Hepatic failure

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  • i. Hepatic encephalopathy: only consistent finding is presence of Alzheimer type II astrocytes
    • 1. type II astrocytes have enlarged vesicular nucleus, marginated chromatin, and scanty cytoplasm with little or no demonstrable GFAP and occur in the deep layers of the cortex, caudate and putamen, globus pallidus, thalamus, and dentate nucleus
    • 2. EEG in hepatic encephalopathy is a characteristic triphasic wave
      • a. Also see triphasic wave in postanoxic encephalopathy along with diffuse burst suppression
    • ii. hepatolenticular degeneration (Wilson’s disease)
    • 1. autosomal recessive disorder caused by mutations in the copper transporting ATPase gene (thought to be involved in the import of copper into the cell) encoded on chromosome 13; Remember: think of Wilson and ‘lucky 13’
    • 2. presents in adolescence and can cause dystonia, spasms, tremor and dementia
    • 3. low serum ceruloplasmin concentration due to increased secretion of copper in the urine
    • 4. histologically putamen and caudate nucleus appear brown and shrunken with neuronal loss, scattered lipid and pigment laden macrophages and type II astrocytes (a.k.a. Alzheimer type II glia but can also have Alzheimer type I glia as well) with spongy degeneration of the cerebral cortex and white matter
      • a. a distinctive feature are Opalski cells in the globus pallidus (round cells with a small central nucleus and abundant, finely granular, esoinophlic cytoplasm)
      • b. Opalski cells may also be seen in non-Wilson’s disease hepatic encephalopathy
    • 5. treatment: D-penicillamine and zinc
      • a. D-penicillamine, polymyxin, and aminoglycosides can lead to a neuromuscular blockade that resembles myasthenia gravis and a patient with myasthenia gravis may have their symptoms aggravated by those agents; steroid treatment may exacerbate the weakness caused by these agents
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