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| Topic: |
Science
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| Number of pages / Number of words: |
3 / 752 |
Essay's paper body
Even in persons with relatively high baseline residual BCKAD activity, episodes of metabolic intoxication can be fatal.
3. Biochemical signs should always be interpreted in the context of dietary leucine tolerance and prevailing clinical circumstances. Dietary leucine tolerance (in mg/kg/day) is defined as the steady-state leucine intake associated with normal growth and plasma leucine within the normal range...
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The authors do not rely on tissue measurements of decarboxylation activity, but classify affected individuals based on their leucine tolerance and metabolic response to illness. Decarboxylation data is from Chuang & Shih ( 2001).
Classic maple syrup urine disease phenotype. Maple syrup odor is evident in cerumen soon after birth and in urine by five to seven days of age...
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General points of the essay
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