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In this article, we analyze myopathies with cores, for which an association to malignant hyperthermia (MH) has been suggested. We discuss the clinical features, the underlying genetic defects, subsequent effects on cellular calcium metabolism, and in vitro muscle responses to MH triggers. We describe in detail central core disease, multiminicore disease,(More)
BACKGROUND Malignant hyperthermia (MH) is a potentially lethal disease triggered by volatile anaesthetics and succinylcholine in genetically predisposed individuals. Because of the heterogenetic nature of MH, a simple genetic-based diagnostic test is not feasible and diagnosis requires an invasive open muscle biopsy followed by the in vitro contracture test(More)
In many cell types agonist-receptor activation leads to a rapid and transient release of Ca(2+) from intracellular stores via activation of inositol 1,4,5 trisphosphate (InsP(3)) receptors (InsP(3)Rs). Stimulated cells activate store- or receptor-operated calcium channels localized in the plasma membrane, allowing entry of extracellular calcium into the(More)
n many cell types agonist-receptor activation leads to a rapid and transient release of Ca 2 ϩ from intracellular stores via activation of inositol 1,4,5 trisphosphate (InsP 3) receptors (InsP 3 Rs). Stimulated cells activate store-or receptor-operated calcium channels localized in the plasma membrane, allowing entry of extracellular calcium into the(More)
Incident Reporting Systems (IRS) are and will continue to be an important influence on improving patient safety. They can provide valuable insights into how and why patients can be harmed at the organizational level. However, they are not the panacea that many believe them to be. They have several limitations that should be considered. Most of these(More)
Plant architecture highly constrains pest infestation but is rarely considered in studies on plant–insect interactions. We analysed the relationships between apple tree architectural traits manipulated by tree training and within-branch development of Dysaphis plantaginea (rosy apple aphid, RAA), a major apple pest, during its multiplication wingless phase(More)
BACKGROUND Malignant hyperthermia (MH) is a rare pharmacogenetic disorder which is characterized by life-threatening metabolic crises during general anesthesia. Classical triggering substances are volatile anesthetics and succinylcholine (SCh). The molecular basis of MH is excessive release of Ca2+ in skeletal muscle principally by a mutated ryanodine(More)
This case is about a 48-year-old woman known with a reduced butyrylcholinesterase activity, who developed prolonged neuromuscular blockade following the unintentional administration of succinylcholine. We took the opportunity to monitor the development of neuromuscular function during this period and blood samples were taken for molecular genetic analysis(More)
BACKGROUND The epidural route is still considered the gold standard for labour analgesia, although it is not without serious consequences when incorrect placement goes unrecognized, e.g. in case of intravascular, intrathecal and subdural placements. Until now there has not been a viable alternative to epidural analgesia especially in view of the neonatal(More)
Many biotic and abiotic parameters affect the metabolites involved in the organoleptic and health value of fruits. It is therefore important to understand how the growers' decisions for cultivar and orchard management can affect the fruit composition. Practices, cultivars and/or year all might participate to determine fruit composition. To hierarchize these(More)