1/60. Asplenia as a cause of sudden unexpected death in childhood.Sudden unexpected death in childhood is rare. The commonest causes of such deaths are a result of fulminating infections of the respiratory or nervous systems. Other causes include unsuspected congenital abnormalities of the heart, acute metabolic disorders, and rarities such as internal hemorrhages and pulmonary thrombosis. Recognition of children with congenital asplenia who are otherwise normal but have an increased susceptibility to overwhelming sepsis is extremely difficult. We reviewed 1763 autopsy files from our institution over 5 years (1990-1995), of which 293 were classified as pediatric cases. The vast majority of the cases were stillbirths and deaths within the first year of life as a result of complex congenital anomalies. Four cases of asplenia were identified in our entire series, 3 of which were of the congenital syndromal variety and 1 of which was a case of isolated sporadic congenital asplenia. All 4 cases of asplenia were analyzed in detail with respect to autopsy findings and cause of death. Severe complex cardiac malformations were present in the congenital syndromal asplenia patients; these other malformations contributed significantly to their death. In this report, we discuss in detail the autopsy findings in a previously healthy 4-year-old girl who presented with a brief 8-hour history of being unwell and died within 4 hours of admission into the hospital. She had sporadic, isolated congenital asplenia complicated by high-grade type 6B pneumococcemia and acute bilateral adrenal hemorrhage (waterhouse-friderichsen syndrome). Previously healthy children who clinically deteriorate very rapidly should have a blood smear done as part of their clinical workup. The detection of Howell-Jolly bodies on a peripheral blood smear can be an indicator of asplenia, and this diagnosis can be confirmed by medical imaging of the abdomen. Such steps may aid in the aggressive management of isolated congenital asplenia and thereby avert untimely death.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/60. A case of sudden death caused by fibromuscular dysplasia.After sudden unexpected death in a previously healthy 42 year old woman, necropsy examination showed myocardial infarction caused by occlusion of a vital coronary artery by fibromuscular dysplasia. This is a rare arterial disease with a clinical onset usually in the third or fourth decades of life. The aetiology is not fully understood but since it affects vital (cerebral and coronary) blood vessels it often has fatal consequences.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/60. Lethal or life-threatening allergic reactions to food.Fatal or life-threatening anaphylactic reactions to food occur in infants, children and adults. Atopic individuals with bronchial asthma and prior allergic reactions to the same food are at a particularly high risk, whereby even the mere inhalation of the allergenic food can be fatal. Not only peanuts, seafood and milk can induce severe, potentially lethal anaphylaxis, but indeed a wide spectrum of foods, according to the different patterns of food sensitivity in different countries. Foods with "hidden" allergens and meals at restaurants are particularly dangerous for patients with food allergies. Similarly, schools, public places and restaurants are the major places of risk. However, the main factor contributing to a fatal outcome is the fact that the victims did not carry their emergency kit with adrenaline (epinephrine) with them. In cases of death where food anaphylaxis is suspected, it is important for forensic reasons to preserve uneaten portions of the food in order to identify (hidden) allergens. It is also important to determine postmortem specific serum IgE, tryptase and histamine levels to document the anaphylaxis. There is a need to raise awareness of the diagnosis and treatment of anaphylaxis among doctors, those called upon to administer emergency medical care, and the public, and also to provide increased support for those with potentially fatal food allergies through the help of patients' organizations, and national and international medical societies. The food industry should ensure a policy of comprehensive labelling of ingredients so that even the smallest amount of potentially lethal foodstuffs can be clearly identified. Finally, the pharmaceutical industry should be persuaded to reintroduce an adrenaline inhaler onto the market.- - - - - - - - - - ranking = 5keywords = life (Clic here for more details about this article) |
4/60. fatal outcome in a case of pontocerebellar hypoplasia type 2.Pontocerebellar hypoplasia (PCH) is a very rare congenital (autosomal recessive) condition with fetal onset. Only a few cases have been published on the basis of both clinical data (symptoms/neuroradiological imaging) and autopsy results. This paper reports on such a case involving a 1.5-year-old male infant. The child suffered from severe psychomotor delay, extrapyramidal dyskinesia and epileptic seizures, but did not exhibit signs of spinal muscular atrophy as related to PCH type 1. magnetic resonance imaging (MRI) at the age of 6 months demonstrated olivo-pontine and bilateral cerebellar hypoplasia. The boy was unexpectedly found dead. autopsy disclosed a severe aspiration of gastric contents as the final cause of death. The neuropathological examination confirmed PCH type 2 (according to Barth [brain Dev., 15 (1993) 411-422]) with marked microcephaly and olivopontocerebellar hypoplasia. Histologically, decreased density of olivo-pontine neurons, reduction of granular and Purkinje's cell layers of the cerebellum, slight astroglial proliferation and fragmented appearance of the dentate nuclei were observed. The immunohistochemical expression pattern was determined using antibodies against glial fibrillary acidic protein, synaptophysin and neurofilament protein. Summarizing, typical features of PCH type 2 were present and proved by clinical course, MRI and autopsy. Despite severe symptoms due to a natural disease this rare neurogenetic entity can become of forensic interest, when sudden unexpected death occurs.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/60. Death due to microvascular occlusion in sickle-cell trait following physical exertion.The heterozygous condition characterized by the presence of hemoglobin AS (sickle-cell trait) occurs in approximately 8% of the American black population. Unlike the homozygous state (sickle-cell disease), sickle-cell trait is not widely recognized as a cause of life-threatening illness or death despite over 30 case reports describing fatal or serious complications of exercise in young black males with this condition. These reports identify heat stress, dehydration, viral illness, and poor physical conditioning as factors which may contribute to exertional rhabdomyolysis and sudden death, suggesting multifactorial etiology. However, since sickling is known to occur postmortem, it remains controversial as to whether the pathogenesis of these exercise related deaths involves microvascular obstruction by sickled erythrocytes. We describe three young black individuals with no significant past medical history who died following physical exertion. In all three cases, postmortem hemoglobin electrophoresis demonstrated hemoglobin AS. In none of the cases was the body temperature found to be elevated. These cases serve to remind the forensic community that, in the proper setting, sickle-cell trait must be viewed as a potentially fatal disorder.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/60. Sudden death from fulminant acute cerebellitis.Acute cerebellitis is postulated to result from viral and/or autoimmune etiologies. This disease has been reported to have a variable course. We report a case of sudden death from acute fulminant cerebellitis in a 13-year-old ballet dancer. Serial CT and MRI demonstrated rapid progression of the disease. Histopathologic correlation is provided. The etiologies, clinical course, therapeutic interventions and postmortem evaluation of this potentially life-threatening condition are briefly reviewed.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/60. Mountain medical mystery. Unwitnessed death of a healthy young man, caused by lightning.A healthy 20-year-old man failed to return home after a jog in the colorado mountains. His lifeless body was found the next day on an exposed mountain slope. The differential diagnosis in such mysterious, unwitnessed mountain deaths includes cardiac arrhythmia, cerebral hemorrhage, pulmonary embolism, seizures, trauma, high-altitude sickness, and hypothermia. The cause of death in this case was established on postmortem examination. The findings of ruptured tympanic membranes and a melted shoe established this as a case of lightning strike fatality. The National lightning Detection Network can be a valuable resource to investigators by providing information on the location and date of lightning strikes in the vicinity of the victim.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/60. cocaine-induced channelopathies: emerging evidence on the multiple mechanisms of sudden death.Sudden death due to cocaine in the absence of myocardial infarction has been attributed to the precipitation of life-threatening arrhythmias not unlike that due to antiarrhythmic drugs. cocaine is a slow on-off sodium blocker and a fast on-off potassium blocker. Effects on repolarization are biphasic: At low concentrations, cocaine delays ventricular recovery, whereas at higher levels, cocaine hastens it. Two distinct clinical profiles emerge from case reports of electrocardiographically documented life-threatening arrhythmias attributed to cocaine. The first is monomorphic slow ventricular tachycardia or idioventricular rhythm that occurs in overdose situations and appears to reflect excessive sodium channel block; it may respond to sodium bicarbonate. The second is torsade de pointes that occurs in recreational users who have underlying risks for this tachycardia (such as fully or partially expressed congenital long qt syndrome) and reflects potassium channel blockade. These clinical observations can be explained by recent findings regarding the electrophysiologic effects of cocaine. Other patterns of severe arrhythmias due to cocaine may yet emerge.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
9/60. Mechanisms of unexpected death in tuberous sclerosis.tuberous sclerosis complex is a protean autosomal dominant disorder characterized by multifocal tissue lesions arising from defects in cellular migration, proliferation, and differentiation. It has an association with sudden death. In the current study, review of all cases of sudden death due to tuberous sclerosis was undertaken at the Forensic science Centre in Adelaide, australia from 1991 to 2001, in addition to an analysis of cases from the literature. There were two local cases where unexpected death had occurred in individuals with known tuberous sclerosis, involving a 31-year-old male (epilepsy), and a 24-year-old female (massive hemorrhage into a renal angiomyolipoma). Fatal mechanisms in cases of tuberous sclerosis may be associated with underlying cardiovascular, renal and cerebral abnormalities. Sudden death may be due to cardiac arrhythmia, epilepsy, and intra-tumoral hemorrhage with additional complications including cardiac outflow obstruction, obstructive hydrocephalus, aneurysm rupture, and spontaneous pneumothorax. An awareness of the highly variable tissue manifestations of tuberous sclerosis and the mechanisms that may be responsible for death is necessary to establish correctly the diagnosis in occult cases (possibly with molecular confirmation), and to chart accurately organ changes in individuals with established disease.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/60. XVIII. Persistent fetal dispersion of the atrioventricular node and His bundle within the central fibrous body.In the hearts of two victims of sudden unexpected death (one age 20 years and the other 11 years) there was abnormally delayed persistence of the pattern of fetal dispersion of the A-V (atrioventricular) node and His bundle within the central fibrous body. This pattern is characterized by a splayed or excessively fragmented histological appearance of these structures viewed in cross-section. Some of the fronds of A-V nodal tissue formed loops connecting one portion of the node to another. Other fragments of nodal tissue appeared isolated within the central fibrous body, occasionally connecting directly to the crest of the interventricular septum. A number of the A-V nodal fragments were undergoing resorptive degeneration. Directly adjacent to the A-V node of the 11-year-old subject there was an island of cartilage within the central fibrous body. Since these anatomically separated fragments of A-V nodal tissue were so numerous and varied widely in size and thickness, in length, in histological organization, and in their apparent state of preservation probably extant during life, it is suggested that they form potential routes for abnormal conduction or impulse formation within the A-V junctional region and that some of these electrophysiological disturbances might be quickly lethal.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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