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propionic acidemia
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propionic acidemia

pathology

Learn about this topic in these articles:

organic acidemias

  • Enzyme defects in urea cycle disorders.
    In metabolic disease: Organic acidemias

    Propionic acidemia is caused by a deficiency of the enzyme propionyl-CoA carboxylase, which results in an accumulation of propionic acid. Individuals with this disorder usually present with life-threatening illness early in infancy. Acidemia, dehydration, low white blood cell count, low muscle tone, and lethargy progressing…

    Read More
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favism
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favism

genetic disorder
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Article History

favism, a hereditary disorder involving an allergic-like reaction to the broad, or fava, bean (Vicia faba). Susceptible persons may develop a blood disorder (hemolytic anemia) by eating the beans, or even by walking through a field where the plants are in flower.

The known distribution of the disease is largely limited to people of Mediterranean origins (Spaniards, Italians, Greeks, Armenians, and Jews). Susceptibility to favism is inherited as a sex-linked trait and appears to be closely related to glucose-6-phosphate dehydrogenase deficiency (q.v.).

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