Liverpool, United Kingdom
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Harigopal S.,Royal Infirmary | Satish H.P.,Royal Liverpool Childrens Hospital | Taktak A.F.G.,University of Liverpool | Southern K.W.,Royal Liverpool Childrens Hospital | Shaw N.J.,Liverpool Womens Hospital
Archives of Disease in Childhood: Fetal and Neonatal Edition | Year: 2011

Aim: To establish a reference range for oxygen saturation (SpO2) in well preterm infants to guide home oxygen therapy using a pulse oximeter and Pulse Oximetry Data Analysis Software (PODS). Methods: SpO2 and heart-Rate profiles of healthy preterm infants receiving mechanical ventilation for less than 6 h and supplemental oxygen for less than 48 h were monitored using a pulse oximeter. The stored data were downloaded from the monitor to a personal computer as individual files. Each infant's files of SpO2 were subsequently displayed in graphic form, and a reference range was constructed using dedicated software, PODS. Results: 43 infants were studied. The median value of all infants mean SpO2 values was 95% (range 92-99%). The median duration of saturations less than 85% and between 85% and 90% were 1% and 2% respectively. Using the study group median, 5th and 95th percentiles, a cumulative frequency curve of time against SpO2 value was constructed (representing the reference range of SpO2 profiles in healthy preterm infants). Conclusion: The SpO2 reference range can be used as an easy and practical guide to compare SpO2 profiles of infants on home oxygen therapy and guide their oxygen therapy.


Koshy G.,Child and Reproductive Health Group | Delpisheh A.,Child and Reproductive Health Group | Brabin B.J.,Child and Reproductive Health Group | Brabin B.J.,Royal Liverpool Childrens Hospital | Brabin B.J.,University of Amsterdam
European Journal of Public Health | Year: 2011

Background: The combined dose response effects of pregnancy cigarette smoke exposure on childhood overweight, obesity and short stature have not been reported. Method: A community based cross-sectional survey of 3038 children aged 5-11 years from 15 primary schools in Merseyside, UK. Self-completed parental questionnaires were used for family characteristics, socio-economic status and parental smoking practices. Children were measured for height and weight and z-scores calculated for parental smoking categories. Results: Of 689 (34.0) mothers who smoked during pregnancy 50.5 smoked ten or more cigarettes daily (heavy smokers). Children of maternal non-smokers had prevalence estimates for overweight, obesity and short stature of 25, 9.6 and 3.2, respectively. Prevalence estimates were higher in children of mothers who were heavy smokers during pregnancy, 31.5 (P=0.001), 15.6 (P<0.001) and 5.5 (P=0.001), respectively. Mean height for age z-scores was lower among heavy maternal (P<0.001) and paternal smokers (P<0.01) compared to non-smokers. Childhood overweight, obesity or short stature were all associated with heavy maternal smoking during pregnancy (all P<0.001). Mean body mass index (BMI) z-scores were higher in boys of mothers who smoked (P=0.043). The adjusted odds ratio for short stature in children of heavy maternal smokers was 2.76 (95 CI 1.21-6.33) and 4.28 (1.37-13.37) if both parents were heavy smokers. The adjusted OR for obesity in children of maternal smokers was 1.61(1.19-2.18). The population attributable risk for short stature was 8.8 (1.1-22.7) for heavy maternal smokers. Conclusion: A dose-response association was observed between pregnancy smoking exposure, short stature and obesity. © 2010 The Author.


Koshy G.,Child and Reproductive Health Group | Brabin B.J.,Child and Reproductive Health Group | Brabin B.J.,Royal Liverpool Childrens Hospital | Brabin B.J.,University of Amsterdam
BMC Medical Research Methodology | Year: 2012

Background: Compliance is a critical issue for parental questionnaires in school based epidemiological surveys and high compliance is difficult to achieve. The objective of this study was to determine trends and factors associated with parental questionnaire compliance during respiratory health surveys of school children in Merseyside between 1991 and 2006. Methods: Four cross-sectional respiratory health surveys employing a core questionnaire and methodology were conducted in 1991, 1993, 1998 and 2006 among 5-11 year old children in the same 10 schools in Bootle and 5 schools in Wallasey, Merseyside. Parental compliance fell sequentially in consecutive surveys. This analysis aimed to determine the association of questionnaire compliance with variation in response rates to specific questions across surveys, and the demographic profiles for parents of children attending participant schools. Results: Parental questionnaire compliance was 92% (1872/2035) in 1991, 87.4% (3746/4288) in 1993, 78.1% (1964/ 2514) in 1998 and 30.3% (1074/3540) in 2006. The trend to lower compliance in later surveys was consistent across all surveyed schools. Townsend score estimations of socio-economic status did not differ between schools with high or low questionnaire compliance and were comparable across the four surveys with only small differences between responders and non-responders to specific core questions. Respiratory symptom questions were mostly well answered with fewer than 15% of non-responders across all surveys. There were significant differences between mean child age, maternal and paternal smoking prevalence, and maternal employment between the four surveys (all p<0.01). Out-migration did not differ between surveys (p = 0.256) with three quarters of parents resident for at least 3 years in the survey areas. Conclusion: Methodological differences or changes in socio-economic status of respondents between surveys were unlikely to explain compliance differences. Changes in maternal employment patterns may have been contributory. This analysis demonstrates a major shift in community parental questionnaire compliance over a 15 year period to 2006. Parental questionnaire compliance must be factored into survey designs and methodologies. © 2012 Koshy and Brabin; licensee BioMed Central Ltd.


Kulkarni A.V.,Hospital for Sick Children | Drake J.M.,Hospital for Sick Children | Kestle J.R.W.,Primary Childrens Medical Center | Mallucci C.L.,Royal Liverpool Childrens Hospital | And 2 more authors.
Neurosurgery | Year: 2010

Background: Endoscopic third ventriculostomy (ETV) has preferentially been offered to patients with more favorable prognostic features compared with shunt. Objective: To use advanced statistical methods to adjust for treatment selection bias to determine whether ETV survival is superior to shunt survival once the bias of patient-related prognostic factors is removed. Methods: An international cohort of children (≤ 19 years of age) with newly diagnosed hydrocephalus treated with ETV (n = 489) or shunt (n = 720) was analyzed. We used propensity score adjustment techniques to account for 2 important patient prognostic factors: age and cause of hydrocephalus. Cox regression survival analysis was performed to compare time-to-treatment failure in an unadjusted model and 3 propensity score-adjusted models, each of which would adjust for the imbalance in prognostic factors. Results: In the unadjusted Cox model, the ETV failure rate was lower than the shunt failure rate from the immediate postoperative phase and became even more favorable with longer duration from surgery. Once patient prognostic factors were corrected for in the 3 adjusted models, however, the early failure rate for ETV was higher than that for shunt. It was only after about 3 months after surgery did the ETV failure rate become lower than the shunt failure rate. CONCLUSIONS: The relative risk of ETV failure is initially higher than that for shunt, but after about 3 months, the relative risk becomes progressively lower for ETV. Therefore, after the early high-risk period of ETV failure, a patient could experience a long-term treatment survival advantage compared with shunt. It might take several years, however, to realize this benefit. © 2010 by the Congress of Neurological Surgeons.


Burwell R.G.,University of Nottingham | Dangerfield P.H.,University of Liverpool | Dangerfield P.H.,Staffordshire University | Dangerfield P.H.,Royal Liverpool Childrens Hospital
Studies in Health Technology and Informatics | Year: 2012

Although considerable progress had been made in the past two decades in understanding the etiopathogenesis of adolescent idiopathic scoliosis (AIS), it still lacks an agreed theory of etiopathogenesis. One problem may be that AIS results not from one cause, but several that interact with various genetic predisposing factors. There is a view there are two other pathogenic processes for idiopathic scoliosis namely, initiating (or inducing), and those that cause curve progression. Twin studies and observations of family aggregation have revealed significant genetic contributions to idiopathic scoliosis, that place AIS among other common disease or complex traits with a high heritability interpreted by the genetic variant hypothesis of disease. We summarize etiopathogenetic knowledge of AIS as theories of pathogenesis including recent multiple concepts, and blood tests for AIS based on predictive biomarkers and genetic variants that signify disease risk. There is increasing evidence for the possibility of an underlying neurological disorder for AIS, research which holds promise. Like brain research, most AIS workers focus on their own corner and there is a need for greater integration of research effort. Epigenetics, a relatively recent field, evaluates factors concerned with gene expression in relation to environment, disease, normal development and aging, with a complex regulation across the genome during the first decade of life. Research on the role of environmental factors, epigenetics and chronic noncommunicable diseases (NCDs) including adiposity, after a slow start, has exploded in the last decade. Not so for AIS research and the environment where, except for monozygotic twin studies, there are only sporadic reports to suggest that environmental factors are at work in etiology. Here, we examine epigenetic concepts as they may relate to human development, normal life history phases and AIS pathogenesis. Although AIS is not regarded as an NCD, like them, it is associated with whole organism metabolic phenomena, including lower body mass index, lower circulating leptin levels and other systemic disorders. Some epigenetic research applied to Silver-Russell syndrome and adiposity is examined, from which suggestions are made for consideration of AIS epigenetic research, cross-sectional and longitudinal. The word scoliogeny is suggested to include etiology, pathogenesis and pathomechanism. © 2012 The authors and IOS Press. All rights reserved.


Nayagam S.,Royal Liverpool Childrens Hospital | Davis B.,Norfolk and Norwich NHS Foundation Trust | Thevendran G.,Tan Tock Seng Hospital | Roche A.J.,Chelsea and Westminster Hospital NHS Foundation Trust
Bone and Joint Journal | Year: 2014

We describe the technique and results of medial submuscular plating of the femur in paediatric patients and discuss its indications and limitations. Specifically, the technique is used as part of a plate-after-lengthening strategy, where the period of external fixation is reduced and the plate introduced by avoiding direct contact with the lateral entry wounds of the external fixator pins. The technique emphasises that vastus medialis is interposed between the plate and the vascular structures. A total of 16 patients (11 male and five female, mean age 9.6 years (5 to 17)), had medial submuscular plating of the femur. All underwent distraction osteogenesis of the femur with a mean lengthening of 4.99 cm (3.2 to 12) prior to plating. All patients achieved consolidation of the regenerate without deformity. The mean follow-up was 10.5 months (7 to 15) after plating for those with plates still in situ, and 16.3 months (1 to 39) for those who subsequently had their plates removed. None developed a deep infection. In two patients a proximal screw fractured without loss of alignment; one patient sustained a traumatic fracture six months after removal of the plate. Placing the plate on the medial side is advantageous when the external fixator is present on the lateral side, and is biomechanically optimal in the presence of a femoral defect. We conclude that medial femoral submuscular plating is a useful technique for specific indications and can be performed safely with a prior understanding of the regional anatomy. © 2014 The British Editorial Society of Bone & Joint Surgery.


Gartshore L.,Royal Liverpool Childrens Hospital
British Journal of Oral and Maxillofacial Surgery | Year: 2010

In 1901, René Le Fort conducted some of the most infamous experiments known to maxillofacial surgery. He was an interesting and highly accomplished man with experience in many fields of surgery. We continue to use much of his research today despite many advances in medicine, yet we know little about the man himself.The aim of this short paper is to introduce you to René Le Fort and to describe the highlights of his experiments, which gave rise to a system of classifying facial fractures. © 2009 The British Association of Oral and Maxillofacial Surgeons.


Gladman G.,Royal Liverpool Childrens Hospital
Paediatrics and Child Health (United Kingdom) | Year: 2013

Asymptomatic cardiac murmurs are a common finding in paediatric patients with some estimates suggesting that 90% of children have a murmur detected at some stage. Most are benign 'innocent' noises or reflect minor structural heart disease of no haemodynamic significance. The degree of parental anxiety invoked by the detection of a murmur in their child is considerable and although in virtually all cases their concerns of significant underlying cardiac disease are unjustified, providing adequate reassurance is a challenge for the health professionals involved. The aim of this article is to guide practitioners in determining which babies or children need urgent referral for a specialist opinion and to provide management suggestions for when a murmur is noted coincidentally during a routine examination. © 2012.


Thompson S.M.,University of Liverpool | Jesudason E.C.,Royal Liverpool Childrens Hospital | Turnbull J.E.,University of Liverpool | Fernig D.G.,University of Liverpool
Birth Defects Research Part C - Embryo Today: Reviews | Year: 2010

Heparan sulfate (HS) is a structurally complex polysaccharide located on the cell surface and in the extracellular matrix, where it participates in numerous biological processes through interactions with a vast number of regulatory proteins such as growth factors and morphogens. HS is crucial for lung development; disruption of HS synthesis in flies and mice results in a major aberration of airway branching, and in mice, it results in neonatal death as a consequence of malformed lungs and respiratory distress. Epithelial-mesenchymal interactions governing lung morphogenesis are directed by various diffusible proteins, many of which bind to, and are regulated by HS, including fibroblast growth factors, sonic hedgehog, and bone morphogenetic proteins. The majority of research into the molecular mechanisms underlying defective lung morphogenesis and pulmonary pathologies, such as bronchopulmonary dysplasia and pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH), has focused on abnormal protein expression. The potential contribution of HS to abnormalities of lung development has yet to be explored to any significant extent, which is somewhat surprising given the abnormal lung phenotype exhibited by mutant mice synthesizing abnormal HS. This review summarizes our current understanding of the role of HS and HS-binding proteins in lung morphogenesis and will present in vitro and in vivo evidence for the fundamental importance of HS in airway development. Finally, we will discuss the future possibility of HS-based therapeutics for ameliorating insufficient lung growth associated with lung diseases such as CDH. © 2010 Wiley-Liss, Inc.


Baillie C.T.,Royal Liverpool Childrens Hospital | Smith J.A.,Royal Liverpool Childrens Hospital
World Journal of Gastroenterology | Year: 2015

Inflammatory bowel disease (IBD) comprises two distinct but related chronic relapsing inflammatory conditions affecting different parts of the gastrointestinal tract. Crohn's disease is characterised by a patchy transmural inflammation affecting both small and large bowel segments with several distinct phenotypic presentations. Ulcerative colitis classically presents as mucosal inflammation of the rectosigmoid (distal colitis), variably extending in a contiguous manner more proximally through the colon but not beyond the caecum (pancolitis). This article highlights aspects of the presentation, diagnosis, and management of IBD that have relevance for paediatric practice with particular emphasis on surgical considerations. Since 25% of IBD cases present in childhood or teenage years, the unique considerations and challenges of paediatric management should be widely appreciated. Conversely, we argue that the organizational separation of the paediatric and adult healthcare worlds has often resulted in late adoption of new approaches particularly in paediatric surgical practice. © The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.

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