Immunology question

Exercise 3

The data in the table below represents part of the laboratory analysis for a five day old female child rushed into hospital unconscious.

Table 2.Laboratory analysis.

Leucine

Isoleucine

Valine

alpha-ketoisocaproate

Analyte	Patient Value Save Time On Research and Writing Hire a Pro to Write You a 100% Plagiarism-Free Paper. Get My Paper	Reference Values
Blood
	Leucine	2470 mol/L	40-158
	Isoleucine	850 mol/L	13-81
Allo-Isoleucine	127 mol/L	<5
	Valine	970 mol/L	85-334
Glutamate	72 mol/L	11-79
Glutamine	806 mol/L	475-746
Phenylalanine	60 mol/L	34-101
	alpha-ketoisocaproate	876 mol/L	24-44
Urine
1559 mol/g creatinine	78-195
463 mol/g creatinine	125-390
415 mol/g creatinine	113-369
287 mol/g creatinine	<15

Questions (exercise 3):

a) What condition is the child suffering from? Explain your ‘diagnosis’ in terms of
each
of the data in the table.

(5 marks)

i) What is the underlying [biochemical] cause of the condition the patient is suffering from? Include details of any relevant enzymatic reactions/pathways.

(3 marks)

j) What‘unusual’ metabolite, not listed above, would you also expect to be found in the urine of the child? Explain how this metabolite might be related to the diagnosed disorder.

(3 marks)

k) Once stabilised the child was maintained on dietary therapy. Outline what form the dietary therapy would take, including any ‘supplements’ that may be added to the basic diet as required. [please note the patients age]

(4 marks)