Abstract:
:Cystic fibrosis is a disease that still causes a reduced life expectancy. The treatment burden remains high for affected individuals with often a combination of multiple oral and inhaled medications, as well as physiotherapy, required on a daily basis. In this article, we look at an overview of the pathogenesis, how this might lead to treatment options and look at some of the available new therapies, all in the aim of increasing life expectancy and reducing treatment burden.
journal_name
Arch Dis Childjournal_title
Archives of disease in childhoodauthors
Davies JC,Ebdon AM,Orchard Cdoi
10.1136/archdischild-2013-304400subject
Has Abstractpub_date
2014-11-01 00:00:00pages
1033-6issue
11eissn
0003-9888issn
1468-2044pii
archdischild-2013-304400journal_volume
99pub_type
杂志文章,评审abstract::Insulin dependent diabetes mellitus presenting in children under five years old exhibits several clinical and management features that differ from diabetes presenting in older children. In this review of the current population of the Oxford children's diabetes clinic, children with diabetes diagnosed aged 0- less than...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.60.12.1144
更新日期:1985-12-01 00:00:00
abstract:OBJECTIVE:To evaluate different methods of examination of the bowel in suspected inflammatory bowel disease. DESIGN:Prospective investigation of all children over a three year period with suspected inflammatory bowel disease. A technetium-99m-HMPAO labelled white cell scan (Tc-WCS), barium follow through examination (...
journal_title:Archives of disease in childhood
pub_type: 临床试验,杂志文章
doi:10.1136/adc.74.1.22
更新日期:1996-01-01 00:00:00
abstract::To determine the natural history and psychological impact of the growth pattern in boys with constitutional delay in growth and puberty (CDGP), 43 boys presenting with short stature due to CDGP were followed up to final height. At presentation mean (SD) chronological age was 14.0 (1.9) years, bone age delay 2.7 (1.0) ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.65.10.1109
更新日期:1990-10-01 00:00:00
abstract::Analysis of both the content and process of examinations is central to planning the appropriate education and training of examiners in paediatric clinical examinations. This paper discusses the case for developing training, reviews the current literature, and suggests the desirable attributes of examiners and the comp...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/adc.2004.055103
更新日期:2005-01-01 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: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::Intestinal D-fructose absorption in 31 children was investigated using measurements of breath hydrogen. Twenty five children had no abdominal symptoms and six had functional bowel disorders. After ingestion of fructose (2 g/kg bodyweight), 22 children (71%) showed a breath hydrogen increase of more than 10 ppm over ba...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.59.8.735
更新日期:1984-08-01 00:00:00
abstract:BACKGROUND AND AIMS:The case fatality rate from meningococcal disease (MD) has remained relatively unchanged in the post antibiotic era, with 20-50% of patients who develop shock still dying. In 1992 a new paediatric intensive care unit (PICU) specialising in MD was opened. Educational information was disseminated to l...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.85.5.386
更新日期:2001-11-01 00:00:00
abstract:OBJECTIVES:To investigate whether meningococcal C conjugate vaccine (MCCV) caused relapse in children with steroid-responsive nephrotic syndrome. DESIGN:A population-based study was conducted using an active surveillance system, developed to assess adverse events following vaccination, which linked hospital record inf...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2006.105247
更新日期:2007-10-01 00:00:00
abstract::The changes in transcutaneous oxygen tension (TcPO2) during 123 observations of 10 care procedures were compared with the intrinsic variability of the TcPO2 at the time of the procedures. Standard deviations of the TcPO2 were measured by planimetry from continuous recording in undisturbed infants during a 10-minute co...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.58.1.20
更新日期:1983-01-01 00:00:00
abstract::At the Birmingham Maternity Hospital the mean cost of caring for surviving infants who require neonatal intensive care ranges from approximately pounds 2500 (for infants above 1500 g birthweight), to pounds 5500 (for infants 1000 to 1499 g birthweight), to pounds 10 000 (for infants less than 1000 g birthweight). The ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.59.11.1064
更新日期:1984-11-01 00:00:00
abstract:AIMS:To develop and implement an evidence based guideline for the treatment of acute asthma using a metered dose inhaler and spacer combination. METHODS:Defined strategies were used for the development and implementation of a guideline, assessed by a prospective, descriptive, study using notes review, and patient, nur...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.84.2.142
更新日期:2001-02-01 00:00:00
abstract:INTRODUCTION:UK screening for congenital hypothyroidism (CH) is based on dried blood spot Thyroid Stimulating Hormone (TSH). Scintigraphy may identify CH subtypes classified as dysplasia, gland in situ (GIS) and ectopia, but is not performed in all centres. We retrospectively investigated the role of scintigraphy to id...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2019-317665
更新日期:2021-01-01 00:00:00
abstract:BACKGROUND:Sweat testing procedures are perceived to vary widely. AIM:To evaluate variability in sweat collection, analysis, and interpretation. METHODS:Questionnaire responses from 30 self selected centres: 15 paediatric centres, and 15 district general hospitals. RESULTS:Centres carried out 30-400 tests per year (...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究
doi:10.1136/adc.82.5.425
更新日期:2000-05-01 00:00:00
abstract::Lipofuscin develops in cells when peroxidation damage occurs. Its development in the liver of patients receiving prolonged parenteral nutrition suggests that peroxidation damage by free radicals has occurred. Deficiencies in antioxidants such as vitamin E may be an important contributing factor. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.60.8.774
更新日期:1985-08-01 00:00:00
abstract::The role of inflammation in adult asthma is well known, involving a cascade of immunological stimulation in which mast cells and eosinophils play pivotal roles. However, the assessment of airway inflammation in children is more difficult as the invasive methods used in adults cannot ethically be used for this purpose ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/adc.82.suppl_2.ii6
更新日期:2000-06-01 00:00:00
abstract::Two Japanese outbreaks of erythema infectiosum were investigated for evidence of human parvovirus infection by a solid phase antibody capture radioimmunoassay based on a monoclonal antibody to human parvovirus. Specific IgM and high concentrations of specific IgG were detected in 37 sera from 27 children with erythema...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.59.11.1016
更新日期:1984-11-01 00:00:00
abstract::A term female infant was admitted to the intensive care unit with the diagnosis of tetralogy of Fallot with critical pulmonary stenosis. On the seventh day of life a long saphenous line was inserted that remained without complications until seven days later when the infant appeared septic. A lumbar puncture demonstrat...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.67.10_spec_no.1201
更新日期:1992-10-01 00:00:00
abstract::We describe a child with primary defect of polymorph bacterial killing associated with systemic lungs erythematosus. We suggest that her autoimmune disease results from chronic bacterial antigen stimulation and propose a hypothetical model linking immunodeficiency with autoimmunity. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.56.5.382
更新日期:1981-05-01 00:00:00
abstract:BACKGROUND:Human metapneumovirus (hMPV) causes lower respiratory tract infections, particularly in young children and the elderly. METHODS:A prospective study was conducted on the clinical characteristics of infants <2 years of age admitted to hospital for respiratory infection and the characteristics of hMPV infectio...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2005.082388
更新日期:2006-04-01 00:00:00
abstract::Levels of calcium, magnesium, and glucose were measured in the blood and cerebrospinal fluid (CSF) of children with febrile convulsions. Calcium and magnesium levels were within the normal range and the blood: CSF ratios were similar to those of normal subjects. Hyperglycaemia was a frequent finding and was reflected ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.51.2.141
更新日期:1976-02-01 00:00:00
abstract::A 6-week-old girl with fever, hypernatraemia, dehydration, and polyuria failed to concentrate urine in response to exogenous vasopressin administration. There was no family history of nephrogenic diabetes insipidus. When she was 15 months old, the infusion of vasopressin did not produce an increase in urinary cyclic-A...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.53.11.unknown
更新日期:1978-11-01 00:00:00
abstract:AIMS:To review the precipitating events, clinical features, treatment, and outcome of macrophage activation syndrome (MAS). METHODS:Retrospective review of cases of MAS from a prospectively collected database of children with rheumatic diseases from 1980 to 2000. RESULTS:Nine patients (eight girls) were considered to...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.85.5.421
更新日期:2001-11-01 00:00:00
abstract:AIM:To identify the experiences of patients, parents or carers when a child/young person is prescribed a new long-term medicine. METHOD:Patients' prescribed a new long-term (>6 weeks) medicine were recruited from a single UK paediatric hospital out-patient pharmacy.A semi-structured questionnaire was administered to p...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2016-311535.58
更新日期:2016-09-01 00:00:00
abstract::Safe and effective paediatric pharmacotherapy requires careful evaluation of the type of drug substance, the necessary dose and the age-appropriateness of the formulation. Generally, the younger the child, the more the attention that is required. For decades, there has been a general lack of (authorised) formulations ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/archdischild-2015-308227
更新日期:2016-07-01 00:00:00
abstract::Six newborn infants with intrauterine supraventricular tachyarrhythmias (five cases of atrial flutter and one of supraventricular tachycardia) are described. Transplacental digitalisation was attempted in three cases. Supraventricular tachycardia associated with hydrops fetalis, detected in a fetus at a gestation of 3...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.61.10.996
更新日期:1986-10-01 00:00:00
abstract::The clinical course of 6 children with primary intestinal lymphangiectuasia who have been treated with low fat medium chain triglyceride-supplemented diets for between 3 and 8 years (4 for longer than 5 years) is described. Though laboratory findings indicate continuing chyle leak, evidence for long-term benefit from ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.50.4.269
更新日期:1975-04-01 00:00:00
abstract::Breast fed infants may be at greater risk of mother to infant hepatitis B virus infection compared with formula fed infants. We studied 85 infants born to 84 hepatitis B surface antigen positive mothers (only two of whom were hepatitis B e positive), and who had received immunisation against hepatitis B virus. Our res...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.60.10.972
更新日期:1985-10-01 00:00:00
abstract::By means of a simple questionnaire and measurements of height, weight, and peak expiratory flow rates 3061 children from city and rural populations were studied. Children with asthma or other respiratory diseases had lower peak expiratory flow rates, and younger children living in rural areas had higher rates. In 2828...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.64.1.96
更新日期:1989-01-01 00:00:00
abstract:OBJECTIVE:To assess the availability and source of guidelines for common infections in European paediatric hospitals and determine their content and characteristics. DESIGN:Participating hospitals completed an online questionnaire on the availability and characteristics of antibiotic prescribing guidelines and on empi...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究
doi:10.1136/archdischild-2015-308255
更新日期:2016-01-01 00:00:00