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Williams A.,University of Cardiff | Kenny D.,The Bristol Royal Hospital for Children | Wilson D.,University of Cardiff | Fagenello G.,The Bristol Royal Hospital for Children | And 5 more authors.
European Journal of Clinical Investigation | Year: 2012

Background Aortic dilatation is the main therapeutic target in patients with Marfan syndrome. Standard treatment with a β-blocker may not lower central pulse pressure - the major objective - because it does not do so in hypertension, unlike angiotensin-converting enzyme inhibitors and calcium-channel blockers. We therefore performed a prospective, randomised, double-blind, crossover trial to compare the effects of these three agents on large artery function and central aortic pressure in patients with Marfan syndrome. Methods and Results Eighteen patients had applanation tonometry, pulse wave analysis and echocardiography, before and after atenolol 75mg, perindopril 4mg and verapamil 240mg, each given for 4weeks, in a random order, with 2weeks between medications. Fourteen patients completed the study. Within-drug comparisons demonstrated that perindopril (-10·3mmHg, P=0·002), verapamil (-9·2mmHg, P=0·003) and atenolol (-7·1mmHg, P=0·01) all reduced central systolic pressure and brachial pressure; central changes were least, and peripheral changes greatest with atenolol but between-drug comparisons (analysis of covariance) were not significant. There was a trend for augmentation to be reduced by perindopril (-6·3%, P=0·05), verapamil (-5·5%, P=0·07) and atenolol (-3·2%, P=0·09). Only atenolol reduced heart rate (by 16%) and delayed expansion in the arch and abdominal aorta (by 8% and 11%) (P<0·001, P<0·01 and P<0·05, respectively, for between-drug comparisons). Conclusions Perindopril, verapamil and atenolol all reduced peripheral and central systolic pressure. As atenolol slowed heart rate and delayed aortic wave travel, β-blockade may have a continuing role in the treatment of patients with Marfan syndrome. © 2012 The Authors. European Journal of Clinical Investigation © 2012 Stichting European Society for Clinical Investigation Journal Foundation.

Mellor C.,The Bristol Royal Hospital for Children | Heckford E.,University of Wales | Frost J.,The Childrens Hospice South West
Paediatrics and Child Health | Year: 2012

This article tracks the development of paediatric palliative care from the appreciation of children's entitlement to high quality palliative care in the 1970s through to the formal recognition of paediatric palliative medicine as a subspecialty by the RCPCH in 2009. It summarizes recommendations from recent key documents with respect to the development of equitable and sustainable paediatric palliative care services and it also outlines opportunities for training in this evolving specialty. We then describe best practice in three areas of paediatric palliative care that are topical at present; advance care planning, transitional care and extubation within a palliative care framework. The principles of practice in each of these areas can be applied to much of paediatrics and it remains the case that much of palliative medicine in children can and should be provided by generalists. However, it is hoped that increased awareness of how paediatric palliative care services are evolving and the specialist care that is available, will enable enhanced collaboration with specialist services when required. © 2011 Elsevier Ltd.

Kelly A.,The Bristol Royal Hospital for Children | Kelly A.,Bristol Royal Hospital for Children | Kelly A.,University of Bristol | Ramanan A.V.,The Bristol Royal Hospital for Children | And 2 more authors.
Paediatrics and Child Health | Year: 2011

Juvenile idiopathic arthritis (JIA) is characterized by joints with swelling, pain, and limitation of movement. The main principle of treatment is to control this active arthritis in order to prevent permanent damage. This review describes the different types of JIA, a stepwise approach to treatment according to the level of disease activity, and the medications used. © 2011 Elsevier Ltd.

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