Abstract:
OBJECTIVE:To review the clinical features, treatment, and outcome of children in the UK with Down's syndrome and acute myeloid leukaemia (AML). DESIGN:A retrospective study of 59 children with Down's syndrome and AML presenting between 1987 and 1995. Data were obtained from hospital case notes, trial records, and by questionnaire. RESULTS:The patients were unusually young (median age, 23 months) with a predominance of megakaryoblastic AML. Two of the seven infants who presented with abnormal myelopoesis aged 2 months or younger achieved complete spontaneous remission. Most of the older children with AML (32 of 52) were treated on recognised intensive protocols but 13 received individualised treatment and seven symptomatic treatment alone. Only four received a bone marrow transplant (BMT) in first remission. For the 45 older children who received chemotherapy the overall survival was 55% (median follow up 4.5 years). Patients on individualised protocols had a similar overall survival and toxic death rate but marginally higher relapse rate than those on standard (intensive) protocols. Children with Down's syndrome treated on the national AML 10 trial had a similar overall survival (70% v 59%) at five years to children of comparable age without Down's syndrome: their improved relapse risk (12% v 38%) offset the slight increase in deaths as a result of treatment toxicity (19% v 11%). CONCLUSION:Neonates with Down's syndrome and abnormal myelopoesis may achieve spontaneous remission, and older children with Down's syndrome and AML can be treated successfully with intensive chemotherapy, without BMT.
journal_name
Arch Dis Childjournal_title
Archives of disease in childhoodauthors
Craze JL,Harrison G,Wheatley K,Hann IM,Chessells JMdoi
10.1136/adc.81.1.32subject
Has Abstractpub_date
1999-07-01 00:00:00pages
32-7issue
1eissn
0003-9888issn
1468-2044journal_volume
81pub_type
杂志文章abstract::During a meningococcal (group A) epidemic, 47 Nigerian children with acute meningococcaemia without meningitis were studied. Their mortality rate was 43% compared with 8% during the whole epidemic. Those presenting with coma and shock had a mortality of 93%, but without shock or coma mortality was only 6%. Coma or sho...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.54.1.44
更新日期:1979-01-01 00:00:00
abstract:AIMS:To describe the hospitalisation history in the first five years of life for all children born in Western Australia (WA) between 1983 and 1992 and diagnosed with intellectual disability (ID). METHODS:Unit record linkage of the WA Midwives Collection, WA Intellectual Disability Database, and the WA Hospital Morbidi...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究
doi:10.1136/adc.2004.062422
更新日期:2005-12-01 00:00:00
abstract::Fluids used for flushing arterial catheters, bronchial lavage, and drug administration are not normally considered when assessing daily fluid intake, yet infants weighing less than 1000 g at birth gained up to 12 ml/kg/day of extra fluid and 2.4 mmol/kg/day (mean) of extra sodium from these hidden sources. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.61.7.695
更新日期:1986-07-01 00:00:00
abstract::The Prematometer is a new instrument for the accurate measurement of the length of small neonates inside incubators. It is capable of giving precise results. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.60.9.837
更新日期:1985-09-01 00:00:00
abstract::We compared the efficacy of the prokinetic agent cisapride with that of Gaviscon (an alginate/alkaline compound) plus Carobel (carob seed flour) in the treatment of gastrooesophageal reflux (GOR). Fifty infants with confirmed GOR received either oral cisapride (0.8 mg/kg/day) or Gaviscon plus Carobel for one month in ...
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.67.5.618
更新日期:1992-05-01 00:00:00
abstract::Seventy six children, 18 boys and 58 girls, aged 0-15.9 (median 1.0) years, with acute pyelonephritis were prospectively studied with a technetium-99m dimercaptosuccinic acid (DMSA) scan during infection and two months later. Fifty nine of these children were also studied two years after the infection. Seventeen child...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.70.2.111
更新日期:1994-02-01 00:00:00
abstract::In this overview, I have attempted to highlight the problems of medical care, particularly paediatric care, in Hungary and to describe the changing nature of its organisation and financing. We hope that through our efforts we can surmount these difficulties, improve the quality of medical care in our country, and crea...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.67.8.1042
更新日期:1992-08-01 00:00:00
abstract::Moderate and severe malnutrition are endemic in much of the developing world and in association with pockets of deprivation in the developed world. The cost in terms of individual and social development is high. The principles of effective management are clearly documented. A low cost, community based treatment progra...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.71.4.297
更新日期:1994-10-01 00:00:00
abstract::Samples from the pharynx, stomach, duodenum or jejunum, and faeces were collected on 7 days between 1st and 28th day from neonates weighing less than 1.5 kg at birth who were fed by transpyloric tube. These were cultured on selective and non-selective media, and the results were expressed in a semi-quantitative manner...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.58.2.115
更新日期:1983-02-01 00:00:00
abstract:OBJECTIVES:Data suggest a potential role for vitamin D in autism spectrum disorder (ASD). We wanted to assess the effect of vitamin D3 supplementation compared with placebo in children with ASD. DESIGN:This was a double-blind, randomised, placebo-controlled trial. SETTING:A paediatric outpatient centre at high latitu...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,随机对照试验
doi:10.1136/archdischild-2017-312783
更新日期:2017-11-01 00:00:00
abstract::We studied two neonates fed diluted formula and excessive water who developed hyponatraemic convulsions; treatment included intravenous hypertonic saline and water restriction. Educating mothers is important to stop recurrences. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.64.5.734
更新日期:1989-05-01 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::Six children are described who developed diarrhoea associated with Clostridium difficile during the course of haemolytic uraemic syndrome. The significance of this infection is discussed within the context of the pathophysiology of haemolytic uraemic syndrome. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.69.2.239
更新日期:1993-08-01 00:00:00
abstract:OBJECTIVE:To study the frequency a diagnosis is made in children with early developmental impairment (EDI), and the contribution made to diagnosis by specific investigations. DESIGN:Retrospective case note review. SETTING:Community, neurodisability and neurology department at a UK tertiary centre. PARTICIPANTS:Child...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2017-312843
更新日期:2017-11-01 00:00:00
abstract::Over a 25 year period, 31 asthmatic children received artificial ventilation for acute asthma at Alder Hey Children's Hospital on 48 occasions. Altogether 47 episodes occurred from 1971-89, with no decline in the number of episodes per year (mean 2.5) over this period. Eight children died during intermittent positive ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.65.12.1324
更新日期:1990-12-01 00:00:00
abstract::Twenty-seven children with unilateral proptosis were investigated over a period of 8 1/2 years. Seventeen (63%) of them had local or disseminated malignancy, a higher proportion than in earlier reports. The frequency of inflammatory lesions, particularly ethmoiditis progressing to proptosis, appeared to have decreased...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.56.7.549
更新日期:1981-07-01 00:00:00
abstract:BACKGROUND:Ataxia telangiectasia (A-T) is a rare multisystem disease with high early mortality from lung disease and cancer. Nutritional failure adversely impacts outcomes in many respiratory diseases. Several factors influence nutrition in children with A-T. We hypothesised that children with A-T have progressive grow...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2015-310373
更新日期:2016-12-01 00:00:00
abstract:BACKGROUND:Cerebral palsy is the commonest cause of severe physical disability in childhood. For many years treatment has centred on the use of physiotherapy and orthotics to overcome the problems of leg spasticity, which interferes with walking and can lead to limb deformity. Intramuscular botulinum toxin (BT-A) offer...
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.83.6.481
更新日期:2000-12-01 00:00:00
abstract::Urinary tract anomalies were prospectively investigated with ultrasound in 29 children with functional constipation. These children were compared before and after treatment with 451 age matched healthy controls without constipation. The bladder residue and upper renal tract dilatation after micturition were significan...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.70.1.56
更新日期:1994-01-01 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 European Respiratory Society guidance on bronchoalveolar lavage (BAL) in children was published in 2000. It recommended taking one BAL specimen from the most affected lobe or from the right middle lobe in diffuse disease. In 2007, the European Respiratory Society modified the recommendations for children...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2009.177618
更新日期:2011-03-01 00:00:00
abstract:BACKGROUND:Our current understanding regarding the aetiology of infantile acute liver failure largely derives from studies conducted by regional liver units. This may introduce selection bias and therefore not provide a true reflection of the wider population. METHODS:Every coagulation screen with a prothrombin time ≥...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2019-317360
更新日期:2020-08-01 00:00:00
abstract::Eighteen children suffering from hay fever were treated with intra-nasal beclomethasone dipropionate (400 mug/day) and an identical placebo aerosol in a double-blind cross-over trial. 17 of the children preferred the intranasal beclomethasone dipropionate, one had no preference, none preferred the placebo. The effect ...
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.50.11.875
更新日期:1975-11-01 00:00:00
abstract::We describe two cases of a non-epileptic florid movement disorder presenting as status epilepticus. Both patients presented with florid jerking of the limbs and eyes. Convulsive status epilepticus related to presumed meningitis or encephalitis was suspected in both cases. The patients received treatment for seizures, ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2009.160044
更新日期:2009-11-01 00:00:00
abstract:AIMS:To determine the prevalence of sleep-disordered breathing (SDB) in a clinical sample of overweight and obese children and adolescents, and to examine the contribution of fat distribution. METHODS:Consecutive subjects without chronic lung disease, neuromuscular disease, laryngomalacia, or any genetic or craniofaci...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2006.101089
更新日期:2007-03-01 00:00:00
abstract:OBJECTIVE:The aim was to compare quality of life (QoL) among children and adolescents with different stages of chronic kidney disease (CKD) and determine factors associated with changes in QoL. DESIGN:Cross-sectional. SETTING:The Kids with CKD study involved five of eight paediatric nephrology units in Australia and ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究
doi:10.1136/archdischild-2018-314934
更新日期:2019-02-01 00:00:00
abstract::Since its first description optic nerve hypoplasia has been identified with increasing frequency, and a range of associated problems have been described. The major neurological and endocrine associations are well established, but those factors that predispose to the development of optic nerve hypoplasia remain unclear...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.65.1.103
更新日期:1990-01-01 00:00:00
abstract:BACKGROUND:The mode of delivery has recently gained attention as another potential perinatal risk factor for childhood obesity but results are conflicting. OBJECTIVE:To examine whether caesarean section is independently associated with childhood obesity after adjusting for a broad range of confounding factors. METHOD...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2012-303459
更新日期:2013-07-01 00:00:00
abstract::Trigeminal neuralgia (TN) is a frequent cause of paroxysmal facial pain and headache in adults. Glossopharyngeal neuralgia (GPN) is less common, but can cause severe episodic pain in the ear and throat. Neurovascular compression of the appropriate cranial nerve as it leaves the brain stem is responsible for the sympto...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.82.4.311
更新日期:2000-04-01 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