Pediatric Optometry

Front 1

1. Mutations in what gene cause retinoblastoma. What do proteins produced by this gene normally do

Back 1

RB1 gene - inhibits cell proliferation by altering the expression of genes which promote cell division

Front 2

two ways to get a mutation of the retinoblastoma gene in a germ (egg or sperm) cell

Back 2

bilateral or multifocal tumors

Front 3

What inheritance pattern is seen with germline mutations of the RB1 gene?

Back 3

AD

Front 4

what is the presentation of germline or inherited RB?

Back 4

kids with hereditary diagnosed earlier than non hereditary.

Front 5

what is the windo of opportunity for metastasis of RB to occur

Back 5

if it mets, it becomes evident withing 18 mo. rare beyond 3 years.

Front 6

presenting signs of RB

Back 6

leukocoria
strab (constant, unilateral),
poor vision,
painful red eye from glaucoma,
orbital cellulitis 2ndry to necrosis, mydriasis, heterochromia, hyphema, hypopyon, uveitis, searching nystagmus

Front 7

vitreous seeding is...

Back 7

endophytic tumors that have pushed into the vitreous

Front 8

Pathognomonic characteristic of RB that helps diagnosis

Back 8

calcification of tumors.

Front 9

most common differentials of RB in north america

Back 9

coats, toxocar, phpv

Front 10

how are anterior dx beyond ora in region of ciliary body detected

Back 10

ultrasound biomicroscopy

Front 11

besides opthalmoscopy, additional tests to dx RB are

Back 11

CT, FA, genetic counseling

Front 12

Longterm sequelae in survivors of germline RB

Back 12

longstanding oncological follow up and genetic counseling

Front 13

differentiates Poor from Good prognosis

Back 13

early detection

Front 14

american society of clinical oncology IDs RB as group 1 disorder = Genetic Testing is

Back 14

Mandatory

Front 15

Achromatopsia clinical signs

Back 15

reduced vision nystagmus, photophobia, day blindness, pupil constriction in dark, hy hyperopia, normal fundus, small central scotoma, complete color blindness

Front 16

achromatopsia inheritance

Back 16

ar

Front 17

genes involved in achromatopsia

Back 17

CNGA3, CNGB3, GNAT2

Front 18

LCA predominant presenting signs

Back 18

poor vision, nystagmus, poor pupillry response to light, oculodigital sign

Front 19

Late signs of LCA

Back 19

enophthalmos, kconus cataract

Front 20

what does the protein normally coded for by ABCA4 do?

Back 20

encodes a transmembrane rim protein in discs of rod and foveal cone outer segments transport of retinoids from photorecepotrs to RPE.

Front 21

how can one twin sister have stargardtz and another twin sister not?

Back 21

association of missense and nonsense mutations of ABCA4.

Front 22

what is the carrier frequency of AR Stargardts in the population

Back 22

1:50-1:100

Front 23

the inheritance in 90% of cases of Stargardtz

Back 23

AR

Front 24

T/F: ROP accounts for most of the diability reltated to premature birth

Back 24

False

Front 25

factors mentioned linked with poor neuron outcome

Back 25

ROP, birth weight, gestational age, CP, anisometropia, RE

Front 26

___% of a BW <1700 develop strabismus. what strab is usually developed

Back 26

20, exotropia

Front 27

what is a major ROP risk factor?

Back 27

degree of imaturity as measured by birth weight or gestational age

Front 28

stage 1 ROP

Back 28

flat gray white demarcation line

Front 29

stage 2 ROP

Back 29

demarcation line increased in volume. neovascular tufts

Front 30

stage 3 ROP

Back 30

extraretinal neovascularization continuous with or disconnected from the posterior border of the ridge. extend in the vitreous

Front 31

stage 4 ROP

Back 31

subtotal retinal detachment, exudative/tractional. may involve fovea 4a or may not 4b

Front 32

stage 5 ROP

Back 32

funnel shaped total retinal detachment.

Front 33

stages of ROP that undergo complete resolution.

Back 33

stage 1 and 2 ROP

Front 34

sign of incomplete resolution of stage 3

Back 34

retinal dragging

Front 35

cryo-rop data...what % of babies with bw 1001-1250g developed any ROP

Back 35

47%

Front 36

% untreated babies had severe residual scarring & vision loss

Back 36

4% had scarring, 2% had vision loss

Front 37

% of babies with bw < 1251g reached stage 3 threshold

Back 37

6%

Front 38

develpment of ROP is more tightly related to

Back 38

post menstrual age

Front 39

by what age will the vast majority of babies have developed sever ROP (defined as stage 3 or more?

Back 39

36 weeks PMA

Front 40

the criteria BW and gestational age that signal the need for ROP screening (dilation and ophthalmoscopy with scleral intenter)

Back 40

all babies <1501 or gestation age <29 weeks OR should screen if 1500-2000 with unstable clinical course & at ristk for ROP

Front 41

surveilance for rop ceases when____.

Back 41

ROP vessels have grown well into zone 3

Front 42

immature retina looks like

Back 42

peripheral -white nonvascular regions
optic disc - grayish double ring appearance
macular area - ill defined with no foveal reflex utntil 36-42 weeks PMA
Retinal arterioles - nor toruous but can become tortuous later

Front 43

at the 5.5 year outcome measure of cryo-rop, what % of children in the treated vs. unreated group had unfavorable structural outcomes?

Back 43

47.1% treated vs. 61.7% untreated

Front 44

by 10 years, what % in the treated vs. untreated group had a good acuity outcome in the cryo-rop study

Back 44

25.2% treated vs. 23.7% untreated

Front 45

how many treated eyes in the cryo-rop study had vas of 20/200

Back 45

45.4%

Front 46

the type of ROP that has highly active ROP and must be considered for early treatment is

Back 46

prethreshold 1

Front 47

define prethreshold 1

Back 47

zone 1 - any stage of ROP+DZ
zone 1 - stage 3
zone 2 - stage 2 or 3 +Dz

Front 48

the type of ROP that should be followed conservatively unless they become prethreshold type 1 or threshold ROP is

Back 48

Prethreshold 2

Front 49

define prethreshold 2

Back 49

zone 1 - stage1 or2 +dz
zone 2 - stage 3 without dz

Front 50

once a decision to treat ROP is made, what is the windo of opportunity

Back 50

1 week. tx should begin within 2-3 days of threshold identification.

Front 51

the occurence of the most popular treatement

Back 51

portable indirect laser

Front 52

which 3 infections if already experienced by the mother prior to preg, pos noo further risk to the fetus

Back 52

primary rubella, varicella

Front 53

which infections can recur during pregnancy and infect the fetus

Back 53

cmv and toxoplas

Front 54

what antibodies are too large to crossthe placenta and thus if present signify immune response of the fetus

Back 54

in humans, the only ab able to cross is maternal IgG. IgM = fetal response

Front 55

signs of autism may appear around the same time children receive a vaccine to prevent what dz

Back 55

MMR prevents rubella

Front 56

incidence of conenital toxoplasmosis

Back 56

1 in 1000 - 1 in 10000

Front 57

blindness hydrocephalus, MR, and deafness could be attributed to what infection

Back 57

toxoplas

Front 58

variability rates of susceptibility (seronegative mothers) bw US and France

Back 58

due to differing dietary and living customs

Front 59

30% change of choriretinits scarring in infants with what dz?

Back 59

toxoplas

Front 60

what can a woman of child bearing age do to prevent cathching toxoplas

Back 60

dont eat undercooked meat, minimize exposure to cats. wash fruits and veges

Front 61

incidence of CMV

Back 61

1% of all newborns

Front 62

how many babies born with CMV are symptomatic

Back 62

10%

Front 63

characteristics that CMV and toxo share

Back 63

jaundice, hepatosplenmegaly, micorcephaly,

Front 64

systemic manifest unique to toxo

Back 64

anemia, fever, intracran calcifications, seizures

Front 65

systemic manifest to CMV

Back 65

hearing loss, rash (blueberry baby)

Front 66

whats cultured to look for CMV?

Back 66

urine and saliva

Front 67

used to treat CMV

Back 67

ganciclovir.

Front 68

usual location of chorioretinitis in HSV

Back 68

peripheral retina, well circumscribed hyperpigmnted scars

Front 69

likelihood of a mother wih primary syphillus having a baby that is affected

Back 69

virutually all

Front 70

early ocular sign of syphillus

Back 70

uveitis

Front 71

hutchinson's triad

Back 71

interstital keratitis, deafness, malformed incisors

Front 72

what is interstital keratitis. what is treatment for it.

Back 72

sectorial or diffuse K edema infiltrated by interstitial vessels. causes K scarring and ghost vessels. topical corticsteroids.

Front 73

another name for chorioretinitis from syphilis

Back 73

pseudoretinitis from syphillus

Front 74

treatment of congenital syphillus

Back 74

10 day course of penecillin

Front 75

congenital varicella is typified by

Back 75

chorioretinitis, cataracts, microphthalmos, horner's neurpathic bladder

Front 76

small infant eyes combines with small pupils, and poor VA _______(increase/decrease) infant's perception of blur

Back 76

decrease

Front 77

retinoscopy (over/under) est. hyperopia in small eyes. by how much is it over/under est?

Back 77

over by 1/3

Front 78

what is the clinical signif of the angler of the papillary axis being larger in infants

Back 78

artificail exotropia causes larger amounts of induced astigmaism.

Front 79

signif RE should be monitored every __ months

Back 79

3

Front 80

combo drops can be prepared from

Back 80

3.75 ml of 2% cyclo, 7.5 ml of 1% T, and 3.75ml of 10% phenyl to equal = .5%T, .5%C, 2.5% phenyl

Front 81

a high accomodative lag can indicate

Back 81

accommodative insufficiency associated with neurodevelopmental delays or low vision

Front 82

1992 guidelines for oral sedation of pediatric patients

Back 82

parent or guardian must be present, immediate access to emergency facilities, personell and equipment, positive pressure o2 delivery system, bp cuff and stuff, emergency cart, appropriate consent forms, insturctions about expected effects of seation with emergency phone number, pre sedation physical, vital signs during sedation and condition at discharge

Front 83

what percent of kids with ambly are detected after school entry

Back 83

50%

Front 84

campbell and charney said

Back 84

what % of kids receiving TX for amblyopiea were first detected in primary medical setting? 25%

Front 85

in older kids, what percent of bv complaints may resolve with treatment of RE

Back 85

nearly 50%

Front 86

what percent of kids age 8-12 years could complete facility with +/-2

Back 86

90%

Front 87

average facility of 8-12 year olds with +/- 2?

Back 87

4.5 cpm

Front 88

after 12 months, what percent of toddlers have hyperopia > 3.0

Back 88

3%

Front 89

most significant refractive risk for developing esotropia

Back 89

high hyperopia

Front 90

what limit of hyperopia results in bilateral amblyopia

Back 90

5D

Front 91

if hyperopia is not fully compensated, ___ is most likely to be reduced

Back 91

stereo

Front 92

how would you correct an amblyopic child with bilateral hyper of 7.0D with a large XP that tropes with full + correction

Back 92

partially correct

Front 93

what percentage of infants have astigmatism>1.0

Back 93

50%

Front 94

most infantile astigmatism is (atr/wtr)

Back 94

atr

Front 95

what axis predicts that astigmatism will persist thruout the critical perios, thus increasinge likelihood of amblyopia

Back 95

- oblique axis

Front 96

what 2 factors in addition to the amount of astig can be used to select which children need RE corrxn of their astig

Back 96

oblique >1.0-2.0 present after the first year, longitudinal measures showing a failure to emmetropinize

Front 97

until age 3.5, a partial correction is advised for astigmatism (T/F)

Back 97

True

Front 98

what additional finding indicates the necessity for full corrxn of astigmatism despite age

Back 98

coexisting bv probs like reduced vergence ranges or facility

Front 99

natural hx of aniso

Back 99

transient

Front 100

risk for stable aniso

Back 100

ambly or strab

Front 101

risk for increasing aniso

Back 101

increased prevalence and degree of myopia

Front 102

when do you use atropine to determine the true amount of aniso

Back 102

if you get a residual ET... use it to look for laten hyper

Front 103

what is standard of care as far as Rxing for aniso is concerned

Back 103

prescribe entire difference in sph refractive error

Front 104

if an infant has a higher degree of ametropia, aniso will probably

Back 104

persist and become associated with strab/ambly

Front 105

MOTAS showed what

Back 105

that most kids dont wear patching like they're supposed to

Front 106

infantile glauc frequently causes

Back 106

aniso myopia

Front 107

natural history of myopia

Back 107

increases in school years (15% at age 15)

Front 108

____ % caucasians and ____% AA have myopie

Back 108

26 and 12

Front 109

the COMETstudy looked at

Back 109

link b/w accomodation and myo development/progression. does PAL slow the rate vs SV lenses.

Front 110

risk factors for higher progression of myopia

Back 110

young age, girls, non AA

Front 111

pals were more effective for who

Back 111

accomodative lag and eso.

Front 112

the CLAMP study looked at

Back 112

the effect of RGPs on the slowing down of myopia

Front 113

define threshold dz

Back 113

5 consecutive or 8 total clock hours of stage 3 ROP in zone 1 or 2 + dz

Front 114

horner's can be a presenting sign of _____ so you should always refer out for an _________

Back 114

neuroblastoma, MRI

Front 115

if children are << (what age) should they be referred out for IV AB for preseptal cellulitis

Back 115

5 yo