婴儿猝死综合征的法医学鉴定

近20多年来,婴儿猝死综合征（sudden infant death syndrome,SIDS）一直是西方发达国家医学界尤其是法医学和儿科学领域研究的热点。目前,随着对SIDS广泛而深入的研究,对其病因、发病特点、危险因素以及致病机制等都有了更明确的认识。虽说近年来SIDS的发病率已有明显下降,在欧美洲、澳洲等发达国家,SIDS仍然是导致1个月到1岁婴儿死亡的首要原因。在亚洲国家和地区,尤其是发展中国家SIDS的报道很少见。本文通过总结文献并结合美国马里兰州近20年（1990—2006）来对SIDS的调查研究资料,介绍SIDS的发展历史、研究现状和新的发展趋势,并对SIDS法医学鉴定程序及鉴定要素进行讨论。     

Sudden infant death syndrome (SIDS)--standardised investigations and classification: recommendations

Sudden infant death syndrome (SIDS) still accounts for considerable numbers of unexpected infant deaths in many countries. While numerous theories have been advanced to explain these events, it is increasingly clear that this group of infant deaths results from the complex interaction of a variety of heritable and idiosyncratic endogenous factors interacting with exogenous factors. This has been elegantly summarised in the "three hit" or "triple risk" model. Contradictions and lack of consistencies in the literature have arisen from diverse autopsy approaches, variable applications of diagnostic criteria and inconsistent use of definitions. An approach to sudden infant death is outlined with discussion of appropriate tissue sampling, ancillary investigations and the use of controls in research projects. Standardisation of infant death investigations with the application of uniform definitions and protocols will ensure optimal investigation of individual cases and enable international comparisons of trends.     

Sudden infant death syndrome (SIDS): a burgeoning medicolegal problem.

This Article presents a summary analysis of the administrative and statutory bases for the documented, prevalent mismanagement of Suddern Infant Death Syndrome (SIDS) cases by a majority of local death investigation agencies in the United States. Herein, Alan P. Cleveland, J.D. advances the theory that the unsatisfactory handling of cases of SIDS by the medicolegal community is the inevitable outgrowth of state laws that expressly require investigative agencies to approach a sudden, unexplained death from the direction of determining first whether or not a criminal act has occurred. In so doing, most statutorily mandated autopsy procedures are socially counterproductive since, in ignoring an acute medical need for supportive family counselling, they often constitute an insuperable obstacle to the effective management of SIDS as a public health problem. The author recommends that a requisite first step in implementing an SIDS management program at the state level is to insulate surviving family members form criminal investigative procedures by appropriate amendment of state laws governing local death investigation systems.     

Sudden infant death syndrome in Japan

Two autopsied cases are presented, one involving a 5-month-old infant, and a 6-month-old infant both of whom died suddenly and unexpectedly. The incidence of sudden infant death syndrome in Japan is 1.2 per 1,000 babies live births. Among all cases autopsied in the departments of legal or forensic medicine in 78 universities or colleges of Japan, the incidence was 15 (0.5%) per 3,329 in 1984 and 20 (0.6%) per 3,150 in 1985.     

Sudden infant death due to asplenia syndrome

An apparently healthy 80-day-old boy died suddenly for no apparent reason. The autopsy revealed that the patient had had congenital asplenia, extensive cardiovascular anomalies, and other organ malformations, including trisegmented lungs, hypoplasia of the corpus callosum and cranial bones, a symmetrical liver, accessory hepatic tissue in the adrenal glands, malrotation of the intestine, and hypoplasia of the greater omentum.     

Sudden infant death syndrome: a subject of medicolegal research

During the last decade, much attention has been paid to the risk factors of sudden infant death syndrome (SIDS). Many researchers have demonstrated that infant-care practices are linked to the risk of SIDS. Prone sleeping, bed sharing, maternal substance abuse, and cigarette smoking have been reported to be significant potentially modifiable risk factors for SIDS. Despite the reports that the incidence of SIDS has decreased by 38% in the United States, it remains the leading cause of death in the first year of life. Deaths resulting from child abuse or neglect inflicted or permitted by their caretakers being second only to SIDS in infant mortalities and some recommendations regarding the differentiation of SIDS and child abuse have generated speculation that some cases of infanticide were misdiagnosed as SIDS. To reach a proper conclusion as to the cause and manner of death of an infant who died suddenly and unexpectedly, investigation must be thorough and professional.     

Sudden infant death syndrome: diagnostic practices and investigative policies, 2004

Using a 2004 population-based survey of all US medical examiner and coroner offices, we examined the characteristics of offices accepting an infant death case and calculated the percentage of offices that had death scene investigation or autopsy policies for the investigation of sudden unexpected infant death (SUID). We also calculated the percentage of offices that used and did not use sudden infant death syndrome (SIDS) as a cause of death, and we compared differences in characteristics among those offices.Of medical examiner and coroner offices, 52% did not report an infant death in 2004. Of the 7957 infant deaths reported, 43% occurred in jurisdictions that experienced 1 or 2 infant deaths. Of the offices that used SIDS as a classification, 34% did not have policies for conducting death scene investigations and autopsies for SUID. At least 5% of offices that reported an infant death did not use SIDS as a cause of death classification. These findings have important implications for understanding recent trends in SIDS and SUID. Supporting the implementation of national standards for investigating and certifying infant deaths could provide guidelines for consistent practices in medical examiner and coroner offices.     

Sudden infant death syndrome: Histological studies on adrenal gland and kidney

Histological observations were made on the adrenal glands and kidneys in ten cases of sudden infant death syndrome (SIDS). The amount of fetal cortex was excessive in the adrenal glands and many glomeruli of fetal form were observed in the kidneys. These findings suggest that the adrenal glands and the kidneys in SIDS cases are more or less immature in development. The immature development in these organs, especially in the adrenal glands, was considered to play a role in the cardiac or respiratory mechanisms in SIDS.     

Sudden death in an 8-week-old infant with Beckwith-Wiedemann syndrome

The authors report a case of a 2-month-old girl diagnosed with Beckwith-Wiedemann syndrome (BWS) who was born prematurely and died suddenly in the hospital just before being discharged. BWS is a malformation syndrome associated with an increased risk of childhood tumors. The major features of BWS are macroglossia, abdominal wall defects, and visceromegaly, frequently leading to premature birth. Due to complex inheritance patterns, a predominance of nonfamilial cases, and the variability in expression of the features (termed incomplete penetrance), the risk of delayed diagnosis is evident. Secondary to hyperplastic pancreatic islands, hypoglycemia occurs frequently, and if not anticipated, adequate measures for prevention of hypoglycemic episodes may be delayed, resulting in possible intellectual deficits. The infant presented here died of natural causes: immaturity of the lungs resulting in marginal respiratory function and compounded by increased risk for asphyxia secondary to the enlarged tongue. The clinical history and findings in this infant are discussed in respect to the genetic syndrome with their relevance to medicolegal examination and the causes and manner of death.     

Sudden infant death syndrome. Morphology update for forensic pathologists--1985

Herein presented are current concepts regarding the sudden infant death syndrome (SIDS) including a definition of the phenomenon and a discussion of its present role in post-neonatal mortality in this country, a few of the recently published results (both clinical and morphological) of the NICHD Cooperative Epidemiologic Study, a report on the status of the so-called "Tissue-Markers for Hypoxia" and a summary of other recent morphologic observations.     

Nonnatural death masquerading as SIDS (sudden infant death syndrome)

This article relates our experiences with accidental and homicidal deaths in reported sudden infant death syndrome (SIDS) cases. Our intent is to alert personnel in medical and health-related professions as well as death investigators (coroners, law enforcement officials, etc.) that a thorough scene investigation and history need to be obtained when a child's death has been reported, since this may modify the type of autopsy to be performed. It should not be assumed that all children less than 1 year of age who die suddenly die from SIDS.     

Sudden death and Angelman syndrome

Angelman syndrome is a condition characterized by developmental delay due to abnormalities in the maternally derived chromosome 15q11-q13. Typical features include impaired expressive language, an ataxic gait, and seizures. Hyperactivity may result in accidental bruises and abrasions, raising issues of possible inflicted injury. A fascination with water may predispose to drowning. A 5-year-old boy with an established diagnosis of Angelman syndrome is reported who died of upper airway obstruction due to massively enlarged tonsils complicating infectious mononucleosis. Assessment of the severity of underlying illness in developmentally delayed children may be difficult due to failure to vocalize worsening symptoms and distress. In addition, signs of upper airway narrowing due to infection in Angelman syndrome may be masked by the sucking and swallowing difficulties that affected individuals may have with drooling and excessive chewing and mouthing behavior.     

Unexpected perinatal death and sudden infant death syndrome (SIDS): anatomopathologic and legal aspects

This work intends to be a review of the recent histopathological findings elicited by research into sudden and unexpected perinatal death and sudden infant death syndrome (SIDS) that have dictated a novel approach to the inherent problems by pathologists, especially those entrusted with forensic medical authority. The new approach stems from the recent advances made in the understanding of neuro- and/or cardiac-conduction-system diseases present in unexpected perinatal death and SIDS. These demand that an accurate morphologic examination be performed of these structures, which modulate respiratory, cardiovascular, digestive, and arousal activities, in all victims of sudden death. A histopathologic study of an ample register of cases of victims of sudden death, either perinatally or in early infancy, has demonstrated frequent alterations both of the autonomic nervous system (especially hypoplasia of the arcuate nucleus) and of the cardiac conduction system (accessory atrioventricular pathways). The present research provides an in-depth study of the many still-controversial aspects underlying perinatal unexpected death and SIDS and is recommended for professionals working in the forensic field, whose greater insight into this problem will allow more complete medicolegal documentation.     

Sudden infant death syndrome and hypertrophy of the palatine tonsil: reports on two cases.

Two cases of sudden infant death syndrome (SIDS) with hypertrophy of the palatine tonsil were reported. The pathogenesis of the SIDS has been clarified (Guilleminault et al., Pediatrics, 68 (1981) 354-360). According to this theory, it is due to a central impairment of the breathing control during sleep, which is particularly pronounced in predisposed subjects. The present cases suggest that the hypertrophied palatine tonsil might contribute as a predisposing factor to the emergence of a SIDS by mechanical impediment to breathing by narrowing of the upper airway.     

Sudden infant death and liver phosphoenolpyruvate carboxykinase analysis

In an effort to substantiate the impaired gluconeogenesis-terminal hypoglycemia hypothesis of sudden infant death syndrome (SIDS), 52 infants ranging from 3 weeks to 7 months of age which had been brought to autopsy were studied. The stomach contents, vitreous humor glucose concentrations, hepatic glycogen content and hepatic phospho-enolpyruvate carboxykinase (PEPCK) activity were measured as part of the laboratory component of the postmortem investigation. The stomach contents, vitreous humor glucose concentrations and liver glycogen content were similar in SIDS/and non-SIDS victims. PEPCK activity was, however, significantly lower in SIDS (p < 0.001) victims and in SIDS with other findings (p < 0.01) victims when compared to non-SIDS victims. Despite the fact that SIDS victims had lower hepatic PEPCK activity and hence potentially lower gluconeogenic capacity, terminal hypoglycemia could not be demonstrated in this group as compared to the SIDS with other findings and the non-SIDS infants. The impaired gluconeogenesis-terminal hypoglycemia hypothesis thus could not be substantiated.     

Sudden infant death in a tropical environment: Singapore's experience

A total of 206 cases of sudden infant deaths examined at the Institute of Science and Forensic Medicine, Singapore, over a 5 year period (1989–1993) were identified to assess the pattern of sudden death in this age group, which was subdivided into the neonatal and post-neonatal periods. A total of 34% (70) of infant deaths occurred in neonatal life and the remaining 66% (136) in the post-neonatal period; 90% of the neonatal deaths were natural, of which over half were due to congenital heart disease and complications of prematurity. Unnatural deaths in this period were uncommon, there being only seven such deaths. In the post-neonatal period, unnatural deaths constituted 25% of the total with trauma and aspiration heading the list. Natural deaths in the post-neonatal period are predominantly due to infections (34%) and a group of sudden natural deaths with minimal findings (31%). The latter group may arguably represent cases of Sudden Infant Death Syndrome (SIDS). The yearly incidence of this group in our study varied between 0.08 to 0.2 per 1000 live births, which is considerably lower than the incidence quoted for Western populations. The criteria for the classification and the impact of sudden infant deaths in Asian countries are discussed.