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1/30. Haemorrhagic shock and encephalopathy syndrome: report of two cases with special reference to hypoglycaemia.

    Haemorrhagic shock and encephalopathy syndrome (HSES) is a devastating disorder affecting infants. So far no cases have been reported in switzerland. It is characterised by the abrupt onset of hyperpyrexia, shock, encephalopathy, diarrhoea, disseminated intravascular coagulation (DIC) and renal and hepatic failure in previously healthy infants. Severe hypoglycaemia has been repeatedly reported in association with HSES. However, the pathophysiology of the hypoglycaemia is not clear. We report on two infants (2 and 7 months old) with typical HSES, both of whom were presented with nonketotic hypoglycaemia. In the first case, plasma insulin was 23 pmol/l at the time of hypoglycaemia (0.1 mmol/l). In the second case, increased values for interleukin-6 (IL-6) (319 pg/ml) and IL-8 (1382 pg/ml) were found 24 hours after admission, whereas IL-1 and tumour necrosis factor-alpha (TNF-alpha) were not measurable. Alpha-1-antitrypsin was decreased (0.6 g/l). In hyperpyrexic, unconscious and shocked infants, HSES should be considered and hypoglycaemia should be specifically looked for. Hypoglycaemia is not caused by hyperinsulinism but may be secondary to the release of cytokines.
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2/30. Influenza virus-induced encephalopathy: clinicopathologic study of an autopsied case.

    BACKGROUND: Rapid progressive encephalopathy with a high fever, consciousness loss and recurrent convulsions has been occasionally reported in children during influenza pandemics in japan since 1995. We examined a 2-year old girl with hemorrhagic shock and encephalopathy syndrome associated with acute influenza a virus infection (A/Nagasaki/76/98; H3N2), to answer several questions for which no histologic or virologic data exist. methods: A clinicopathologic study using immunohistochemical staining and viral genome detection by reverse transcriptase polymerase chain reaction (RT-PCR) was performed with this autopsied case. RESULTS: The virus antigen was positive in CD8 T lymphocytes from the lung and spleen. The virus infected a very limited part of the brain, especially Purkinje cells in the cerebellum and many neurons in the pons, without inducing an overt immunologic reaction from the host. The RT-PCR used for detecting the hemagglutinin gene demonstrated positive bands in all frozen tissues and cerebrospinal fluid taken at autopsy and not in samples obtained on admission. CONCLUSIONS: The pathologic change induced by the direct viral invasion cannot be responsible for all of the symptoms, especially for the rapid and severe clinical course of the disease within 24-48 h after the initial respiratory symptoms. Together with the rapid production of several inflammatory cytokines, the breakdown of the blood-brain barrier may induce severe brain edema and can be a major pathologic change for the disease. Any therapeutic strategy to control this multistep progression of the disease could be effective.
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keywords = shock
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3/30. Hemorrhagic shock and encephalopathy syndrome: neurologic features.

    Hemorrhagic shock and encephalopathy syndrome (HSES) is a severe disease that affects previously healthy infants of less than 1 year of age and is associated with significant mortality and neurologic morbidity. It is characterized by sudden onset of shock, convulsions and coma, bleeding due to severe coagulopathy, fever, diarrhea, metabolic acidosis, and hepatorenal dysfunction. central nervous system involvement with recurrent seizures and brain edema is the most common cause of high mortality and neurological morbidity. In this report, we describe four patients of HSES and review the initial and follow-up neurological features, electroencephalography findings, and the results of neuroradiological examinations of this catastrophic illness.
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ranking = 0.85714285714286
keywords = shock
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4/30. Aspirating subdural effusions, so called brain stem shock.

    A shift of blood into the head during negative pressure aspiration of subdural haematomas in an infant has been demonstrated, and also that aspiration may restart bleeding. An estimate of the elasticity of a 47 cm circumference skull has been obtained. It is suggested that the observed changes in distribution of blood are sufficient to explain the occasional deaths of infants after aspiration of subdural haematomas and that so-called "brain stem shock" need not be invoked.
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ranking = 0.71428571428571
keywords = shock
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5/30. Abnormal magnetic resonance images in hemorrhagic shock and encephalopathy syndrome.

    Hemorrhagic shock and encephalopathy syndrome is an acute childhood illness that involves the rapid onset of multisystem failure, including central nervous system, renal, cardiovascular, hepatic, and hematologic dysfunction, and often leads to death or serious neurologic damage. We report the first case of a child with hemorrhagic shock and encephalopathy in which magnetic resonance imaging was used to define the cortical hemorrhagic involvement.
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6/30. Complete recovery from hemorrhagic shock and encephalopathy.

    We describe three infants (aged 9 weeks to 4 months) with the classic features of hemorrhagic shock and encephalopathy syndrome, including sudden onset of shock, neurologic disturbance, bleeding, disseminated intravascular coagulation, and impaired hepatic and renal function. Unlike the cases previously described, all three children recovered rapidly without evidence of long-term neurologic damage. These findings may modify the perception that this disorder has a uniformly bad outcome.
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ranking = 0.85714285714286
keywords = shock
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7/30. Brain damage after heat stroke.

    Cerebellar syndromes and radiologic cerebellar atrophy after hyperpyrexia have occasionally been reported, mostly in neuroleptic malignant syndromes, but neuropathologic studies are extremely rare. We studied 3 patients (a 74-year-old woman, a 63-year-old man, and an 80-year-old man) who had heat stroke during heat waves in france. One patient had generalized seizures and died 28 hours after admission. The other patients survived one month and 2 months after admission; both had palatal myoclonus, and in one case, magnetic resonance imaging showed high signal intensity in the cerebral peduncles. The main neuropathology in the 3 cases was severe diffuse loss of purkinje cells associated with heat shock protein 70 expression by Bergmann glia. In situ end labeling was negative in surviving purkinje cells, suggesting that the mechanism of neuronal death was not apoptosis. Degeneration of purkinje cells axons resulted in myelin pallor of the white matter of the folia and of the hilum of the dentate nuclei. dna internucleosomal breakages were identified by in situ end labeling in the dentate nuclei and centromedian nuclei of the thalamus and were associated with degeneration of the cerebellar efferent pathways: superior cerebellar peduncles, decussation of the superior cerebellar peduncles (Wernekinck commissure), and dentatothalamic tract. These findings suggest that the mechanisms of neuronal death in the dentate nuclei and centromedian nuclei of the thalamus was different from that in purkinje cells and more likely resulted from deafferentation. Ammon's horn and other areas susceptible to hypoxia were spared. These observations confirm the selective vulnerability of purkinje cells to heat-induced injury and involvement of the cerebellar efferent pathways in palatal myoclonus.
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keywords = shock
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8/30. Haemorrhagic shock encephalopathy and sudden infant death.

    In 2 pairs of non-identical twins, haemorrhagic-shock encephalopathy syndrome developed in 1 co-twin while the other died of sudden infant death syndrome. The twin pairs were aged 3 and 4 months, respectively, and no cause was identified. We suggest that stress protein deficiency may underlie both syndromes.
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ranking = 0.71428571428571
keywords = shock
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9/30. Lactic acidosis in childhood.

    Children with chronic metabolic acidosis should be investigated to determine the presence of an organic acid, especially when the plasma electrolyte profile shows a deficiency of anion. One of the organic acids that should be looked for in such a patient is lactic acid. Lactic acidosis due to tissue hypoxia is a well-known phenomenon (e.g., in shock and cardiopulmonary disease) and has not been discussed in this essay; nor has lactic acidosis due to exogenous causes like infusion of fructose or sorbitol, or admiministration of phenformin. Chronic lactic acidosis in infancy is a rare condition. It may be associated with glycogen storage disease Type 1, fructose diphosphatase deficiency, methylmalonic acidemia, propionic acidemia, pyruvate carboxylase or dehydrogenase deficiency and Leigh's subacute necrotizing encephalomyelopathy (SNE). Some patients with chronic lactic acidosis do not have nay of these diseases and comprise an "idiopathic" group. This is a heterogeneous group, probably having several different causes for the metabolic error. In Leigh's SNE, a metabolic block in the formation of thiamine triphosphate in brain has been demonstrated and has been attributed to the presence of an inhibitor of thiamine pyrophosphate-adenosine triphosphate (TPP-ATP) phosphoryl transferase in body fluids. The inhibitor has also been encountered in cases of intermittent cerebellar ataxia and of primary hypoventilation (Ondine's curse), which may represent variants of Leigh's disease. Increased blood levels of lactate, pyruvate and alanine frequently are encountered in SNE, but it still is not clear whether they are due to a primary or secondary disturbance in the catabolism of pyruvate. Disturbed lactate and pyruvate metabolism has also been encountered in isolated cases of mental retardation and growth failure, in mitochondrial myopathies and in polyneuropathies, and may be expected to occur in Wernicke's encephalopathy. Finally, it has been noted in malignancy and in association with other rare metabolic disorders.
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keywords = shock
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10/30. Postpartum pulmonary embolus as an unusual cause of cortical blindness.

    A pregnant patient was delivered by cesarean section with blindness occurring in the postoperative period. Diagnostic evaluation revealed the presence of massive pulmonary embolus associated with cardiogenic shock. The differential diagnosis of blindness during pregnancy is discussed.
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keywords = shock
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