metabolic diseases of the liver l.
Download
Skip this Video
Loading SlideShow in 5 Seconds..
Metabolic diseases of the liver PowerPoint Presentation
Download Presentation
Metabolic diseases of the liver

Loading in 2 Seconds...

play fullscreen
1 / 13

Metabolic diseases of the liver - PowerPoint PPT Presentation


  • 212 Views
  • Uploaded on

Metabolic diseases of the liver. Central role in metabolism Causes and mechanisms of dysfunction Clinical patterns of metabolic disease Clinical approach to problem-solving Specific disorders . Liver’s central role in metabolism.

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'Metabolic diseases of the liver' - hisa


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
metabolic diseases of the liver
Metabolic diseases of the liver
  • Central role in metabolism
  • Causes and mechanisms of dysfunction
  • Clinical patterns of metabolic disease
  • Clinical approach to problem-solving
  • Specific disorders
liver s central role in metabolism
Liver’s central role in metabolism

Pathways involve CHO, protein, lipid, trace element and vitamin metabolism

  • Synthesis

e.g. serum proteins

  • Detoxification/degradation

e.g. ammonia

  • Regulation

e..g. blood glucose

metabolic function of the liver
Metabolic function of the liver

Reactions disturbed by

  • Congenital deficiency of enzymes
  • Nutritional deficiency/excess of substrate
  • Toxic/chemical damage to organelles
  • Hypoxic/ischaemic insult
  • Secondary to metabolic effects of disease
metabolic disorders
Metabolic disorders
  • of amino acid metabolism
  • of bile acid synthesis and metabolism
  • of carbohydrate metabolism
  • of bile flow and excretion
  • of mitochondrial function
  • of peroxisomal function
  • of copper metabolism
metabolic dysfunction inherited disease
Metabolic dysfunction : inherited disease
  • Essential product deficit

e.g. G-6-P deficiency

(Glycogen storage I)

  • Precursor accumulation

e.g. OTC deficiency

(Hyperammonaemia)

  • Alternative pathway activation

e.g. Aminoacidopathy

  • Combinations of above
mitochondrial damage
Mitochondrial damage
  • Inhibition of beta oxygenation of fatty acids

leads to microvesicular steatosis

  • Interference with oxidative phosphorylation

leads to insufficient ATP generation

  • Impairment of the respiratory chain

leads to excess ROS with lipid peroxidation

  • Increase in permeability transition

leads to cell death (apoptosis)

mitochondrial dysfunction
Mitochondrial dysfunction
  • Inborn enzyme deficiencies involving:

fatty acid oxygenation

organic acids

lactate metabolism

oxidative phosphorylation

urea cycle

Episodic decompensation precipitated by intercurrent stress

toxic damage to liver
Toxic damage to liver
  • Mitochondrial damage

Drugs (antivirals, salicylate, valproate, tetracycline)

Toxins (hypoglycin, atractyloside)

  • Endothelial damage to hepatic veins

Drugs (cytotoxic drugs)

Toxins (Senecio, aflatoxin, pyrrolizidine)

  • Glutathione depletion and cell death

Drugs (paracetamol)

Hypoxic ischaemia

mechanisms of centrilobular necrosis
Mechanisms of centrilobular necrosis
  • Sepsis
  • Shock induced ischaemia
  • Congestive heart failure
  • Toxicity from drugs and poisons

made worse by

Malnutrition

Infection

Fasting

Exercise

pathologic manifestations of metabolic disease of the liver
Pathologic manifestations of metabolic disease of the liver
  • No structural abnormalities evident but severe functional disturbance
  • Hepatocyte injury leading to apoptosis, necrosis, cirrhosis or tumours
  • Storage of lipid, glycogen or other products manifesting as hepatomegaly
clinical patterns of metabolic disease involving the liver
Clinical patterns of metabolic disease involving the liver
  • Newborn acute metabolic crisis

mimics sepsis

  • Severe vomiting and failure to thrive
  • Recurrent episodes of vomiting and encephalopathy with acidosis
  • Progressive retardation or seizures with hepatomegaly
  • Hepatomegaly with/without jaundice and failure to thrive/grow normally