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Old 09-06-2014
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Arrow Practice Question #6

An 9-month-old boy is brought to the pediatrician by his parents because he has recently lost the ability to crawl or hold his toys. On examination the patient is tachypneic and breathing with considerable effort; the liver is palpable five fingerwidths below the right costal margin. X-ray of the chest reveals cardiomegaly. He has some difficulties with sitting upright. Despite a number of interventions, the childís symptoms continue to worsen until his death 2 weeks later.
On autopsy, it is likely that this patientís cells will contain an accumulation of
which of the following substances?

(A) Glucose
(B) Glycogen
(C) Oxaloacetate
(D) Pyruvate
(E) Urea
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Old 09-06-2014
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The answer is b
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Old 09-06-2014
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Seems like B glycogen storage disease " pompe's disease
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Old 09-06-2014
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Pompe's so glycogen. I hate biochem!
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Quote:
Originally Posted by MananaMD View Post
An 9-month-old boy is brought to the pediatrician by his parents because he has recently lost the ability to crawl or hold his toys. On examination the patient is tachypneic and breathing with considerable effort; the liver is palpable five fingerwidths below the right costal margin. X-ray of the chest reveals cardiomegaly. He has some difficulties with sitting upright. Despite a number of interventions, the child’s symptoms continue to worsen until his death 2 weeks later.
On autopsy, it is likely that this patient’s cells will contain an accumulation of
which of the following substances?

(A) Glucose
(B) Glycogen
(C) Oxaloacetate
(D) Pyruvate
(E) Urea
YES Guys.. GJ!

The correct answer is B.
This patient has Pompe’s disease, a glycogen storage disorder.
Pompe’s disease is an autosomal recessive disease that is characterized by a deficiency or defect in lysosomal α-1,4-glucosidase, that is necessary for the dissolution of the polymer linkages in glycogen. In its absence, glycogen accumulates to toxic levels in both the cytoplasm and lysosomes.

(A)Glucose is stored as glycogen in the cells and is also present in blood. However, hyperglycemia is not responsible for the symptoms observed in this patient.
(C)Oxaloacetate is the first intermediate in the Krebs cycle. It is regenerated with each turn of the cycle but is not present in excessive amounts in the cell.
(D)Pyruvate is a component of the cellular respiration pathway and an intermediate in gluconeogenesis. It is not stored in cells in any significant quantity.
(E)Disorders of the urea cycle leading to nitrogen accumulation in the body results in progressive lethargy and coma but generally do not cause the myopathy observed in this child.
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