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Nicu Bort > Items To Review > Flashcards

Items To Review Flashcards

1
Q

Pompes Disease

A

GSD2,

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2
Q

PKU (and a similar disorder)

A

Deficiency of PAH (converts phenylalanine to tyrosine), P toxic to brain, T needed for melanin = fair eyes/hair
Similar disorder - tetrahydrobiopterin (PAH cofactor)

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3
Q

Abnormal odor and IEM:
Maple syrup
Boiled cabbage
Fish
Sweaty foot

A

MSUD
Tyrosinemia
3 methylcrotonylglycinuria
Glutaric aciduria 2

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4
Q

Galactosemia

A

Galactose 1 phosphate uridyltransferase def (GALT)
Poor feeding lethargy jaundice cataracts E. coli sepsis
Tx: dietary restriction (no lactose). Even with tx can have mental retardation.
Girls have premature ovarian failure.

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5
Q

MCAD def

A

Most common d/o fatty acid oxidation.
Usually asymptomatic as neonate. Can have vomiting, HM, lethargy, coma, seizures with fasting or an illness.
Increased C6-10 acylcarnitines.

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6
Q

Ectopic atrial contraction

A

180-240 bpm
Speeds up and slows down
Atrial contraction before vent ctx

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7
Q

HLHS management

A

Norwood palliation. Atrial septectomy and convert RV into the main ventricle for pulm and systemic flow. MPA and Ao are connected, MPA disconnected from PAs. Shunt placed from PA to lungs.
Then Glenn then fontan.

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8
Q

Tx for SVT
Tx for AV reentry tachycardia

A

SVT - digoxin
AVRT - amiodarone

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9
Q

Hydrocortisone mechanism

A

Inhibit NO synthase
Upregulate adrenergic receptors
Upregulate angiotensin II receptors
Inhibit catecholamine metabolism.
Increase intracellular Ca.

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10
Q

Milrinone mechanism

A

Phosphodiesterase 3 inhibitor. Decreases breakdown of cAMP = increases Ca influx to cells.
Inotropic, inodilator, lusitrope.

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11
Q

Norepi mechanism

A

Activates alpha1,2 and beta1 receptors to increase SVR and CO. Greater systemic constriction than pulmonary

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12
Q

Ebstein anomaly associated with which EKG abnormality

A

RBBB. Also WPW

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13
Q

DM CHD

A

VSD
DORV
TA

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14
Q

HCM in IDM

A

Histologically different than congenital HCM. Caused by insulin and hyperglycemia. Supportive care, can be reversible, treatment includes beta blockers to improve ventricular output.

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15
Q

Most common cause hypertrophic cardiomyopathy and Tx

A

Noonans
Beta blocker

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16
Q

How to assess atrial communication in fetal echo for HLHS

A

Flow reversal thru pulmonary vein in atrial systole

17
Q

Dobutamine mechanism

A

Acts on alph. and beta without releasing norepi. Increased myocardial contractility and vasodilation of peripheral vasculature

18
Q

CCHDs

A

HLHS, PA intact septum, ToF, TAPVR, TGA, TA, TA

19
Q

PHACES

A

Posterior fossa anomalies
Hemangioma
Arterial anomalies
Cardiac
Eye anomalies

20
Q

LEOPARD syndrome

A

Lentigines
It is Noonans with lentigines

21
Q

Graves

A

> 90% babies born to graves moms habe subclinical thyrotoxicosis. fT4 peak at 5 days. 1-5% are diagnosed.

PTU drug of choice. Methimazole is teratogenic

22
Q

Smith lemli optiz

A

7a dehydrocholesterol reductase deficiency.
Impaired choles synth. Cardiac, cleft palates, syn poly dactyly , amb gebitalia

23
Q

Goldenhar syndrome

A

Ear abnormalities, eyelid notching abnormalities (eyelid coloboma), spinal deformity like scoliosis

24
Q

Failed hearing screen sensorineural deficit

A

Hearing aid trail if no improvement by 1 year then cochlear implant may be good, better to get by 12 mo

25
Q

Follow up schedule for ROP if treated with becavizumab

A

45-55 weeks PMA and up to 65 weeks

26
Q

3 types of lymphatic malformations

A

Macrocystic - cystic hygroma usually head and neck
Micro cystic - can look like vesicle clusters that leak lymphatic fluid
Mixed

27
Q

When to fu ROP in 1 week

A

If zone 1 any stage or any stage 3

28
Q

Brachial plexus injuries
- what innervates upper middle and lower roots
- what root gives Horner syndrome
- what are the clinical palsies
- 4 palsy groups

A
  • C5-6, C7, C8-T1
  • lower root
  • Erb (upper c5-7, waiters tip, intact grasp). Klumpke (grasp absent, rare), and total
  • 1- c5-6, 90% recovery rate, 2- 65%
29
Q

Covid isolation

A

Vertical - test 24-72 h and remove from isolation if negative
Horizontal - test at 0 and then 5-7 days and isolate 10 days regardless
Need droplet for low flow and airborne for high flow

Nicu Bort (1 decks)

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