Abstracts


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English Multi-Agency Approach to the Investigation of Sudden Unexpected Deaths in Infancy and the Care of Bereaved Families
Peter J. Fleming

Experience of the investigation of unexpected infant deaths in several countries has identified recurring instances of two types of error in such investigations - both equally destructive and undesirable - 1) inadequate investigation failing to identify child neglect or abuse, or 2) innocent parents being wrongly accused of harming their child. Studies of the use of multiagency investigation of unexpected infant deaths have shown that the needs of bereaved parents for help and support and the need for statutory agencies to investigate unexpected infant deaths need not be seen as conflicting or incompatible. The implementation in England of a co-ordinated multiagency approach to the investigation of unexpected deaths in infancy has led both to improved care and support of families and to higher standards in the investigation of such deaths, making optimal use of all available forensic, clinical and epidemiological skills and evidence. This chapter describes the rationale behind the English approach and the preliminary effects of its implementation over the past few years.


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Sudden Unexpected Death in Infancy (SUDI) - The Role of the Pathologist
Roger W. Byard

The involvement of a pathologist with forensic and pediatric training in all stages of the assessment of sudden and unexpected infant death (SUDI) is crucial as pathologists are among a limited group of medical practitioners who have been trained in evaluating the interaction of injuries, disease processes, and post-mortem changes. However problems exist, with variations in the quality of pediatric autopsy practice and in diagnostic categories that are applied. While the development of standard definitions and protocols has improved this situation, use of the term SUDI as an umbrella term has also assisted in evaluating trends and reducing the impact of diagnostic shift. The following paper reviews the contributions that may be made by pathologists in cases of SUDI, from an initial evaluation of a death scene, through the autopsy process, discussions with families, research and participation in multidisciplinary death review committees.


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Pediatric Immune Dysfunction and Health Risks Following Early-Life Immune Insult
Rodney R. Dietert and Judith T. Zelikoff

Chronic pediatric diseases arising from early-life immune insult and postnatal immune dysfunction are among the most prevalent health challenges for children. Diseases such as childhood asthma and allergies, chronic otitis media, type 1 diabetes, childhood leukemia and pediatric celiac disease all feature dysfunctional immune responses and/or inappropriately skewed immune capacities. Additionally, these disorders have been increasing in incidence in recent years with previously identified risk factors unable to fully account for the change. Still, some treatment approaches target the initial health complaint and its symptoms without fully addressing either the underlying immune dysfunction of the initial disease or the likelihood for additional associated health risks in later life. Therefore, it is useful to understand both the nature of the immune dysfunction as well as the reported associated health risks. This review characterizes those pediatric immune dysfunctions produced by well-studied immunotoxicants and provides a matrix of those health risks that appear to be linked together via the underlying pediatric immune dysfunction. This information could lead to: 1) improved identification and treatment of underlying immune dysfunction, 2) long-term approaches to health management, and 3) improved prenatal and neonatal prevention strategies to avoid environmentally-induced immune insult and developmental immunotoxicity.


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Some Controversial Theories for SIDS
Mechtild M.T. Vennemann, T. Fracasso and Edwin A. Mitchell

Almost 20 years ago prone sleeping position was established as a risk factor for sudden infant death syndrome (SIDS) and with risk reduction campaigns which largely focused on this one factor, the incidence of SIDS has declined by 50-80% in most of the countries where campaigns were conducted. However, the pathophysiological cause or causes of SIDS are not yet known, although many theories have been proposed. This paper examines several of the more controversial theories for SIDS causation.

In 1997 a link between Helicobacter pylori and SIDS was proposed. Initial positive results were not confirmed. More recently there is new evidence that H. pylori may play a role in some cases but these results need to be confirmed by others. Anaphylaxis caused by milk is an older theory, which has its merits, but needs to be verified with new methods. The toxic gas theory was interesting but had flaws. "Toxic gases" have not been produced in an environment remotely resembling that found in a cot. Proponents of the theory have recommended wrapping the cot mattress in polythene to prevent the postulated gases reaching the baby, but there is no evidence that this has had any effect. The proponents have been very vocal in the lay media despite evidence that disproves this theory. No further evidence is needed for the final rejection of this theory. The harm and benefits of immunisations are a controversial topic in the lay press, although seldom in the scientific literature. As the age of infants dying from SIDS is similar to that when immunisation is given, it has been postulated that there is a causal link. Several large case-control studies have shown that immunisations are not a risk factor for SIDS and recent a Meta analysis in fact reported that immunisation halve the risk for SIDS compared with infants who had not been immunized.

In conclusion, while the cause or causes of SIDS remains unknown new theories will be proposed and this is to be welcomed. These theories should be first discussed within the scientific community. Debating theories and preliminary findings in the lay media risks confusing parents of young infants and takes attention away from established risk factors and recommendations.


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Bed Sharing and the Risk of Sudden Infant Death: Parents Need Clear Information
Edwin A. Mitchell

Bed sharing is a major risk factor for sudden infant death syndrome. This risk is increased when the mother smokes or smoked in pregnancy, or when the parent has drunk alcohol or taken drugs. This risk is further increased in younger infants. The mechanism of sudden infant death with bed sharing is unknown, but airway obstruction, thermal stress and head covering have all been suggested. The benefit from bed sharing has only been established for breastfeeding, although other benefits are claimed. There is a small group of infants that has been shown to be at no increased risk of SIDS with bed sharing, namely infants of mothers who do not smoke, who are aged 3 months or more, and whose mothers have not taken alcohol or drugs and do not co-sleep on a sofa. Recommendations on how to bed share safely are not evidence based. Without this knowledge parents cannot make an informed choice on whether or not to bed share. Parents should be advised to place the baby to sleep in its own cot next to the parents' bed for the first six months.


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International Trends in Sudden Infant Death Syndrome and Other Sudden Unexpected Deaths in Infancy: Need for Better Diagnostic Standardization
Fern R. Hauck and Kawai O. Tanabe

Purpose: The aim of this paper is to compare international trends in sudden infant death syndrome (SIDS) and postneonatal mortality (PNM) since the introduction of SIDS risk reduction and safe sleep campaigns, offer possible explanations for differences, and to provide recommendations to improve consistency in classifying and reporting sudden unexpected deaths in infancy (SUDI) internationally. Methods: SIDS and postneonatal mortality rates were obtained for 15 countries from 1990 through the year for which most recent data were available. Results: SIDS rates have declined in all countries, with reductions well over 50% for most countries. These declines are attributed to SIDS risk reduction campaigns, which achieved success primarily in reducing rates of prone sleeping among infants. The largest declines generally occurred in the first few years after initiation of national campaigns, and there are concerning indications that these rates have reached plateaus in many countries. Conclusions and Recommendations: Diagnostic accuracy is essential to monitor and compare trends in SIDS and other sudden unexpected infant deaths. This requires establishing sudden infant death definitions and diagnostic categories that are agreed upon widely. National and local campaigns need to be reenergized to continue the early successes made in reducing SIDS incidence. Finally, data collection needs to be easy to access and this would best be accomplished by national vital statistics agencies posting data in a uniform way on their websites.


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Sudden Unexplained Death in Infancy and Long QT Syndrome
Jonathan R. Skinner

After more than 30 years of research into the hypothesis that long QT syndrome (LQTS) might be a cause of arrhythmic sudden infant death, we are now at the point where we can state with certainty that some sudden unexplained deaths in infancy, about 10%, are indeed due to long QT syndrome. The evidence for this lies in large population ECG screening programmes, post-mortem molecular genetic testing of sudden infant death victims, and some informative case reports. The cardiac sodium channel gene SCN5A (LQTS type 3) is the most common culprit, but LQTS types 1,2, 6, 9 and 12 have also been found. There is also new evidence that other arrhythmic syndromes sometimes cause SUDI, in particular short QT syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT). These conditions are also due to disordered cardiac ion channel function like LQTS, and are usually inherited in an autosomal dominant fashion. There remain, however, many unanswered questions, most particularly whether all populations are affected equally, and what should clinicians do with this knowledge? Should newborn ECG screening become mandatory? How should we best investigate SUDI at post mortem in order to diagnose LQTS? This review summarises the evidence to date and addresses these questions.


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Never Sleep with Baby? Or Keep Me Close But Keep Me Safe: Eliminating Inappropriate "Safe Infant Sleep" Rhetoric in the United States
Lee T. Gettler and James J. McKenna

Creating public health messages regarding how mothers should sleep close and safely with their babies is tricky and complex. It requires an appreciation of what exactly the term "sleeping with baby" and "co-sleeping" can mean. It also requires sensitivity to what parents will or can do if told emphatically "never sleep with your baby." In the United States, well-intentioned public health messages from prominent government agencies about safe infant sleep have increasingly used language that equates "safe infant sleep" with the absence of the mother. Many messages seemingly imply that all forms of "co-sleeping" are dangerous and that those parents that practice it are acting irresponsibly. Messages such as "babies sleep safest alone" conflict with both laboratory and epidemiological findings as well as with recommendations from most medical organizations, including the American Academy of Pediatrics, who state that mothers and babies should sleep on separate surfaces close together in the same room. Moreover, studies reveal that breastfeeding and forms of co-sleeping, including both roomsharing and bedsharing, are functionally interdependent and that many mothers worldwide find that they can manage their own and their infant's needs more easily by adopting at least intermittent bedsharing. Hence, simple, unqualified recommendations against ever bedsharing are not likely to be followed. According to recent studies the most effective public health recommendations are likely to be those that educate parents and facilitate parents in implementing bedsharing safeguards alongside their own choices. This approach does not exclude informing parents of what we know can be dangerous about some bedsharing practices, nor where and when it should be avoided altogether. Rather, it acknowledges that while separate surface co-sleeping in the form of roomsharing should always be recommended, nonetheless, many parents will appreciate and benefit from the opportunity to learn how to reduce the risks associated with bedsharing.


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Creating Change: How Knowledge Translates into Action for Protecting Babies from Sudden Infant Death?
Stephanie Cowan

We know how to protect babies from sudden infant death syndrome (SIDS) and have had considerable success in doing so. Yet babies continue to die in non-supine positions, unsafe sleeping environments and exposed to smoking. Why? Understanding what underpins the success to date is essential to the design of strategies for the final stage of prevention. This paper reviews influences on changing SIDS mortality, describes the practice of creating change as it relates to protecting babies from sudden infant death, and presents three principles that emerge from the success to date to focus the design of research and intervention programmes for ending the SIDS story.


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Sudden Infant Death Syndrome: Implications of Altered Physiological Control During Sleep
Rosemary S.C. Horne, Nicole B. Witcombe, Stephanie R. Yiallourou and Heidi L. Richardson

A failure of cardiorespiratory control mechanisms, together with an impaired arousal from sleep response, is believed to play an important role in the final event of the Sudden Infant Death Syndrome (SIDS). The 'Triple Risk Model' describes SIDS as an event that results from the intersection of three overlapping factors: [1] a vulnerable infant, [2] a critical developmental period in homeostatic control, and [3] an exogenous stressor. In an attempt to understand how the Triple Risk Hypothesis is related to infant cardiorespiratory physiology many researchers have examined how the known risk factors for SIDS alter infant physiology and arousal particularly during sleep. This review discusses the association between the three components of the Triple Risk Hypothesis, the major risk factors for SIDS (prone sleeping and maternal smoking), together with three "protective" factors (breastfeeding, pacifiers and swaddling), and cardiovascular control and arousability from sleep in infants, and discusses their potential involvement in SIDS.


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Role of Voluntary Organisations
Joyce Epstein

Voluntary organisations, working closely with scientists and doctors in some 15 countries, play a key role in promoting research into Sudden Infant Death, providing support for bereaved families, disseminating infant care messages to help prevent deaths and improving investigations when a baby dies. This paper provides an overview of the role of voluntary organisations including giving examples of innovative projects and programmes to tackle the problems of sudden infant death.


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Sudden Unexpected Death in Infancy and the Dilemma of Defining the Sudden Infant Death Syndrome
Henry F. Krous

Sudden unexpected death in infancy (SUDI) is an umbrella label that some employ to encompass all sudden unexpected infant deaths, whether or not explained, while others restrict its use to cases in which the cause of death is uncertain, but possibly due to asphyxia as may occur, for example, with sleeping prone, face down on a soft sleep surface, and/or being found with the head covered. Since sudden infant death syndrome (SIDS) is a diagnosis of exclusion, there is an inevitable interface between it and those cases whose deaths are potentially caused by unsafe sleep environments. This interface is especially blurred given the lack of definitive, easily identifiable postmortem marker(s) for SIDS. Therefore, present SIDS definitions are imprecise and its diagnosis remains one of exclusion. Improved death scene investigation has resulted in a diagnostic shift away from SIDS towards other causes of death such as positional asphyxia or undetermined. Unfortunately incomplete death scene investigation has hampered evaluation of the real circumstances of death in too many of the cases further contributing to confusion. In this report, the purposes for and primary definitions of SIDS are delineated. Subsequent discussion focuses on the increasing challenge to incorporate risk factors and the underlying pathology germane to the pathophysiology of SIDS into future definitions. This challenge is matched by the need to develop affordable and widely available testing that will identify pathology relevant to medical examiners and others charged with certifying the cause and manner of death.


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Commentary on the Multi-Agency Approach to the Investigation of SUDI
Torleiv O. Rognum

To use the diagnosis SIDS, investigation of the death scene is a prerequisite. In some countries death scene investigation is performed by ordinary police, in some states in the US it is performed by the medical examiner, but in many countries no death scene investigation is done at all. There is general agreement in the SIDS community that the death scene should be investigated by a specialist team including medical and forensic experts. This requirement has been a challenge for the legislators and legal experts who claim that entering homes without consent is violation of human rights. This challenge has been overcome in the UK where after a new law was passed on April 1, 2008, professor PJ Fleming and his co-workers successfully have developed a multi-agency approach which may be a model for other countries.


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Perspectives on Bed-Sharing
Peter S. Blair

The successful and dramatic reduction in cot deaths has come about not so much because of a better understanding of causal mechanisms of sudden death but rather from identifying risk factors in the infant sleeping environment; in particular placing young infants on their front to sleep. More recently bed-sharing has been identified as a potential risk factor and similar efforts are being made in some countries to advise against this care practice. However prone sleeping is not a culturally widespread behaviour; introduced in the 20th century partly based on observations showing how premature infants thrived (and still do) when placed in this position in incubators. If we are going to advise against a behaviour such as bed-sharing that is common to many cultures over many thousands of years we need to address some basic questions. This review asks i) Is there any benefit to bedsharing? ii) Is bed-sharing in itself a 'risk-factor' for SIDS? and iii) By advising against bed-sharing will we do any harm?


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The Death-Scene Investigation
Martin Schlaud

The death-scene investigation is an important and increasingly recognized step in the process of diagnosing Sudden Infant Death Syndrome (SIDS). According to current definitions, information from death-scene investigations is required when SIDS diagnoses are made. Due to differences in national jurisdictions, however, there are differences in the methods used and the professions involved in routinely performed death-scene investigations. Therefore any international comparability of death-scene data is limited.

Only a few epidemiological studies have used thorough death-scene investigations in a systematic way, including a standardized, objective observation of the scene in cases and reference data from the general population. These studies gave close insights into the circumstances of infant death, but their complex protocols are mostly not feasible for routine use. For that purpose, manageable death-scene investigation protocols need to be distilled from the ones used in complex studies, taking into account their results.

Whilst protocols for post-mortem examinations and definitions for SIDS have been largely standardized and agreed internationally, this step is still missing for death-scene investigations. If routinely obtained death-scene data were standardized and, thus, comparable, this would have a potential of generating new hypotheses that eventually lead to a better understanding of the underlying mechanisms and to more effective measures of prevention.


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Neurological Assessment of Early Infants
Yasuyuki Futagi, Yasuhisa Toribe and Yasuhiro Suzuki

For early diagnosis of children with neurodevelopmental disorders, neurological assessment during early infancy and follow-up examinations are essential. However, to make the neurological assessment effective, some knowledge regarding the examination procedures and interpretation of the results is required. There have been many articles on the neurological assessment of early infants, but ones that clearly delineated its diagnostic significance have been surprisingly few. Based on our own experience and the literature, in this review we will discuss the diagnostic significance of neurological assessment of early infants, including of primitive reflexes (crossed extensor reflex, suprapubic extensor reflex, heel reflex, Galant response, asymmetric tonic neck reflex, plantar grasp response, and Babkin reflex), involuntary movements (ankle clonus and tremor), and Vojta's postural reactions. Neurological assessment of these reflexes is simple and useful for early detection of infants at high risk for a neurodevelopmental disorder that might later develop.