Abstract:
:Experience gained from liver studies in 450 patients with cystic fibrosis, seen in a 38 year period from 1964 to 1992, is surveyed. Of these, 31 (7%) showed findings that indicated multilobular cirrhosis. There was a slight but not significant male predominance: 19 males against 12 females. Liver disease had its onset during childhood in most cases. The natural course of liver disease and of cirrhosis is protracted. All patients were routinely evaluated by way of: (i) clinical examination, (ii) biochemical studies and specifically estimation of transaminases and gamma glutamyltransferase, and (iii) liver imaging, ultrasonography, and computed tomography. The study aimed to detect early liver disease, that is multilobular cirrhosis and its complications, with a view to optimal introduction of treatment with ursodeoxycholic acid as this drug shows promise for preventing or stabilising the cirrhotic process. Effects of surgical treatment on portal hypertension are surveyed. These include portacaval shunting, partial splenectomy (considered the procedure of choice), liver transplant in the event of liver failure, or a triple transplant (liver, lungs, and heart) if necessary. One triple transplant was successfully performed in a boy of 10 years with a 2 year follow up.
journal_name
Arch Dis Childjournal_title
Archives of disease in childhoodauthors
Feigelson J,Anagnostopoulos C,Poquet M,Pecau Y,Munck A,Navarro Jdoi
10.1136/adc.68.5.653subject
Has Abstractpub_date
1993-05-01 00:00:00pages
653-7issue
5eissn
0003-9888issn
1468-2044journal_volume
68pub_type
杂志文章abstract::A novel form of severe, X linked mental retardation associated with alpha thalassaemia (ATR-X syndrome) has recently been described. Two affected cousins are described, one of whom has an unusually mild haematological phenotype. HbH inclusions, which are the hallmark of this disease, were only detected in the peripher...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.70.5.439
更新日期:1994-05-01 00:00:00
abstract::Gross neuropathy consequent upon selective malabsorption of vitamin B12 was diagnosed in a 6 year old Bangladeshi girl brought to Great Britain for further investigation of an unexplained illness of three years' duration. The initial peripheral blood count was normal. Treatment with vitamin B12 has led to substantial ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.59.6.575
更新日期:1984-06-01 00:00:00
abstract::Obesity is becoming a common clinical headache for child health professionals, as the increase in professional and media concern has not been matched by effective clinical solutions. Added to this is considerable confusion about both the definitions of obesity and about which groups of children and adolescents actuall...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/adc.2004.062224
更新日期:2005-04-01 00:00:00
abstract::Vision screening of 13 and 15 year old children by school nurses in Oxfordshire has declined in recent years due to the pressure of other commitments. A study was carried out to evaluate the likely consequences of failing to provide vision screening for secondary schoolchildren, in order to make a decision about the f...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.70.1.14
更新日期:1994-01-01 00:00:00
abstract:AIMS:To study the prevalence of tuberculosis infection among children in household contact with adults having pulmonary tuberculosis, and identify the possible risk factors. METHODS:Children under the age of 5 years who were in household contact with 200 consecutive adults with pulmonary tuberculosis underwent tubercu...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2003.044255
更新日期:2005-06-01 00:00:00
abstract::The partial androgen insensitivity syndrome occurs in 46,XY subjects with phenotypes ranging from perineoscrotal hypospadias with cryptorchidism and micropenis (mild undervirilisation) to clitoromegaly and partial labial fusion (marked undervirilisation). Within an affected family, wide variation in the degree of geni...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.68.4.453
更新日期:1993-04-01 00:00:00
abstract::Doppler ultrasound was used to measure blood flow velocity in the anterior cerebral artery of six premature infants with posthaemorrhagic hydrocephalus, before and after intermittent cerebrospinal fluid (CSF) drainage, on 23 occasions. There was a significant increase in mean blood flow velocity after the drainage pro...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.69.1_spec_no.74
更新日期:1993-07-01 00:00:00
abstract:AIMS:To study how respiratory symptoms reported by children, with or without spirometry, could help to discriminate those with asthma from those without. METHODS:Respiratory symptoms (frequent cough, frequent phlegm, and wheezing) reported by 1646 schoolchildren (aged 8-12 years) in a respiratory questionnaire and the...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2003.033688
更新日期:2004-06-01 00:00:00
abstract::In a family in which the father carried a balanced translocation between chromosomes 15 and 22 two of his children had Prader-Willi syndrome and an unbalanced chromosome complement, having lost the proximal bands from the long arm of chromosome 15. His four other surviving children were normal but carried a balanced t...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.62.8.841
更新日期:1987-08-01 00:00:00
abstract:OBJECTIVE:To assess the benefit of nebulised amiloride added to the standard inpatient treatment of a respiratory exacerbation in cystic fibrosis. DESIGN:Prospective, randomised, double blind, placebo controlled trial. SUBJECTS:27 cystic fibrosis patients (mean age 12.8 years). SETTING:Two hospitals in Leeds, UK. R...
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.73.5.427
更新日期:1995-11-01 00:00:00
abstract:BACKGROUND:Mentoring is important for personal and professional development of doctors. Peer mentoring is a core skill in the UK paediatric postgraduate curriculum. However, there is a paucity of peer mentoring programmes aimed at postgraduate doctors in training (postgraduate trainees), and there are no such schemes w...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2013-304277
更新日期:2014-02-01 00:00:00
abstract::A combined test of anterior pituitary function was used on 21 normal children. It shows that a comprehensive evaluation can be made of growth hormone, thyroid stimulating hormone, adrenocorticotrophic hormone, and gonadotrophin reserve. Simultaneous assessment of those peripheral glands associated with the anterior pi...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.53.4.301
更新日期:1978-04-01 00:00:00
abstract:OBJECTIVE:There is limited information on the psychosocial impact of growing up with Silver-Russell syndrome (SRS), characterised by slow growth in utero leading to short stature in adulthood. Such information could aid families in making difficult treatment decisions and guide management strategies for health professi...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2018-314952
更新日期:2019-01-01 00:00:00
abstract::Of the 265 children aged between 6 and 18 months admitted to hospital in a 26-month period each with his first febrile convulsion, there were 64 who satisfied our criteria for a simple febrile convulsion. Of these, 43 (random) were entered into a double-blind trial of continuous sodium valproate versus phenobarbitone,...
journal_title:Archives of disease in childhood
pub_type: 临床试验,杂志文章
doi:10.1136/adc.55.3.171
更新日期:1980-03-01 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::Remarkable advances have been made over the past decade in defining the burden of newborn mortality and morbidity and stillbirths, and in identifying interventions to address the major risk factors and causes of deaths. However, progress in saving newborn lives and preventing stillbirths in countries lags behind that ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/archdischild-2013-305557
更新日期:2015-02-01 00:00:00
abstract:OBJECTIVE:Assays based on interferon gamma (IFNgamma) are an exciting new development for screening for latent tuberculosis infection (LTBI) in adults, but there are limited data on their effectiveness in children. Nevertheless new National Institute for Health and Clinical Excellence (NICE) guidelines recommend their ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2006.106617
更新日期:2008-03-01 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: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::This study evaluated the accuracy of the interpretation of the chest film in delineating localised abnormalities of ventilation and perfusion, as well as the overall severity of airways obstruction, exercise tolerance, and clinical condition in children with cystic fibrosis. Radiographic findings in various regions of...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.56.2.106
更新日期:1981-02-01 00:00:00
abstract::A cohort of 164 children born within the defined boundaries of greater Manchester during the years 1981 to 1984 inclusive were identified as having bilateral sensorineural hearing loss, giving a prevalence of 1.2/1000 population of 1/817 births. Ninety three children developed an additional conductive hearing loss sec...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.65.7.757
更新日期:1990-07-01 00:00:00
abstract::Mortality from cystic fibrosis throughout childhood is more than twice as high in England and Wales than it is in the State of Victoria, Australia. Possible reasons for this difference are reviewed. ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.59.1.71
更新日期:1984-01-01 00:00:00
abstract::Ninety-six Birmingham children with sickle cell disease were studied prospectively between 1969 and 1979. Thirty-five were homozygotes for HbS (SS), 12 had sickle thalassaemia (S thal), and 23 were double heterozygotes for HbS and C (SC). Twenty-six whose family studies were incomplete were classified as SS or S thal ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.56.9.676
更新日期:1981-09-01 00:00:00
abstract::A 9 month old boy presented with acute respiratory distress and was found to have a left pleural effusion. The chylous nature of the effusion, multiple bony lytic lesions, and splenic cysts lead to the diagnosis of congenital lymphangiomatosis with chylothorax. Surgical intervention including pleurectomy was required ...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.64.7.1058
更新日期:1989-07-01 00:00:00
abstract::In acute paediatrics, overtreatment and overdiagnosis is encountered in threshold and developed countries. The reason for overtreatment may be similar in the two settings, namely the mere availability of invasive procedures, which may lead to their inappropriate use. Physiological healing processes should be integrate...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/adc.2007.124479
更新日期:2008-03-01 00:00:00
abstract::Fine needle aspiration biopsy (FNAB) offers a simple outpatient technique for specimen collection in child tuberculosis suspects with peripheral lymphadenopathy. To perform FNAB with mycobacterial culture on an outpatient basis requires use of a sterile transport medium to facilitate bedside inoculation, maintain orga...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2009.164038
更新日期:2010-01-01 00:00:00
abstract::A preterm infant with classic phenylketonuria required rather less than 90 mg/kg of phenylalanine and between 270 and 290 mg/kg tyrosine daily to achieve a rate of weight gain of around 20 g/kg per day. Using Lofenalac as the low phenylalanine food, the intake of tyrosine, an essential amino acid for patients with phe...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.60.3.263
更新日期:1985-03-01 00:00:00
abstract:BACKGROUND:The epidemiology of congenital infections is ever changing, with a recent resurgence in syphilis infection rates seen in the UK. Identification of congenital infection is often delayed; early recognition and management of congenital infections is important. Testing modalities and investigations are often lim...
journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/archdischild-2020-318841
更新日期:2021-02-01 00:00:00
abstract::To assess the bacteriological quality of unpooled expressed breast milk, a pilot bottle sample of each donation was examined before the milk was given to the neonate. Provided the milk did not contain greater than 2500 organisms/ml or potential pathogens it was used unheated. Milk containing between 2500 and 5000 orga...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.54.10.760
更新日期:1979-10-01 00:00:00
abstract::Thirty preterm infants (gestational age 26 to 30 weeks) were investigated by cross sectional echocardiography using a 5 or 7.5 MHz transducer positioned in the suprasternal notch or the left subclavicular position to enable visualisation of the aortic arch, main pulmonary artery, left pulmonary artery, and ductus arte...
journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.59.4.341
更新日期:1984-04-01 00:00:00