Royal College of Paediatrics and Child Health British Paediatric Surveillance Unit 16th Annual Report 2001-200214th14Annual Report 1998/99 The British Paediatric Surveillance Unit (BPSU) welcomes invitations to give talks on the work of the Unit and takes every effort to respond positively. Enquiries should be made direct to the BPSU office. The BPSU positively encourages recipients to copy and circulate this report to colleagues, junior staff and medical students. Additional copies are available from the BPSU office, alternatively the report can be viewed via the BPSU website. Published September 2002 by the: British Paediatric Surveillance Unit A unit within the Research Division of the Royal College of Paediatrics and Child Health 50 Hallam Street London W1W 6DE Telephone: 44 (0) 020 7307 5671 Facsimile: 44 (0) 020 7307 5694 E-mail: [email protected] Website: http://bpsu.rcpch.ac.uk Registered Charity no 1057744 ISBN: 1 900954 76 1 © British Paediatric Surveillance Unit 2002 British Paediatric Surveillance Unit – Annual Report 2001-2002 Compiled and edited by Richard Lynn, Hilary Kirkbride, Mike Preece, and Jugnoo Rahi, September 2002 Membership of Executive Committee 2001/2002 Dr Christopher Verity* Outgoing chair Professor Michael Preece# Chair Dr Claire Bramley# Scottish Centre for Infection and Environmental Health Dr Angus Clarke* Dr Allan Colver Professor Richard Cooke** Royal College of Paediatrics and Child Health Research Division Professor Denis Gill Royal College of Physicians (Ireland) Ms Linda Haines Royal College of Paediatrics and Child Health Research Division Dr Patricia Hamilton Royal College of Paediatrics and Child Health Dr Alun Elias-Jones Royal College of Paediatrics and Child Health Dr Ian Jones* Scottish Centre for Infection and Environmental Health Dr Christopher Kelnar* Dr Hilary Kirkbride Medical Adviser (infectious disease) Dr Gabrielle Laing Mr Richard Lynn Scientific Co-ordinator Professor Catherine Peckham ICH (London) Dr William McGuire Professor Neil McIntosh ++ Dr Angus Nicoll Public Health Laboratory Service Dr Jugnoo Rahi Medical Advisor (non-infectious disease) Dr Martin Richardson Professor Brent Taylor* Mrs Carol Youngs Parent and Carers Committee representative Dr Roderick MacFaul* Department of Health (observer) Dr Simon Lenton Department of Health (observer) * retired in 2001 ** Retired April 2001 # September 2001 ++ April 2001 i Contents Membership of Executive 5 Surveillance studies undertaken in 2001 Committee 2001/2002 i Cerebrovascular disease, stroke & like illness 12 Congenital cytomegalovirus 14 Congenital rubella 16 Encephalitis in children two months to three years 18 Foreword 1 HIV/AIDS infection in childhood 20 by Professor Mike Preece, Internal abdominal injuries due to child abuse Chairman of the BPSU Executive Committee in children under 14 years 22 Progressive intellectual & neurological deterioration 25 1 Introduction Thrombosis in childhood 28 Aims of the BPSU 3 Vitamin K deficiency bleeding 29 Key challenges 3 6 New studies for 2002 2 How the surveillance Suspected fatal adverse drug reactions 32 system works Congenital toxoplasmosis 32 Selection of studies for inclusion in the scheme 4 Severe complications to varicella 34 The reporting system 4 Langerhan cell histiocytosis 35 Follow-up and confirmation of case reports 6 Difficulties in case reporting 6 The use of complementary data sources 7 Funding 7 7 The international perspective 37 Appendices 3 Surveillance activities in 2001 Appendix A Completed studies 45 Participation in the scheme 8 Appendix B Published papers 48 Workload of those reporting 10 Appendix C Recent presentations 49 Appendix D Support groups & contacts 50 Appendix E Contact addresses 51 4 Main findings of studies undertaken in 2001 11 ii Foreword This is my first Foreword for the Annual Report and the past of various projects run through the BPSU. It was a very interesting year has seen a number of changes and innovations. Chris and stimulating session endorsed from the feedback received Verity has stepped down as Chairman and I succeeded him last by delegates. November. Already I have discovered what a hard act he is to follow. The past five years under his Chairmanship has been a There is an increasing anxiety concerning confidentiality and particularly successful period for the BPSU, but they have also consent. In the past the BPSU has carried out, with ethics seen a number of significant challenges. I will discuss some of approval, its surveillance, in most cases, without prior consent the more notable issues later. from patients or their families. Great care is taken that the data collected is managed in an appropriate manner and that minimal A number of other stalwarts have also left the Executive identifying data is retained. Ideally it should be completely Committee. Richard Cooke has come to the end of his term as anonymised, but sometimes it is necessary to keep minimal Vice-President (Research) and is succeeded by Neil MacIntosh. identifiers to avoid duplication when data comes from multiple I thank Richard for his support in past years. Ian Jones, who sources. If prior consent were needed it would seriously hamper represented the Scottish Centre for Infection and Environmental the Units work (in that the reporting process would become Health (SCIEH), Rodderick MacFaul from the Department of more complex and prolonged); in one study undertaken by a Health (DH), Peter Kearney representing the Royal College of similar surveillance system where consent was sought (because Physicians of Ireland (RCPI), Angus Clarke, Chris Kelnar, and there was a need to approach families with a questionnaire) the Brent Taylor all leave the committee and deserve thanks for their whole project was significantly hampered. There is also the fear support over the years. Their places have been taken by Clare that failure to give consent, if asked, might lead to biased Bramley from SCIEH, Simon Lenton from DH, Denis Gill for the ascertainment that would undermine the validity of the whole RCPI, Martin Richardson, William McGuire, and Allan Colver; surveillance process. This issue was the subject of a highly to them all, welcome. relevant publication in the BMJ in May (C. M. Verity and A. Nicoll, Consent, confidentiality, and the threat to public health In April the BPSU successfully hosted the second International surveillance. Br.Med.J. 2002; 324:1210-1213). Network of Paediatric Surveillance Units (INoPSU) conference, held over two days at York University, in conjunction with the Recent legislation that bears upon the handling of patient Annual Scientific meeting of the College. The first day brought identifiable data places the BPSU’s activities at risk and this has together 20 representatives from 12 of the 14 national surveillance been further threatened by statements from the General Medical units. Dr Chris Verity, Richard Lynn and I represented the UK. Council on confidentiality and consent. One possible way Countries represented at the meeting were Germany, Netherlands, forward lies in the Health and Social Care Act 2001, which Australia, New Zealand, Republic of Ireland, Wales, Switzerland, contains a facilitating clause (Section 60) that would allow certain Canada, Malaysia, Portugal and Greece. The aims of INoPSU specified surveillance activities to be continued, under closely are to facilitate communication between existing units; encourage regulated conditions, if an adequate case can be made in terms the sharing of information between researchers and to assist in of public health benefit. We are studying this with care. Over the development of new units. With the final aim in mind the the past nine months the Nuffield Trust have sponsored an Portuguese and Irish Paediatric Surveillance Units were accepted extensive consultation on the issues of confidentiality and as full INoPSU members whilst the Greece/Cyprus Unit was secondary use of health data and the outcome of this will be accepted as an affiliate until such time as it has fulfilled the presented at a joint meeting with the Royal Society of Medicine requirements for entry. Topics discussed included funding on 28th November 2002. This will be an important occasion for problems, the difficulties with data collection and handling and the future working of the BPSU. the need for multi-national rare disease surveillance. One ongoing concern is the completeness of ascertainment and A series of lectures on the second day demonstrated the work some study investigators in this Report have voiced anxieties of the INoPSU; around 100 paediatricians attended the open about this. Contributing factors that might reduce ascertainment session. Following an introduction by Professor Elizabeth Elliott are: low returns of the orange cards; delays in following-up of the Australian Paediatric Unit on the workings of the INoPSU positive reports by the investigators; poor returns of completed the session continued with seven more specific presentations questionnaires; and situations when children with the condition 1 of interest are seen by professionals outside the orange card mailing list. At present the return rate of cards remains high at over 90%, and though it has fallen slightly in recent years, we make regular efforts to maximize returns. Delays in the follow-up of positive case reports can only be managed by the investigators, but there are several ways in which they can maximize returns: prompt contact with informants and clear and unambiguous case definitions and questionnaires are the most important.