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free120/Block 3/Question#21 (reveal difficulty score)
A 6-day-old breast-fed boy is brought to the ...
Galactose 1-phosphate ๐Ÿ” / ๐Ÿ“บ / ๐ŸŒณ / ๐Ÿ“–
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submitted by โˆ—bwdc(697)
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What we have here is a congenital intolerance to breast milk: galactosemia, in which the body cannot convert galactose to glucose (resulting in an accumulation of Galactose 1-phosphate). They then list the findings and tests used to diagnose it. Lactose (the disaccharide in milk) is composed of glucose+galactose.

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okokok1  also you can cross out the other answers because: what builds up in essential fructosuria: fructose what builds up in Hereditary fructose intolerance: Fructose-1-Phosphate what builds up in galactokinase def.: galactose what builds up in classic galactosemia: Galactose-1-P. Hence, this is the only correct choice of the answers the stem gave. +1



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submitted by โˆ—nwinkelmann(366)
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If you didn't understand the tests in the stem, this should help:

Urine reducing substance test: https://study.com/academy/lesson/reducing-vs-non-reducing-sugars-definition-comparison.html

Glucose oxidase test: catalyzes the oxidation of glucose to hydrogen and D-glucono-delta-lactone. If there is no glucose present (i.e. with galactosemia), the test will be negative because there is nothing for the test to catalyze.

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drpee  TLD-Watch? Reducing sugars include galactose, lactose, and fructose. +8
chaosawaits  drpee be the real MVP +
chaosawaits  All monosaccharides are reducing sugars. Lactose and maltose are common dietary reducing polysaccharides. Sucrose is not a reducing sugar. +



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submitted by meli(0)
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UWORLD 1067: Fructose, glucose, and galactose are reducing sugars and can be detected by a copper reduction test, which nonspecifically detects the presence of reducing sugar. A urine dipstick, uses glucose oxidase to ascertain the presence of urinary glucose and will not test positive in the presence of fructose or galactose.

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