Conditions

Question 1

Congenital heart disease cyanotic

Answer 1

oxygen rich blood mixes with oxygen poor blood
hypoxia is the main concern
malnutrition is a concern

Question 2

Congenital heart disease acyanotic

Answer 2

shunting of blood occurs from the left side of the heart to the right side - CHF is a concern

Question 3

Congenital heart disease pre op

Answer 3

PN initiated early and advanced to full calorie and protein goals until enteral feeding can be achieved
EN as long as infant is hemodynamically stable- human milk preferred option but when unavailable standard infant formula is reasonable

Question 4

congenital heart disease post op

Answer 4

as son as gut function return - transition to EN
feeding eval should be done due to high risk of vocal cord injury
oral intake is the goal

Question 5

protein losing enteropathy

Answer 5

abnormal loss of protein from the digestive tract or inability of digestive tract to absorb protein
most prominent after fontan procedure to treat CHD

Question 6

chylothorax

Answer 6

complication of pediatric cardiac surgery
accumulation of chyle in the pleural space
challenge is maintaining fluids and electrolytes while minimizing the lymphatic leak
low fat diets with MCT

Question 7

heart transplant

Answer 7

correct malnutrition before transplant
post transplant- limit sweets and foods high in cholesterol, fat, and salt
supplement vit d and Ca (1200-1500 mg)

Question 8

pediatric cardiomyopathy

Answer 8

myocardium is abnormally enlarged
40% of children receive a heart transplant within the 1st 2 years of diagnosis

antioxidants important
foods that trigger acute phase response should be avoided (excess carbs and sat fat) add foods that are anti-inflammatory
CoQ10 and Selenium of special interest

Question 9

CKD

Answer 9

irreversible renal injury that is progressive in nature
hypertension and proteinuria play a role in the progression of CKD to ESRD
Yearly Vit D supplement if levels <30 ng/mL
Monitor Na intake, potassium restricted, and phosphorous limited

Question 10

Neonatal Choleostasis

Answer 10

provide ample quantities of fat-soluble vitamins

formula with ratio of 1:1 MCT:LCT- optimal fat balance

Question 11

Wilson’s disease

Answer 11

hepatic copper stones
reduce foods high in copper
increase Zn supplementation

Question 12

Intestinal failure

Answer 12

Loss of intestinal length or competence is below the minimal amount necessary to maintain normal digestion and absorption of nutrients and fluids for weight gain and growth in children independent of PN

Question 13

Asthma

Answer 13

chronic oral corticosteriod use leads to growth failure, sodium retention, voracious appetite, excessive weight gain, hypertension, glucose intolerance, obesity, and decreased bone density
Adequate Ca and Vit D supplementation important

Question 14

Brunchopulmonary Dysplasia

Answer 14

common in preterm infants with positive pressure ventilation and oxygen
energy 140-150 kcal/kg
Ca monitored every 2 weeks
avoid excess carbs- respiratory quotient

Question 15

Cystic fibrosis

Answer 15

sodium chloride- requires supplementation - inadequate intake could be life threatening
PI (pancreatic insufficiency)- requires PERT
CF foundations recs:
weight for length @ 50% by 2yo
2-20yo BMI >50%
Adults BMI 22-women 23-men

Question 16

Intrauterine Growth restriction

Answer 16

placental transport insufficiency (oxygen, glucose, AA) which may be related to local uterine factors, maternal diabetes, hypertension, or other maternal or placental pathology

Question 17

Low HDL treatment

Answer 17

increase exercise

Question 18

High TGs treatment

Answer 18

increase physical activity
increase omega 3s
decrease simple carbs

Question 19

High LDL treatment

Answer 19

increase fiber

increase physical activity

Question 20

Recommendations for lipid control

Answer 20

Fiber: DGA
Simple carbs: DGA
dietary cholesterol <200 mg
trans fat: avoid
saturated fats: <7% total calories
omega 3s: DGA
physical activity : DGA

Question 21

Lipid Profile (high)

Answer 21

TC >200 mg/dL
LDL > 130 mg/dL
non HDL >145 md/dL
TG (0-9) >100 mg/dl (10-19) >130 mg/dL
HDL <45 mg/dL

Question 22

Lipid screening

Answer 22

Universal: b/w 9-11 yo & 17-21 yo
Patients at risk: 2-8 or 12-16
risk factors:
immediate family w/ CVD before <55 M <65 F
parent with total cholesterol > 240 mg/dL
family hx not available (adopted)
obesity, smoking, hypertension, diabetes, renal disease, chronic inflammatory disease, HIV, retinoic acid, anticonvulsants, oral contraceptives