Nutrition Case study of Maple Syrup Urine Disease GB is the second born child of a Lebanese family. He has an older sister who is normal. He was born after an uncomplicated pregnancy and labor. His birth weight is 3.2kg. He seemed normal and had no immediate neonatal complications. He fed well and was alert. At 7 days of age, GB stopped sucking and became lethargic. He was readmitted to the hospital. He was afebrile but had 3 episodes of apnea. The mother noticed that there was a sweet odor of the urine. Blood test and urine AA chromatography on 24-h urine collection were done.   Normal plasma (mg/100mL) Plasma (mg/100ml) Patient urine (mg/24h) Leucine 1.5-3.0 57.49 Too high to read Isoleucine 0.8-1.5 14.10 41.1 Valine 2.0-3.0 14.76 77.28 Thus, GB was diagnosed with Maple Syrup Urine Disease. Thiamin responsiveness was evaluated: 510mg/kg/d of oral thiamin were prescribed for a period of 3 weeks. No improvement was noticed. GB was put from first day on BCAA-free diet. Part 1 1-Assess the patient. 2-Calculate energy, protein and BCAA needs. 3-Translate the prescription into diet with Similac and MSUD Analog (composition shown in Table below).

Surgical Tech For Surgical Tech Pos Care
5th Edition
ISBN:9781337648868
Author:Association
Publisher:Association
Chapter14: General Surgery
Section: Chapter Questions
Problem 1CS
icon
Related questions
Question
100%

Nutrition Case study of Maple Syrup Urine Disease

GB is the second born child of a Lebanese family. He has an older sister who is normal. He was born after an uncomplicated pregnancy and labor. His birth weight is 3.2kg. He seemed normal and had no immediate neonatal complications. He fed well and was alert. At 7 days of age, GB stopped sucking and became lethargic. He was readmitted to the hospital. He was afebrile but had 3 episodes of apnea. The mother noticed that there was a sweet odor of the urine. Blood test and urine AA chromatography on 24-h urine collection were done.

 

Normal plasma (mg/100mL)

Plasma

(mg/100ml)

Patient urine (mg/24h)

Leucine

1.5-3.0

57.49

Too high to read

Isoleucine

0.8-1.5

14.10

41.1

Valine

2.0-3.0

14.76

77.28

Thus, GB was diagnosed with Maple Syrup Urine Disease. Thiamin responsiveness was evaluated: 510mg/kg/d of oral thiamin were prescribed for a period of 3 weeks. No improvement was noticed. GB was put from first day on BCAA-free diet.

Part 1

1-Assess the patient.

2-Calculate energy, protein and BCAA needs.

3-Translate the prescription into diet with Similac and MSUD Analog (composition shown in Table below).

Expert Solution
steps

Step by step

Solved in 4 steps

Blurred answer
Similar questions
  • SEE MORE QUESTIONS
Recommended textbooks for you
Surgical Tech For Surgical Tech Pos Care
Surgical Tech For Surgical Tech Pos Care
Health & Nutrition
ISBN:
9781337648868
Author:
Association
Publisher:
Cengage