Great Ormond St Hospital for Children

London, United Kingdom

Great Ormond St Hospital for Children

London, United Kingdom

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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 | Year: 2010

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.

Morris J.K.,Queen Mary, University of London | Waters J.J.,Great Ormond St Hospital for Children | de Souza E.,Queen Mary, University of London
Prenatal Diagnosis | Year: 2012

Objective: Pregnant women who receive a high screening risk result for Down, Edwards or Patau syndrome are offered diagnostic tests that carry a risk of miscarriage. This study determined how many women had such tests per syndrome diagnosis. Method: The number of tests per Down, Edwards or Patau syndrome diagnosis adjusted for maternal and gestational age at diagnosis was calculated using routine data from 18 (95%) cytogenetic laboratories in England and Wales in 2008. Results: There were 19326 tests that identified 1118 diagnoses of Down syndrome and 615 of Edwards and Patau syndromes. There were eight chorionic villus samplings (CVS) per syndrome diagnosis compared with 16 amniocenteses (gestational age adjusted). The lowest number of tests per diagnosis (three for CVSs and for amniocentesis) resulted from an abnormal ultrasound scan. Among pregnant women, 2.9% had an invasive diagnostic test. If a CVS and an amniocentesis increase the risk of a miscarriage by 1% and 0.5%, respectively, approximately one miscarriage for every 14 Down, Edwards or Patau syndrome diagnosis would have occurred. Conclusion: A simple measurement of the population impact of screening for Down syndrome can be calculated using data already collected. Annual estimates should be produced to monitor the national fetal anomaly screening programme. © 2012 John Wiley & Sons, Ltd.

Peters C.J.,University College London | Peters C.J.,Great Ormond St Hospital for Children | Kayemba-Kays S.,University College London | Geary M.P.P.,Rotunda Hospital | And 2 more authors.
Journal of Clinical Endocrinology and Metabolism | Year: 2013

Background: Gestational diabetes is associated with increased birth size. Blood glucose concentrations within the nondiabetic range affect birth size but whether this influences postnatal growth is unclear. Methods: We measured fasting blood glucose concentrations (FBG) in 1650 singleton Caucasian pregnancies at 12 and 28 weeks' gestation and related values to birth weight and weight at 12 and 24 months of age. Pregnancies complicated by antepartum hemorrhage, gestational diabetes, preeclampsia, and prematurity were excluded. Results: Mean maternal age was 30 years and 49% were primiparous. There was a weak relationship between birth weight (z score) and FBG at 12 (r=0.1; P<.006) and 28 (r=0.1; P<.001) weeks. FBG at 12 and 28 were correlated (r=0.3; P<.001). Mothers at 12 and 28 weeks of pregnancy with higher FBG were shorter and heavier. The relationship between FBG at 12 and 28 weeks and birth weight was not observed in primiparous women and FBG was not associated with weight at any postnatal time point. Conclusions: These data suggest that in a low-risk United Kingdom pregnancy cohort FBG concentrations in the nondiabetic range affect birth weight in multiparous women. The effect is small (50 g change in birth weight/1 mmol/L FBG change) and does not persist into postnatal life. This implies a limited role for maternal glucose status within the normal range in determining size in infancy. © 2013 by The Endocrine Society.

Bladen M.,Great Ormond St Hospital for Children | Main E.,University College London | Hubert N.,Great Ormond St Hospital for Children | Koutoumanou E.,Institute of Child Health | And 2 more authors.
Haemophilia | Year: 2013

Joint damage from bleeding episodes leads to physical or functional limitations in people with haemophilia. Various factors may influence the frequency and severity of joint damage. This study examined whether age, prophylaxis, history of high-titre inhibitors (HTI) and bleeding events influenced the Haemophilia Joint Health Score (HJHS) in children. Medical and physiotherapy notes of boys with severe haemophilia, aged 4-18 years, were reviewed to identify factors associated with increased HJHS. The HJHS of 83 boys (median age: 11) ranged from 0 to 25, with 44/83 (53%) having a score of zero. The median HJHS was 0 (mean 2.6). In the non-HTI group, the HJHS for boys on late prophylaxis was 2.68 times higher than those who started early and the HJHS was on average 10% higher for every additional recent bleed. In this group the odds of having a zero score fell by 30% for every year increase in age. Boys with a history of HTI had higher HJHS scores than the non-HTI group, and age, number of recent bleeds and tolerized status were positively associated with HJHS. The score rose on average by 28% for every year of age and by 76% for non-tolerized boys. This study provides further evidence supporting early prophylaxis use and the importance of immune tolerance therapy. The HJHS is a useful tool for identifying and tracking changes in joint health with respect to therapy or disease progression. With improvements in haemophilia treatment, the disproportionate number of zero scores will continue to make interpretation of the HJHS challenging. © 2013 John Wiley & Sons Ltd.

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 | Year: 2012

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.

Harding S.A.,Great Ormond St Hospital for Children | Nischal K.K.,Great Ormond St Hospital for Children | Nischal K.K.,University College London | Upponi-Patil A.,Great Ormond St Hospital for Children | Fowler D.J.,Great Ormond St Hospital for Children
Ophthalmology | Year: 2010

Purpose: To report our experience of deep anterior lamellar keratoplasty (DALK) in children. Design: Retrospective case note review. Participants: Nine patients (13 eyes) aged from 13 weeks to 14 years, 11 months at the Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children National Health Service (NHS) Trust, London, United Kingdom. Methods: A study of all pediatric patients undergoing DALK from February 2002 to October 2008 was undertaken. Deep anterior lamellar keratoplasty was attempted in 9 children (13 eyes); the procedure was successful in 11 eyes, and 2 eyes progressed to penetrating keratoplasty (PKP). One eye underwent repeat DALK. Preoperative examination included electrophysiology, ultrasound biomicroscopy (UBM), and slit-lamp biomicroscopy. Main Outcome Measures: Complications and visual acuity at last follow-up. Results: Five patients had mucopolysaccharidoses (MPS), 3 patients had scarring presumed to be infectious, and 1 patient had keratoconus. Because of the failure of follow-up and loose sutures, 1 child with MPS had an epithelial rejection and the operation was repeated successfully. All grafts showed good graft clarity 10 to 80 months after grafting with visual acuities ranging from 0.28 to 1.0 logarithm of the minimum angle of resolution. Two children with nonspecific causes of scarring showed good visual acuities 24 to 51 months post-DALK. Two children who had conversion to PKP were lost to follow-up because they had moved abroad. In 4 of the 5 children with MPS, established techniques of DALK could not be performed because of excessive glycosaminoglycans (GAGs) in the stroma. Ultrasound biomicroscopy was used to guide trephination depth in the first instance. In 1 child with MPS, viscodissection was successfully used. All clinically diagnosed scars were histologically confirmed, and electron microscopy of corneal buttons confirmed the diagnosis in patients with MPS. Conclusions: Deep anterior lamellar keratoplasty should be considered in children with MPS and partial-thickness scars. In MPS, viscodissection and the "big bubble" technique may not be useful if there are excessive GAGs in the stroma. Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references. © 2010 American Academy of Ophthalmology.

Slater R.,University College London | Worley A.,Great Ormond St Hospital For Children | Fabrizi L.,University College London | Roberts S.,University College London | And 3 more authors.
European Journal of Pain | Year: 2010

While human infants can display distinctive behavioural and physiological spinal cord and brainstem responses to noxious stimulation, it is not known whether cortical neurons are specifically activated by noxious stimuli in newborns. Here, using a novel approach to time-lock an EEG recording to a clinically required heel lance, we show the presence of a distinct nociceptive-specific potential in newborn infants (35-39 weeks postmenstrual age). The potential can be observed in single trials in the central electrodes (Cz and CPz) and using principal component analysis is characterised by a positivity that occurs at approximately 560 ms post-stimulus (N420-P560; P, positive; N, negative). The magnitude of the nociceptive-specific potential is not dependent on sleep state, whereas an earlier potential (N150-P260-N430), which is sleep-state dependent, is evoked by both noxious and non-noxious stimulation. These results provide the first direct evidence of specific noxious-evoked neural activity in the infant brain and suggest that newborn infants are capable of the sensory-discriminative aspects of pain experience. © 2009 European Federation of International Association for the Study of Pain Chapters.

Brierley J.,Great Ormond Street Hospital for Children | Linthicum J.,Great Ormond St Hospital for Children | Petros A.,Great Ormond Street Hospital for Children
Journal of Medical Ethics | Year: 2013

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.

Colville G.,St Georges Hospital | Colville G.,Great Ormond St Hospital For Children | Pierce C.,Great Ormond St Hospital For Children
Intensive Care Medicine | Year: 2012

Purpose: To establish longitudinal rates of post-traumatic stress in a cohort of child-parent pairs; to determine associations with poorer outcome. Method: This was a prospective longitudinal cohort study set in a 21-bed unit. In total 66 consecutive admissions aged 7-17 years were screened with one parent at 3 and 12 months post-discharge. Measures used were the Children's Revised Impact of Event Scale (CRIES-8) and the SPAN (short form of Davidson Trauma Scale). Results: In total 29 (44 %) child-parent pairs contained at least one member who scored above cut-off 12 months after discharge, with scores increasing over time for 18 parents and 26 children. At 3 months, 28 (42 %) parents and 20 (32 %) children scored above cut-off; at 12 months the rates were 18 (27 %) parents and 17 (26 %) children. Parents scoring above cut-off at 12 months were more likely to have had a child admitted non-electively (100 % vs. 77 %, p = 0.028); had higher 3-month anxiety scores (11.5 vs. 4.5, p = 0.001) and their children had higher post-traumatic stress scores at 3 months (14 vs. 8, p = 0.017). Children who scored above cut-off at 12 months had higher 3-month post-traumatic stress scores (18 vs. 7, p = 0.001) and higher Paediatric Index of Mortality (PIM) scores on admission (10 vs. 4, p = 0.037). Conclusions: The findings that (a) nearly half of families were still experiencing significant symptoms of post-traumatic stress 12 months after discharge; (b) their distress was predicted more by subjective than by objective factors and (c) many experienced delayed reactions, indicate the need for longerterm monitoring and more support for families in this situation. © Copyright jointly held by Springer and ESICM 2012.

Sala D.,Great Ormond St Hospital For Children | Chomto S.,Great Ormond St Hospital For Children | Hill S.,Great Ormond St Hospital For Children
Transplantation Proceedings | Year: 2010

Introduction: Short bowel syndrome (SBS) is a malabsorptive state that occurs following extensive small intestinal resection. The most severely affected children require intravenous feeding/parenteral nutrition (PN). Two common causes in infancy/early childhood are intestinal resection for volvulus and resection of necrotic small intestine in infants with gastroschisis. The aim of this study was to review the long-term outcomes of children with severe intestinal failure who remained dependent on PN for many months or years. We evaluated the outcomes among gastroschisis cases versus those following resection of mid-gut volvulus or congenital short gut. Methods: We reviewed the case records over a 10-year period from 1997 of children presenting by 5 years of age with volvulus and/or congenital short gut or gastroschisis and who were dependent on long-term/home PN. We obtained data regarding the diagnosis, the small intestinal length, the presence of the ileo-cecal valve, the survivals, and the incidences of weaning from PN. Results: Six children underwent resection following volvulus or congenital short gut syndromes and 7 had gastroschisis. Ten of the 13 children underwent surgery as neonates and 3 from 2-5 years of age. The residual small intestinal length was <10 cm in 1, 10-25 cm in 4, 25-50 cm in 5, 50-100 cm in 2, and >100 cm in 1 case. The ileo-cecal valve was removed in 8 children. All children were treated with PN after surgery for at least 7 months. All 7 children with gastroschisis and 1 with <10 cm small intestine after intestinal resection for volvulus still required PN after 14-120 months. Three with gastroschisis died. All 5 children with volvulus and >10 cm small intestine were weaned from PN, thriving at our review 7 months-7 years later. Conclusions: Infants and young children with short gut and >10 cm small intestine gain intestinal autonomy. In contrast, those with gastroschisis have poor outcomes and should be referred for intestinal transplantation assessment more readily than those with after intestinal resection for volvulus short gut. Crown Copyright © 2010.

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