Pre-Test 3 Flashcards
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
Hair tuft on spine think what associated features
Occult spinal dysraphism –> cause neurological spinal damage
Inborn error of metabolism that can lead to thromboembolus
Homocystinuria
Ocular ice pack test for
Myasthenia Gravis (ach autoantibodies) –> will show improvement of weakness of eyes muscles
What is Todd paralysis
24-48 hours of paralysis after a seizure
Tay-Sachs features
Lack of B-hexosaminidiase A in WBCs
Macular cherry red spots and exquisite sensitivity to noise
Angiokeratomas in young kids think? def? other key feature?
Fabry
Lacks a-galactosidase
Painful crisis of extremities and abdomen
Myotonic muscular dystrophy features
Distal muscle weakness
Cataracts
Bladness, facial immobility
TB head CT (w/ contrast)
Exudate in basal cisterns w/ enhancement
Low low glucose on LP but neg culture and all lymphocytes
Pontine glioma featurs
CN VII abnormalities
Unsteady gait 2/2 spasticity
Behavioral changes
Subacute sclerosing pancephalitis seen after?
Measles
Neuro issue seen with kids w/ left to right shunting cardiac defects
Brain abscesses
Cushings triad
Hypertension, bradycardia, irregular respirations
3 things seen from craniopharyngioma
Poor growth, DI, and papilledema
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)
Young girl who loses loss of hand function and loss of ability to socialize
Rhett syndrome (MECP2 mutation)
Calcifications of congenital toxo
Scattered and “soft appearing”
“Ash leaf” spots seen with
Tuberous sclerosis
SMA caused by
Degeneration of anterior horn cells in the spinal chord
Vasovagal syncope can have twitching why?
Brief ischemia of higher cortical levels
“Milk maid grip” or sequential tightening and relaxing seen with
Sydenham chorea –> seen after strep infections
What is Sturge-Webber
PHACE (posterior fossa malformations, hemiangiomas, arterial anomalies, coarctation, eye abnormalities)
port wine stain along v3
14 year old with precocious puberty who develops goiter
McCune albright
How long does rubella usually last
3 days
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
