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Wong I.B.Y.,National University of Singapore | Nischal K.K.,Great Ormond St Hospital for Children | Nischal K.K.,Institute of Child Health
Journal of AAPOS

Children are affected by some common external diseases, including allergic conjunctivitis and blepharokeratoconjunctivitis. This workshop aims to familiarize readers with the clinical features of each along with common presentations and to discuss strategies for managing these conditions, with emphasis on newer drugs and therapies. The other group of external diseases that commonly present in children comprises persistent punctate erosions and persistent epithelial defects. Etiology is varied, and making the correct diagnosis requires a systematic approach with close inspection of the microenvironment of the eye. Common causes and treatment modalities will be discussed. Types of lubrication and how they may best be used also will be outlined. © 2010 American Association for Pediatric Ophthalmology and Strabismus. Source

Brierley J.,Paediatric and Neonatal Intensive Care Unit | Linthicum J.,Great Ormond St Hospital for Children | Petros A.,Paediatric and Neonatal Intensive Care Unit
Journal of Medical Ethics

Religion is an important element of end-of-life care on the paediatric intensive care unit with religious belief providing support for many families and for some staff. However, religious claims used by families to challenge cessation of aggressive therapies considered futile and burdensome by a wide range of medical and lay people can cause considerable problems and be very difficult to resolve. While it is vital to support families in such difficult times, we are increasingly concerned that deeply held belief in religion can lead to children being potentially subjected to burdensome care in expectation of 'miraculous' intervention. We reviewed cases involving end-of-life decisions over a 3-year period. In 186 of 203 cases in which withdrawal or limitation of invasive therapy was recommended, agreement was achieved. However, in the 17 remaining cases extended discussions with medical teams and local support mechanisms did not lead to resolution. Of these cases, 11 (65%) involved explicit religious claims that intensive care should not be stopped due to expectation of divine intervention and complete cure together with conviction that overly pessimistic medical predictions were wrong. The distribution of the religions included Protestant, Muslim, Jewish and Roman Catholic groups. Five of the 11 cases were resolved after meeting religious community leaders; one child had intensive care withdrawn following a High Court order, and in the remaining five, all Christian, no resolution was possible due to expressed expectations that a 'miracle' would happen. Source

Doherty G.M.,Kings College | Aurora P.,Great Ormond St Hospital for Children
Paediatric Respiratory Reviews

Lung transplantation is well-established in the treatment of end-stage lung disease in children. Our understanding of the problems associated with transplantation has increased rapidly over the past 25 years. Recent figures suggest this knowledge is starting to translate into improvements in management and survival. The common indications for lung transplantation in children, the process of assessment and the outcomes and complications of transplantation are reviewed. We discuss briefly some of the ethical issues relevant to lung transplantation and review strategies for the future. This information may help the respiratory paediatrician prepare potential candidates and their families for the process of assessment and help him or her anticipate common problems that may occur. © 2009 Elsevier Ltd. All rights reserved. Source

White B.,University College London | Nicholls D.,Great Ormond St Hospital for Children | Christie D.,University College London | Cole T.J.,University College London | Viner R.M.,University College London
International Journal of Obesity

Background: Psychological comorbidities of obesity are well recognised. However, the role of childhood psychological problems in the aetiology of later obesity has been little studied. Design: Secondary analyses of a national birth cohort (1970 British Cohort Study). Analysis: Logistic regression models to predict obesity risk at 26, 30 and 34 years related to hypothesised predictors: maternal and teacher reported child psychological function at 5 and 10 years (general behavioural, conduct, emotional or attentional/hyperactivity problems) and maternal psychological function. Results: General behavioural problems at age 5 years increased the risk of obesity at 30 and 34 years. Persistence of these problems through childhood further increased the obesity risk. Inattention/hyperactivity at 10 years similarly increased risk of obesity at 30 years (adjusted odds ratios (AOR) 1.3). Chronic conduct problems at 5 and 10 years also increased the obesity risk at 30 years (AOR 1.6 (1.1, 2.4) P<0.05). Childhood emotional disorders and maternal psychological function were not associated with adult obesity. Conclusion: Children with early and persistent behavioural problems, particularly conduct problems, hyperactivity and inattention in early and mid-childhood are at an increased risk of obesity in adult life. The promotion of child and adolescent mental health and well-being may form an important part of future obesity prevention strategies. The promotion of healthy eating and activity should form part of secondary prevention and management strategies for children with disruptive behaviour disorders. © 2012 Macmillan Publishers Limited All rights reserved. Source

Prasad R.,Queen Mary, University of London | Chan L.F.,Queen Mary, University of London | Hughes C.R.,Queen Mary, University of London | Kaski J.P.,Inherited Cardiovascular Diseases Unit | And 7 more authors.
Journal of Clinical Endocrinology and Metabolism

Context: Classic ACTH resistance, due to disruption of ACTH signaling, accounts for the majority of cases of familial glucocorticoid deficiency (FGD). Recently FGD cases caused by mutations in the mitochondrial antioxidant, nicotinamide nucleotide transhydrogenase, have highlighted the importance of redox regulation in steroidogenesis. Objective: We hypothesized that other components of mitochondrial antioxidant systems would be good candidates in the etiology of FGD. Design: Whole-exome sequencing was performed on three related patients, and segregation of putative causal variants confirmed by Sanger sequencing of all family members. A TXNRD2-knockdown H295R cell line was created to investigate redox homeostasis. Setting: The study was conducted on patients from three pediatric centers in the United Kingdom. Patients: Seven individuals from a consanguineous Kashmiri kindred, six of whom presented with FGD between 0.1 and 10.8 years, participated in the study. Interventions: There were no interventions. Main Outcome Measure: Identification and functional interrogation of a novel homozygous mutation segregating with the disease trait were measured. Results: A stop gain mutation, p.Y447X in TXNRD2, encoding the mitochondrial selenoprotein thioredoxin reductase 2 (TXNRD2) was identified and segregated with disease in this extended kindred. RT-PCR and Western blotting revealed complete absence of TXNRD2 in patients homozygous for the mutation. TXNRD2 deficiency leads to impaired redox homeostasis in a human adrenocortical cell line. Conclusion: In contrast to the Txnrd2-knockout mouse model, in which embryonic lethality as a consequence of hematopoietic and cardiac defects is described, absence of TXNRD2 in humans leads to glucocorticoid deficiency. This is the first report of a homozygous mutation in any component of the thioredoxin antioxidant system leading to inherited disease in humans. Copyright © 2014 by the Endocrine Society. Source

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