In a different instance of two infants suspected to have a defi- ciency of the PDH complex because of severe lactic acidosis, tissue biop- sies could not be taken to measure URINARY EXCRETION OF PYRUVIC AND a-KETOGLUTARIC Acids Pyruvic Acid (umol/mg of creatinine) a-Ketoglutaric Acid (µmol/mg of creatinine) Age Special Diet or Drugs Patient G. M. 5 wk enzyme activities. Instead it was no- ticed that there were high concentra- tions of organic acids in the urine. Typical results are tabulated in the ta- ble on the right. Identify the enzyme defect and explain why it presents this metabolic pattern. What other meta- bolic breakdown products likely were also detected especially following a high protein diet but are not listed? Name the defective enzyme. 2.26 3.57 7½ wk 2.10 3.39 11 wk 1.35 4.63 12 wk After 18-hr fast 1.87 0.44 High-protein, low-carbohy- drate diet 15 wk 1.62 5.38 Patient D. M. 1 day 13 days 0.87 0.44 Thiamine (0.45 3.44 24 days 0.33 2.47 4 wk 0.53 1.23 6 wk Thiamine 0.42 1.39 12 wk Thiamine 0.32 0 80
In a different instance of two infants suspected to have a defi- ciency of the PDH complex because of severe lactic acidosis, tissue biop- sies could not be taken to measure URINARY EXCRETION OF PYRUVIC AND a-KETOGLUTARIC Acids Pyruvic Acid (umol/mg of creatinine) a-Ketoglutaric Acid (µmol/mg of creatinine) Age Special Diet or Drugs Patient G. M. 5 wk enzyme activities. Instead it was no- ticed that there were high concentra- tions of organic acids in the urine. Typical results are tabulated in the ta- ble on the right. Identify the enzyme defect and explain why it presents this metabolic pattern. What other meta- bolic breakdown products likely were also detected especially following a high protein diet but are not listed? Name the defective enzyme. 2.26 3.57 7½ wk 2.10 3.39 11 wk 1.35 4.63 12 wk After 18-hr fast 1.87 0.44 High-protein, low-carbohy- drate diet 15 wk 1.62 5.38 Patient D. M. 1 day 13 days 0.87 0.44 Thiamine (0.45 3.44 24 days 0.33 2.47 4 wk 0.53 1.23 6 wk Thiamine 0.42 1.39 12 wk Thiamine 0.32 0 80
Chapter11: Endocrine System
Section: Chapter Questions
Problem 1CS
Related questions
Question
Transcribed Image Text:In a different instance of
two infants suspected to have a defi-
ciency of the PDH complex because
of severe lactic acidosis, tissue biop-
sies could not be taken to measure
URINARY EXCRETION OF PYRUVIC AND a-KETOGLUTARIC ACIDS
Pyruvic Acid
(итol/ mg of
creatinine)
a-Ketoglutaric
Acid (µmol/mg
of creatinine)
Age
Special Diet or Drugs
Patient G. M.
5 wk
2.26
3.57
enzyme activities. Instead it was no-
ticed that there were high concentra-
tions of organic acids in the urine.
Typical results are tabulated in the ta-
ble on the right. Identify the enzyme
defect and explain why it presents this
metabolic pattern. What other meta-
bolic breakdown products likely were
also detected especially following a
high protein diet but are not listed?
Name the defective enzyme.
7½ wk
2.10
3.39
11 wk
1.35
4.63
12 wk
After 18-hr fast
1.87
0.44
High-protein, low-carbohy-
drate diet
15 wk
1.62
5.38
Patient D. M.
1 day
13 days
0.87
0.44
Thiamine
0.45
3.44
24 days
0.33
2.47
4 wk
0.53
1.23
6 wk
Thiamine
0.42
1.39
12 wk
Thiamine
0.32
0.80
13 wk
Thiamine & lipoate
2.05
3.12
15 wk
Thiamine, lipoate, riboflavin
1.13
0.97
7 mo
0.31
1.64
14 mo
2.32
0.13
Normal
Adults°
<0.04
<0.15
Values for infants are probably not significantly different.
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