1/224. Infantile disseminated visceral giant cell arteritis presenting as sudden infant death.The rare clinicopathological entity 'disseminated visceral giant cell arteritis' (DVGCA) was first described in 1978. It is characterized by widespread small-vessel giant cell angitis and extravascular granulomas. A normal and healthy 7-month-old boy who presented unexpectedly with sudden infant death syndrome (SIDS) is reported. Histological examination at autopsy revealed giant cell angitis of the aorta, common carotid, coronary, pulmonary, celiac, mesenteric and common iliac arteries. There were also granulomas in the tracheal wall and liver. To our knowledge, this is the first documented case of DVGCA occurring in an infant younger than 12 months of age. A review of the literature on DVGCA is presented in this report, and the differential diagnosis is discussed.- - - - - - - - - - ranking = 1keywords = sudden, death (Clic here for more details about this article) |
2/224. Sudden death due to ventricular septal defect.Ventricular septal defects (VSD) are usually considered non-life-threatening, usually closing spontaneously or causing symptoms of congestive heart failure, which can be surgically treated in time to save the patient's life. Despite the usually benign clinical course of VSD, serious arrhythmias occur in 16-31% of patients. Sudden death accounted for one-third of all deaths in a series of medically managed patients and occurred in 4.2% of patients in a study of VSD and arrhythmias. Cardiac hypertrophy is the common denominator in all cases reported in detail of VSD-associated sudden death. We have encountered four cases of sudden unexpected death from VSD in infants ranging in age from 1 week to 3 and (1/2) months. In each case there was cardiomegaly and in one case there was pulmonary arteriolar medial thickening, with extension of smooth muscle into small intralobular vessels. In half of our cases the attending physician was sued for malpractice. We believe that VSD in infants and young children are potentially life-threatening malformations which warrant careful clinical follow-up.- - - - - - - - - - ranking = 0.86287574987291keywords = sudden, death, sudden death (Clic here for more details about this article) |
3/224. Multiple acyl-coenzyme a dehydrogenase deficiency: diagnosis by acyl-carnitine analysis of a 12-year-old newborn screening card.We report a family who experienced an unexplained neonatal death. Twelve years after the death, we retrospectively diagnosed multiple acyl-coenzyme a dehydrogenase deficiency by demonstrating an abnormal acyl-carnitine profile in the child's archived newborn screening card, using tandem mass spectrometry.- - - - - - - - - - ranking = 0.15158744579024keywords = death (Clic here for more details about this article) |
4/224. Inborn defects of fatty acid oxidation: a preventable cause of SIDS.Inborn errors of fatty acid oxidation, including medium chain acyl CoA dehydrogenase (MCAD) deficiency are readily detectable and treatable metabolic disorders in which recognition of symptoms is important. Symptoms occur when there is fasting, often associated with illness. If not diagnosed, these inborn errors of metabolism can result in sudden death classified as SIDS. These disorders can be diagnosed by ordering plasma or blood spot acylcarnitine profiles.- - - - - - - - - - ranking = 0.20811341250219keywords = sudden, death, sudden death (Clic here for more details about this article) |
5/224. Histiocytoid cardiomyopathy: three new cases and a review of the literature.Histiocytoid cardiomyopathy (HC), a rare arrhythmogenic disorder, presents as difficult-to-control arrhythmias or sudden death in infants and children, particularly girls. Three cases are described with autopsy findings. In two cases, yellow-tan nodules were grossly visible in the myocardium; in the third case, no gross lesions were identified. Microscopic examination in all three cases revealed multiple, scattered clusters of histiocytoid myocytes which on ultrastructural examination were filled with abnormal mitochondria, scattered lipid droplets, and scanty myofibrils. These pathologic findings are similar to those previously described. The pathogenesis of this entity remains controversial. It was recently proposed that this disorder is X-linked dominant with the associated gene located in the region of Xp22.- - - - - - - - - - ranking = 0.20811341250219keywords = sudden, death, sudden death (Clic here for more details about this article) |
6/224. myocarditis due to salmonella virchow and sudden infant death.A strain of salmonella virchow was isolated in the myocardium of a 1 1/2 month child who died suddenly. The source of contamination was the water of a family aquarium containing turtles.- - - - - - - - - - ranking = 0.92420627710488keywords = sudden, death (Clic here for more details about this article) |
7/224. Mitochondrial dna point mutations detected in four cases of sudden infant death syndrome.The aim of this study was to investigate the tRNA(Leu(UUR)) gene and the first part of the ND1 gene in mitochondrial dna (mtDNA) in cases of sudden infant death syndrome (SIDS). A total of 158 cases of SIDS and 97 controls were included in the study, and the base pairs in the range 3230-3330 were investigated using polymerase chain reaction (PCR) and temporal temperature gradient electrophoresis (TTGE). If a band shift was detected by TTGE, the area investigated and the D-loop was sequenced. Three different point mutations (T3290C, T3308C and T3308G) were detected in four of the SIDS cases, while none of the controls were mutated. We also found a high D-loop substitution rate in these four cases. The findings indicate that mtDNA mutations may play a role in some cases of SIDS.- - - - - - - - - - ranking = 1keywords = sudden, death (Clic here for more details about this article) |
8/224. Familial neonatal SIDS revealing carnitine-acylcarnitine translocase deficiency.A patient with a severe phenotype of carnitine-acylcarnitine translocase deficiency (CATR)(McKusick 212138) is reported. Prior to birth, a defect in beta-oxidation was suspected because of neonatal death of six siblings. Dietary treatment during neonatal adaptation and the subsequent six months of life and a trial of carnitine supplementation are reported. The rapidity with which long chain fatty acid metabolites can accumulate and induce secondary carnitine deficiency within a few hours after birth in an infant with CATR is noteworthy. CONCLUSION: High rates of glucose suppressed neonatal lipolysis in this infant, but did not seem sufficient to avoid secondary carnitine deficiency as in severe forms of CATR. Therefore simultaneous use of insulin and glucose may be necessary to control neonatal lipolysis. carnitine supplementation and the possible adverse effects of MCT systematically administrated, should be further assessed in patients with CATR.- - - - - - - - - - ranking = 0.07579372289512keywords = death (Clic here for more details about this article) |
9/224. Sudden and unexpected infant death due to an hemangioendothelioma located in the medulla oblongata.Herein, we report the case of a 4-month-old male infant dying suddenly and unexpectedly. Post mortem examination was requested with clinical diagnosis of sudden infant death syndrome (SIDS). Histological examination showed instead the presence of an hemangioendothelioma located in the medulla oblongata. Sudden unexpected death is the commonest form of death among babies between 1 month and 1 year of age. Although the vast majority of these fatalities are related to SIDS, a very small percentage is due to primary neoplasm. Necroscopy studies of sudden infant death should always include an accurate histological examination of the medulla oblongata on serial sections but seldom do.- - - - - - - - - - ranking = 0.7789686144756keywords = sudden, death (Clic here for more details about this article) |
10/224. pathology of the heart and conduction system in a case of sudden death due to a cardiac fibroma in a 6-month-old child.A 6-month-old female infant considered to be in good health died suddenly and unexpectedly. Post-mortem examination was requested, with clinical diagnosis of sudden infant death syndrome. Gross examination revealed, however, the presence of a cardiac mass 4.5 X 4 x 3.5 cm in diameter. Histological examination of the heart confirmed the presence of a cardiac fibroma. In the present case, the sudden death could have been due to the left ventricular outflow obstruction, as much as to conductive disturbances caused by overstretching and compression of the atrioventricular node and of the bundle branches. Hemodynamic and conductive abnormalities are presumed to have provoked bradycardia degenerating into ventricular fibrillation and sudden death. Necroscopy studies of sudden death should always include histological examination of the cardiac conduction system but seldom do.- - - - - - - - - - ranking = 1.7832918726565keywords = sudden, death, sudden death, cardiac (Clic here for more details about this article) |
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