Flashcards in Peds Test and Tx Deck (24):
What is the screening test for PKU?
Phe levels in the blood
Guthrie test in urine - coloration test for phe in the urine
What should be avoided in PKU.
High protein diet
What foods are recommended in PKU?
Cereals, starches, fruits, vegetables, Phe-free milk
What is the tx for foreign body in the vagina of a child?
Irrigation with warm water (usually toilet paper)
Removal with calcium alginate swab
What is the tx for constipation in children?
Limit cow's milk to less than 24 ounces per day
Increase dietary fiber
What is the first step to identifying bowel obstruction?
ABXR to rule out pneumoperitoneum that would require immediate surgery
What is the next step after an Ab XR to identify a bowel obstruction?
What is the best step in management in a child with exposure to lead?
Measure venous lead level
What is tx for lead level less than 44?
Repeat level in a month
What is the tx for lead level between 45-69?
DMSA - oral succimer or inpatient EDTA
What is the tx for lead levels over 70?
Chelation therapy: dimercaprol plus EDTA
What is the enzyme deficient in Gaucher's?
What are the sx of Gaucher's?
Erlenmeyer flask deformity of distal femur
What is the enzyme deficient in Nieman-picks?
What is the enzyme deficient in Krabbe's?
What is the enzyme deficient in Tay Sachs?
What are the sx of Krabbe's?
Death in first 3 years of life
What are the sx of Tay Sachs?
Slow loss of motor skills beginning around 3-6 months
Cherry red spot n macula
What are the sx of Nieman picks?
Cherry red spot
Recurrent pulmonary disease
What kind of cells are found in Gaucher's?
Crinkled papa macrophage
What is the deficiency in Fabry's?
Alpha galactosidase A leads to buildup of ceramide trihexoside
What are the sx of Fabry's disease?
Increased risk if TE
Mass with calcifications on CT or US + leukoria