Oronasal blood in sudden infant death.

Oronasal secretions are observed frequently in sudden infant death syndrome (SIDS), but overt blood is uncommonly reported. The literature on oronasal blood in sudden infant death is limited. The goal of this study was to determine the frequency of oronasal blood in sudden infant deaths and to examine possible causative factors. Oronasal blood was described in 28 (7%) of 406 cases of sudden infant death. Oronasal blood could not be attributed to cardiopulmonary resuscitation in 14 cases, including 10 (3%) of 300 cases of SIDS, 2 (14%) of 14 accidental suffocation cases, and 2 (15%) of 13 undetermined cases. Eight of the 10 infants in cases of sudden infant death were bedsharing: 5 with both parents, 2 between both parents. The infant in 1 SIDS case was from a family that had had three referrals to Child Protective Services. Oronasal blood not attributable to cardiopulmonary resuscitation occurs rarely in SIDS when the infant is sleeping supine in a safe environment. Bedsharing may place infants at risk of suffocation from overlaying. Oronasal blood observed before cardiopulmonary resuscitation is given is probably of oronasal skin or mucous membrane origin and may be a sign of accidental or inflicted suffocation. Sanguineous secretions that are mucoid or frothy are likely of remote origin, such as lung alveoli. The use of an otoscope to establish the origin of oronasal blood in cases of sudden infant death is recommended.     

Simultaneous sudden infant death syndrome: a proposed definition and worldwide review of cases.

Epidemiologic studies of sudden infant death syndrome (SIDS), the leading cause of death of infants during the postperinatal period (7-365 days), have mainly focused on the deaths of single infants. Simultaneous sudden infant death syndrome (SSIDS), the death of a pair of twins occurring at the same time, has received limited attention within the medical community. To the authors' knowledge, this article is the first to describe the 41 SSIDS cases cited in the world literature from 1900 to 1998 by the location of death, a summary of the circumstances surrounding the deaths, and evaluation of these cases in terms of a proposed definition of SSIDS. This evaluation critiques whether the 41 pairs of SSIDS cases adhere to a newly proposed definition of SSIDS. Twin infant deaths must meet all three criteria to be considered SSIDS. The study found that only 12 pairs of twins met all three criteria (29.2%), nine pairs met two criteria (21.9%), alternative cause of death was offered in five pairs of twins (12.1%) and in the remaining 15 pairs (36.6%), only limited information was available; therefore, no conclusions could be reached.     

Comparative analysis of differences by gender in sudden infant death syndrome in Hungary and Japan

We examined the sex ratio in sudden infant death syndrome (SIDS) cases in Hungary, in Tokyo and Japan between 1985 and 1996. From all the infant death cases in Hungary 395 (240 male, 155 female) were SIDS (odds ratio (OR)=1.179, with 95% confidence interval (CI)=0.961, 1.446), in Japan 4348 (2550 male, 1798 female) were SIDS (OR=1.145, with 95% CI=1.076, 1.218) and in Tokyo 307 (178 male, 129 female) were SIDS (OR=1.128, with 95% CI=0.894, 1.423). Male infants showed a significantly higher birth rate than females. The male infants are more vulnerable (p<0.005), however, higher mortality among male infants should not be considered a characteristic feature for SIDS.     

Pulmonary siderophages and unexpected infant death

It has been proposed that the presence of siderophages in the lungs of infants who die unexpectedly should be considered a marker of a previous hypoxic event, which may preclude a diagnosis of sudden infant death syndrome. The authors retrospectively reviewed all infant deaths (<1 year old) going to autopsy at the Denver Office of the Medical Examiner from January 1999 to January 2001. Lung sections were stained with Prussian blue, and siderophages were counted in 20 high-power fields per lobe sampled. Cell counts were performed by two independent pathologists who were blinded to history and cause of death, with good reproducibility. Iron stain results were then categorized by average number of siderophages per 20 high-power field (category 1 = <5, category 2 = 5-100, category 3A = 100-500, category 3B = >100 in a single lobe, category 4 = >500). The results were subsequently correlated to case history, autopsy findings, and cause/manner of death. Forty-three cases were reviewed. The causes of death included sudden infant death syndrome (16), asphyxia (5), undetermined (6), and other (16). Those deaths were categorized by the above criteria as follows: category 1. (32), category 2. (6), category 3. (4), and category 4. (1). All sudden infant death syndrome deaths were in category 1. Categories 1 and 2 also included deaths in which hypoxia might have been present before death because of such factors as pneumonia and congenital heart disease. Categories 3 and 4 included a known homicidal asphyxia in which repeated episodes of intentional smothering were documented, 2 probable asphyxias, 1 nonaccidental trauma, and 1 undetermined. All 5 cases had questionable circumstances surrounding the death of the infant. Pulmonary siderophages were described in only 1 of the 43 autopsy reports. It was concluded that pulmonary siderophages can be markedly increased in cases of repeated asphyxia. Siderophages may also be increased in cases where hypoxia may have been present for another reason, but not to the same degree. Siderophages are not increased in sudden infant death syndrome. Because iron-laden macrophages often are not recognized on routine examination with hematoxylin and eosin staining, iron stains may be helpful in the evaluation of infant deaths. If siderophages are present in increased amounts without an obvious explanation, further investigation is warranted.     

Virological analysis in the diagnosis of sudden children death: a medico-legal approach

Infections are considered to be an important cause of unexpected death in children. It has also been assumed that respiratory viruses are involved in the genesis of sudden infant death syndrome (SIDS). The Spanish National Institute of Toxicology and Forensic Sciences act as the forensic reference centre for Spain. We analyse the experience of this centre in the virological study of 64 cases of sudden children death where viral serology, virological cultures, herpesviruses polymerase chain reaction (PCR) and electron microscopy were performed. According to pathological findings, death could only be attributed to an adenovirus infection in one amygdalitis with upper airways stenosis and asphyxia. Human herpes virus 6 (HHV-6) was detected by PCR in one case with pathological findings characteristic of SIDS. Recent infection by respiratory syncytial virus (RSV), Epstein-Barr virus (EBV) and cytomegalovirus (CMV) were also detected. Meanwhile, 85.9% of the cases yielded negative viral results. Twenty-eight infants were finally categorised as SIDS. Pathological findings of infection were detected in 12 patients despite the negativity of viral analyses. Although viral infection is an uncommon cause of sudden children death, a complete microbiological investigation will help to solve the puzzle of SIDS. Definitive guidelines for microbiological analyses need to be updated whilst new pathogens are discovered or new techniques are implemented in order to clarify unsolved cases.     

Sudden death of an infant with 'an early epileptic encephalopathy'.

This article reports an autopsy case of sudden death of an infant with an infrequent encephalopathy involving epileptic episodes. The infant was a 1-year and 10-month-old boy, who had a history of the first convulsive seizures in the third month after birth. The clinical diagnosis was described as 'an early infantile epileptic encephalopathy with suppression-bursts' (Ohtahara syndrome). On a winter day, he was collapsed following a high fever and was already dead on the arrival at a hospital. The body was small for the age and poorly nourished. The autopsy and postmortem magnetic resonance imaging scan (MRI) of formalin-fixed brain revealed advanced unsymmetric brain atrophy with cortical dysplasia, which were prominent in the left temporal and right occipital lobes, and sclerotic atrophy of the parahippocampal gyri, additionally showing a feature of the olivo-ponto-cerebellar atrophy. However, the cause of death was pathologically and microbiologically determined as bacterial bronchopneumonia following pulmonary infection of the influenza A virus. In sudden death cases of physically handicapped infants, the investigation of viral infection in consideration of an epidemiological survey is important even when the death can be pathomorphologically explained.     

Desquamative interstitial pneumonia in an infant. Mimicry of sudden infant death syndrome

A 15-week-old infant girl, without a prior history of overt illness, was found dead while sleeping between her two parents. The gross examination at autopsy showed only congested lungs, and the initial diagnosis was sudden infant death (SID). On microscopic examination, a desquamative interstitial pneumonia (DIP) was observed. The widespread, patchy intraalveolar histiocytic desquamation was associated with lymphocytic infiltration of bronchiolar and aveolar walls, which together provided convincing evidence that an interstitial pneumonitis was the cause of death. A viral etiology seems most likely in view of the accompanying chronic inflammation of bronchial submucosal glands.     

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.     

Cardiac lesions in sudden infant death syndrome

Sequential morphological changes as found in the hearts of 250 sudden infant death syndrome (SIDS) infants are described. Detailed examination of macroscopic and microscopic lesions reveal that all SIDS infants had identifiable lesions at the time of their death. The lesions can best be described as selective focal anoxic muscle fibre necrosis at chronologically different developmental stages. The extent of these lesions vary markedly from case to case, from a minimal muscle fibre eosinophilia through contraction band formation, myocytolysis, stromal condensation to scar formation. The morphological variations in the lesions amongst individual cases can be interpreted as relating to the time interval of the development of the lesions. The intramural and coronary arteries in some cases are also affected showing intimal hyperplasia. Although these sequential morphological aberrations are not specific and typical to SIDS infants only, they were present in all SIDS infants in this series.     

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.     

Histologic thyroid gland findings in the newborn infant and infant, with special reference to sudden infant death

The morphological picture of the thyroid gland, the only endocrine organ with a follicle structure, allows a limited conclusion to be drawn with respect to the functional state in spite of any physiological variability. The thyroid of the newborn shows total colloid release and collapse of the follicles. The regular structure of the thyroid will be rebuilt within several weeks after birth. Total colloid absorption can be found in cases of stress-activated thyroids as well as in cases of death due to freezing. A histological examination was done on 88 thyroids of fetuses, newborns and infants. About 70% of the results on 27 cases of sudden infant death syndrome (SIDS) may be interpreted as a morphological correlate of a premortal chronic or recurrent stress reaction. The value of these results is discussed.     

Toxicology and sudden infant death

One hundred thirty cases of sudden infant death occurring in Wayne County, Michigan, (population 2.7 million) were analyzed for possible drugs. The toxicological protocol has been outlined. Six cases were found to be positive, and in five of these the drugs found had been prescribed for a variety of illnesses. In one case methadone was found in the blood of an infant whose mother was undergoing methadone treatment for drug addiction, the drug being transmitted through breast milk. In none of the 102 cases of sudden infant death syndrome (SIDS) included in the study did the toxicological results affect the diagnosis. In our study, toxicological analyses never contradicted an initial diagnosis of SIDS, and, therefore, we feel that this diagnosis should be made promptly based on investigative and autopsy findings.     

Homicide as a cause of the sudden infant death syndrome

The homicidal asphyxiation of a 10 1/2-month-old male infant and the attempted asphyxiation of his 4-month-old sibling, documented by parental confession, is presented as evidence that murder may sometimes be mistaken as sudden infant death syndrome (SIDS). A review of the literature of the relationship between murder and SIDS deaths reveals the suspicions of some physicians but few published cases; this reflects not only the difficulties of making a determination of murder by suffocation, since no injuries may be present, but also a lack of awareness among physicians who must evaluate infant deaths. It is recommended that murder should be considered in the differential diagnosis of sudden, unexpected death in infants and that the autopsy should include full-body x-rays and at least an initial look at the social history of the child.     

Increase of pulmonary density of macrophages in sudden infant death syndrome

A 1996 cytodensitometric study found increased cellular density in the pulmonary parenchyma of infants who died of sudden infant death syndrome (SIDS). The present study clarifies these results in quantifying the density of immunohistochemical subtyped inflammatory cells. Histomorphometry was used to compare the density of macrophages, granulocytes and T and B lymphocytes in the lungs of two groups of infants. From the post-mortem records of infant deaths between 1983 and 1995, 29 (mean age = 5 months) were randomly selected including 16 cases of SIDS and 13 who died of other non-pulmonary causes. Densities of immunoreactive cells were measured under blind conditions in the parenchyma. The mean density of macrophages was significantly higher in cases of SIDS compared with the controls (P = 0.0318), but there were no differences for the lymphocytes and the granulocytes. These morphometrical results must be interpreted within the methodological limits of this study, especially the non-uniform level of lung inflation between selected subjects. However, the differences in level of inflation are not sufficient to explain the observed increase of macrophage density. Indeed, the mean values of alveolar surface area, which represent an indirect measure of lung inflation, are not significantly different between the two groups. Increase of pulmonary macrophage density in SIDS agrees with three non-exclusive hypotheses: (1) an abnormal inflammatory reaction by expression of Th1 helper cell phenotype activation; (2) consequence of passive smoking; and (3) post-agonal mechanisms. Bacterial superantigens produced by toxigenic bacteria in the respiratory tract could play a role as a trigger factor that initiates a fatal cascade with overproduction of cytokines leading to death. The significant increase of pulmonary macrophage density would be the morphological expression of this potential mechanism of death.     

Beta-endorphin in the brainstem, pituitary, and spinal fluid of infants at autopsy: relation to sudden infant death syndrome

Immunohistochemical localization of beta-endorphin was studied in the pituitaries and medullas of forty human infants at autopsy. beta-Endorphin immunoreactivity was found in anterior pituitary cells in all cases. In the medulla, beta-endorphin immunoreactivity was found in the neurons of the medial and lateral cuneate nuclei in ten out of the forty cases. In eight of these ten cases, the infants died of causes other than sudden infant death syndrome (SIDS). Only two of 25 SIDS cases had demonstrable beta-endorphin in the brainstem nuclei. Beta-endorphin levels in the spinal fluids of all the cases showed no correlation to cause of death, age or gender.     

Serum tryptase levels in sudden infant death syndrome in forensic autopsy cases

An elevated serum tryptase concentration is considered to be a specific marker for systemic mast-cell activation, a central feature of anaphylaxis, which has been observed in some cases of sudden infant death syndrome (SIDS). However, it is still unclear whether anaphylaxis is involved in the etiology for SIDS. In the present study, we measured serum tryptase levels in 21 infants with SIDS, and 14 control infants from forensic autopsy cases by Uni-CAP TRYPTASE Fluoroenzyme immunoassay system, which detects both alpha- and beta-tryptase. The assay did not show any significant elevation of tryptase levels in the SIDS group compared with controls. Additionally, increased concentrations of tryptase were not observed in any SIDS case. Our results indicated that anaphylaxis does not seem to be involved in the etiology of SIDS.     

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.     

An autopsy case of an infant with Joubert syndrome who died unexpectedly and a review of the literature

The present report describes the unexpected death of a 6-month-old female infant who had been clinically diagnosed with Joubert syndrome. This is a relatively rare congenital neurological disorder characterized by hypoplasia/aplasia of cerebellar vermis, which transmits information from the body to the cerebellum, and is associated with respiratory dysfunction, abnormal eye movements, and developmental delay. The infant was found dead in bed and the immediate cause of death was determined as aspiration of vomit which may have been induced by a neurological disorder related to hypoplasia of the cerebellar vermis. These findings, together with a review of previous clinical case reports, suggest that Joubert syndrome should be considered as a predisposition to sudden unexpected death in infants mainly due to aspiration or complicated infection.     

An analysis of the usefulness of specific stages in the pathologic investigation of sudden infant death

Retrospective analysis of autopsy findings in 60 infants who had been found unexpectedly dead in their cribs or beds in South Australia from 1994 to 1998 was undertaken to determine the diagnostic usefulness of individual stages in the postmortem investigation. Positive findings occurred in 2 of 43 scene examinations (3%), 2 of 60 external examinations (3%), 2 of 11 radiologic examinations (18%), 8 of 60 internal examinations (13%), 7 of 60 histologic examinations (12%), and 3 of 58 microbiologic examinations (5%). No positive findings were detected on toxicologic screening. Not every case underwent each diagnostic step. This gave alternative diagnoses to sudden infant death syndrome (SIDS) in 15 cases (25%). This study demonstrates an increase in the percentage of cases of unexpected infant death due to causes other than SIDS; it also shows the diagnostic yield of individual stages in the postmortem evaluation of such cases. Negative findings were important in giving validity to the diagnosis in the 45 cases that were ultimately designated as SIDS.     

Changing trends in the diagnosis of sudden infant death

A study of 114 consecutive cases of unexpected infant death that occurred in South Australia over a 5-year period from January 1994 to December 1998 was undertaken. There were 45 deaths attributed to sudden infant death syndrome (SIDS), 19 to natural causes, 21 to accidents. and 5 to homicides; 24 cases were listed as "undetermined." Although there has been a genuine and continued decline in SIDS numbers in this population, there has also been an increase in the diagnosis of cases of accidental asphyxia due to unsafe sleeping environments and of cases in which the family background and autopsy findings suggested more complex mechanisms. The change in diagnostic profile has followed the introduction of more rigorous clinical history review, death scene examination, and autopsy testing. Thus, although diagnostic outcomes have altered in this population, it is more likely the result of more careful interpretation of the extensive investigations that are now undertaken rather than arbitrary reclassification.