Abstract:
:The transport characteristics of the placenta, which favour higher phenylalanine concentrations in the fetus than in the mother, and regression data of head circumference at birth against phenylalanine concentration at conception in maternal phenylketonuria (PKU), suggest that treatment of maternal PKU should ideally aim to maintain plasma phenylalanine concentration within the normal range throughout pregnancy. A patient with classical PKU was treated from before conception by aiming to maintain plasma phenylalanine concentration within the range 50-150 mumol/l and tyrosine within the range 60-90 mumol/l. The diet was supplemented with phenylalanine-free amino acids (100-180 g/day) and tyrosine (0-5 g/day). Plasma amino acid concentrations were monitored weekly by amino acid analyser. Dietary phenylalanine intake ranged from 6 mg/kg/day at conception to 30 mg/kg/day at delivery. Normal weight gain and fetal growth were maintained throughout the pregnancy. A normal baby was born at term with a head circumference of 35.5 cm; at 1 year of age no abnormality is detectable. These results show that with careful monitoring and compliance it is possible, and may be advisable, to maintain plasma phenylalanine concentration within the normal range in the management of PKU pregnancy.
journal_name
Arch Dis Childjournal_title
Archives of disease in childhoodauthors
Thompson GN,Francis DE,Kirby DM,Compton Rdoi
10.1136/adc.66.11.1346subject
Has Abstractpub_date
1991-11-01 00:00:00pages
1346-9issue
11eissn
0003-9888issn
1468-2044journal_volume
66pub_type
杂志文章abstract::Forty three infants were assigned randomly to a ready-to-feed infant formula or a standard formula that required reconstitution from powder. Despite similar nutrient composition of the two formulas those fed the powdered formula had significantly increased body weight and skinfold thickness gains, and became significa...
journal_title:Archives of disease in childhood
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1136/adc.67.7.935
更新日期:1992-07-01 00:00:00
abstract::Infant feeding methods used by mothers who smoke and by mothers who do not, who delivered in one maternity hospital, were compared to determine if smoking affected breast feeding. Significantly more of the smoking group chose either to bottle feed from delivery or, having been discharged from the hospital breast feedi...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.58.5.378
更新日期:1983-05-01 00:00:00
abstract::A family with multiple congenital scalp defects, over two generations and probably genetically determined, is described. Although alarming in appearance, surgical intervention is not indicated at least for small lesions. The risk of haemorrhage and meningitis is emphasized. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.50.12.958
更新日期:1975-12-01 00:00:00
abstract:BACKGROUND:The Coventry Consensus in 1998 recommended a single height measurement of all children at school entry or around the age of 5 years and prompt referral of children with height <0.4th centile for further assessment, in order to identify undetected and treatable asymptomatic growth disorders. AIM:To determine...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.88.6.477
更新日期:2003-06-01 00:00:00
abstract:OBJECTIVE:To examine the efficacy and safety of torasemide in children with chronic heart failure (HF). METHODS:102 children with chronic HF who had received oral torasemide were analysed. Of these, 62 (de novo group) were newly diagnosed as having HF and were given torasemide as a diuretic. The remaining 40 (replacem...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2007.129239
更新日期:2008-09-01 00:00:00
abstract::The introduction of enteric coated pancreatic enzyme supplements in the early 1980s was undoubtedly one of the major advances in the care of children with cystic fibrosis. Further refinements in the presentation of these preparations inevitably followed, to improve patient acceptability and compliance. The emergence o...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/adc.74.5.464
更新日期:1996-05-01 00:00:00
abstract::Two neonates with severe culture proven pertussis were successfully treated with salbutamol 0.5 mg three times daily. There was a demonstrable decrease in the total number of coughing spasms, their mean duration and severity. In one baby this was shown by the transcutaneous oxygen tension measurements. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.61.6.600
更新日期:1986-06-01 00:00:00
abstract::Maternal transmission of hepatitis B virus infection in relation to the hepatitis B e antigen/antibody system and serum hepatitis B virus-DNA were evaluated. Results indicate that hepatitis B virus-DNA analysis can identify hepatitis B serum antigen positive mothers who may transmit infection to their offspring. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.60.1.56
更新日期:1985-01-01 00:00:00
abstract::Female genital mutilation (FGM) is almost always performed on children and consequently paediatricians should have a central role in the detection and prevention of FGM. FGM has no health benefits and can cause lifelong damage to physical and psychological health. Extensive migration of FGM practising communities mean...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/archdischild-2014-307234
更新日期:2016-03-01 00:00:00
abstract::The practice of general paediatricians in the initial evaluation of congenital hypothyroidism (CHT) was assessed. This was performed by a questionnaire survey of paediatricians in East Anglia of whom 84% responded. Nineteen of 25 clinicians based in seven district hospitals managed children with CHT. The median number...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.77.4.339
更新日期:1997-10-01 00:00:00
abstract:AIMS:To evaluate the clinical efficacy of sublingual immunotherapy (SLIT) in respiratory allergy in children. METHODS:A systematic literature review was conducted. The search was focused on all the double blind (and double dummy if necessary) studies. SEARCH STRATEGY:Medline, Embase, Cochrane Controlled Trial Registe...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/adc.2003.030411
更新日期:2004-07-01 00:00:00
abstract::A boy with the dysmorphic features of Noonan's syndrome and pulmonary valve stenosis who had evidence of hypoparathyroidism and abnormal T lymphocyte numbers in the neonatal period is reported. He had a normal karyotype but molecular analysis revealed a submicroscopic deletion within chromosome 22q11, the region delet...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.68.2.187
更新日期:1993-02-01 00:00:00
abstract::The auditory function of 370 infants, drawn from both low and high risk groups, was tested before postnatal discharge using three tests: standard auditory brain stem responses (ABR), automated analysis of ABR, and automated analysis of evoked otoacoustic emissions (OAE). All infants failing any neonatal test had furth...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.66.10_spec_no.1124
更新日期:1991-10-01 00:00:00
abstract::Bilateral diaphragmatic pacing was successfully performed in an 18-month-old child with congenital central hypoventilation syndrome (Ondine's curse) as an alternative to long-term mechanical ventilation. Subsequent complications were related more to cor pulmonale and tracheostomy care than to the pacing itself. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.55.11.901
更新日期:1980-11-01 00:00:00
abstract:AIM:To measure drug adherence in children with mild asthma receiving long term prophylactic treatment. METHODS:Double blind randomised placebo controlled trial. Patients received inhaled budesonide 100 microg or 200 microg daily, or placebo for 27 months. All participants were asked to inhale medication or placebo fro...
journal_title:Archives of disease in childhood
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1136/adc.83.4.330
更新日期:2000-10-01 00:00:00
abstract::Albuminuria was studied in 40 diabetic children before and after a standardised exercise test, and also in 21 normal children; the results are expressed as geometric mean (95% range) of urine albumin:urine creatinine ratio (mg/mg). There was no significant difference between the mean resting albumin:creatinine ratio i...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.60.4.305
更新日期:1985-04-01 00:00:00
abstract::During the 6-year period from 1 October 1971 to 30 September 1977, 13 (about 1%) of 1225 admissions to hospital with asthma developed severe ventilatory failure (peak arterial PCO 2 greater than 8 kPa). Mean age was 4.1 years (2.3--7.9), and on average each patient had been admitted to hospital on 5 occasions during t...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.53.9.714
更新日期:1978-09-01 00:00:00
abstract:AIM:To determine whether emergency hospital admission rates (EAR) for common paediatric conditions in Greater London are associated with measures of child well-being and deprivation. DESIGN:Retrospective analysis of hospital episode statistics and secondary analysis of the Index of Multiple Deprivation (IMD) 2007 and ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2009.180125
更新日期:2011-03-01 00:00:00
abstract::Eight hospitals reported 781 non-iatrogenic accidents occurring to patients and visitors under 16 years of age during an 18 month period up to October 1989. Accidents more often involved boys and children aged 3 to 5 years old. Falls from a height, slips, and striking accidents were common by day and falls by night. A...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.66.9.1047
更新日期:1991-09-01 00:00:00
abstract:BACKGROUND:Some victims of sudden infant death syndrome (SIDS) are found with their heads covered with bedclothes, but the significance of this is uncertain. The aim of this review is to describe the prevalence of head covering, the magnitude of the risk and how far the suggested causal mechanisms agree with current ep...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/adc.2007.136366
更新日期:2008-09-01 00:00:00
abstract::The association of weight for height and triceps skinfold with seven respiratory symptoms has been examined using logistic regression analysis in 7800 5 to 11 year old children (6200 in England and 1600 in Scotland). The results support the view that overweight children have a greater liability to some respiratory sym...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.59.10.940
更新日期:1984-10-01 00:00:00
abstract:INTRODUCTION:Intravenous paracetamol (acetaminophen) has recently been registered for treatment of pain in neonates but the pharmacodynamics, including effects on body temperature, have not been reported. METHODS:A pooled analysis on body temperature recordings in neonates exposed to intravenous paracetamol was perfor...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2010.203778
更新日期:2011-03-01 00:00:00
abstract::Coeliac disease occurred at the same age in MZ twins. The diagnosis was confirmed by histology of the small intestine, rapid response to a gluten-free diet, and relapse after reintroduction of gluten. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.54.5.395
更新日期:1979-05-01 00:00:00
abstract::The clinical response to sodium cromoglycate treatment was compared with its concentration in serum. Twenty five children with asthma entered a 10 week trial of two baseline weeks followed by eight weeks of treatment by the inhalation of 20 mg of sodium cromoglycate spincaps four times a day. Individual clinical respo...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.63.6.592
更新日期:1988-06-01 00:00:00
abstract:BACKGROUND:Short-course oral corticosteroids are commonly used in children but are known to be associated with adverse drug reactions (ADRs). This review aimed to identify the most common and serious ADRs and to determine their relative risk levels. METHODS:A literature search of EMBASE, MEDLINE, International Pharmac...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,meta分析,评审
doi:10.1136/archdischild-2015-309522
更新日期:2016-04-01 00:00:00
abstract:BACKGROUND:A paucity of evidence regarding the natural history of congenital pulmonary airway malformations (CPAMs) and pulmonary sequestration (PS) has resulted in a divergence in management strategy of asymptomatic cases. METHODS:We describe the long-term clinical course of 119 children diagnosed with these lesions ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2016-311233
更新日期:2017-09-01 00:00:00
abstract::The objectives of this study were to determine whether or not the probiotic Lactobacillus GG can colonise the immature bowel of premature infants and if so, does colonisation result in a reduction of the size of the bowel reservoir of nosocomial pathogens such as enterobacteriaceae, enterococci, yeasts or staphylococc...
journal_title:Archives of disease in childhood
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1136/adc.69.5_spec_no.483
更新日期:1993-11-01 00:00:00
abstract:BACKGROUND:Faecal impaction is frequently observed in children with chronic constipation. The term megarectum is often used to describe this finding. AIM:To evaluate rectal functioning and rectal measures in constipated children with a filled rectum, in order to define the terms faecal impaction, enlarged rectum, and ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.83.1.52
更新日期:2000-07-01 00:00:00
abstract:BACKGROUND:The number of trips to the tropics taken by children with chronic health disorders (CHDs) is increasing. METHODS:All of the children with CHDs who attended two international vaccination centres in France before travelling to the tropics were included in a prospective, exposed/unexposed study. Each child was...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2015-309436
更新日期:2016-11-01 00:00:00
abstract:AIM:To investigate the safety of bronchoscopy and endobronchial biopsy in children with difficult asthma, and discuss the ethical issues associated with the procedure. METHODS:A three year prospective observational study was performed in two tertiary paediatric respiratory centres specialising in the management of chi...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究
doi:10.1136/adc.84.5.423
更新日期:2001-05-01 00:00:00