CHILDREN TALK ABOUT DEATH

ABSTRACT. Raimbault. G. (Inserm U.158, Hôpital des Enfants Malades, Paris, France). Children talk about death. Acta Paediatr Scand, 70:179, 1981–As a psychoanalyst in a pediatric nephrology ward, I have listened to children talking about dying and death. As soon as a child can express himself freely on this subject, no further age-dependent progress concerning the concept of death can be observed. Faced with disease and death, the child is led to the same images and conclusions, to the same order of ideas as the adult. This clearsightedness does not prevent the appearance of mechanisms of defence against the idea of death. Faced with the silence of the adult, the dying child shuts himself up in loneliness. However, the child does not want to be alone: he craves to be recognized. During the time left before his death, to be recognized is for him synonymous with: "to be recognized near death". To let a child talk about his death is to let him talk about his life, to bring him the only possible help, that is, to be with him till the end.     

Distinguishing sudden infant death syndrome from child abuse fatalities

Fatal child abuse has been mistaken for sudden infant death syndrome. When a healthy infant younger than 1 year dies suddenly and unexpectedly, the cause of death may be certified as sudden infant death syndrome. Sudden infant death syndrome is more common than infanticide. Parents of sudden infant death syndrome victims typically are anxious to provide unlimited information to professionals involved in death investigation or research. They also want and deserve to be approached in a nonaccusatory manner. This clinical report provides professionals with information and suggestions for procedures to help avoid stigmatizing families of sudden infant death syndrome victims while allowing accumulation of appropriate evidence in potential cases of infanticide. This clinical report addresses deficiencies and updates recommendations in the 2001 American Academy of Pediatrics policy statement of the same name.     

Working with families after a sudden child death

Every child's death is a tragedy. Many paediatricians feel unprepared to support bereaved families after a sudden child death, and are concerned about what may or may not be permitted with concurrent Sudden Unexpected Death in Childhood (SUDIC) and coronial investigations. After a child death, parents need to be able to say goodbye to their child, they require information both about the cause of death and the process of investigations, parents may also require emotional support. This review describes how to support bereaved families from the time of death and in the weeks and months that follow.     

Problems on the diagnosis of sudden infant death syndrome

Various autopsy cases of sudden unexpected death (SUD) in infancy were examined at the Tokyo Medical Examiner's Office between 1985 and 1994. More than half of the SUD were diagnosed as sudden infant death syndrome (SIDS), but a number of other causes, such as mechanical asphyxia, were also diagnosed. SIDS is diagnosed by autopsy, but there are no clear diagnostic criteria differentiating SIDS from other causes of SUD. SUD is diagnosed as SIDS when other causes are excluded, but it is difficult to distinguish between SIDS and mechanical asphyxia. There was not a large difference in autopsy findings, or in death scene or statistical data, between SIDS and non-SIDS cases. In their estimation of the diagnostic ratio of SIDS to other causes of death, medical examiners might be divided into three groups: ‘SIDS tolerationist’ examiners think that SUD should be positively diagnosed as SIDS, insofar as another cause of death is not proved clearly. A second group of examiners might be regarded as ‘SIDS exclusionist’; these consider microscopic findings or peculiar death scenes as important contributing factors leading to death. The third group represents a middle stance somewhere between these two. We thought that (forensic) pathologists as well as medical examiners in Japan might have differing stances on SIDS diagnosis. The statistical analysis of SIDS in certain research areas may be affected by the diagnostic ‘preference’ of pathologists belonging to a certain institute.     

Role of infection in the death of children with acute lymphoblastic leukaemia.

Twenty-four consecutive deaths from a total of 70 children receiving treatment for acute lymphoblastic leukaemia (ALL) have been reviewed. An attempt has been made to ascribe the cause of death to either infection, haemorrhage, the leukaemia itself, or a combination of these factors. No child was free of infection at death. Infection, with or without haemorrhage, was responsible for the deaths of all 15 children whose leukaemia had not relapsed. Although infection was present at death in all 9 children whose leukaemia had relapsed, the leukaemia process itself was also a major contributing factor. Viruses were associated with death in many of the children and may be emerging as important pathogens in children with ALL. Familiarity with a protocol may be an important factor in the prevention of fatal infections in such children. Centralization of treatment is necessary if this expertise is to be acquired.     

Suffocation,shaking or sudden infant death syndrome: Can we tell the difference?

Given that the diagnosis of sudden infant death syndrome (SIDS) remains one of exclusion, problems still exist in distinguishing possible cases from those where the deaths were due to accidental or inflicted suffocation. The term 'SIDS' cannot be used unless a complete autopsy examination has been performed, augmented by review of the clinical history and examination of the death scene. In the absence of a cause of death, a more suitable designation in the presence of inflicted injury is 'undetermined' rather than 'SIDS'. Use of standard autopsy and death scene protocols that have been endorsed by professional bodies will improve the investigation of unexpected infant death and will increase the likelihood of diagnosing subtle disorders that may be confused with SIDS.     

Synergistic effect of influenza a virus on endotoxin-induced mortality in rat pups: a potential model for sudden infant death syndrome

Sudden infant death syndrome is the most common cause of postneonatal infant mortality in the developed world. It is a diagnosis of exclusion with peak age of incidence between 2 and 6 mo. Fifty to 63% of these infants have a preexisting upper respiratory tract infection before death. We hypothesized that the immature immune system may be altered by a primary infection, preventing a protective response after secondary challenge. To mimic dual infection, we used a nonlethal strain of a rat-adapted influenza A virus and a sublethal dose of endotoxin to establish a model that results in pathology and death in 12-d-old rat pups similar to that seen in infants dying of sudden infant death syndrome. Mortality only occurred when specific criteria such as timing between infectious insults and developmental age of the pup were met. Results suggest that mortality is caused by a rapid systemic shock event rather than lung-specific damage. Gross pathologic findings such as lung petechiae and liquid blood around the heart on necropsy were consistent with those seen in infants dying of sudden infant death syndrome. Histopathologic lesions including subendocardial hemorrhage and mild cortical thymocyte necrosis were found with greater severity and frequency in dually challenged animals. Macrophage subpopulation in rat-adapted influenza A virus-inoculated animals was significantly elevated in the spleen at the time of death. Our model suggests that the developing immune system can be primed to respond in an exaggerated way to a second immune challenge resulting in unexpected death.     

Do the right children have necropsies?. Analysis of selected determinants.

An analysis of the frequency of post-mortem examinations was conducted in the paediatric age group. Among the factors found to influence necropsy rates were (i) age at death, the frequency of necropsy decreasing with age; (ii) cause of death, necropsy frequency varying with major diagnostic groups; (iii) investigation conducted before death, necropsy frequency decreasing when the clinical picture was ill-defined; and (iv) place of death, necropsy being less frequent when death occurred outside hospital. These findings imply (a) that necropsy practices may be governed by habit and convenience as well as by desire to complete the clinical picture, and (b) that more equitable balances in such practices should be sought if there is to be improvement in the quality of mortality data.     

The sudden infant death syndrome gene: does it exist?

BACKGROUND: Sudden infant death syndrome (SIDS) is in a difficult position between the legal and medical systems. In the United Kingdom, prosecutors have for years applied the simple rule that 1 unexpected death in a family is a tragedy, 2 are suspicious, and 3 are murder. However, it seems that the pendulum has now swung to the opposite extreme; mutations or polymorphisms with unclear biological significance are accepted in court as possible causes of death. This development makes research on genetic predisposing factors for SIDS increasingly important, from the standpoint of the legal protection of infants. The genetic component of sudden infant death can be divided into 2 categories, ie (1) mutations that give rise to genetic disorders that constitute the cause of death by themselves and (2) polymorphisms that might predispose infants to death in critical situations. Distinguishing between these 2 categories is essential, and cases in which a mutation causing a lethal genetic disorder is identified should be diagnosed not as SIDS but as explained death. GENETIC ALTERATIONS THAT MAY CAUSE SUDDEN INFANT DEATH: Deficiencies in fatty acid metabolism have been extensively studied in cases of SIDS, and by far the most well-investigated mutation is the A985G mutation in the medium-chain acyl-CoA dehydrogenase (MCAD) gene, which is the most prevalent mutation causing MCAD deficiency. However, <1% of sudden infant death cases investigated have this mutation, and findings of biochemical profiles seen in specific fatty acid oxidation disorders in a number of such cases emphasize the importance of investigating fatty acid oxidation disorders other than MCAD deficiency. Severe acute hypoglycemia may cause sudden death among infants, but only rare novel polymorphisms have been found when key proteins involved in the regulation of blood glucose levels are investigated in cases of SIDS. The long QT syndrome (LQTS) is another inherited condition proposed as the cause of death in some cases of sudden infant death. The LQTS is caused by mutations in genes encoding cardiac ion channels, and mutations in the genes KVLQT1 and SCNA5 have been identified in cases initially diagnosed as SIDS, in addition to several polymorphisms in these 2 genes and in the HERG gene. In addition, genetic risk factors for thrombosis were investigated in a small number of SIDS cases; the study concluded that venous thrombosis is not a major cause of sudden infant death. GENE POLYMORPHISMS THAT MAY PREDISPOSE INFANTS TO SUDDEN INFANT DEATH UNDER CERTAIN CIRCUMSTANCES: Many SIDS victims have an activated immune system, which may indicate that they are vulnerable to simple infections. One reason for such vulnerability may be partial deletions of the complement component 4 gene. In cases of SIDS, an association between slight infections before death and partial deletions of the complement component 4 gene has been identified, which may indicate that this combination represents increased risk of sudden infant death. There have been a few studies investigating HLA-DR genotypes and SIDS, but no association has been demonstrated. The most common polymorphisms in the interleukin-10 (IL-10) gene promoter have been investigated in SIDS cases, and the ATA/ATA genotype has been reported to be associated with both SIDS and infectious death. The findings may indicate that, in a given situation, an infant with an unfavorable IL-10 genotype may exhibit aberrant IL-10 production, and they confirm the assumption that genes involved in the immune system are of importance with respect to sudden unexpected infant death. Another gene that has been investigated is the serotonin transporter gene, and an association between the long alleles of this gene and SIDS has been demonstrated. Serotonin influences a broad range of physiologic systems, as well as the interactions between the immune and nervous systems, and findings of decreased serotonergic binding in parts of the brainstem, together with the findings in the serotonin transporter gene, may indicate that serotonin plays a regulatory role in SIDS. It has also been speculated that inadequate thermal regulation is involved in SIDS, but investigations of genes encoding heat-shock proteins and genes encoding proteins involved in lipolysis from brown adipose tissue have not found evidence of linkages between common polymorphisms in these genes and SIDS. A number of human diseases are attributable to mutations in mitochondrial DNA (mtDNA), and there are several reasons to think that mtDNA mutations also are involved in SIDS. Both a higher substitution frequency and a different substitution pattern in the HVR-I region of mtDNA have been reported in SIDS cases, compared with control cases. A number of coding region mtDNA mutations have also been reported, but many are found only in 1 or a few SIDS cases, and, to date, no predominant mtDNA mutation has been found to be associated with SIDS. CONCLUSIONS: All mutations giving rise to metabolic disorders known to be associated with life-threatening events are possible candidates for genes involved in cases of sudden infant death, either as a cause of death or as a predisposing factor. It is necessary to distinguish between lethal mutations leading to diseases such as MCAD and LQTS, and polymorphisms (for instance, in the IL-10 gene and mtDNA) that are normal gene variants but might be suboptimal in critical situations and thus predispose infants to sudden infant death. It is unlikely that one mutation or polymorphism is the predisposing factor in all SIDS cases. However, it is likely that there are "SIDS genes" operating as a polygenic inheritance predisposing infants to sudden infant death, in combination with environmental risk factors. For genetically predisposed infants, a combination of, for instance, a slight infection, a prone sleeping position, and a warm environment may trigger a vicious circle with a death mechanism, including hyperthermia, irregular breathing, hypoxemia, and defective autoresuscitation, eventually leading to severe hypoxia, coma, and death.     

Cardiac pathology in sudden unexpected infant death

OBJECTIVES: To determine the spectrum of cardiac pathology and circumstances of death in infants with sudden unexpected death and to define the impact of sudden cardiac deaths to overall sudden infant death.Study design: Retrospective analysis of all autopsies of infants with sudden death 7 days to 2 years of age between January 1987 and December 1999 in the province of Québec (Canada). RESULTS: Eighty-two cases of sudden death with cardiac pathology were found, representing 10% of the total number of sudden infant deaths. A structural malformation was present in the majority of cases (54%); however, cardiac pathology in anatomically normal hearts was also common (46%). Most (64%) anatomic malformations were detected before death compared with 13% of nonstructural heart disease. Although a major proportion of children were found dead during sleep, a significant number were described as being awake at time of death (32%). CONCLUSIONS: Heart disease is present in a significant percentage of autopsies of infants with sudden death. Structural heart malformations predominate, although nonstructural pathologic features of the heart are common and usually unrecognized before an autopsy is performed. Cardiac pathologic features are frequent when the child is witnessed to be awake at the time of sudden death.     

Medico-legal child deaths in Istanbul: data from the Morgue Department

BACKGROUND: Before determining health policies, finding solutions to problems and taking precautions, one should define the problems and their regional and national dimensions. Data about causes of death vary from country to country and, therefore, it is clear that precautions should be based on regional data and needs, which will be more effective. METHOD: The authors reviewed deaths among children aged 1 month to 18 years, whose autopsies were performed in the Morgue Department, State Institute of Forensic Medicine, Istanbul, Turkey, between 2000 and 2002. RESULTS: The authors evaluated autopsy reports about 736 child deaths. The mean age of the children was 8.77 +/- 6.25 years and 62.8% of the children were male. As to causes of death, the most frequent was asphyxia. Almost half of them were caused by mechanical asphyxia due to drowning. A total of 48.5% of the deaths were accidental. There was a significant difference in causes of death between children aged 11 years or younger and those aged over 11 years. Death due to nontraumatic conditions and poisoning was predominant among children aged 11 years and younger, while death due to gunshot wounds and stab wounds was predominant among children aged more than 11 years. There was a significant difference in causes of death between females and males. CONCLUSION: Asphyxia, poisoning and blunt traumatic injuries were found to be the leading causes of death, which can be prevented or decreased by certain precautions. As a result, new regulations should be enacted to protect children against accidents, injuries and hazards and a child protection program is needed in Turkey.     

Death, nasomaxillary complex, and sleep in young children

This is an investigation of anatomical and sleep history risk factors that were associated with abrupt sleep-associated death in seven children with good pre-mortem history. Seven young children with abrupt deaths and information on health status, sleep history, death scene report, and autopsy performed in a specialized unit dedicated to investigation of abrupt death in young children were investigated Seven age and gender matched living children with obstructive-sleep-apnea (OSA) were compared to the findings obtained from the dead children. Two deaths results from accidents determined by the death scene and five were unexplained at the death scene. History revealed presence of chronic indicators of abnormal sleep in all cases prior death and history of an acute, often mild, rhinitis just preceding death in several. Four children, including three infants, were usually sleeping in a prone position. Autopsy demonstrated variable enlargement of upper airway soft tissues in all cases, and in all cases, there were features consistent with a narrow, small nasomaxillary complex, with or without mandibular retroposition. All children were concluded to have died of hypoxia during sleep. Our OSA children presented similar complaints and similar facial features. Anatomic risk factors for a narrow upper airway can be determined early in life, and these traits are often familial. Their presence should lead to greater attention to sleep-related complaints that may be present very early in life and indicate impairment of well been and presence of sleep disruption. Further investigation should be performed to understand the role of upper airway infection in the setting of anatomically small airway in apparently abrupt death of infants and toddlers.     

Investigating sudden unexpected death in infancy and early childhood

Sudden unexpected death is one of the most frequent ways of dying in the first year of life after the neonatal period. It is however, much less frequent after the first birthday. Investigations into the cause of death are very important, for a significant proportion of these sudden deaths can be explained only after a thorough investigation. Of the causes identified, infection is the most frequent cause; metabolic disorders and cardiovascular diseases play a role as well, although the proportion of cases is much smaller. There is now evidence that cardiac channel gene mutations also play an important role; however, identification of these conditions relies on costly testing that is not readily or widely available. The physician's role as primary care provider is critical in ensuring that families understand the results of the investigation into their child's death. It is important that everything be done to identify the cause of death so that no such tragedy recurs in the same family.     

Sports preparticipation cardiac screening: what about children?

Sudden cardiac death in young athletes is a devastating event. Screening programs have been proposed to prevent sudden cardiac death in young athletes. Mortality rates and causes of death differ among young adults and children. Children have a considerably lower incidence of sudden cardiac death. Data lack to compare athletes and non-athletes in childhood, but 40–50 % of sudden cardiac death in this age group seems to be related to exercise. Screening programs including history and physical exam are not very sensitive or specific and will result in important numbers of false-positives and false-negatives. Especially, interpretation of ECG in children is different from ECG in adults, with less accurate diagnoses as a consequence. Secondary prevention by widespread education of simple resuscitation techniques and use of automatic external defibrillators if available will probably save as many lives as any screening program. Conclusion: Sufficient data are lacking to support general preparticipation screening with history, physical exam, and ECG in competitive children. Nevertheless, the impact of such a program, together with secondary preventive measures, should be evaluated in large prospective studies.     

CARDIOVASCULAR CAUSES FOR SUDDEN INFANT DEATH

Although sudden infant death syndrome (SIDS) is a cause for sudden infant death, other causes should be ruled out before diagnosing SIDS. Cardiac causes for sudden infant death include viral myocarditis, congential heart disease particularly congential aortic stenosis, endocardial fibroelastosis, and anomalous origin of the left coronary artery from the pulmonary artery. Other cardiac conditions that may result in sudden death include rhabdomyomas of the heart in tuberous sclerosis and conduction system disorders. The most frequent conduction system disorders resulting in sudden death include histiocytoid cardiomyopathy, congential heart block that may be associated with maternal lupus erythematosus, arrhythmogenic right ventricular dysplasia, noncompaction of the left ventricle, and long QT syndromes.     

Kawasaki syndrome. Clinical report of 5 cases]

The paper reports five cases of Kawasaki's syndrome diagnosed during the period 1985-89. The mean age of patients was 20 months (4/12; 4 years and 5/12). Symptomatology gradually regressed in four of these patients with a return to good health and normal cardiological controls. The fifth patient died suddenly following a cardiorespiratory failure when his general conditions were showing a marked improvement. Given that cardiac involvement is reported to be the sole cause of death in Kawasaki's syndrome, this was probably the cause of the patient's death. This event together with the presence of a number of barely symptomatic forms, which certainly escape correct diagnosis, may explain some of the cases of sudden death in early childhood. In the present case the child died suddenly due to an early stage of heart failure on awakening and may be considered a classic example of "cot death". Although the treatment protocol is well defined, it is not yet known to what extent the cardio-vascular apparatus is influenced by therapy in cases of children with Kawasaki's syndrome.     

Investigation of unexplained infant deaths in Jerusalem, Israel 1996-2003.

BACKGROUND: Sudden infant death syndrome (SIDS) is a diagnosis of exclusion that may be assigned only after investigations including a forensic autopsy are performed to exclude possible organic and environmental causes of death. Israeli society is influenced by the Jewish and Islamic faiths, which permit autopsy only under selected circumstances. Against this background, we carried out a study to determine what examinations are performed to investigate unexplained infant deaths in Jerusalem, Israel. METHODS: We examined hospital, Ministry of Health and Ministry of Interior records of unexplained infant deaths in the Jerusalem district from the years 1996-2003. RESULTS: Ninety six cases were identified from all sources. Forty nine (51%) infants were brought to a hospital at or near the time of death. Studies to determine the cause of death were performed in 54% of cases for which medical records were available for review. These studies included bacterial cultures (44%), skeletal surveys (12%), computerised tomography (3%) and metabolic studies (3%). Only one forensic autopsy was performed, and in no instance was the death site examined by medical personnel. There was a high rate of retrospective review by district health physicians. The most frequently assigned cause of death was SIDS. CONCLUSIONS: : The capacity of public health officials and forensic pathologists to investigate unexplained infant deaths is strongly affected by the legal, religious and political milieu in which they work. Efforts should be made to develop socially acceptable methods of improving the quality of infant death investigations in Jerusalem.     

American Academy of Pediatrics: Distinguishing sudden infant death syndrome from child abuse fatalities

In most cases, when a healthy infant younger than 1 year dies suddenly and unexpectedly, the cause is sudden infant death syndrome (SIDS). SIDS is more common than infanticide. Parents of SIDS victims typically are anxious to provide unlimited information to professionals involved in death investigation or research. They also want and deserve to be approached in a nonaccusatory manner. This statement provides professionals with information and guidelines to avoid distressing or stigmatizing families of SIDS victims while allowing accumulation of appropriate evidence in potential cases of death by infanticide.     

Evolution and significance of the triple risk model in sudden infant death syndrome

Sudden infant death syndrome (SIDS) is a leading cause of death in infants, although the mechanisms leading to death remain unclear. Multiple theories have emerged over time, with one of the most influential hypotheses being the triple risk model. This model, first devised in 1972 and later revised in 1994 by Filiano and Kinney, is still widely used in assisting with conceptualising and understanding sudden death in infancy. This model has evolved over time, with each version stressing that SIDS is likely to occur when certain risk factors coincide, suggesting that the lethal mechanisms in SIDS are likely to be multifactorial. All versions of the triple risk model from 1972 to the present have emphasised the complexity of SIDS and serve as useful guides for current and future research into the enigma of sudden and unexpected death in infancy.