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Step 2 High Yield > Peds Test and Tx > Flashcards

Peds Test and Tx Flashcards

0
Q

What should be avoided in PKU.

A

High protein diet

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1
Q

What is the screening test for PKU?

A

Phe levels in the blood

Guthrie test in urine - coloration test for phe in the urine

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2
Q

What foods are recommended in PKU?

A

Cereals, starches, fruits, vegetables, Phe-free milk

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3
Q

What is the tx for foreign body in the vagina of a child?

A

Irrigation with warm water (usually toilet paper)

Removal with calcium alginate swab

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4
Q

What is the tx for constipation in children?

A

Limit cow’s milk to less than 24 ounces per day
Increase dietary fiber
Laxative

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5
Q

What is the first step to identifying bowel obstruction?

A

ABXR to rule out pneumoperitoneum that would require immediate surgery

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6
Q

What is the next step after an Ab XR to identify a bowel obstruction?

A

Contrast enema

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7
Q

What is the best step in management in a child with exposure to lead?

A

Measure venous lead level

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8
Q

What is tx for lead level less than 44?

A

Repeat level in a month

No meds

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9
Q

What is the tx for lead level between 45-69?

A

DMSA - oral succimer or inpatient EDTA

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10
Q

What is the tx for lead levels over 70?

A

Chelation therapy: dimercaprol plus EDTA

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11
Q

What is the enzyme deficient in Gaucher’s?

A

Glucocerebrosidase

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12
Q

What are the sx of Gaucher’s?

A 
Hepatomegaly
Anemia
Leukopenia
TCP
AVN 
Bone pain
Osteopenia 
Erlenmeyer flask deformity of distal femur
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13
Q

What is the enzyme deficient in Nieman-picks?

A

Sphingomyelinase

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14
Q

What is the enzyme deficient in Krabbe’s?

A

Galactocerebrocidase

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15
Q

What is the enzyme deficient in Tay Sachs?

A

Hexosaminidase A

16
Q

What are the sx of Krabbe’s?

A 
Hyperacusis
Optic atrophy 
Spasticity
Seizures
Perineural neuropathy 
Death in first 3 years of life
17
Q

What are the sx of Tay Sachs?

A 
Slow loss of motor skills beginning around 3-6 months
Cherry red spot n macula
Hearing loss
Dysphagia
Vision loss
18
Q

What are the sx of Nieman picks?

A 
Cherry red spot
Hepatomegaly 
Ab distention/pain
Recurrent pulmonary disease 
dysarthria 
Ataxia
Dystonia
19
Q

What kind of cells are found in Gaucher’s?

A

Crinkled papa macrophage

20
Q

What is the deficiency in Fabry’s?

A

Alpha galactosidase A leads to buildup of ceramide trihexoside

21
Q

What are the sx of Fabry’s disease?

A 
Angiokeratomas
Telangectasia 
Renal failure 
HTN 
Parenthesias/neuropathic pai. 
Hypohidrosis
Increased risk if TE
22
Q

Mass with calcifications on CT or US + leukoria

A

Retinoblastoma

23
Q

What is the gold standard for diagnosing Duchenne’s?

A

Genetic testing

Step 2 High Yield (20 decks)

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