Rapid Responses to:
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Rapid Responses: Rapid Responses published:
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The future human cost of the UK's Muddle, Mistrust and Recklessness.
- Woody Caan (23 November 2004)
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National surveillance for congenital rubella
- Pat A Tookey (6 January 2005)
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Reduced dataset needed for NCAS
- Sally M Bradley, Dr Michael Robinson, Consultant Paediatrician, Salford Royal Hospitals NHS Trust, Stott Lane, Salford M6 8HD. (8 February 2005)
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Underreporting of congenital anomalies may have been understated
- Mike Joffe (22 February 2005)
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Re: Underreporting of congenital anomalies may have been understated
- Patricia A Boyd, Helen Dolk, Ben Armstrong, Judith Rankin, Sam Pattenden (14 March 2005)
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Underreporting of congenital anomalies caused by hormonal contraceptives and nutritional deficiencies
- Ellen C G Grant (15 March 2005)
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Update
- Woody Caan (3 May 2005)
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Woody Caan, Professor of public health APU, Chelmsford, Essex CM1 1SQ.
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Boyd's paper on serious and patchy deficiencies in the UK system for 'surveillance' of congenital anomalies merits an urgent national response [1]. Over several years the Government has lost the trust of many parents over the use of standardised Measles Mumps and Rubella vaccine [2]. Maternal rubella infection in pregnancy is associated with a constellation of congenital problems, some obvious and some subtler in presentation [3]. Unless we improve our system to achieve accurate and consistent monitoring of patterns of anomalies, we simply won't know if a spread of rubella is leading to increased disability.... until it becomes so obvious that we have missed our chance to contain the hazard. 1 Boyd PA, Armstrong B, Dolk H, Botting B, Pattenden S, Abramsky L, Rankin J, Vrijheid M, Wellesley D. Congenital anomaly surveillance in England--ascertainment deficiencies in the national system. BMJ 2004; 0: bmj.38300.665301.3Av1 2 Caan W. Daunting challenges which face us call for solidarity and widespread support. Health Service Journal 2002; 17 January: 20-21. 3 Cutts FT, Best J, Siqueira MM, Engstrom K, Robertson SE. Guidelines for surveillance of congenital rubella syndrome and rubella. Geneva: World Health Organization, 1999. Competing interests: Used to work in the area of sensory disabilities; current RNID member. |
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Pat A Tookey, Senior Lecturer Institute of Child Health, 30 Guilford St, London WC1N 1EH
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Woody Caan raises specific concerns about the surveillance of congenital rubella, in the light of Boyd and colleagues' paper on congenital anomaly surveillance (1). The National Congenital Rubella Surveillance Programme (NCRSP) was established in 1971 to monitor the effect of the newly introduced rubella vaccination policy on congenital rubella incidence in England, Wales and Scotland. Other sources of data on rubella infection in pregnancy include laboratory reports to the Health Protection Agency, and rubella associated terminations reported to the Office for National Statistics. The number of infants born with congenital rubella and reported to the NCRSP declined from about 50 a year 1971-75 to just over 20 a year 1986-90, and rubella associated terminations from an average of 750 to 50 a year. Since 1990 congenital rubella has been one of the rare conditions of childhood monitored through the British Paediatric Surveillance Unit's active monthly surveillance scheme (2). Substantial outbreaks of rubella last occurred in the British Isles in the spring of 1996, mainly affecting young men; 12 infants with congenital rubella were reported to the NCRSP later in the year, almost all of them within two months of birth (3). Between 1997 and 2004 10 infants with confirmed congenital rubella were notified, and the majority of these were born to women who acquired their infection abroad (4). As Woody Caan points out, there are a variety of problems associated with congenital rubella, and it is possible that infants without obvious manifestations at birth (for example those with sensorineural hearing loss alone) may not be diagnosed and reported; however, infants with symptoms at birth are likely to be reported in a consistent manner and any rise in birth prevalence of congenital rubella should be evident. We should certainly be concerned about the possibility of a resurgence of rubella and congenital rubella if MMR uptake continues at the present level, and it is vital that the established surveillance systems are maintained. Pregnant women and health professionals must continue to be aware of the risks of rubella infection in pregnancy. Guidelines on managing suspected infection in pregnancy are available (5), and any suspected cases of congenital rubella should be reported through the BPSU, or direct to the NCRSP. References 1. Boyd PA, Armstrong B, Dolk H, Botting B, Pattenden S et al. Congenital anomaly surveillance in England - ascertainment deficiencies in the national system. BMJ 2005; 330: 27 2. Verity C and Preece M. Surveillance for rare diseases by the BPSU. Arch Dis Child 2002; 87: 269-71 3. Tookey PA and Peckham CS. Surveillance of congenital rubella in Great Britain, 1971-96. BMJ 1999; 318: 769-70 4. Tookey P. Surveillance Report: Rubella in England, Scotland and Wales. Eurosurveillance Monthly 9 (4) April 2004; 21-22 5. Morgan-Capner P. and Crowcroft NS. Guidelines on the management of, and exposure to, rash illness in pregnancy. Commun Dis Public Health 2002; 5(1): 59-71 Pat Tookey, email: p.tookey@ich.ucl.ac.uk Competing interests: Co-ordinator of the National Congenital Rubella Surveillance Programme |
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Sally M Bradley, GPwSI public health Salford Primary Care Trust, St James's House, Pendleton Way, Salford M6 5FW, Dr Michael Robinson, Consultant Paediatrician, Salford Royal Hospitals NHS Trust, Stott Lane, Salford M6 8HD.
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Editor- Population surveillance can be judged using a series of attributes including sensitivity, timeliness, representativeness, positive predictive value, acceptability, flexibility and simplicity [1]. The evaluation of surveillance systems needs to focus on the appropriate balance of these attributes. Boyd et al acknowledge that case ascertainment of the National Congenital Anomaly System (NCAS) is “very low (40%)” even though the researchers only included “major defects for which the ascertainment is high, agreement on case definition by all registries is good, and ICD10 lists specific codes”[2]. It is disconcerting that only half the cases of Down’s Syndrome and one-third of all chromosomal syndromes are picked up. Experience locally demonstrates problems with the simplicity and acceptability of the system; clinicians find it difficult to know what to notify. NCAS guidance contains over 37 conditions and in excess of 200 ICD codes for the data collected; it also contains a list of over 18 excluded conditions and 31 conditions not to be considered as congenital anomalies [3]. ONS advise to err on caution and report if in doubt whether an anomaly should be reported In Salford alert warnings have been issued as higher than expected number of certain conditions have been reported. Investigation has found inaccurate reporting particularly of conditions which do not need to be reported (there may also be underreporting) and therefore the need for greater clinical knowledge of NCAS requirements. Boyd et al recommend a basic surveillance dataset for local registers and the NCAS, and the extension of local registers to the whole country. We suggest that whilst this is considered a reduced dataset be introduced for the NCAS to improve ascertainment for major conditions. At present it appears that NCAS might be unable to pick up an increase in minor defects whose inclusion only increases the complexity of the system. [1] Detels R, Mc Ewen J, Beaglehole R, Tanaka. Oxford Textbook of Public Health. Oxford: Oxford University Press, 2002. [2] Boyd PA, Armstrong B, Dolk H, Botting B, Pattenden S, Abramsky L et al. Congenital anomaly surveillance in England-ascertainment deficiencies in the national system. BMJ 2005;330:27-9. [3] Office for National Statistics. The National Congenital Anomaly System: A guide for users and suppliers. London: ONS, 2001 Competing interests: None declared |
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Mike Joffe, Acting Head of Epidemiology Chemical Hazards and Poisons Division, Health Protection Agency, Chilton, Didcot, Oxon OX11 0RQ
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Dear Sir Boyd et al [1] have performed a valuable function in documenting the extent of underreporting in the National Congenital Anomaly System (NCAS). The high degree of variation by type of defect and by geographical area is important information that has not previously been readily available, and the lack of variation with degree of socioeconomic deprivation is reassuring. On the positive side, for cleft lip and palate and for limb reductions at least two-thirds of anomalies are reported as being covered by the NCAS system, even when terminations of pregnancy are included in the denominator. However, Boyd et al understate the extent of underreporting. Because three of the four local registers that they used are not entirely population based, some of the pregnancies/births in the wards that they studied would have occurred outside the hospitals covered by those registers. The authors have tried to minimise this problem by excluding wards where fewer than 80% of mothers delivered in hospitals reporting to the register, which was a sensible course of action. The implication is that up to 20% of the pregnancies may have fallen outside the local system in some wards, leading to underreporting by the “gold standard”. This could be partially checked by ascertaining those births that are within the NCAS but not the local register. In addition, the four registers used in the study cover only about 109,000 births annually. There are well over half a million births in England each year, for example 589,851 in 2003 [2], so that the four registers used by Boyd et al cover well under half of the approximately 50% of births in England that are covered by local registers. It is not clear why the other local registers were not used in the study, and in particular whether it was considered that they are not of the same standard as the four that were chosen, or whether their exclusion was merely for practical reasons. If health protection is to be taken seriously for reproduction and development, it is essential to have adequate systems for surveillance of outcomes, including congenital anomalies. Boyd et al’s conclusions therefore deserve support, that high-quality local registers should be extended to cover the whole country, and that the data collection system should include terminations of pregnancy. References 1. Boyd PA, Armstrong B, Dolk H, Botting B, Pattenden S, Abramsky L, et al. Congenital anomaly surveillance in England – ascertainment deficiencies in the national system. BMJ 2005;330:27-29. 2. Office for National Statistics. Birth statistics: Review of the Registrar General on births and patterns of family building in England and Wales, 2003. London: ONS. http://www.statistics.gov.uk/downloads/theme_population/FM1_32/FM1no32.pdf [accessed 22 Feb 2005] Competing interests: None declared |
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Patricia A Boyd, Clinical Geneticist, Senior Research Fellow National Perinatal Epidemiology Unit OX3 7LF, Helen Dolk, Ben Armstrong, Judith Rankin, Sam Pattenden
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Dear Sir, Joffe highlights the concern our paper(1) details of patchy and low ascertainment to the National Congenital Anomaly System (NCAS). He raises two methodological questions which we would like to clarify. He asks whether we understated the degree of underascertainment of NCAS since some of the regional registers were not entirely population based, even though we reduced the study area to wards where at least 80% of births to resident mothers took place in hospitals reporting to the register. This resulted in a total coverage of between 94% and 100% of all births in each of the four registry study areas, and 96.9% overall. Joffe is therefore right that we underestimated underascertainment of NCAS, but only by 3% overall. We adjusted for this underascertainment, as explained in other publications from this study (2,3) ,when calculating prevalence rates and investigating their variation, but not in this comparison of numbers between the registers and NCAS. Joffe also asks how the four congenital anomaly registers were chosen, since they cover one fifth of England and Wales, whereas registers now cover about half of England and Wales. They were selected as the registers which were long enough established to provide sufficient years of analysis for an investigation of the degree of geographical variation and clustering in congenital anomaly rates. Other registers have been established more recently but still fall far short of covering the entire country. PA Boyd
1. Boyd PA; Armstrong B; Dolk H; Botting B; Pattenden S; Abramsky L; Rankin J; Vrijheid M, and Wellesley. Congenital anomaly surveillance in England - ascertainment deficiencies in the national system. BMJ. 2005; 330:27-29. 2. Dolk H, Armstrong B, Vrijheid M, Stone D, Rankin J, Abramsky L, et al. A study of the geographical variation in overall rates of congenital abnormalities and the rates of specific abnormalities. Report to Department of Health, 2003. www.eurocat.ulster.ac.uk/pubdata 3. Rankin J; Pattenden S; Abramsky L; Boyd PA; Jordan H; Stone D; Vrijehid M; Wellesley D, and Dolk H. Prevalence of congenital anomalies in five British regions 1991-99. Archives of Disease in Childhood. Fetal and Neonatal Ed 2005. Accepted for Publication. Competing interests: None declared |
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Ellen C G Grant, physician and medical gynaecologist Kingston-upon-Thames, KT2 7JU,UK
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The failure to record the numbers of foetal abnormalities in early terminated pregnancies is regrettable.1 This must have helped encourage the increased use of contraceptive hormones, progesterones and oestrogens, which are known to be teratogenic.2-4 In 1976 the Oxford/FPA contraception study reported “a surprisingly low incidence” of congenital abnormalities among live infants born to nulliparous women who had never taken hormonal contraceptives compared with nulliparous ever users (0.4% compared with 3.8%).5 The particularly high incidence of spina bifida between 1964 and 1972 was attributed to the use of hormonal pregnancy tests.6 These tests involved taking as much as a nine months’ dose of oral contraceptive progesterones and oestrogens. Since then the introduction of scans in early pregnancy have increased early terminations of apparently unrecorded numbers of abnormal foetuses. The “morning after” emergency contraceptive pill also contains a large dose of a synthetic progesterone, levonorgestrel, and is therefore potentially teratogenic.7,8 Congenital anomalies developing in any surviving foetuses, who are later aborted, should be recorded but prescription records are not being kept. Use of hormones before or during pregnancy increases the likelihood of essential nutrient deficiencies or imbalances which are also major cause of teratogenesis and poor health in children.9 1. Boyd PA, Armstrong B, Dolk H, et al. Congenital anomaly surveillance in England - ascertainment deficiencies in the national system. BMJ 2005; 330:27-29. 2 Murthy BKP, Prema K. Sister-cromatid exchanges in oral contraceptive users. Mutation Research 1979: 68:149- 52. 3 Biri A, Civelek E, Karahalil B, Sardas S. Assessment of DNA damage in women using oral contraceptives. Mutat Res. 2002; 521: 113-9. 4 Lejeune J, Prieur M. Oral contraceptives and trisomy 21. A retrospective study of 730 cases, Annals of Genetics 1979: 22:61-66. 5 Vessey MP, Doll R, Peto R, et al. A long-term follow-up study of women using different methods of contraception. Br J Obstet Gynae 1976; 8: 373-424. 6 Gal I, et al. Hormonal pregnancy tests and congenital abnormalities. Nature 1967; 216: 83. 7 Grant ECG. Levonorgestrel is hazardous http://bmj.com/cgi/eletters/329/7459/182#70964, 14 Aug 2004 8 Grant ECG. Hormonal contraceptives should not be OTC http://bmj.com/cgi/eletters/329/7459/182#71252, 17 Aug 2004 9 Grant ECG. Nutritional supplements to prevent pregnancy complications. http://bmj.com/cgi/eletters/329/7458/152#67502, 16 Jul 2004 Competing interests: None declared |
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Woody Caan, Professor of public health APU, Chelmsford, Essex CM1 1SQ.
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May I draw readers' attention to the recent report by Amy Cawthorne and colleagues in the Eastern Region Public Health Observatory [1]? This has the widest and most recent coverage of surveillance available. Inconsistent and under reporting of congenital anomalies make detection and quantification of time trends and geographical foci: unreliable. This report concludes the patchy, wobbly systems are "currently inadequate" [1]. Correspondence to the eBMJ suggests there are many stakeholders in various places wanting to get this surveillance right: perhaps a good time to all link up? 1 Cawthorne A, Evans S, Halliday S. Congenital anomaly screening surveillance in the East of England. INpho 12. Cambridge: ERPHO, 2005. Competing interests: Used to work in the area of sensory disabilities; current RNID member. |
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