Failure To Thrive, Hepatomegaly, and Seizures! - USMLE Forums
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Old 07-22-2012
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Question Failure To Thrive, Hepatomegaly, and Seizures!

An 18-month-old boy who recently moved to the area with his family is brought to the clinic by his mother because of a 1-month history of night-time seizures. The boy is below the fifth percentile for weight and height and below the tenth percentile for head circumference. His mother says that he always seems hungry and she feeds him frequently. Examination shows a protuberant abdomen with thin extremities. His liver is palpated to be enlarged. Which of the following is the most appropriate part of this patientís management?

a) Glucose feeding throughout the night
b) Iron addition once in the morning and at nighttime
c) Medium chain fatty acid addition in the afternoon
d) Tyrosine supplementation before each meal
e) Vitamin B12 injection each morning

Please give the diagnosis
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Old 07-22-2012
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A.....is it type 1 glycogen storage disease??????
hypoglycemia-->seizures, hepatomegaly, growth retardation.....

another closer diagnosis would be MCAD def but i doubt its clinical spectrum include hepatomegaly.....
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Old 07-22-2012
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Ya seems Von- gierke syndrome.
Hepatomegaly
Severe hypoglycemia- if don't feed him for whole day and may be hypoglycemia is cause of mid night seizures.
Go management would be feeding him in night.


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Old 07-22-2012
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I thought it is kwashiorkor but no option for that. Perfectly fits Kwashiorkor though i think
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Old 07-22-2012
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I thought it is kwashiorkor but no option for that. Perfectly fits Kwashiorkor though i think
Ya it seems kwashiorkor too but night seizures can't be defined by kwashiorkor . But still I am not sure about mine answer.
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Old 07-23-2012
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the signs,,, seen Kwashiorkor a kind of severe malnutrition, adding more calories and protein may correct it, so fatty calorics source coulde be medium chain fatty acid
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Old 07-23-2012
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Fasting type of hypoglycemia caused ketotic hypoglycemia in childhood
most common cause of hypoglycemia from 18 months to mid -childhood
multiple etiologies
maple syrup urine disease,galactosemia ,hereditary fructose intolerance,von gierke's glycogen storage disease,carnitine deficiency
diagnosis
prolonged fast
must satisfy whipple's triad:
Symptoms occur
hypoglycemia is demonstrated
symptoms are relieved by glucose
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Old 07-23-2012
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Quote:
Originally Posted by Casandra View Post
An 18-month-old boy who recently moved to the area with his family is brought to the clinic by his mother because of a 1-month history of night-time seizures. The boy is below the fifth percentile for weight and height and below the tenth percentile for head circumference. His mother says that he always seems hungry and she feeds him frequently. Examination shows a protuberant abdomen with thin extremities. His liver is palpated to be enlarged. Which of the following is the most appropriate part of this patientís management?

a) Glucose feeding throughout the night
b) Iron addition once in the morning and at nighttime
c) Medium chain fatty acid addition in the afternoon
d) Tyrosine supplementation before each meal
e) Vitamin B12 injection each morning

Please give the diagnosis
I think is von-gierke -defien. glu-6phosp. will be A
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Old 07-27-2012
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Default correct answer :)

Correct answer is a) Glucose feeding throughout the night

The boy has Type I glycogen storage disease (von Gierke disease). This is caused by a deficiency of the enzyme glucose-6-phosphatase, which prevents the liver from producing free glucose from glycogen and from gluconeogenesis. The result is severe hypoglycemia during fasting periods. The glycogen accumulates in the liver and kidneys, enlargening both organs. Sleep is a fasting state, and severe hypoglycemia during this time can cause seizures. By providing glucose during the night, glucose levels will not drop to such low levels. A deficient glukose-6-phosphatase causes the accumulation of glucose-6-phosphate, which prevents the conversion of lactate to pyruvate, resulting in elevated lactate.
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