Pre-Test 3 Flashcards by Robby Skinner

Acute disseminated encephalomyelitis features? Tx?

Autoimmune demyelinating disease in children under 10
Can follow URIs and many other viral diseases
MRI will show white plaques across the brain
Tx is steroids

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Hair tuft on spine think what associated features

Occult spinal dysraphism –> cause neurological spinal damage

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Inborn error of metabolism that can lead to thromboembolus

Homocystinuria

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Ocular ice pack test for

Myasthenia Gravis (ach autoantibodies) –> will show improvement of weakness of eyes muscles

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What is Todd paralysis

24-48 hours of paralysis after a seizure

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Tay-Sachs features

Lack of B-hexosaminidiase A in WBCs

Macular cherry red spots and exquisite sensitivity to noise

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Angiokeratomas in young kids think? def? other key feature?

Fabry
Lacks a-galactosidase
Painful crisis of extremities and abdomen

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Myotonic muscular dystrophy features

Distal muscle weakness
Cataracts
Bladness, facial immobility

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TB head CT (w/ contrast)

Exudate in basal cisterns w/ enhancement

Low low glucose on LP but neg culture and all lymphocytes

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Pontine glioma featurs

CN VII abnormalities
Unsteady gait 2/2 spasticity
Behavioral changes

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Subacute sclerosing pancephalitis seen after?

Measles

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Neuro issue seen with kids w/ left to right shunting cardiac defects

Brain abscesses

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Cushings triad

Hypertension, bradycardia, irregular respirations

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3 things seen from craniopharyngioma

Poor growth, DI, and papilledema

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Most common site of brain tumors in kids under 1? 1-10 years? > 10?

Under 1: Supratentorial (choriod plexus tumors and teratomas
1-10: Infratentorial
> 10: Supratentorial (astrocytomas)

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Young girl who loses loss of hand function and loss of ability to socialize

Rhett syndrome (MECP2 mutation)

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Calcifications of congenital toxo

Scattered and “soft appearing”

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“Ash leaf” spots seen with

Tuberous sclerosis

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SMA caused by

Degeneration of anterior horn cells in the spinal chord

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Vasovagal syncope can have twitching why?

Brief ischemia of higher cortical levels

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“Milk maid grip” or sequential tightening and relaxing seen with

Sydenham chorea –> seen after strep infections

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What is Sturge-Webber

PHACE (posterior fossa malformations, hemiangiomas, arterial anomalies, coarctation, eye abnormalities)
port wine stain along v3

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14 year old with precocious puberty who develops goiter

McCune albright

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How long does rubella usually last

3 days

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What should you do while waiting for final results in a kid born to an HIV + mom

PCP ppx (tmp-smx)

Reyes syndrome death usually from

Cerebral edema and subsequent herniation

Tinea capitus tx

Griseofulvin

In an infant who appears otherwise normal, sudden onset of fever and high left shift suggests

Pneumococcal bacteremia

High fever goes away as rash appears

Roseola

Most common rabies animal in US

Raccoon

Lyme dz rash called

Erythema chorinicum migrans

Chronic watery frothy diarrhea

Cryptosporidium | *can tx with nitazoxanide*

When does t. spiralis cause the Poo

1-7 days after undercooked pork | *muscle involvement can last*

Ig elevated in WAS

IgE and IgM

Job syndrome features

Disorder of phagocytic chemotaxis | Hyper IgE, eczema like rash, and recurrent severe staph infections

Gonorrhea culture

Thayer-Martin

URI with rapid progression to respiratory distress and pneumothorax what kind of pna

Staph

Hgb A2 increased in

B-thal trait

What is cyclic neutropenia? tx?

AD disease where neutropenia occurs about every 3 weeks | Tx is g-csf

First step in suspected Hodgkin's dz

CXR to look for suspicious mediastinal mass

Newborn presentation very similar to Rh incompat but not

Hereditary spherocytosis

How does glucose 6 phosphatase def often present

3-4 months old with failure to thrive, hypoglycemia, hepatomegaly, and acidosis

Erlenmeyer flask like long bones seen with

Gaucher disease (b-glucocerebrosidase def)

Third degree heart block seen in kids born to what moms

Those with lupus

Penis and ball size to indicate puberty

Penis longer than 2.5 cm | Testicular volume > 3 cm

Waardenburg features

White forelock Heterochromia irides Congenital deafness Lateral displacement of inner canthi

Most common cause of juvenile hypothyroidism

Lymphocytic

Galactosemia kids increased risk of

GBS sepsis | *Also jaundice, hepatosplenomegaly, and elevated Thyroid function tests*

Citrulline supplementation for

OTC def

FAS similar to what enzyme def

PKU (MR, microcephaly, heart and vascular malformations)

Pt's born normal who develop hepatosplenomegaly and MR likely have

a storage disease

Hurler's features

Def of a-1-iduronidase | Umbilical hernia, kyphoscoliosis, deafness, cloudy corneas, and claw hand deformity

Inc wilms tour risk with what syndrome

Beckwith-Wiedemann

Pseudohypoparathyroidism features

High PO4, Low Ca (but PTH levels high) | Also brachydactly, obesity with round face, and increased bone density (but delayed bone age)

What can be seen after immobilization from a fracture? Complications?

Hypercalcemia Complications include nephropathy, constipation and anorexia *serum to urine Ca to test for the hypercalciuria*

Skin of CAH

Hyperpigmented

When does vomiting begin in CAH

3rd week of life

What can help alkaptonuria

Absorbic acid | *rember black urine, homogenistic acid def*

Big Marfan's CV risk

Aortic dissection

Ca and PO in nutritional rickets

PO4 is low and serum Ca is usually normal (but will see hypocalciuria*

Why hypoglycemia in pre-term kids

Glycogen and fat stores are diminished

Vit D-resistant rickets

RTA can't reabsorb phosphate or activate bit D | Low PO4, normal Ca

Tingling in the hands followed by seizures could be due to

Hypoparathyroidism --> High PO4 and low Ca

Medullary thyroid Ca secretes

Calcitonin (but levels of Ca and PO4 normal)

What is Laurence-Moon-Beidl syndrome

AR trait causing MR, hypogonadism, polydactyl, and retinitis pigmentosum

DI concentrations of Na and K

Both will be high

Addison's crisis Na and K

Hyponatremic and hyperkalemic | *resorption of Na and excretion of K impaired*

Upper extremity abnormalities and cardiac abnormalities is what? Can also be missing?

Holt-Oram | Can also be missing pectoralis muscles

First sign of puberty in boys

Scrotal and testicular growth

Tanner 2 in boys

Penile enlargement and sparse pubic hair

What should you do for any adolescent suicide attempt

Hospitalize

Most frequent complication of accutane

Dry eyes, skin, and mouth

Chief benefit of benzoyl peroxide on acne

Antibacterial activity

Grade 1 concussion

No LOC and return to baseline in 15 minutes | *can go back into the game after, but not after another*

Odd contraindication to contact sports

Having one eyeball

What is Prehn sign

Epididymitis vs torsion | Lifting the scrotum should help epididymitis and make torsion worse

Painless genital ulcer that has resolved leading to unilateral draining lymphadenitis

Lymphogranuloma venereum

Chancroid features

Painful ulcer and bilateral draining inguinal lymphadenopathy

Pre-Test 3 Flashcards

(78 cards)