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Le Doare K.,Evelina Childrens Hospital
Archives of disease in childhood. Education and practice edition | Year: 2014

OBJECTIVE: To present a structured approach for an outpatient consultation of a child with recurrent mouth ulcers.METHOD: Review of literature and description of approach followed in our unit.CONCLUSIONS: The literature emphasises the need to consider local and systemic causes for oral ulceration in a child. Focused history and examination are key in establishing the cause and in order to ensure appropriate management. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Sinha M.D.,Evelina Childrens Hospital
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association | Year: 2012

The aim of this study was to evaluate in non-hypertensive children following renal transplantation (TX) the rates and determinants of transition to hypertension. Retrospective case note review of all current paediatric kidney transplant patients in the UK. At baseline (6 months following TX), all included subjects were non-hypertensive with systolic and/or diastolic clinic blood pressure (BP) ≤95th percentile while on no anti-hypertensive therapy. We assessed progression from optimal (systolic and/or diastolic clinic BP<50th percentile), normal (systolic and/or diastolic clinic BP≥50th but <90th percentile) and pre-hypertension (systolic and/or diastolic clinic BP 90th-95th percentile) to hypertension (systolic and/or diastolic clinic BP>95th percentile). If systolic and diastolic BP levels belonged to different categories, the higher of the two levels were used for categorization. At baseline, 146 of 524 (27.9%) children (106 male) median [inter-quartile range (IQR)] age 7.8 years (4.8, 11.8) were non-hypertensive and not on any anti-hypertensive therapy; there were 34 patients (23.2%) with optimal BP, 90 (61.6%) with normal BP and 22 (15.1%) with pre-hypertension. They were followed up for a median of 2.0 (1.0, 4.0) years post-TX. At the end of follow-up, BP was optimal in 37 patients (25.3%), normal in 35 (24.0%), high normal in 2 (1.4%) and 72 (49.3%) had progressed to hypertension. The Kaplan-Meier estimated time at which 50% of patients developed hypertension was 2.0 years for the pre-hypertension and 3.0 years in the normal BP group as opposed to 40% risk at 7-year post-TX in the optimal group (P=0.001 between the three groups). The differences between BP groups remained significant after adjustment for all risk factors on multivariate analysis. Just over 49% of our initially non-hypertensive patients progressed to hypertension following TX. BP needs careful monitoring post-TX and ideally should be maintained in the 'normal' and 'optimal' range.

Lim M.,Evelina Childrens Hospital
Developmental Medicine and Child Neurology | Year: 2011

Inflammation of the central nervous system is a prominent feature in many childhood neurodegenerative conditions, with various studies demonstrating the upregulation of the innate and adaptive immune system. Recent evidence also suggests that this inflammatory process can contribute to further neurodegeneration. Furthermore, immunosuppression in mouse models of a few lysosomal storage disorders has demonstrated that attenuation of this immune response can influence the clinical and neuropathological progression. However, there are significant challenges before this finding translates to patient care. Treating inflammation in neurodegenerative conditions requires the identification of the time point when inflammation becomes pathogenic, after which the safest therapeutic strategies are required to target the various components and confounders of inflammation. Nevertheless, as the progress made towards effective gene-, cellular-, and enzyme-based therapy in most of these disorders has been disappointing, treating pathogenic inflammation may offer the clinician another therapeutic strategy in managing these devastating disorders. © The Author. Journal compilation © Mac Keith Press 2011.

Chin C.,Evelina Childrens Hospital
Artificial Organs | Year: 2013

Congenital heart disease (CHD) is relatively common and there are treatment options for virtually every form of CHD. With good care, the majority of babies can survive to adulthood; however, CHD remains a significant cause of death in infancy and neonates are especially vulnerable. In order to manage these patients, a clear understanding of the underlying pathophysiology and the planned surgical procedure is important. However, attention to detail and meticulous care are equally important, and, in addition, there are several management strategies that may standardize practice and can improve outcome. © 2013, The Authors. Artificial Organs © 2013, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

Sharland G.,Evelina Childrens Hospital
Archives of Disease in Childhood: Fetal and Neonatal Edition | Year: 2010

Congenital heart disease (CHD) is a common form of congenital malformation associated with significant morbidity and mortality. Antenatal diagnosis of lifethreatening forms of CHD may help to improve survival and morbidity as well as allowing parental choice and preparation. The diagnosis of CHD during fetal life can be made with a very high level of diagnostic accuracy in tertiary centres dealing with high-risk pregnancies. However, most cases of CHD will occur in low-risk groups and will only be detected by screening at the time of obstetric ultrasound scans. The concept of antenatal screening for CHD was introduced in the UK over 20 years ago, and current national guidelines recommend that the heart should be examined at the time of the obstetric anomaly scan. However, there remains a large regional variation in prenatal detection rates of CHD. Widespread teaching and training is required to achieve a more uniform standard but this will be associated with significant resource implications.

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