Abstract:
BACKGROUND:Achondroplasia is associated with foramen magnum stenosis (FMS) and significant risk of morbidity and sudden death in infants. A sensitive and reliable method of detecting infants who require decompressive surgery is required. This study aims to describe the incidence and severity of FMS in an unselected, sequential series of infants using a novel MRI score and retrospectively correlate severity with clinical examination and cardiorespiratory sleep (CRS) studies. METHODS:The Achondroplasia Foramen Magnum Score (AFMS) was developed and scores were retrospectively correlated with clinical and CRS data over a 3-year period. RESULTS:Of 36 infants (M:F, 18:18), 2 (5.6%) did not have FMS (AFMS0); 13 (36.1%) had FMS with preservation of the cerebrospinal fluid (CSF) spaces (AFMS1); 3 (8.3%) had FMS with loss of the CSF space but no spinal cord distortion (AFMS2); 13 (36.1%) had FMS with flattening of the cervical cord without signal change (AFMS3); and 5 (13.9%) had FMS resulting in cervical cord signal change (AFMS4). Mean Total Apnea and Hypopnea Index (TAHI) for AFMS0-4 was 3.4, 6.41, 2.97, 10.5 and 25.8, respectively. Severe TAHI had a specificity of 89% but only a 59% sensitivity for AFMS3-4. Neurological examination was normal in 34/36 (94%) patients. Overall, 9/36 (25%) infants required neurosurgery with minimal surgical complications. CONCLUSIONS:Clinical examination and CRS have a low sensitivity for predicting the effects of foramen stenosis on the spinal cord. Routine screening with MRI using AFMS can aid in detecting early spinal cord changes and has the potential to reduce infant morbidity and mortality.
journal_name
Arch Dis Childjournal_title
Archives of disease in childhoodauthors
Cheung MS,Irving M,Cocca A,Santos R,Shaunak M,Dougherty H,Siddiqui A,Gringras P,Thompson Ddoi
10.1136/archdischild-2020-319625subject
Has Abstractpub_date
2021-02-01 00:00:00pages
180-184issue
2eissn
0003-9888issn
1468-2044pii
archdischild-2020-319625journal_volume
106pub_type
杂志文章abstract:OBJECTIVE:To ascertain mortality from 1 to 18 years, and predictors of mortality. DESIGN:Long-term follow-up of population cohort born 1989-1992. SETTING:Births in South East Thames Region. PATIENTS:346 children with bilateral cerebral palsy (CP). INTERVENTIONS:Not applicable. MAIN OUTCOME MEASURES:Mortality; pred...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2009.172841
更新日期:2011-11-01 00:00:00
abstract:AIMS:To describe the epidemiology of children with traumatic brain injury (TBI) admitted to paediatric intensive care units (PICUs) in the UK. METHODS:Prospective collection of clinical and demographic information from paediatric and adult intensive care units in the UK and Eire between February 2001 and August 2003. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究
doi:10.1136/adc.2005.072405
更新日期:2005-11-01 00:00:00
abstract::Chromatographic analysis of sugars in the stools of 40 normal newborn infants has shown the presence of an oligosaccharide in 85% of samples. The oligosaccharide has been shown to contain fucose (6-deoxygalactose), glucose, and galactose and is present in normal breast milk. In addition fucose has also been found in t...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.53.11.913
更新日期:1978-11-01 00:00:00
abstract::Adrenal cortical carcinoma in a 4 1/4-month-old girl was treated by surgery in combination with actinomycin D, cyclophosphamide, and 5-fluorouracil given daily for 5 days every third week for 13 1/2 months. Postoperative hypertension and raised 24-hour 17-hydroxy- and 21-oxosteroids suggested residual microscopical tu...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.53.4.342
更新日期:1978-04-01 00:00:00
abstract::Recent studies of Guillain-Barré syndrome in adults have shown that the requirement for assisted ventilation correlates closely with a poor outcome, and the need for ventilation is now being used as an indication for plasmapheresis. As comparable studies in children have not been performed we reviewed our experience o...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.62.3.288
更新日期:1987-03-01 00:00:00
abstract:OBJECTIVES:To investigate outcome, management, and prevention in Munchausen syndrome by proxy, non-accidental poisoning, and non-accidental suffocation. DESIGN:Ascertainment through British Paediatric Surveillance Unit and questionnaires to responding paediatricians. SETTING:The UK and Republic of Ireland, September ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究
doi:10.1136/adc.78.3.217
更新日期:1998-03-01 00:00:00
abstract::A retrospective study was made of 30 children with Leber's amaurosis (congenital retinal blindness). 24 presented with severe visual impairment, typical ophalmological findings, and absent electroretinograms. 6 other children, though presenting with marked visual loss and absent electroetinograms were later shown to b...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.52.5.399
更新日期:1977-05-01 00:00:00
abstract::A 9 year old girl with symptoms of the Sjögren syndrome showed interstitial lymphocytic infiltrate on renal biopsy. Two years later she had clinical and biological evidence of systemic lupus erythematosus, associated with a typical glomerulonephritis. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.59.2.175
更新日期:1984-02-01 00:00:00
abstract::We have measured total work of breathing before and after the inhalation of water, salbutamol, and ipratropium bromide, given as nebulised solutions, in 39 studies on 25 infants with acute, severe bronchiolitis. Twenty minutes after nebulised water, mean work of breathing per minute was increased by 4% and work per li...
journal_title:Archives of disease in childhood
pub_type: 临床试验,杂志文章
doi:10.1136/adc.58.4.279
更新日期:1983-04-01 00:00:00
abstract:OBJECTIVE:To assess the natural history of respiratory symptoms not labelled as asthma in primary schoolchildren. DESIGN:Repeat questionnaire survey of subgroups identified from a previous questionnaire survey after a two year delay. SUBJECTS:The original population of 5321 Sheffield children aged 8-9 years yielded 4...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.75.5.385
更新日期:1996-11-01 00:00:00
abstract::Three children with cystic fibrosis developed steatorrhoea unresponsive to changes in pancreatic supplements. The final diagnoses were chronic giardiasis, stagnant loop syndrome, and Crohn's disease. Refractory intestinal symptoms in cystic fibrosis merit further investigation. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.63.12.1496
更新日期:1988-12-01 00:00:00
abstract:OBJECTIVE:To assess the risk of skin cancer in persons treated with neonatal phototherapy (NNPT) for jaundice. DESIGN:Retrospective cohort study. SETTING:Grampian Region, Scotland, UK. DATA SOURCE:Aberdeen Maternity and Neonatal Databank. NNPT exposure was abstracted from paper records spanning 1976-1990. Follow-up ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2009.179275
更新日期:2010-10-01 00:00:00
abstract:OBJECTIVE:Children with Down syndrome are at high risk of obstructive sleep apnoea (OSA) and screening is recommended. Diagnosis of OSA should be confirmed with multichannel sleep studies. We aimed to determine whether home pulse oximetry (HPO) discriminates children at high risk of OSA, who need further diagnostic mul...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2017-314409
更新日期:2018-10-01 00:00:00
abstract:BACKGROUND:Chronic abdominal pain (CAP) is common among schoolchildren, but risk factors for its onset are still largely unknown. AIMS:To determine the frequency of onset of CAP in schoolchildren and investigate risk factors for its development. METHODS:1411 schoolchildren aged 11-14 years were recruited from schools...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究
doi:10.1136/adc.2006.115089
更新日期:2007-12-01 00:00:00
abstract::Retrospective analysis of glycosylated haemoglobin concentrations in a diabetic outpatient clinic over three and a half years showed a small seasonal variation in the mean value with a peak in mid February and a nadir in mid August. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.62.9.959
更新日期:1987-09-01 00:00:00
abstract:AIMS:To determine whether the British 1990 growth charts are valid for cross sectional and longitudinal use in primary school children. METHODS:A total of 694 children aged 7-10 years from 10 Leeds primary schools were weighed and measured annually over three years by an expert auxologist. SD scores were calculated us...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.83.4.298
更新日期:2000-10-01 00:00:00
abstract:BACKGROUND:Several studies have shown an increased incidence of neurodevelopmental impairment in very preterm survivors at school age compared with controls. AIM:To compare findings in the same cohort at 8 years and 15 years. METHODS:A total of 151 of the 224 eligible infants born before 33 weeks of gestation from 19...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2002.006676
更新日期:2004-03-01 00:00:00
abstract:BACKGROUND:Paediatric early warning scores (PEWS) are widely used as an adjunct to support staff in recognising deterioration in hospitalised children. Relatively little is known about how staff use these systems. OBJECTIVE:To examine the completeness and accuracy of PEWS recording in hospitalised children in a tertia...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2018-316248
更新日期:2019-12-01 00:00:00
abstract::A percutaneous lung biopsy sample was used to diagnose interstitial lung disease in nine children aged less than 42 months. Fibrosing alveolitis was diagnosed in eight children and Pneumocystis carinii pneumonia in one child. Complications associated with the procedure were minimal and the results of the biopsy sample...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.70.2.143
更新日期:1994-02-01 00:00:00
abstract:AIMS:To review the results of long term high dose intravenous immunoglobulin treatment. METHODS:162 treatment years in 18 patients with hypogammaglobulinaemia who received intravenous immunoglobulin treatment between 1973 and 1993 were reviewed. RESULTS:A mean dose of 0.42 g/kg immunoglobulin resulted in a mean troug...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.74.6.527
更新日期:1996-06-01 00:00:00
abstract::Transient hyperphosphatasaemia is usually a chance finding arising from the use of biochemical screening profiles. Clinical correlates in cases so far described have been non-specific. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.59.10.996
更新日期:1984-10-01 00:00:00
abstract::Thirty-two children with a past diagnosis of coeliac disease were reinvestigated by means of gluten challenge. They had received a gluten-free diet for a mean period of 6-5 years (range 0-25-11-0) before gluten challenge, and had a mean age of 9-9 years (range 3-0-15-3) at the onset of challenge. Small intestinal biop...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.53.6.449
更新日期:1978-06-01 00:00:00
abstract::The temperatures of 587 children were taken before and after diphtheria/tetanus/pertussis (DTP) or diphtheria/tetanus (DT) vaccine. Only slight temperature increases were found but these were notably more frequent after plain than adsorbed DTP vaccine preparations and the frequency increased with each successive dose....
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.58.11.921
更新日期:1983-11-01 00:00:00
abstract::Thirty seven healthy infants (18 born at full term and 19 preterm) were studied serially with cross sectional and Doppler echocardiography to compare their postnatal circulatory adaptation. Pulmonary artery pressure was assessed by its inverse relationship with the ratio of pulmonary artery time to peak velocity and r...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.65.1_spec_no.24
更新日期:1990-01-01 00:00:00
abstract:BACKGROUND:Acute respiratory infections (ARI) cause 3 million deaths in children worldwide each year. Most of these deaths occur from pneumonia in developing countries, and hypoxaemia is the most common fatal complication. Simple and adaptable indications for oxygen therapy are important in the management of ARI. The c...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/adc.2008.138362
更新日期:2009-01-01 00:00:00
abstract::Metabolic rhythms were studied over 24 hours in eight adolescent diabetic patients during treatment with porcine insulin and after transfer to human insulin. Despite an increase in dose with human insulin no significant changes were found in fasting blood glucose, 24h mean blood glucose, or glycosylated haemoglobin co...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.61.4.341
更新日期:1986-04-01 00:00:00
abstract:OBJECTIVE:To investigate the accuracy of morphine infusions prepared for neonates in relation to the label strength and to identify the differences in deviation between infusions made in neonatal intensive care unit (NICU) and those dispensed ready-to-use from pharmacy. METHODS:Unused portions of morphine solution for...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2013-304522
更新日期:2013-12-01 00:00:00
abstract:OBJECTIVES:To assess bone and collagen turnover in asthmatic children treated with dry powder budesonide from the Turbuhaler and dry powder beclomethasone dipropionate from the Diskhaler in a dose of 800 micrograms/day. SUBJECTS:Thirteen prepubertal children with asthma. DESIGN:Open crossover study with two treatment...
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.73.6.524
更新日期:1995-12-01 00:00:00
abstract::The reliability and validity of the North American paediatric appropriateness evaluation protocol (PAEP) for use in paediatric practice in Britain was tested. The protocol was applied to 418 case records of consecutive emergency admissions to three Yorkshire district general hospitals. The PAEP ratings were then compa...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究
doi:10.1136/adc.77.4.294
更新日期:1997-10-01 00:00:00
abstract::A 9 year old girl presenting with multiple glomus tumours is reported. Multiple glomus tumours are rare, often asymptomatic lesions, with a familial tendency and found in a more proximal location than their solitary counterparts. In our report four close relatives had similar lesions. Indications for surgical excision...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.61.8.801
更新日期:1986-08-01 00:00:00