The mitochondrial trifunctional protein (TFP) is a multienzyme complex of the fatty acid beta-oxidation cycle. It is composed of four alpha-subunits (HADHA) harboring long-chain enoyl-CoA hydratase and long-chain L-3-hydroxyacyl-CoA dehydrogenase (LCHAD) and four beta-subunits (HADHB) harboring long-chain 3-ketoacyl-CoA thiolase (LKAT). Mutations in either subunit can result in TFP deficiency w...

Opening of a high-conductance pore in the mitochondrial inner membrane induces onset of the mitochondrial permeability transition (mPT). Cyclosporin A and trifluoperazine inhibit this pore and block necrotic cell death in oxidative stress, Ca2+ ionophore toxicity, Reye-related drug toxicity, pH-dependent ischaemia/reperfusion injury and other models of cell injury. Confocal fluorescence microsc...

BACKGROUND Many inborn errors of metabolism (IEMs) may present as sudden infant death (SID). Nowadays, increasing numbers of patients with IEMs are identified pre-symptomatically by population neonatal bloodspot screening (NBS) programmes. However, some patients escape early detection because their symptoms and signs start before NBS test results become available, they even die even before the ...

Reye's syndrome is most frequently seen in children but has also been described in adults. This syndrome is usually associated with ingestion of 5-aminosalicylates (ASA) or infection with influenza A, influenza B, or varicella virus. A case of Reye's syndrome in a 47 year old, previously healthy woman precipitated by ingestion of ASA and acute hepatitis A virus infection is described. Reye's sy...

Evidence of circulating endotoxin was sought in children with Reye's syndrome, on the thesis that severe hepatic failure is likely to result in loss of capacity to detoxify intestinal endotoxins entering the circulation. A modification of the Limulus assay was used to demonstrate high levels of endotoxin-like activity (E.L.A.) in nine comatose patients with Reye's syndrome and in one of the two...

limitations, risks and complications, and instructions for monitoring devices. Part 5 consists of a chapter on the care of the technologically dependent child. One disadvantage of this partitioning is that aspects of a particular system are not always covered in the expected section; e.g., cerebral death is covered in Part 1 and therefore is not located in the "neurologic" section of Part 3. Th...

Intelligent Tutoring Systems (ITS) represent a substantial portion of knowledge from human tutors, therefore they are able to provide a better learning guidance than Computer Assisted Instructions (CAI). One of the most important goals of an ITS is to provide feedback tailored to the learner unique needs. There are several educational activities that are supported by these systems such as: prob...

REFERENCES [1] Wu, J. Theory and Application of Partial Functional Differential Equations. Springer Verlag, New York, 1996. [2] Martín, J.A., Rodríguez, F., Company, R. Analytical solution of mixed problems for the generalized diffusion equation with delay. Mathematical and Computer Modelling, 40 (2004), 361--369. [3] Reyes, E., Rodríguez, F., Martín, J.A. Cálculo eficiente de soluciones numéri...

Three children with Reye's syndrome are described. One child died, the second had mild and transient illness, and the third had recurrent episodes. In all 3 children a muscle biopsy showed pronounced infiltration of the myofibres with fat microdroplets as shown by the oil red 0 stain and by electron microscopical examination. We suggest that needle biopsy of muscle may be a quick and safe aid t...

Schultz et al (2008) raised the question whether regression into autism is triggered, not by the measles-mumps-rubella (MMR) vaccine, but by acetaminophen (Tylenol) given for its fever and pain. Considerable evidence supports this contention, most notably the exponential rise in the incidence of autism since 1980, when acetaminophen began to replace aspirin for infants and young children. The i...