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Springett A.,Queen Mary, University of London | Draper E.S.,University of Leicester | Rankin J.,Northumbria University | Rounding C.,University of Oxford | And 4 more authors.
Birth Defects Research Part A - Clinical and Molecular Teratology | Year: 2014

Background: Exomphalos occurs in 2.2 per 10,000 births with 76% of these babies surviving to discharge. The aim of this study was to determine the birth prevalence and survival of babies with this anomaly in England and Wales. Methods: Six BINOCAR regional congenital anomaly registers in England and Wales (covering 36% of births) between 2005 and 2011 provided cases for this study. Cases included live births, stillbirths (24+ weeks' gestation), late miscarriages (20-23 weeks' gestation), and terminations of pregnancy with fetal anomaly. Results: The overall birth prevalence was 3.8 (95% confidence interval [CI]: 3.6-4.0) per 10,000 births; 1.4 (1.2-1.6) for isolated cases, 1.2 (1.1-1.4) for cases with multiple anomalies, and 1.2 (1.1-1.4) for cases with chromosomal anomalies. The live birth prevalence was 0.8 (0.7-0.9), 0.5 (0.4-0.6), and 0.1 (0.0-0.1) per 10,000 live births, respectively. Edwards syndrome, congenital heart defects, and nervous system anomalies were the most common anomalies associated with exomphalos. A prenatal diagnosis was made in 83% of isolated, 95% of multiple, and 99% of chromosomal cases. Fifty-five percent of isolated and multiple cases were live born, whereas 85% of cases with chromosomal anomalies resulted in a termination of pregnancy with fetal anomaly. The 1-year survival of live born babies with an isolated exomphalos was 92% compared with 81% in cases with multiple anomalies and 27% in cases with chromosomal anomalies (p<0.001). Conclusion: We report a higher birth prevalence than has previously been reported. The proportion of infants surviving with exomphalos remained unchanged over the time period. © 2014 Wiley Periodicals, Inc. Source

Reguerre Y.,University of Angers | Martelli H.,University Paris - Sud | Rey A.,CNRS Gustave Roussy Institute | Rogers T.,Institute of Child Life and Health | And 6 more authors.
European Journal of Cancer | Year: 2012

Background: Localised pelvic rhabdomyosarcomas (pRMS) are rare tumours with a poorer prognosis than the majority of RMS. This study analysed patient outcome according to the type of local therapy delivered and the effect of disease-related factors on prognosis. Patients and methods: 97 children with localised pRMS were enrolled in the SIOP-MMT84, 89 and 95 studies. After primary surgery or biopsy, all children received ifosfamide/actinomycin/vincristine- based chemotherapy. Radiotherapy and surgery were planned in patients failing to achieve complete remission. Results: Median age at diagnosis was 52 months [5 months-18 years]. IRS staging was I for five patients, II for 15 and III for 77. Patients had embryonal RMS (N = 41), alveolar RMS (N = 29), botryoid RMS (N = 3), or not otherwise specified RMS (N = 24). Outcome: 87 patients achieved local control (90%), 37 relapsed (43%), mainly locally (84%). With a median follow-up of more than 10 years [4-22 years], 5-year OS was 66% (95% CI: 56-75%) and EFS was 52% (95% CI: 42-61%). Among the 18 IRS-I/II patients treated without radiotherapy, 15 survived. Seven out of the 20 IRS-III patients treated without local therapy died. In multivariate analysis, IRS staging, age greater than 10 years and lymph node involvement had a negative impact on OS. Perineal/perianal locations had a trend towards a worse prognosis. Conclusion: pRMS still have a relatively poor prognosis. Radiotherapy or brachytherapy is necessary for all IRS-III patients including those with radiological complete remission after neoadjuvant chemotherapy with or without surgery. Radiotherapy may be withheld in IRS-I patients and children under 3 years with IRS-II pRMS. © 2012 Elsevier B.V. All rights reserved. Source

Phillips R.J.,University of Bristol | Al-Zamil H.,University of Bristol | Hunt L.P.,Institute of Child Life and Health | Fortier M.A.,Laval University | Lopez Bernal A.,University of Bristol
Molecular Human Reproduction | Year: 2011

Prostaglandins (PGs) are important factors in the physiology of human parturition and the control of uterine contractility. We have characterized the expression of 15 genes from all stages of the PG pathway in human pregnant and non-pregnant (NP) myometrium and in other uterine tissues at delivery, and the results show patterns indicative of different capacities for PG synthesis and catabolism in each tissue. In placenta, the PG synthase expression profile favours production of PGD 2, PGE 2 and PGF 2, with high levels of PG transporters and catabolic PG dehydrogenase suggesting rapid PG turnover. Choriodecidua is primed for PGE 2, PGF 2 and PGD 2 production and high PG turnover, whereas amnion expresses genes for PGE 2 synthesis with low levels of PG transporters and dehydrogenase. In umbilical cord, PGI 2 synthase is highly expressed. In pregnant myometrium, PGI 2, PGD 2 and PGF 2 synthases are highly expressed, whereas PG dehydrogenase is underexpressed. Myometrium from women with spontaneous or induced labour had higher expression of the PGH 2 synthase PTGS2 than tissue from women not-in-labour. Myometrium from NP women had lower levels of PG synthases and higher levels of PG dehydrogenase than pregnant myometrium. Discriminant function analysis showed that expression of selected genes in myometrium could distinguish groups of women with different modes of labour from each other and from NP women. In cultured myometrial cells, there was a dose-dependent stimulatory effect of interleukin 1β and tumour necrosis factor α on PTGS2, PTGES and AKR1B1 (PGF synthase) expression. © The Author 2010. Published by Oxford University Press on behalf of the European Society of Human Reproduction and Embryology. All rights reserved. Source

Siassakos D.,Southmead Hospital | Siassakos D.,University of Bristol | Bristowe K.,University of the West of England | Hambly H.,Speech Kingdom | And 6 more authors.
Simulation in Healthcare | Year: 2011

Introduction: Patient satisfaction is an important healthcare outcome and communication with clinical staff is an important determinant. Simulation could identify problems and inform corrective action to improve patient experience. Methods: One hundred eight randomly selected maternity professionals in 18 teams were videoed managing a patient-actor with a simulated emergency. The trained patient-actor assessed the quality of staff-patient interaction. Clinicians scored teams for their teamwork skills and behaviors. Results: There was significant variation in staff-patient interaction, with some teams not having exchanged a single word and others striving to interact with the patientactor in the heat of the emergency. There was significant correlation between patientactor perceptions of communication, respect, and safety and individual and team behaviors: number, duration, and content of communication episodes, as well as generic teamwork skills and teamwork behaviors. The patient-actor perception of safety was better when the content of the communication episodes with them included certain items of information, but most teams failed to communicate these to the patient-actor. Conclusion: Some aspects of staff-patient interaction and teamwork during management of a simulated emergency varied significantly and were often inadequate in this study, indicating a need for better training of individuals and teams. © 2011 Society for Simulation in Healthcare. Source

Marcovecchio M.L.,University of Cambridge | Tossavainen P.H.,University of Cambridge | Acerini C.L.,University of Cambridge | Barrett T.G.,University of Birmingham | And 6 more authors.
Diabetes Care | Year: 2010

OBJECTIVE - Familial predisposition to hypertension has been associated with the development of diabetic nephropathy in adults, but there are limited data in adolescents. Our aim was to assess whether parental ambulatory blood pressure (ABP) was associated with ABP and albumin excretion in young offspring with type 1 diabetes. RESEARCH DESIGN AND METHODS - Twenty-four-hour ABP monitoring was performed in 509 young offspring (mean ± SD age 15.8 ± 2.3 years) with type 1 diabetes, 311 fathers, and 444 mothers. Systolic (SBP) and diastolic blood pressure (DBP) measurements during 24 h, daytime, and nighttime were calculated. Three early morning urinary albumin-tocreatinine ratios (ACRs), A1C, and anthropometric parameters were available for the offspring. RESULTS - All paternal ABP parameters, except for nighttime SBP, were independently related to the offspring's ABP (24-h SBP β = 0.18, 24-h DBP β = 0.22, daytime SBP β = 0.25, daytime DBP β = 0.23, and nighttime DBP β = 0.18; all P < 0.01). Maternal 24-h DBP (β = 0.19, P = 0.004), daytime DBP (β = 0.09, P = 0.04), and nighttime SBP (β = 0.24 P = 0.001) were related to the corresponding ABP parameter in the offspring. Significant associations were found between the offspring's logACR and maternal ABP. The association with 24-h DBP (β = 0.16, P = 0.02), daytime DBP (β = 0.16 P = 0.02), and nighttime DBP (β = 0.15 P =0.03) persisted even after adjustment for the offspring's ABP. Mothers of offspring with microalbuminuria had higher ABP than mothers of offspring without microalbuminuria (all P < 0.05). CONCLUSIONS - In this cohort, parental ABP significantly influenced offspring blood pressure, therefore confirming familial influences on this trait. In addition, maternal ABP, particularly DBP, was closely related to ACR in the offspring, suggesting a dominant effect of maternal genes or an effect of the intrauterine environment on microalbuminuria risk. © 2010 by the American Diabetes Association. Source

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