (n = 19) delivery. The saturated fatty acids C10:0 and C12:0 and the p...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s004310050573
更新日期：1997-02-01 00:00:00
abstract：:The light- and electron-microscopic structure of articular and costal cartilage in a case of achondrogenesis type I has been described. The most characteristic ultrastructural change in the chondrocytes was conspicuous dilatation of the rough endoplasmatic reticulum (RER) which contained amorphous electronopaque mater...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF00442406
更新日期：1980-06-01 00:00:00
abstract：:This study aimed to estimate the number of infants who died of unrecognized congenital adrenal hyperplasia (CAH) in Austria and the Czech Republic within the past 13 years, before the introduction of adequate neonatal screening. The study was based on retrospective analysis of neonatal screening cards of 242 infants w...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-006-0154-8
更新日期：2007-01-01 00:00:00
